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An 84-year-old-woman underwent a non-elective surgery because of an ankle fracture. Her weight and height were 62 kg and 150 cm, respectively, with body mass index of 27.6 kg/m2. She had left upper arm surgery eight days earlier in the same hospital. She fell in the bathroom while on the ward during the early morning two days previously. She hit her head and fractured her right ankle. She was unconscious when she was found by a nurse, but soon regained consciousness. She was not aware that she fainted. Her oxygen saturation (SpO2) was 88%, but increased to 94% with oxygen via a mask, with a blood pressure of 105/59 mmHg and heart rate 95 bpm. Although subsequent computed tomography (CT) of the head confirmed traumatic subarachnoid hemorrhage, with a small amount of blood in a region of the occipital sulci, no therapeutic intervention was deemed necessary for the head injury. However, she needed surgery for open reduction and internal fixation of the ankle fracture. She had atrial fibrillation, tachycardia of 120 bpm, diaphoresis and fever, and looked unstable at presentation before induction of anesthesia in the operating room (OR), although she responded appropriately to questions.\nThe surgery was performed under general anesthesia using desflurane and air combined with femoral triangle block and sciatic nerve block through a popliteal approach. Her airway was secured with a supraglottic device (#4 i-gel, Japan Medicalnext Co., Osaka). The surgery took one hour. Her anesthesia course of two hours and five minutes was unstable. Her heart rate varied between 95 and 140 bpm, systolic blood pressure varied between 70 and 110 mmHg, and frequent intermittent administration of phenylephrine was needed. Her SpO2 was 92-98%, though FO2 was maintained at 50%. Her body temperature was 38.5℃. After restoration of spontaneous respiration and confirming eye-opening to verbal stimuli, we removed the supraglottic device. She looked pale, although her SpO2 was 94% with an oxygen mask at 4 L/min. Because of her unstable hemodynamics and unexplained low SpO2 during and after surgery, we performed POC ultrasonography with a portable device (SonoSite M-Turbo, Fujifilm, Tokyo) using a sector probe (SonoSite, P21x) to rule out disorders in the heart and lung.\nLung sliding was identified on both lung surface, excluding possibility of pneumothorax. We found no fluid collection in the thorax. The apical four-chamber view revealed, however, a dilated right ventricle (RV), with reduced free wall motion of the RV with normokinesis of the apical RV wall (McConell’s sign) and a moderate tricuspid regurgitant (TR) jet. The peak TR pressure gradient was 41 mmHg. Further, we confirmed a D-shaped left ventricle (LV) during diastole in the parasternal short axis view along with a round-shaped dilated inferior vena cava in the subcostal short axis view. Ultrasonography did not detect any thrombus in the right heart or in the femoral veins. These images were not stored in the device, because the examination was not part of a routine work-up. Because there were no such abnormalities in the previous recordings of echocardiography before the first surgery, we diagnosed an acute RV overload, presumably resulting from a PE. We urgently ordered multidetector CT. CT angiography demonstrated filling defects in the right and left pulmonary arteries with an RV/LV diameter ratio of 1.5 (). CT venography showed only a small thrombus in the right popliteal vein and no residual thrombus in the greater veins such as the popliteal, femoro-iliac, or inferior vena cava. Her SpO2 was thereafter stable above 97% with oxygen supplementation via face mask for the following two days. She was carefully managed by cardiologists with oral anti-coagulant therapy (edoxaban). She was discharged from the hospital two weeks later.
The patient was a 30-year-old female with jaundice. Blood analysis at a local hospital revealed severe liver failure, and she was transferred to our hospital. She was diagnosed as having acute liver failure of unknown etiology and underwent medical treatment, including plasma exchange and transfusion; however, her liver function did not improve. LT was planned, but there was no suitable living donor, and she was placed on the DDLT wait list. DDLT was performed with right tri-segment split graft 13 days later. At the time of abdominal closure, the transplanted graft showed ischemia in segment IV. The graft volume to the standard liver volume ratio was 123.4 % (graft volume = 1381 g), the cold ischemic time was 10 h and 32 min, the warm ischemic time was 50 min, and the anhepatic time was 1 h and 5 min. The native liver showed subtotal necrosis.\nAlthough no severe complications occurred after surgery, she developed a fever and increased total bilirubin level on post-operative day (POD) 5 (Fig. ). Blood analysis revealed decreased platelets, coagulation abnormalities, and severe inflammation. Computed tomography on POD 6 revealed necrosis of segment IV of the transplanted graft (Fig. ). She was diagnosed as having DIC based on the Japanese acute-phase DIC score (seven points) [], and rTM was begun at 380 U/kg/day. Thereafter, inflammation, liver function, and coagulation abnormalities dramatically improved and the fever resolved (Figs. and ). However, on POD 12, the pleural effusion drainage from the chest tube became bloody. The anticoagulant effect of rTM was considered the cause and was discontinued. On POD 14, the pleural effusion drainage became serous, and on POD 19, the quantity of pleural effusion was minimal and the chest tube was removed. Thereafter, she made good progress and was discharged from hospital on POD 28.\nrTM was effective for DIC after DDLT with right tri-segment split graft in this case, and we believe that necrosis of segment IV of the split graft caused the DIC.\nDIC is a severe possible complication after LT and is a systemic disorder characterized by continuous intravascular coagulation related to an underlying disease. The liver produces most of the body’s coagulation factors, fibrinolytic compounds, and coagulation inhibitor factors. However, the dramatic coagulation disorders and fibrinolysis seen with DIC occur systemically after liver transplantation. Coagulation and fibrinolysis are markedly degraded in end-stage liver failure patients well before surgery, and these functions are degraded further after surgery because of the intra-operative anhepatic time and delayed time to improved graft function. After transplantation, the competing effects of acute inflammation with coagulation and fibrinolysis lead to thrombotic microangiopathy and systemic inflammatory response syndrome making it difficult to grasp the disease symptoms and treatment [].\nCauley et al. [] recently aimed to determine the current risk of graft failure in adult recipients after split LT by analyzing data from the United Network for Organ Sharing registry, assessing 889 split liver grafts performed from 1995 to 2010. Similar to previous analyses from the USA, the authors noted a significantly increased risk of graft failure in split grafts compared with whole grafts. In Japan, Sakamoto et al. showed that complications related to the operation were more frequent in split LT. They reported that the occurrence of bile leakage in extended right-lobe grafts was generally related to the viability of segment IV. The blood supply to segment IV, which arises primarily from the left-side vasculature, may be sacrificed during the splitting procedure and may increase the risk of parenchymal necrosis and bile leakage with an incidence of approximately 20–30 %. Segment IV-related complications may be directly associated with high rates of graft loss and mortality []. These data might include cases that develop DIC after surgery with necrotic areas in the graft, as in our case. Post-operative DIC can also occur following hepatectomy because liver partition and portal vein ligation for staged hepatectomy can cause post-operative ischemia in segment IV []. Monitoring hepatectomy patients for post-operative DIC is important with intra-operative visible hepatic ischemia.\nrTM is a novel anticoagulant agent composed of the active, extracellular domain of thrombomodulin that regulates the imbalanced coagulation system by reducing excessive activation of thrombin []. In Japan, it has been reported that rhTM potentially reduces the morbidity and mortality in patients with sepsis-induced DIC [–]. Similarly, an international phase IIb clinical trial of rTM in patients with sepsis and suspected DIC [] suggested that this treatment is both efficacious and safe, stimulating enthusiasm for the application of rTM in critical care. rTM also has an inhibitory effect on high-mobility group box 1 (HMGB1) by an anti- inflammatory action []. HMGB1 is an intranuclear protein that was originally identified as a DNA-binding protein but has since been recognized as a late-phase mediator during sepsis []. HMGB1 is also known to act as a pro-coagulant as well as a pro-inflammatory mediator for septic organ dysfunction. Hepatocyte nuclei contain high levels of HMGB1, which is released into the blood after ischemic-reperfusion injury to the liver and hepatocyte necrosis []. Therefore, rTM is a useful treatment for DIC after LT.
A female caucasian baby was born by cesarean section at 39 weeks of gestation to a 37-year-old mother. Family medical history was unremarkable. The pregnancy had a normal course until 32 weeks of gestation when an obstetric ultrasound (US) showed the presence of a cystic abdominal mass of about 3 cm in diameter, localized between the bladder and the umbilicus, which was suggestive of an urachal cyst without other structural alterations of the urinary apparatus. At birth, APGAR score was 9/10 and birth weight 3200 g.\nAt physical examination, a vascular lesion (4 cm × 4 cm) was detected in the right lower abdominal quadrant. The presence of a soft suprapubic mass was also appreciated, without alterations of the overlying skin. The patient was otherwise asymptomatic.\nThe dermatologist confirmed the diagnosis of complex vascular malformation: it consisted in a venocapillary superficial lesion associated with multicystic lymphangiomatous lesions in the subcutaneous tissues, involving the right lower abdomen and the homolateral inguinal area and tight. Also, a small angiomatous lesion of the right first toe with no dysmetria of the affected limb was detected at physical examination.\nTable summarizes the main clinical and radiologic findings in our patients, which led to the diagnosis of multicystic lymphangioma, and the major steps in the management and follow-up.\nOn the second day of life the abdominal US was performed, and no congenital urachus abnormalities were detected, but rather some hypo-anechoic cysts of about 3.1 × 0.5 cm that extended from the umbilical to the right inguinal area, reaching the medial surface of the thigh, with a little extremity just above the homolateral knee (Fig. ). The Doppler technique showed some vascular signals from the adipose tissue where these cystic lesions were located. A magnetic resonance imaging (MRI) scan with contrast was then performed. In consideration of the age of the patient, sedation was needed during the exam. At MRI, several lacunas with cystic appearance were observed in the subcutaneous adipose tissue of the right lower abdomen. These structures had variable size (maximum 3 cm of diameter) and were hypointense in T1 and hyperintense in T2 sequences. Cysts seemed to be restricted to the subcutaneous tissues since no signals of muscular or abdominal cavity involvement were evident. After the intravenous contrast administration, no significant enhancement of the cysts’ content was seen. All MRI characteristics confirmed the diagnosis of multicystic lymphangioma (Fig. ).\nAs surrounding abdominal organs were not involved and compressive complications on adjacent organs were not present, given the possibility of spontaneous regression of the lesion, a “wait and see” strategy was adopted, and the patient was closely monitored through a multidisciplinary follow-up program.\nA size reduction and progressive discoloration were macroscopically appreciated, and progressive regression was confirmed through serial US evaluations.\nSixteen months later, the presence of a new cystic formation (3.6 cm × 2.1 cm × 1.9 cm) with irregular morphology and corpuscular content was detected at the US scan, referable to an intracystic bleeding. Since the patient was asymptomatic and no other complications were detected, the conservative approach was carried on.\nDuring the following months, some millimetric angiokeratomas developed.\nAlmost 3 years later, the cystic lymphangioma appears significantly reduced in extension, and the cysts now have a maximum diameter of 0.8 to 1 cm. No alterations or compressions of adjacent organs were present. The patient is 3 years old, has normal neurodevelopment and does not have any abdominal disturbances. No compression on adjacent organs nor dysmetria at the inferior limbs is present.\nHowever, given the risk of bleeding or ulceration of the superficial angiokeratomas, pulsed dye laser seems now the best treatment choice.[
The patient is a 26-year-old woman with a 13-year history of chronic abdominal pain that started after an episode of presumed "food poisoning." The pain typically lasted 5-6 hours per day, was triggered by eating or bowel movements, and was often severe enough to induce vomiting. It was causing significant interference with her daily activities, both from the distraction of the pain itself and from the side effects associated with pain medication. The patient found an article about PMM performed on a series of six cancer patients and requested evaluation and consideration for the operation. At the time of our first evaluation, the patient had already undergone multiple colonoscopies with no abnormalities identified other than some nonspecific inflammation. She had also undergone endoscopy with biopsy of her gastrointestinal tract, in addition to a pill camera study. These revealed only small intestinal overgrowth. She even underwent a diagnostic laparotomy that did not show any abnormalities. She carried a diagnosis of severe irritable bowel syndrome with constipation (IBS-C) with visceral hypersensitivity syndrome. Regarding prior therapies, she had tried a multitude of diets without relief, and had even gone on total parenteral nutrition (TPN) for months for bowel rest with no resolution of her symptoms. Notably, her insurance company had denied other surgical options including a spinal cord stimulator and an intrathecal morphine pump. On exam, she was neurologically intact other than diffusely reduced deep tendon reflexes, more so in the upper extremities. Given the nature and severity of the pain, the extensive and unrevealing prior workup and therapeutic strategies, and the patient’s wishes, we offered a PMM as a treatment option for her clearly visceral origin pain. We discussed the risks in detail, as well as the uncertainty given the paucity of clinical experience with PMM in non-malignant pain. Despite these cautions, the patient and family wished to proceed with surgery.\nThe patient underwent an uneventful PMM via a T7 laminectomy. The technique used was that of a simple transverse crush injury to the PSDC system as first described by Nauta and colleagues in 2000 []. Figure depicts the post-operative magnetic resonance imaging (MRI) of the lesion.\nIntraoperatively, she had no decrement in her somatosensory evoked potential (SSEP) monitoring. Anticipating difficulty with post-operative pain control from the patient’s tolerance to opioids, liposomal bupivacaine (EXPAREL®) was injected intraoperatively into the paraspinal muscles and wound edges. Over the first two post-operative days, she was uncertain of any benefit. Then on post-operative day three, she had a bowel movement which was the first in many years without associated severe pain. By two-week follow-up, the patient had experienced no episodes of abdominal pain since the procedure and had weaned her pain medication from 2 mg of oral hydromorphone every 4-5 hours pre-operatively to 2 mg every 12 hours for incisional pain. On exam, she was motor intact with preserved light touch and proprioception in her lower extremities. The only detectable deficit was diminished sensation to vibration on her great toe pads bilaterally. Her gait was normal and Romberg testing was negative. Regarding sensory changes, she reported only mild numbness of the vulva but not of the vagina and some numbness on the insides of her feet. By her eleven-week follow-up, she was pain free off all opioid medication. She was able to eat whatever food she liked and was having normal pain free bowel movements. Also, the previous numbness/tingling in her vulva had resolved, and she had regained full sensation. She was having no balance difficulties, nor was she having bowel or bladder function issues. Moreover, she was again able to exercise and succeeded in losing over 10 pounds since surgery. Her only perceptible abnormality was some tingling in her toes bilaterally and around her incision on her back. Notably, her sexual function was normal, and she was able to achieve orgasm as pre-operatively. Overall, the patient was extremely pleased with the results of the operation.
A 37-year-old Caucasian female with history of obesity and iron-deficiency anemia presented with painful left lower extremity swelling. A physical examination revealed an edematous, tender, and mildly erythematous left lower extremity. Mild tachycardia was noted. A lower extremity venous duplex ultrasound showed an extensive, occlusive deep venous thrombosis (DVT) from the left common femoral vein to the calf veins. Also, there was occlusive DVT within the left common and external iliac veins. A computer tomographic (CT) imaging of the chest with contrast revealed pulmonary emboli within the subsegmental pulmonary artery branches in the right lower lobe. Heparin was administered intravenously. Catheter-directed venous thrombolysis was done, and complete clearing of the left iliofemoral DVT was achieved. Intravenous heparin was switched to rivaroxaban, and the patient was discharged home. A week later, the patient presented with right-sided pleuritic chest pain. A CT of the chest with contrast revealed new acute pulmonary emboli within the distal left lower lobar artery and the basilar segmental pulmonary arteries of the left lower lobe. A venous duplex ultrasound showed extensive left lower extremity DVT from the left common femoral vein to the calf veins and occlusive thrombus within the left external iliac vein and the left common iliac vein. Intravenous heparin drip was initiated. Inferior vena cava filter was placed. Catheter-directed venous thrombolysis was performed, and complete thrombolysis of the left lower extremity DVT was noted within 24 hours. Three days later, heparin was stopped, and a low molecular weight heparin, lovenox, was started. Within 24 hours of starting lovenox, the patient noticed increased left leg tightness and worsening pain. A venous duplex ultrasound was performed and again revealed extensive, occlusive DVT from the left common femoral vein to the left popliteal vein and a nonocclusive DVT in a small left external iliac vein. By this time, platelet count had decreased from 448 × 109/L at the time of admission to 147 × 109/L (normal, 150–400 × 109/L). Enzyme-linked immunosorbent assays (ELISA) were performed in order to evaluate for the presence of heparin associated platelet antibodies (HAPA) and antiphospholipid antibodies (APLA). Lovenox was stopped, and argatroban was started. TPA thrombolysis with aspiration thrombectomy was performed, and a follow-up venography demonstrated complete removal of thrombus from the popliteal, femoral, and common femoral veins. Three days later, while the patient was on argatroban, a venous duplex ultrasound again demonstrated extensive, occlusive DVT in the distal veins (posterior tibial, peroneal, and gastrocnemius) extending to the popliteal, femoral, deep femoral, and common femoral veins. Argatroban was stopped; arixtra, 10 mg daily, was started, and monitoring with serial venous duplex ultrasound was continued. Three days after starting arixtra, partial recanalization of DVT within the left external iliac vein and the left femoral popliteal system was demonstrated. Platelet count nadir at 134 × 109/L. HAPA was strongly positive 1.412 (normal, <0.4 OD). Serotonin release assay was positive with 82% (normal, ≥20%) serotonin release with low-dose heparin (0.1 U/mL) and 0% (normal, =20%) serotonin release with high-dose heparin (100 U/mL). Antiphospholipid antibody profile demonstrated elevated anti-β2 glycoprotein 1, anticardiolipin, and positive lupus anticoagulant (). Once platelet count stabilized and DVT recanalization was achieved, Coumadin was started. Coumadin was overlapped with arixtra for 5 days after INR reached therapeutic level. Three months after the initial hospital encounter, antiphospholipid antibodies levels were still elevated, confirming the diagnosis of antiphospholipid syndrome. We recommended against pregnancy giving her significant thrombotic risk, and the risk of fetal toxicity with Coumadin.
A 41-year-old Asian man presented to our hospital after an automobile accident in which he sustained a severe crush injury to his right foot that resulted in formation of a soft-tissue lesion in the weight-bearing heel and instep area. After the debridement, the size of a full-thickness defect at the medial and anterior aspects of the heel measured 6cm in length and 4cm in width (Figure ). Because the extensive zone of injury involved the proximal instep area, an ipsilateral instep island flap was not possible. Therefore, a plan was made to cover the defect using a free instep flap.The patient was placed in the prone position, and both legs were abducted for simultaneous flap elevation and preparation of the recipient site. The operation was performed under tourniquet control for better visualization. Debridement of the defect was performed, and the stumps of the posterior tibial artery and vein, as well as the tibial nerve, were indentified and dissected proximal to the defect. In harvesting the instep flap from the contralateral foot, the skin paddle was designed 7cm in length and 5cm in width and marked in the instep area (Figure ), so that it was slightly larger than the defect to allow for flap-moulding. Flap dissection began with an incision along the distal border of the flap down to the fascia, which then was incised and dissected in a plane superficial to the abductor hallucis muscle. The distal branches of the medial plantar artery were carefully indentified and divided. The intermuscular septum between the abductor hallucis muscle and the flexor digitorum brevis containing the medial plantar artery, nerve and its fasciocutaneous perforators was raised in continuity with the flap. During flap dissection, the medial plantar nerve and the cutaneous branches of the first toe were preserved, but cutaneous nerve fascicles were retained within the flap. To obtain adequate length of these fascicles, we performed interfascicular dissection of the medial plantar nerve proximally. To facilitate flap harvest, the abductor hallucis muscle was divided at its origin, exposing the neurovascular pedicle deep into it. The neurovascular pedicle of the medial plantar artery and venae comitans was dissected proximally up to the common origin of the medial and lateral plantar artery from the posterior tibial artery (Figure ). The abductor hallucis muscle was reattached, and the donor site was covered with a split-thickness skin graft taken from the thigh (Figure ).\nSo as not to sacrifice an important artery in the traumatized foot of our patient, inset of the flap into the defect was followed by end-to-side anastomosis of its medial plantar artery to the recipient posterior tibial artery. The venous anastomosis was carried out end-to-end. One artery and two venae comitans were anastomosed. An epineurial window was meticulously made on the medial surface of the tibial nerve to ensure nerve-sprouting from sensory nerve fascicles. The cutaneous nerve fascicles of the flap were then anastomosed end-to-side with the recipient tibial nerve. When the tourniquet was freed, vascularization of the flap was noted (Figure ).The donor and recipient sites healed without any complications during 3 weeks of follow-up (Figure ). Two months after surgery, the patient was able to wear normal footwear and had a normal gait. At his 3-month follow-up examination, the patient subjectively reported an increase in sensation at the recipient site compared to immediately after surgery. Static two-point discrimination was 20mm compared to 18mm of the contralateral normal side at the 6-month follow-up visit. At 30 months after surgery, no ulceration in the flap due to poor contact with the shoe had occurred.
A 24-year-old unmarried woman was admitted to the Department of Neurology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China because of severe unresponsiveness and twitching in October 2019. Subsequent computed tomography of the skull showed low-density shadows of the left temporal lobe and bilateral frontal lobe. Magnetic resonance imaging (MRI) of the skull and intracranial blood vessels showed direct enhancement of the cranial veins and brain. Diffusion imaging of the brain showed multiple filling defects, abnormal enhancement and diffuse signals at the distal end and branch of the superior sagittal sinus, the left transverse sinus-sigmoid sinus-internal jugular vein and the right transverse sinus. The patient’s prothrombin time was 13.4 s, prothrombin activity was 77% and D-dimer was 0.88 μg/ml. The patient was considered to have CVST with brain parenchymal damage (cerebral oedema and venous infarction). Therefore, the patient underwent whole cerebral angiography via femoral artery catheterization and catheterization thrombolysis. After the operation, the patient was given comprehensive treatments, such as thrombolysis, sedation and lowering of intracranial pressure.\nThe patient’s bone marrow aspiration result was verified to be normal. To identify the cause of CVST in such a young woman, a thorough medical history was taken and the patient reported a history of increased menstrual flow for 6 months, increased vaginal discharge and a peculiar smell for 1 month. Prior routine examination of leucorrhoea in the local hospital showed inflammatory changes. After taking oral anti-inflammatory drugs for 1 month, the condition did not improve. Then, the patient was given an urgent examination with uterine accessory ultrasound to prompt the following question: is this condition uterine disease? MRI of the pelvic cavity demonstrated an enlarged cervix with an irregular shape. The neurologist asked the gynaecologist for two consultations in total. The first physical examination found that there was redness and swelling at the vaginal opening and that the tumour was limited to the vaginal opening, and then a biopsy was performed. Five days later, the second physical examination showed that there was a mass of approximately 9 cm in diameter in the vagina, covered with pus and stained with blood, and the pedicle could not be touched (). After the patient's neurological symptoms improved, she was transferred to the Department of Gynaecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China for gynaecological treatment in November 2019.\nAfter being transferred to the Department of Gynaecology, a gynaecologist (L.M.) performed two physical examinations and found that the tumour was progressively prolapsed. The diameter had increased from 11 cm to 14 cm, of which the pedicle could not be touched (). The patient's cancer antigen (CA) 125 level was 36.4 U/ml. The pathological examination of the vaginal mass confirmed a tumour. Therefore, the patient was given laparoscopic total hysterectomy and bilateral salpingo-oophorectomy. During the operation, it was found that the patient's cervical tumour was approximately 15 × 15 × 15 cm, touching the thick pedicle, which was approximately 2.5 cm in diameter. What was confusing for the physicians was that the patient's uterus was found to be completely inverted and adhered to the left ovary and the front wall of the rectum, but there was no mass in the uterus. The two fallopian tubes were stuck together and the appearance of both ovaries was normal (). As there was no evidence of extrauterine spread of the tumour at the time of surgery, the tumour was staged as clinical group I based on the Intergroup Rhabdomyosarcoma Study (IRS)-I. Finally, postoperative histology confirmed embryonic cervical rhabdomyosarcoma with a tumour size of 18 × 16 × 7 cm, which invaded the whole layer of the cervical wall, and a tumour thrombus was seen in the vessel (). Immunohistochemistry revealed the following: CD10 (++), CD117 (–), CD34 (–), CD68 (+), CKpan (−), desmin (++), DOG1 (–), Ki-67 (90%+), LCA (–), lysozyme (–), MyoD1 (–), myogenin (+), S-100 (–), SMA (–) and vimentin (+). However, the patient declined further chemotherapy and radiotherapy, and then she was discharged.\nTwo months later in January 2020, the patient quickly showed signs of relapse. A physical examination revealed a mass of approximately 8 × 8 × 5 cm in the vaginal stump that was crumbly and bled when touched (). Based on the IRS treatment scheme, adjuvant chemotherapy with vincristine, actinomycin-D and cytoxan was administered (2 mg vincristine on day 1 + 0.4 mg actinomycin-D on days 2–6 + 0.4 g cytoxan on days 2–6; intravenous infusion). As the patient’s condition progressed rapidly, the vaginal mass progressively increased to 13.53 × 9.92 × 10.74 cm in size () and there were lung metastases and para-aortic lymph node metastases during the second chemotherapy session. The patient refused subsequent chemotherapy and was discharged. Two months later, the patient died of complications. The reporting of this study conforms to CARE guidelines.Written informed consent was obtained from the patient to publish this case report and the patient was reassured that all of her information would be deidentified.
A 43-year-old male underwent tooth extraction for jaw pain and experienced heavy bleeding; prompting imaging that revealed a large mass of the left maxilla, invading the hard palate and orbital floor. He underwent prophylactic endovascular coil embolization of his left IMA prior to biopsy of the mass to prevent further hemorrhage. He was diagnosed with a solid type adenoid cystic carcinoma of the left maxilla, T4N0M0. He was referred to Otolaryngology-Head and Neck Surgery and underwent hemimaxillectomy with a three-segment stacked fibula free flap reconstruction guided by preoperative virtual surgical planning. The vascular pedicle did not have sufficient length to anastomose to branches of the external carotid artery, so a vein graft was used to create a Corlett loop reaching from the neck to the reconstructive site. Initial attempts to anastomose to the facial artery were complicated by thrombosis; thus, the distal external carotid artery was used and the flap successfully revascularized. The patient recovered uneventfully and underwent adjuvant proton irradiation with concurrent chemotherapy. Following the completion of radiation, he developed dehiscence of the posterior extent of the reconstructed palate that was successfully managed with a palatal obturator.\nEight months after treatment completion, the patient removed a small wire from his maxillectomy cavity that was consistent with a vascular coil from his previous IMA embolization. CT angiography was obtained notable for partial coil extrusion from a left IMA branch, with a separate vascular blush concerning for pseudoaneurysm of another IMA branch (). Several days later, he developed left-sided epistaxis, which was controlled with nasal packing and subsided after approximately 30 minutes. This was felt to represent a possible sentinel bleed; although his ECA was ligated proximally, there was thought to be sufficient backflow to fill the pseudoaneurysm and cause symptomatic bleeding, as has been reported previously [].\nDue to inability to access patient’s IMA via the ECA as it had previously been utilized for free flap reconstruction, options considered included endoscopic exploration of the maxillectomy cavity for direct vessel ligation; neck exploration or percutaneous approach to access the distal stump of the ECA; or retrograde access for IMA endovascular embolization via the undissected STA. The decision was made to attempt retrograde access via the STA.\nThe patient underwent a left preauricular incision to isolate the STA from surrounding parotid parenchyma by the Head & Neck Surgery team. Under microscopic guidance, the STA was cannulated with an 18-gauge angiocatheter () by the Neurosurgery team, which was secured in place with 2-0 silk suture. A rotating hemostatic valve (Abbott Laboratories, Chicago, IL) was attached to the angiocatheter and a retrograde angiogram was performed, confirming STA access (). An Echelon 10 microcatheter (Medtronic, Minneapolis, MN) and a Synchro 14 microwire (Stryker, Kalamazoo, MI) were then used to select the proximal left IMA for digital biplane angiography. Hand-injected control angiography confirmed appropriate microcatheter positioning, demonstrating opacification of the left IMA and branches, with nonspecific contrast blush including the left sphenopalatine arteries. Thereafter, liquid embolization using Onyx® Liquid Embolic System (Medtronic, Minneapolis, MN) was performed to occlude the IMA. A final angiogram demonstrated satisfactory occlusion of the left IMA without evidence of extravasation, aneurysm or pseudoaneurysm (). The STA was decannulated and ligated, and the preauricular incision was closed primarily.\nThe patient tolerated the procedure well and was discharged on postoperative day 1 in stable condition. In follow-up on postoperative day 10, he had no further evidence of epistaxis or other symptoms.
The patient is a 35 year-old woman who was diagnosed with GSD Ib at the age of 10 months. Dietary treatment was started with good metabolic control. Since the age of 9 years the patient has received G-CSF treatment for neutropenia and recurrent infections. Since the age of 10 she has suffered from inflammatory bowel disease. The clinical course was further complicated by recurrent abdominal abscesses. At age 25, subtotal colectomy was performed due to long-distance stenosis of the transverse colon with an abscess in the right upper abdomen. Postoperative wound healing was impaired and required surgical wound revision and closure. Pancreatic fibrosis was observed at age 28, exocrine pancreatic insufficiency was diagnosed at age 31, and supplementation with pancreatin was initiated. Hepatosplenomegaly was observed, and the thrombocyte count was significantly reduced to around 40 G/l, possibly due to the massive splenomegaly. At age 33, the patient presented with acute abdominal pain in the lower abdomen. MRI was suggestive of a pelvic abscess, enlarged pelvic retroperitoneal and mesenterial lymph nodes as well as inflammatory alterations of the intestine. The patient had bloody stools, and her overall clinical condition was reduced. Antibiotic therapy with meropenem, ampicillin and metronidazole was started. Explorative laparotomy with extensive resection of the small bowel (side-to-side jejuno-jejunostomy 70 cm distal of the ligament of Treitz and end-to-end jejuno-jejunostomy 150 cm distal of the ligament of Treitz, resection of the stenosing ileodescendostomy) was performed. Abdominal bleeding occurred in the postoperative course, which required 4 further laparotomies within 3 weeks. Secondary surgical closure of the wound could be performed 4 weeks after the initial operation. However, within the consecutive weeks a large wound dehiscence developed with severely impaired wound healing. At its maximum the wound had a diameter of 29 × 18 cm. Surgical revision of the abdominal wall defect after 1 year was not of permanent success. A colonoscopy was performed alongside this procedure, which revealed mild to moderate inflammatory activity. Supplementation with protein powder as well as arginine and glutamine was started to promote wound healing, but was also without success.\nAt age 35 the patient first presented to our metabolic clinic. Her body weight was 50 kg, body length 153 cm and BMI 21.4 kg/m2. She was in good metabolic control under a GSD diet with regular meals every 2 h during the day and continuous nocturnal feeding with tube feeding formula and maltodextrin (composition of diet assessed by a 3-day dietary protocol: carbohydrates 47% of energy intake, fat 34% of energy intake, and protein 18% of energy intake). Continuous glucose monitoring was performed using a Dexcom G6 device. Transaminase activities (AST 13 U/L, ALT 19 U/L, normal 10–35 U/L), serum triglycerides 140 mg/dL (< 150 mg/dL), uric acid 5.4 mg/dl (2,4–5,7 mg/dL) and lactate 1.0 mmol/L (normal 0,5–1,6 mmol/L) were normal. Total protein, albumin and pre-albumin concentrations in serum were also within the respective reference ranges (7.6 g/L (normal 6.4–8.3 g/L), 4.2 g/L (normal 3.5–5.2 g/L) and 0.21 g/L (normal 0.2–0.4 g/L), respectively). Abdominal ultrasound still showed massive hepatosplenomegaly (liver 17.8 cm, normal < 15 cm; spleen 27.1 cm, normal < 14 cm). Thrombocyte count was severely reduced with 42 G/L, whereas the hemoglobin level was normal at 11.6 g/dL. The patient had about 10–11 loose to watery stools per day. The Crohn’s Disease Activity Index (CDAI) was 398. This index is the gold standard to determine current activity of Crohn’s disease. A CDAI score < 150 is defined as remission of Crohn’s disease, while a value of greater than 450 is defined as severe disease. The Crohn-like disease was treated with budesonide 3 mg/day, 5-aminosalicylic acid 3 × 1 g/day, vitamin E 800 mg/day and loperamide 2 mg. Stool calprotectin was within the normal range (42 mg/kg, normal < 50 mg/kg), CRP was normal (0.7 mg/dl, normal < 1 mg/dl), and the blood sedimentation rate was 25 within the 1st hour (normal < 20 mm). The clinical condition was stable but the overall well-being was markedly impaired, the appetite was reduced. The abdominal wall defect was 8 cm × 6.5 cm and oozing (Fig. ). Colonoscopy or capsule endoscopy was not performed due to the high risk of abdominal complications. Anogenital lesions had not occurred within the last 20 years, oral lesions only after special triggers such as dental cleaning.\nAfter informed consent for this individual treatment the patient was admitted for the initiation of empagliflozin therapy. The starting dose was 5 mg/day, and the dose was subsequently increased to 2 × 5 mg/day on day 2, and 10 mg/5 mg on day 3. Absolute neutrophil count at admission was 1600/μl under 1.05 μg G-CSF/kg/day. The dietary regimen was continued as usual, and no relevant hypoglycemias were observed. The minimal blood sugar was 4.3 mmol/L. The patient could be discharged on day 4. Within the following weeks the neutrophil count increased, and the G-CSF dose could subsequently be decreased stepwise as shown in Fig. . The appetite and well-being improved. The stool frequency also decreased to 5 to 7 stools per day, and loperamide could be terminated. Wound-healing distinctly improved and the abdominal wall defect slowly granulated (Fig. ). After 1 month, the empagliflozin dose was increased to 2 × 10 mg/day. Administration of GCSF was terminated 41 days after the start of empagliflozin (Fig. ). No major side effects of empagliflozin were observed, such as urogenital infections, hypoglycemia and ketoacidosis. Albumin concentration remained stable within the normal range (4.2 g/L, normal 3.5–5.2 g/L). The body weight remained stable during the treatment period, and no relevant change in thrombocyte count or haemoglobin level was observed. The CDAI score decreased to 184 on day 50 of empagliflozin treatment. Fasting tolerance also slightly increased. The wound size on day 85 of treatment was about 3 × 1.5 cm.\nAs PMN from GSD Ib patients are known to show impaired ROS production and an increased apoptosis phenotype, we analysed both apoptosis as well as reactive oxygen production in neutrophils from our patient (Fig. ) on day 50 of empagliflozin treatment. As shown in Fig. a, spontaneous apoptosis of isolated PMN did not differ compared with healthy control cells. Furthermore, apoptosis could be efficiently reduced by supplementation of cell culture media with GM-CSF (Fig. b). PMN apoptosis could be fully abrogated by the pan-caspase inhibitor Q-VD, an effect which was even more pronounced in patient PMN compared to control cells (Fig. c). We next measured reactive oxygen species of patient and control PMN after PMA stimulation and observed no significant differences, further confirming the stabilized phenotype of patient PMN under treatment with empagliflozin.
A 29-year-old female patient had first reported to the ENT Department of the Cantonal Hospital in St. Gallen, Switzerland, in 2001, with severe and progressive nasal blockage. Symptoms had begun 5 years earlier but had been left untreated. She was however known to have an aspirin and histamine intolerance. In addition, she had suffered from symptoms that had been interpreted as hay fever and asthma since her youth and reported a positive family history for nasal polyposis.\nPhysical findings documented at the time included a normal appearing external nose, complete bilateral obstruction of the nasal cavities by nasal polyps, and anosmia. The anosmia was determined by testing with Sniffin' sticks whereupon the patient was able to identify 3 out of 12 possible odors.\nA CT scan showed hypoplastic frontal sinuses and a complete obstruction of all the other paranasal sinuses, with normal bony anatomy of the external nose. After medical treatment with topical and systemic steroids failed, the patient underwent an endoscopic sphenoethmoidectomy and maxillary sinus fenestration in 2002. Upon histologic examination of the polyp tissue respiratory mucosa with severe chronic eosinophilic inflammation was found. Postoperative treatment included topical and systemic corticosteroids. Aspirin desensitisation therapy was repeatedly discussed but declined by the patient. The sense of smell returned and the patient now identified correctly 11 out of 12 possible odors. The patient was free of recurrence during 5 months of follow-up in the hospital before continuing follow-up in the vicinity of her home. During this further follow-up, the sense of smell was not documented.\nIn March 2014, the 38-year-old patient was again referred to the ENT Department of Cantonal Hospital in St. Gallen, Switzerland, because of complete bilateral nasal obstruction with polyps visible in both nares and a progressive widening of the bony and cartilaginous nose. The patient reported a recurrence of nasal obstruction and a loss of sense of smell over the last 2-3 years and an increasingly noticeable nasal deformity in the last 12 months. Also pressure was felt over the maxillary but not frontal sinuses frequently. Postnasal drip symptoms were present in most days. Nasal treatment consisted of sporadic application of topical mometasone spray. Bronchial asthma symptoms had been stable with regular inhalation with formoterol and budesonide and regular systemic check-ups had shown no sign of bronchiectasis.\nClinical examination showed a distinct broadening and bilateral enlargement of the nasal pyramid (). Anterior rhinoscopy revealed massive nasal polyps obstructing the cavum and vestibule of the nose on both sides (). On a CT scan, a complete obliteration of the nose and paranasal sinuses as well as thinning and massive expansion of the nasal bone was seen (). A comparison to the CT scan from 2002 was not possible. Since the picture data was archived for 10 years and then destroyed in 2012, the written radiologist's report describes no nasal bone thinning or expansion. The patient's test results with Sniffin' sticks again showed an anosmia (identification of 2 out of 12 possible odors). Medical treatment consisting of topical and systemic steroids failed and an endonasal endoscopic polypectomy and revision ethmoidectomy were performed in May 2014. Simultaneously, the bony nasal vault was narrowed by forced digital compression without osteotomies (). The bone appeared to be thinned sufficiently to give in upon digital compression. Impression of the bony nasal sidewall resulted in a visible depression in the overlying skin which was corrected by a transcartilaginous decollement of the skin and subcutaneous tissues in a supraperiosteal plane. No nasal packing was inserted. A nasal cast was applied and left in place for one week.\nHistology of the polyps again showed squamous epithelium and respiratory mucosa with light to medium signs of inflammation, some fibrosis, and severe eosinophilia. The patient reported a dramatically improved nasal air passage. A strict postoperative regimen including nasal saline irrigation and topical application of mometasone spray and fluticasone nasal drops was installed. At 3-month follow-up, minimal polypoid tissue was noted in the ethmoidal region, the nasal air passage remained free, and the patient reported a return of the sense of smell and was able to identify correctly all 12 possible odors in the Sniffin' sticks test. The form of the exterior nose remained stable (). Again an aspirin desensitization was discussed but declined by the patient. The most recent follow-up in January 2015 (8 months after surgery) showed stable findings.
A 65-year-old woman presented with left breast lumpiness to the symptomatic unit and appointment regarding triple breast assessment clinic was arranged.\nPhysical examination of the symptomatic left breast revealed no suspicious findings.\nBreast radiology showed a mammographic asymmetry within the upper outer quadrant of the left breast (). The level of suspicion for malignancy was low, and this was therefore assigned BI-RADS 4A.\nThis persisted on additional views. High-resolution sonography revealed no suspicious focal mass. There were only some minimal fibrocystic changes which could potentially correlate with indeterminate mammographic findings. However, the sonographic examination was noncontributory.\nThe imaging was reviewed and decisions regarding core biopsy with titanium clip placement were made.\nPatient informed the team that had some mild adverse skin reactions to base metals previously; namely common materials such as copper, lead, nickel or aluminum used in costumes or jewellery. There was no history of previous medical interventions, autoimmune diseases, and atopic dermatitis, and she was not aware of any allergy or prior contact with titanium.\nSince the asymmetry in keeping with an area of interest was well seen on mammography, an open coil–shaped hydro-mark titanium clip was chosen to enable the potential follow-up and exact correlation with sonography. The hydro-mark clips are stainless, based on hydrogel technology, and consist of both hydrogel and titanium. These markers hydrate the site of interest after deployment and subsequently enable long-term visualization with both ultrasound and T2-weighted MRI sequence. Moreover, the left-sided mammographic abnormality regarding our patient was nonpalpable. Thus, the titanium-based clip was used in order to mark the sampled site and provide good correlation across all other breast imaging modalities that could be used for further verification in the future if required.\nPost-clip mammogram confirmed that the location of the marker corresponded well with the mammographic abnormality ().\nSubsequent histopathological examination revealed benign fibrotic changes with no evidence of malignancy or atypia. Thus, given the positive final diagnosis, no further imaging was required, and the patient was discharged.\nHowever, 7 weeks later, the same person re-presented with increasing pain and discomfort within the upper outer quadrant of the left breast.\nHigh-resolution ultrasound examination and mammography were again performed. No drainable collection or abscess were visualized (). The marker clip was identified, and the location correlated with patient’s tenderness (). Otherwise, the appearances on imaging were unremarkable.\nTherefore, following a multidisciplinary meeting, MRI and second professional opinion were requested. These were noncontributory, demonstrated neither any new significant findings nor suggested to alter the management.\nThe woman continued to be symptomatic, felt persisting increasing discomfort, itching, and insisted on the removal of the metallic component. There were no associated dermatologic or systemic manifestations. An abscess or other drainable collection post-biopsy were excluded with an ultrasound and suspicion of adverse reaction to titanium was raised.\nHowever, the patient was informed that it is still uncertain if any invasive interventions would fully treat her symptoms.\nPossible options regarding surgical and radiological interventional procedures were discussed.\nFollowing conversations and permission granted in full knowledge of risks and benefits, the patient decided to undergo ultrasound-guided vacuum clip excision. The procedure was successfully performed under local anesthesia. The titanium clip within the tissue sample was identified ().\nNo immediate or delayed complications were observed. Moreover, the undesired symptoms subsequently resolved. The total duration of the titanium clip implantation within the affected breast was 15 weeks. Histopathological examination demonstrated lymphocytic infiltration of the tissues (). No overt foreign body granuloma was identified. Histology in combination with clinical history and breast radiology were suggestive of a delayed reaction and sensitization to titanium material.
A 49-year-old man with a history of acromegaly was admitted to our hospital with the concern of recurrent shortness of breath and dyspnea on exertion during the previous 2 years, and he had experienced an episode of presyncope 2 weeks prior without any further evaluation. He was a chef in a local restaurant for almost 30 years. He had no family history of any diseases and no past history of hypertension, diabetes mellitus, sleep apnea, or sudden cardiac death. He did not smoke or consume alcohol. The patient provided a history of stereotactic radiosurgeries twice in a decade or so and adherence to treatment with a somatostatin analog (octreotide given 40 mg once per month through intramuscular injection) at the time of diagnosis 20 years before. The patient was overweight and moderately nourished. He was 1.85 m (73 inches) tall, weighed 134 kg, and had a body mass index of 39 kg/m2. His blood pressure was 110/60 mmHg, and his heart rate was 92 beats/min with sinus rhythm. He had distinct skeletal features that included prominent superciliary arches and nose bridge, enlargement of the tongue and lip, and large hands and feet. Cardiac auscultation revealed irregular premature beats and pathological third heart sound, and a systolic murmur was discovered over the apex and aortic area. Bilateral extensive borders of cardiac dullness were noted. His physiological reflexes were present without any pathology. An electrocardiogram demonstrated sinus rhythm with wide (160 ms) QRS duration of left bundle branch block (LBBB) (Fig. ). The patient’s condition was classified as New York Heart Association (NYHA) stage III–IV.\nOn admission, magnetic resonance imaging showed pituitary macroadenoma. Given the symptoms described, we arranged blood testing of myocardial injury markers showing an elevated brain natriuretic peptide level of 740 pg/ml indicating cardiac failure (Table ). Hormone laboratory tests performed subsequently demonstrated excessive secretion of GH and IGF-1, twofold greater than the reference normal upper limit, which was consistent with pituitary macroadenoma (Table ). Other routine analyses of liver and renal function were roughly normal.\nA Holter monitor was ordered for underlying arrhythmias to explain the patient’s dyspnea, chest discomfort, and presyncope. It demonstrated sinus rhythm with an average heart rate of 68 beats/min, frequent ventricular premature beats, and nonsustained ventricular tachycardia (up to 2200 ms) (Fig. ).\nA chest x-ray showed a cardiothoracic ratio (CTR) of 78%. Echocardiography showed diffuse impairment of left ventricular (LV) systolic motion, reaching an LVEF of 16%. We noted hypertrophy of the ventricular septum at 18 mm, ventricular dilation, with LV diameter of 72 mm. The right ventricle and atrium and the left atrium were also dilated with moderate mitral regurgitation and mild tricuspid regurgitation. There was no associated systolic anterior motion (SAM) of the mitral valve. Dyssynchrony of the biventricular systolic motion was apparent.\nGiven an exertional component to the symptoms together with echo presentations in order to better exclude ischemic cardiomyopathy, coronary angiography was performed, which showed normal coronary arteries without stenosis, and left ventriculography applied simultaneously revealed an EF of 20% with diffuse LV hypokinesis.\nGiven the patient’s previous medical history of acromegaly, the absence of obstructive coronary artery imaging findings or segmental dyskinesia, family history of hypertrophic cardiomyopathy (HCM), symmetric hypertrophy, as well as absence of SAM of the mitral valve, acromegaly-induced cardiomyopathy was confirmed, which was absolutely opposed to coronary heart disease (CHD) and HCM.\nThese results indicated that it was probably not a case of hereditary cardiomyopathy; therefore, we diagnosed the patient as having secondary dilated cardiomyopathy due to acromegaly, even taking it a step further progressing to congestive heart failure secondary to acromegaly-induced dilated cardiomyopathy.\nChronic excess of GH and IGF-I secretion affects cardiac morphology and performance [], so etiological treatment for acromegaly-induced cardiomyopathy is crucial to suppressing GH secretion or blocking GH action for the sake of reversing acromegaly-induced cardiomyopathy. The mainstay of treatment acknowledged globally is surgical resection of the pituitary adenoma [], which was unfortunately considered high-risk given our patient’s cardiac condition (NYHA stage III–IV). Although stereotactic radiosurgery combined with somatostatin analogs and GH antagonists administrated previously were effective in suppressing hormones, they could not help his cardiac function. Therefore, we carefully administered diuretics, vasodilators, angiotensin-converting enzyme inhibitor (ACEI), β-blockers, and spironolactone for management of heart failure following the current guidelines []; in the meantime, octreotide (200 μg/day) was administered for the control of GH excess. After good compliance of pharmacotherapy and a regular medical examination regimen for nearly half a year, the serum GH and IGF-1 concentrations decreased from 32.50 ng/ml to 1.98 ng/ml and 627.00 ng/ml to 229.10 ng/ml, respectively, but the patient was hospitalized again because of uncontrollable cardiac failure. Accompanied by the normalization of GH and IGF-1 levels, the patient’s cardiac function did not seem to take a favorable turn upon readmission. Though echocardiography showed a recovered EF value from 16% to 28%, a significant ventricular mechanical dyssynchrony was detected as formerly. Electrophysiological study was performed using a nonaggressive stimulation protocol, which revealed a nonsustained ventricular monomorphic tachycardia []. In the presence of overt ventricular dyssynchrony, complete LBBB, LVEF< 35%, inducible ventricular tachycardia, and symptomatic heart failure despite guideline-directed medical therapy, surgical indication was rarely assessed by neurosurgeons, and stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects. Therefore, we boldly recommended cardiac resynchronization therapy with defibrillator (CRT-D) implantation based on device implantation official guidelines [, ]. The patient underwent CRT insertion finally and was discharged to home 5 days later, pharmacotherapy continued as usual (Fig. ).\nTelephone follow-up was arranged, and the patient claimed symptom improvement following the device insertion 1 month later and was basically back to normal life. We required that he return for follow-up at 1 month, 3 months, and 6 months after the interventional therapy. The patient has been followed in our outpatient clinic for nearly half a year now. During his last visit, echocardiography identified improved LVEF of 54%, and a chest x-ray showed reduced CTR of 60%. The patient was in NYHA functional class II (Fig. ).
A 35-year-old male patient presented in our out-patient department (OPD) in June 2014 with diplopia, ptosis, and excessive fatigability and was diagnosed with AchR antibody-positive generalized MG. Contrast enhanced computerized tomography (CECT) of the chest revealed an ill-defined heterogeneously enhancing lesion in the anterior mediastinum. He was treated with cholinesterase inhibitors (pyridostigmine) and low dose of oral steroids with success. He was doing well till December 2014 when he developed relapse of symptoms in the form of diplopia, ptosis, and fatigability with additional manifestation of dysarthria and dysphagia. His doses of pyridostigmine were titrated to maximum possible doses and a short tapering course of oral steroids was given with addition of a steroid-sparing drug, azathioprine, which was titrated up to 150 mg in divided doses. Patient slowly improved and underwent thymectomy (diagnosed with hepatitis B virus-positive in preoperative screening) in February 2015. Histopathology was suggestive of thymoma B1 with cystic degeneration. He was doing well on symptomatic treatment, till end of January 2016 when he developed relapse of symptoms with dyspnea following an episode of fever with cough. He was admitted in ICU on February 2016 in crisis and was kept on ventilator after endotracheal intubation. He went for five cycles of PE without much improvement and subsequently IVIG (2 g/kg) was infused over 5 days, and he started showing improvement and was weaned off ventilator after 5 weeks in March 2016.\nAfter 24 hours of removal of the ventilator support of 5 weeks, the patient again needed the ventilator support. After 2 weeks of failed attempt of weaning of the ventilator support, IVIG was infused again for 5 days without any success. We went for five cycles of PE in May 2016 without any success and four cycles of rituximab (300 mg/m2) were then infused in June 2016 with partial success when he intermittently managed to stay off the ventilator for few hours. After much interdisciplinary deliberation to rule out any possible cause responsible for refractory state crisis, we repeated CECT again to rule out thymoma as well as sent hepatitis B DNA copies. Tenofovir was added after gastroenterologist consultation. The patient remained ventilator dependent on polypharmacy with our constant attempt to titrate symptomatic and immunosuppressive therapy till October 2016 and we faced multiple challenges in the form of ventilator-associated pneumonia and recurrent leucopenia. Azathioprine could not be titrated more than 150 mg due to leucopenia and Tenofovir was stopped after consideration of its potential to cause leucopenia. The patient again went for 10 cycles of alternate-day PE in October 2016 and slowly he was weaned off the ventilator completely in November 2016 after 7 months on the ventilator. He was transferred out to wards after 9 months of ICU stay. Over the next two months, the patient developed ptosis and diplopia, despite being on maximum tolerable doses of symptomatic and immunosuppressive treatment for which an additional 10 cycles of PE were administered. Finally, his tracheostomy closure was done in January 2018 after a period of 11 months. The patient went for a total of 45 cycles of PE, 2 cycles of IVIG infusion, and 1 cycle (four doses—300 mg/m2) of rituximab during his hospital stay and presently he is doing well on symptomatic and immunosuppressive therapy.
A 45-year-old male presented with painless and progressive swelling on the right frontal region over 2 years prior to examination. According to patient history, the “swelling” started over his eyebrow and progressively increased in size. He underwent a biopsy at an outside facility and the pathology report diagnosed neurofibroma but patient failed to follow-up. He was later referred to our hospital after the lesion enlarged in size and he had developed difficulties opening his eyes. He had no history of trauma, bone pain, systemic disease, or neurological symptoms. Physical examination found an extensive scalp lesion that measured 30 × 20 cm in size, extending from the right orbital rim toward the contralateral side and the parietal region. Upon palpation the lesion was firm but without any tenderness []. The skin on the tumorous area was adherent to the underlying soft tissue and the lesion was immobile. There was no visible venous dilatation or any audible bruit over the lesion. A small post biopsy scar was present at the middle of the lump. Neurological examination was unremarkable and the patient did not bear any signs of NF-1 as well as in their family tree.\nComputed tomography (CT) scan of the head revealed a calvarial soft tissue mass predominantly located in right frontoparietal with infiltrating mass on the frontal region and associated right frontal bone defect [Figure and ]. Magnetic resonance imaging (MRI) of the brain revealed a soft tissue mass that was iso-hypointense on T1 showing lobulated mass at frontal region [], which enhanced homogenously after contrast administration []. The mass extends to the frontal base viewed by coronal plane [] and various intensity on T2 imaging []. The tumor showed extension from extracranial compartment through the bony defect into the right frontal lobe and frontal base. The decision was made to pursue resection.\nThe patient was positioned supine with back slightly elevated 20° and without any head fixation. A wide marginal excision with 4 cm distance from neoplasm margin was performed. At surgery, tumor tissue was found to be soft, fleshy, moderately vascular, and mostly encapsulated with some areas displaying ill-defined margins. The mass was eroding through the internal table of the bone and infiltrated the dura mater as well as the intradural compartment. The mass was highly vascular and bled easily when touched. The bone at the right frontal region appeared moth-eaten and was removed with rongeurs until a normal hard and thick border was identified. A wide intracranial portion of the lesion was removed without any involvement of brain parenchyma, and a fascia lata graft was used for duroplasty. The postoperative bone defect measuring 10 × 10 cm was closed using a titanium mesh. A vascularized free flap was raised from anterolateral thigh and sewn in by the plastic surgeon to close the skin defect. The patient's neurological status remained intact postoperatively. He was discharged from the hospital uneventfully.\nOver a period of 6 months, the patient was seen in regular follow-up when a recurrence was seen on routine imaging and also new complaint of ptosis on left side. The images show local recurrence on right frontal lobe; interestingly, a new lesion was prominent on contralateral cavernous sinus []. Patient underwent surgery for the right frontal lobe mass and 20 gray external beam radiosurgery for cavernous sinus lesion. There was partial response as the ptosis complaint got better and mass shrink nearly 50% of its size. After 2 years the tumor recurred again on both right frontal and also cavernous sinus, but the patient refused any other medical intervention. Patient died two-and-a-half years after initial diagnosis of this disease.
A 70-year-old man presented in 2017 after discovery of a hypervascular, expansile, 7 cm pancreatic body mass involving the splenic vein on routine surveillance CT scan (). He endorsed 1 year of diarrhea and steatorrhea that he had not sought medical care for but denied weight loss, appetite changes, fatigue, pain, or jaundice. He had a history of RCC and underwent a left nephrectomy and adrenalectomy in 2002. Additionally, he developed adenocarcinoma of the ampulla of Vater and was treated with pancreaticoduodenectomy in 2003.\nHe developed pulmonary metastases from the RCC and was treated with right lung segmentectomy in 2006 and three bilateral CyberKnife treatments in 2010, 2011, and 2013. He had surveillance CT scans roughly every 6 months from the discovery of the 2010 lung metastasis until 2015 and then yearly thereafter. He never received any chemotherapy for either the RCC or PDA. He is a former smoker.\nUnfortunately, records of his prior malignancies, treatments, and surgeries were limited. His metastatic workup consisted of a CT scan of the head, chest, abdomen, and pelvis, which revealed no other sites of tumor except the 7 cm mass in the body of the pancreas, and testing his carcinoembryonic antigen (CEA) tumor marker level, which resulted as 0.9 ng/mL. The two main differential diagnoses were metastatic RCC versus recurrent PDA. Imaging characteristics, timeline of the mass development, and normal CEA level were the investigations used to determine that metastatic RCC was most likely and supported the decision to resect the mass.\nThe patient underwent completion pancreatectomy and splenectomy to resect his newly discovered pancreatic lesion. Surgical exploration and dissection revealed that the previous pancreaticoduodenectomy had been completed entirely antecolic. The gastrojejunostomy was able to be left intact throughout the duration of the procedure, whereas the hepaticojejunostomy was reconstructed due to its initial position being too far anterior in relation to the current surgical bed. Due to vascular invasion, a part of the portal superior mesenteric–splenic vein junction was resected with primary venovenostomy. His postoperative course was uncomplicated, and he was discharged on postoperative day 6.\nDue to his apancreatic status, he received diabetic teaching, was commenced on parenteral insulin, and referred to an endocrinologist for follow-up. He was also counseled on pancreatic enzyme supplementation and scheduled to have repeat CT scans at 4 months postoperatively, and then, every 3 months thereafter provided no active disease progression. Due to his splenectomy and asplenic status, he received appropriate vaccinations for encapsulated organisms: Pneumococcus, Meningococcus, and Haemophilus Influenzae.\nGrossly, a 6.5 × 6.2 × 4.5 cm well-circumscribed mass is located within the pancreatic parenchyma (). On cut surface, it is glistening, red-purple, and slightly lobulated, with foci of hemorrhage. Histologically, the tumor has alveolar and solid growth pattern, with rich network of small thin-walled blood vessels (). At higher power, the lesional cells have clear cytoplasm with distinct cell borders, with large and round nuclei (). The morphological features in conjunction with the history are consistent with a metastatic clear cell RCC. The margins are negative, and 1/6 lymph nodes is positive for metastatic carcinoma.\nThe patient followed up with his medical oncologist who advised against adjuvant chemotherapy or radiation therapy, opting for imaging surveillance for now despite the one positive lymph node.
An 8-month-old female patient was brought to the dentist by her mother with a complaint of an ulcerative growth in the hard palate. Her mother had noticed an erupting tooth in the anterior part of the upper gums when the patient was 3 months old. She also noted a small ulcerated swelling in the same region about 3 months ago. The lesional area was impacted with food debris resulting in foul odor. There was bleeding from the lesional site on manipulation. There was no other complaint except for occasional mild rashes on the scalp and trunk. The patient was afebrile, and active with no disturbance during sleep.\nOn examination, there were no significant extra-oral findings. Intra-oral examination revealed an ulcerative growth present in the alveolus of the right maxilla extending from the region of 51 to 55 t and also extending onto hard palate, measuring 1.5 cm × 1.5 cm in size. The ulceration had a raised edge. The crown of 52 was noted within the lesion and was mobile []. The other teeth present in the oral cavity were the erupting 71 and 81. The clinical impression was a slow growing ulcerative lesion of the right maxillary alveolus extending onto the hard palate.\nThe posterior-anterior and lateral skull views showed a cystic lesion present in the right maxillary region, and a radiological diagnosis of a maxillary alveolar cyst was given. There was no lesion extending to the skull vault.\nComputed tomography (CT) scan showed a nearly spherical mass in the right side of the palate measuring 1.37 cm (anteroposteriorly) × 1.44 cm (width) × 0.63 cm (height). Destruction of the adjacent alveolar process of the maxilla and anterior aspect of the hard palate on the right side was noted along with the loss of normal bony crypts of upper teeth. Tooth 52 was pushed out prematurely. Multiple enlarged lymph nodes were observed in the neck bilaterally. No focal lesion was seen in the visualized lung apices.\nBlood investigation showed no significant findings. During biopsy, all the crowns of the developing teeth were seen floating within the lesion. The dental surgeon was unable to separate the lesion from the surrounding normal tissue; hence tissue had to be obtained as piecemeal.\nThe gross specimen consisted of one large and several small soft tissues. All the soft-tissue bits were white to dark brown in color and firm in consistency. The largest tissue measured 1.3 cm × 0.3 cm × 0.6 cm while, the smaller soft-tissues collectively measured 1.0 cm × 0.3 cm × 0.6 cm in size.\nMicroscopically the soft-tissues showed ulcerated hyperplastic parakeratinized stratified squamous epithelium with a moderately collagenous connective tissue stroma that was diffusely infiltrated with pale staining cells resembling histiocytes. The lesional cells had an indistinct eosinophilic cytoplasm and exhibited indentation of nuclei. Varying numbers of eosinophils, lymphocytes and plasma cells were interspersed among the lesional cells []. Abundant hemorrhagic foci were evident. The lesional cells stained positive for S100 and CD1a []. Odontogenic epithelial cells, dentine and enamel matrix were also present. As the lesional cells were positive for CD1a a diagnosis of LCH was confirmed.\nOur patient was referred to the oncology department where she was staged as a multisystem high risk case. Our patient was totally asymptomatic with the multisystem involvement being totally unrecognized leading to delay in parents seeking treatment. The patient was placed under ARM B protocol for LCH consisting of an initial treatment with prednisolone, vinblastine, etoposide and a continuation treatment with oral 6-mercaptopurine followed by pulses of oral prednisolone, vinblastine and etoposide. Despite the treatment plan adopted, the baby succumbed to her disease within 6 months after the initial diagnosis.
A Caucasian male in his early 60s presented to the McLaren Macomb Emergency Department (ED) with a complaint of bilateral hip pain and inability to ambulate. The pain had been present for the past four-to-five days and was progressive in nature. The patient admitted to being fairly active and normally able to ambulate without the assistance of a cane or walker up until symptoms began. He described his pain as 10 out of 10 constant pain in the anterior bilateral hip joints with mild radiation into his lumbar back and groin, with no radiation into his legs. His pain increased with ambulation but improved with rest. He attempted to take three doses of aspirin at home without any relief in his symptoms and began using a walker to help him ambulate.\nThe patient had an extensive medical history. This history included sigmoid diverticulitis with sigmoid colectomy in 2012, deep vein thrombosis, duodenal ulcer, benign prostatic hypertrophy and hypertension. He also suffered from chronic obstructive pulmonary disease, chronic thrombocytopenia, peripheral vascular disease, atherosclerotic coronary artery disease and paroxysmal atrial fibrillation. He had been diagnosed with end stage renal disease and was on hemodialysis. He subsequently had multiple hemodialysis catheter infections with bacteremia (MRSA), obstructive uropathy with chronic hydronephrosis, and several peritoneal and pelvic abdominal abscesses. He denied having noticed any recent changes in his ostomy output.\nThe patient’s past surgical history included inferior vena cava filter placement, cholecystectomy, sigmoid colectomy with end colostomy, numerous cystoscopies, bilateral ureteral stents, peroneal abscess incision and drainage, esophagogastroduodenoscopy, colonoscopy, several IR drainages of pelvic abscesses, exploratory laparotomy with enterolysis, temporary catheter placement, and numerous arteriovenous fistula creations.\nThe patient took amiodarone, Prilosec, Imdur, and aspirin. He had no allergies to any medications. He denied use of nicotine, alcohol or any illicit drugs. He lived with and took care of his elderly mother. His father had died from a cerebral vascular accident and his mother had a history of coronary artery disease.\nPhysical exam revealed a well-nourished, well-hydrated male who was in no acute distress. He had been brought from home via ambulance as ambulation was too painful. His vital signs demonstrated hypotension with a blood pressure of 71/51. Patient admits that this was his typical baseline. His pain was worse on the right than the left. He had increased tenderness to log roll, axial compression, and passive flexion of the right hip when compared to the left.\nHe had intact two-point discrimination and light touch in the L2-S1 nerve distributions bilaterally. He had intact +2 out of 4 dorsalis pedis and posterior tibial pulses found with Doppler ultrasound. He elicited 5 out of 5 muscle strength in his bilateral lower extremities. There was no tenderness to palpation over the greater trochanteric region of either hip. He had a negative straight leg raise bilaterally. The patient had severe brawny edema and dryness to his lower extremities.\nLaboratory studies demonstrated a normocytic anemia, chronic thrombocytopenia, hyponatremia, and chronic kidney disease. His sedimentation rate and c-reactive protein levels were elevated. When the patient was sent over for x-ray imaging, left hip, right hip, and pelvis x-rays demonstrated no abnormalities. Lumbosacral x-ray demonstrated mild scoliosis with diffuse moderate degenerative change. (Image 1) The patient was offered pain medication but refused any other treatment. He was admitted to the hospital due to his intractable bilateral hip pain and inability to ambulate. Orthopedics, Nephrology, and Physical Therapy/Occupational Therapy consults were placed.\nOrthopedics was the first service to evaluate the patient on hospital Day 2 and ordered a CT of the abdomen and pelvis with contrast. This study found that superior to the bladder, there was a 4.9 x 3.8 cm fluid collection with peripheral enhancement concerning for abscess. (Image 2) Internal Medicine then evaluated the patient and reviewed the results of the CT scan. Blood cultures were obtained and the General Surgery service was consulted. A repeat CT with oral contrast was ordered by General Surgery on hospital Day 3. This test showed that the adjacent loops of the intestines along the anterior and superior margin of the fluid collection were partially opacified without obvious extravasation of oral contrast in the region, suggesting fistula (i.e., communication with the bowel). (Image 3)\nThe blood cultures grew out E. coli x 2 after 24 hours. The patient was placed on Zosyn and vancomycin. On hospital Day 5, Interventional Radiology performed CT guidance drainage of the pelvic abscess with catheter placement. Culture results would later grow E. coli. The Urology service was also consulted as the patient had history of hydronephrosis due to obstructive uropathy with bilateral stent placement. This service was asked to evaluate the need for stent replacement and integrity of the bladder with its close approximation to the abscess.\nOn hospital Day 6, the patient went to the operating room with Urology for a bilateral retrograde urogram. The study demonstrated findings consistent with communication of the urinary bladder with the abscess cavity previously drained. The stents were exchanged, a urinary catheter was placed and a urine sample was obtained for which culture results came back positive for E.coli. The patient remained on Zosyn.\nAs the patient continued to experience significant bilateral hip pain, an MRI of the patient’s thoracic/lumbar spine and bilateral hips was ordered on Day 7. A thoracic spine MRI demonstrated a small central disc protrusion at the Thoracic 4-T5 level. No spinal canal stenosis or foraminal encroachment. The MRI of the lumbar spine and bilateral hips was unable to be completed since the patient could not tolerate the pain long enough to have the study performed.\nThe pelvic drain was removed between hospital Days 9 and 11, although progress notes are unclear as to the specific day. On hospital Day 15, the patient was discharged to a subacute rehabilitation unit on a two-week course of oral Augmentin. He had worked with Physical Therapy throughout his hospital stay. The patient slowly regained some ability to ambulate but it is unknown whether or not this was from resolving his pelvic abscess or due to rehabilitation with Physical Therapy. He was to follow up with the urologist in two-to-three weeks for urinary catheter removal.
A 49-year-old woman with a past medical history of pulmonary embolism presented for IVC filter removal, which was initially placed approximately three months prior to this hospitalization. She was not a smoker and had no history of chronic lung disease or occupational hazards. At that time, the patient had right leg deep vein thrombosis and right sub-segmental pulmonary artery embolism. Hematological workup was inconclusive and it was presumed to be provoked by prolonged bed rest in the setting of bariatric surgery. The patient had a history of intracranial aneurysm in the posterior circulation so long-term anticoagulation was not thought to be a safe option. She got IVC filter placed via the right femoral approach.\nIn the present hospitalization, interventional radiology (IR) guided IVC filter removal was done in the operating room (OR). Under X-ray fluoroscopy, a snare was inserted into the internal jugular vein, and the IVC filter was withdrawn from its hook (Figure ). Per the OR notes, it was a difficult approach requiring manipulation of the snare. The patient was transferred after the procedure into an observation room.\nWithin an hour after the procedure, she developed pleuritic chest pain, hypotension, presyncope, and shortness of breath. She denied any other systemic symptoms like nausea, vomiting, diarrhea, joint pain, and bowel or bladder symptoms. Her physical examination showed a blood pressure of 85/60 mmHg, a pulse of 122 beats per minute, a respiratory rate of 26 breaths per minute, and an oxygen saturation of 92%. She had a poor inspiratory effort. Her jugular venous pressure was elevated (9 cm of H2O). On cardiovascular examination, her heart sounds were markedly diminished, and the point of maximal impulse was nonpalpable. No murmurs, gallops, or rubs were appreciated. Her chest was clear on auscultation. The results of her abdominal and neurological examinations were also unremarkable. Her pertinent laboratory findings revealed PT/INR of 3.4, a potassium level of 3.1 mEq/L, and hemoglobin of 10.6 g/dL. The other laboratory tests were unremarkable. Her chest X-ray revealed low lung volumes with bibasilar subsegmental atelectasis. An electrocardiogram (EKG) revealed sinus tachycardia, electrical alternans, low voltage QRS complexes, and a prolonged QT interval (Figure ).\nBedside echocardiography (echo) at the symptom onset showed a large anterior, small to moderate lateral and posterior pericardial effusion. Left ventricular and right ventricular functions were grossly normal. Transthoracic echo revealed moderate to severe pericardial effusion, 2.1 cm in the largest dimension with right ventricular collapse during diastole consistent with the tamponade effect (Figure ). It also showed a large echogenic mobile structure, consistent with pericardial thrombus.\nShe was started on intravenous fluid resuscitation and immediately transferred to the OR. With an emergent pericardiocentesis, 300 mL of blood and a large clot was removed, and a pericardial window was placed. Postoperative transthoracic echocardiography (TTE) revealed that pericardial blood volume was significantly improved with only a small amount left anteriorly. The pericardial thrombus was no longer evident. She remained in the hospital for two days after the procedure. The drainage catheter was removed on the second day when it drained less than 25 ccs of fluid over the day. She was subsequently discharged in a stable condition, and an outpatient follow-up was advised.
A 2-month-old baby girl weighing 3.5 kg presented with a huge swelling on the left side of her neck. The swelling was small in size when noticed at birth, which progressed gradually to the present size [Figures and ]. The child presented with a huge swelling with restricted mouth opening and difficulty in feeding. Examination revealed swelling on the left side of the neck sized 10 cm × 10 cm, which was cystic, non-tender and extending from the angle of the mandible to the clavicle. The skin over the swelling looked normal with no local rise of temperature. The swelling was huge and presented with difficulty in swallowing. In anticipation of rupture of the swelling and aspiration of its contents and also because it could lead to airway obstruction, it was decided to operate the child.\nPre-operative evaluation was thoroughly carried out. Evaluation for concurrent anomalies like Down's syndrome, Turner syndrome or congenital heart defects was done. The size and extent of the neck mass was defined carefully in an effort to detect the potential for airway compromise and to avoid soft tissue trauma during intubation.[] Chest X-ray was done to exclude the presence of chest infection and intrathoracic extension of the tumour.\nA rescue tracheostomy by the surgeon was available as a standby during induction.[]\nBecause cystic hygroma presents with difficult airway challenge to the anaesthesiologist, a difficult airway cart was kept ready. The child was premedicated with intravenous (IV) atropine 70 mcg. A shoulder roll was used to keep the child at optimal laryngoscopic position as the child had a larger occiput compared with the rest of the body. Because a larger tongue in the child could obstruct the airway after induction, an inhalational induction was considered.[]\nAs sevoflurane was not available in our institute, the child was induced with halothane in oxygen by face mask. A straight blade laryngoscope was used as children have an anterior, cephalad placed larynx and short and stout epiglottis.[] Halothane does not have a noxious smell and is still commonly used for the gaseous induction of anaesthesia in places where budgetary concerns limit the use of sevoflurane.[] At first attempt, laryngoscopy was difficult as the vocal cords were not visualised and only the epiglottis could be visualized, which was shifted to the right side due to displacement of the soft tissues. The child was ventilated with mask and a second attempt of laryngoscopy was made. This time the glottis could be seen after shifting the soft tissues towards the left side by the assistant and the trachea was successfully intubated with uncuffed endotracheal tube (ETT) size 3.5 mm, and it was fixed just 1 cm beyond the vocal cords in order to avoid accidental extubation. Anaesthesia was maintained with 66% nitrous oxide in oxygen with controlled ventilation with Jackson Rees modification of Ayre's T piece. Inj. Fentanyl 8 mcg and Inj. atracurium 2 mg was administered and supplemental doses 0.1 mg/kg were used as and when necessary. Blood loss throughout the surgery was calculated to be about 100 ml and was replaced. The cysts were multiloculated and excised completely. All the vital signs were stable in the intra-operative period. At the end of surgery, the child was reversed with Inj. Neostigmine 0.05 kg/kg and Atropine 0.01 mg/kg. In view of possible collapse of trachea and obstruction to airflow, it was decided not to extubate the trachea in view of the difficulties faced during intubation.\nThe child was shifted to the Neonatal intensive care unit and was extubated on the second post-operative day after return of adequate muscle power, respiratory efforts, cry and movements.
Our case is an 11-year-old Sudanese girl, residing in Qatar, who presented to the pediatric outpatient department with an 18-month history of progressive abdominal distention. The parents had been attributing the abdominal distention to weight gain, especially as the child remained otherwise asymptomatic. They had in fact been encouraging the girl to lose weight. The parents sought medical advice at a local health center, due to flu-like symptoms. The examination at the health center was impressive for signs of massive abdominal distention and she was admitted to our tertiary care hospital promptly for further investigations with a preliminary diagnosis of ascites. Apart from the progressive abdominal distention, there was no other contributory history suggestive of any underlying malignancy, liver disease, heart failure, or undiagnosed renal problems. The girl did not complain of any abdominal pain, constipation, urinary retention, or respiratory distress secondary to her abdominal distention. Our patient had achieved menarche 1 year ago with infrequent menstrual cycles; her first day of the last menstrual period was two weeks prior to her admission. There was no history of menorrhagia or dysmenorrhea.\nUpon examination, the child was noted to be in good general condition with normal vital signs for age and in no apparent pain or distress. Her weight was 64.5 kg, which was above the 95th centile for her age. Apart from the massive abdominal distention, she was thin built. Her general examination did not reveal any clubbing, pallor, icterus, peripheral edema, or lymphadenopathy. There were no stigmata of chronic liver disease. Abdominal examination revealed a huge uniformly distended abdomen (maximum diameter was 105 cm), extending from the pelvis to the xiphisternum with full flanks. There were no visible dilated veins on the abdomen. Palpation did not reveal any tenderness or masses; fluid thrill was positive. She had normal female genitalia. Her respiratory, cardiovascular, and nervous system examinations were unremarkable. A bedside urine dipstick did not reveal any proteinuria.\nAs the physical findings detected a fluid thrill, her preliminary investigations were directed towards finding a likely explanation for what seemed like a massive ascites. Her preliminary laboratory work-up which included a complete blood count, peripheral smear, serum electrolytes, renal and liver functions tests were within normal. As our patient did not show any signs of chronic liver or renal disease, there was a strong concern among the treating physicians that the presumed ascites could be secondary to an underlying abdominal malignancy. At this stage, the pediatric oncology team was consulted and tumor markers which included Ca125 (6 U/mL), CEA 0.7 microgram/L, alpha-fetoprotein (<1.7 IU/mL) and beta-hCG (<5 IU/L) along with Uric acid 297 micromol/L, and LDH (174 U/L) were ordered, all of which were within normal limits.\nAn urgent transabdominal ultrasonogram of the abdomen confirmed the suspicion of massive ascites (). The possibility of requiring a diagnostic paracentesis was discussed with the family once the MRI of the abdomen and pelvis reasonably ruled out any underlying malignancy.\nThe MRI of the abdomen and the pelvis () revealed that what was visualized as massive ascites by the sonographer was in fact a large homogenous well defined unilocular huge cystic abdominopelvic mass which measured 39 × 29 × 18 cm in dimension, occupying the entire abdomen and pelvis and bulging into the anterior abdominal wall. No solid component could be noted within the mass lesion. No loculation or septation was seen given the likelihood of serous cyst adenoma of the right ovary.\nA lower abdominal midline incision was made revealing the peritoneum. An elliptical incision is carefully made through the ovarian cortex to the cyst wall.\nWhen the cyst wall was reached, blunt and sharp dissection using surgical scissors was used to separate the cyst wall from the surface of the ovary. Intraoperative visualization did not reveal any abnormality of the left adnexal structures. The cyst was aspirated prior to its delivery and gave 13000 milliliters of fluid. The patient underwent right ovarian cystectomy with complete preservation of both ovaries and fallopian tubes. It weighed 13 kg and contained 13 liters of fluid. () Histopathological examination of the cyst revealed simple tubal-type epithelium confirming the diagnosis of a serous cystadenoma of the right ovary, consistent with the preoperative MRI diagnosis.\nOur patient did well after surgery and was discharged on the fourth postoperative day. Her discharge weight was 48 kg. Upon follow-up a week after her surgery she showed an excellent recovery and will continue to have regular follow-up in our outpatient clinic with an ultrasound examination every three months for early detection of any recurrence.
We present the unique case of a 28-year-old male patient displaying a complex clinical picture with mental retardation and various behavioural problems since birth. Symptoms of the autism spectrum comprising difficulties in social interaction and communication are reported since childhood. Additionally, he suffers from auto-aggressive tics in terms of beating himself with objects against his head and lower jaw, head movement tics and simple vocal tics. Striking dysmorphic features are not evident.\nExcept a one-time bleeding in week 20 of gestation, pregnancy had been without any complications. No infections, medication, smoking, or intake of alcohol or drugs during pregnancy was reported. The patient was delivered in week 40 of gestation with the help of a ventouse due to irregular cardiac activity. During delivery there were minor signs of birth asphyxia. Birth weight was 2.900 g (25th percentile), birth length 51 cm (50th percentile) and head circumference 33 cm (<3rd percentile). During infancy a prominent frontal fissure was conspicuous. A premature ossification of the sagittal fissure could not be detected.\nThe patient showed psychomotor retardation: he walked alone only by 26 months and was not able to sit alone falling over to one side without shoring up even at the age of 2. Furthermore, tics in terms of eye blinking as well as a muscular hypotonia were described. The patient’s parents reported early autistic features such as difficulties in social communication and interaction with avoiding eye contact and poor interest in social interaction. Development of speech was delayed (first words with 18 months). He refused body contact and demonstrated stereotypic patterns of behaviour such as filling bowls without showing any variations.\nWhen examined at the age of 27 months, the patient presented some special facial features such as synophrys, epicanthus, modelled ears, a deep joined thumb and microcephaly. His weight was 10 kg (3rd percentile), his length 88 cm (25th percentile) and his head circumference 46 cm (2 cm above the 3rd percentile). The patient only spoke a few words and never a whole sentence. Mostly, he only repeated the words he had heard before in terms of an echolalia. A considerable general delay of development with severe perceptual disturbance and autistic traits was diagnosed.\nSince the age of two years, the patient shows relevant aggressive symptoms such as throwing his head on the floor or biting into items. He needs extensive support concerning all activities of daily living and requires constant daily routines. During nursery school, auto-aggressive symptoms exacerbated in terms of head banging behaviour injuring his jaw and ears. At the age of 3 years, he started grinding his teeth. Later on, he presented head throwing movements against his left shoulder and banging of one row of his teeth against the other. There was a pattern of aggravation of the tic symptoms during stressful situations. He repeatedly showed refusal of meals and sleeping disturbances. At the age of 3 years, once there was a query febrile convulsion associated with an infection. Apart from that, there was no evidence for further seizures and clearly no epilepsy.\nAccording to ICD-10 and DSM-5 classification, the clinical features of the patient are consistent with early infantile autism as well as with Tourette syndrome.\nIn the clinical examination, the patient presented with cauliflower-ears as a result of his head banging behaviour and subsequent repetitive ear injuries as well as various injuries of all kinds and healing stages. Dysmorphic features or additional external malformations were not noted, there was no evidence of internal abnormalities (heart, eye, inner ear) either. The patient showed a preserved ability of speech comprehension with rare speech production.\nOur patient is the first son of non-consanguineous healthy German parents. His younger brother (24 years old) was diagnosed with an autism spectrum disorder (Asperger syndrome). There was no evidence of syndromal-secondary autism in the brother. He did not show intellectual impairment, dysmorphic signs or organic conditions such as a heart malformation or epilepsy. The mother’s paternal grandparents were cousins. They had five healthy children. In their further progeny three mentally retarded persons were reported, one granddaughter (died at the age of approximately 27 years) and two great-grandchildren (a boy and a girl). The exact diagnosis was unknown to our patient’s parents. Consanguinity of the retarded relatives’ parents was denied. One of the patient’s cousins (the son of his father’s brother) was said to suffer from clinically apparent dyslexia and perhaps an autism spectrum disorder. A disablement of a cousin of our patient’s father (son of his father’s sister) could not be specified further. An assessment of the father revealed no clinical characteristics, neither with respect to striking dysmorphic features, the occurrence of tics nor with respect to autistic symptoms. Further relatives with neurodevelopmental disorders were not reported on the father’s side of the family.\nConventional R-banded karyotyping of the patient was performed according to standard protocols with a resolution of approximately 500 bphs and revealed a structurally and numerically normal male karyotype (46,XY) in all 21 metaphases examined.\nFurthermore, the genomic DNA of the patient was examined by microarray-analysis (CytoSureTM Constitutional v3 Array 180 k, OGT (Oxford Gene Technology)) according to the manufacturer’s instructions. After hybridization, the array was scanned with the SureScan Microarray scanner (Agilent), the results were analyzed using CytoSure interpret software v.4.9 (OGT) against the Genome Reference Consortium human genome GRCh37 (hg19). Molecular karyotyping revealed a heterozygous deletion of approximately 719 kb (267 contiguous oligonucleotides) out of the chromosomal region 2q24.3 (karyotype according to ISCN (International System for Human Cytogenetic Nomenclature) (2016): arr[GRCh37] 2q24.3(165471418_166190427)× 1). The deletion encompasses the five genes GRB14 (exon 1 to intron 2–3), COBLL1, SLC38A11, SCN3A and SCN2A (exon 1 to intron 14–15) listed in OMIM (Online Mendelian Inheritance in Man) (Fig. ). With FISH (fluorescence in situ hybridization) analysis using probe RP11-150F4 (Empire Genomics) located at 2q24.3 the deletion could be confirmed. There is no evidence in literature that the microdeletion we detected is a common variant in the Caucasian population [, ]. “Orphanet” states a prevalence rate of a 2q24 microdeletion < 1/1000000 (worldwide) ().\nThe subsequently performed chromosome analysis of the parents, FISH with RP11-150F4 included, showed a normal female karyotype in the mother and a normal male karyotype in the father with no evidence of a deletion or rearrangement at 2q24.3. Therefore, it can be asserted that the deletion in the patient is a de novo mutation. The patient’s younger brother (suffering from Asperger syndrome) was shown to have a normal male karyotype without a deletion or rearrangement at 2q24.3.\nMagnetic resonance imaging conducted in 2014 showed no abnormalities of the patient’s brain. In 1991, a detailed investigation was performed with a metabolic screening of serum, urine and cerebrospinal fluid revealing normal results. In the EEG, there was a slow baseline activity without any epilepsy suspicious potentials. A proton-spectroscopy showed no abnormalities of N-acetyl-aspartate, choline and phosphor-creatinine levels. An ophthalmologic investigation exhibited no deviations. In 1997, gastrointestinal passage was unsuspicious, and in an X-ray of the brain no stenosis of the sagittal fissure was confirmed.
Mrs. E was a 57-year-old lady with a 12-year history of COPD, with an FEV1 of 30 %. Furthermore, she had a past medical history of carcinoma of the breast, type 2 diabetes mellitus and left ventricular failure (LVF), which was diagnosed on an echocardiogram (ECHO) in July 2014. Despite a diagnosis of COPD she continued to smoke 20–30 cigarettes/day.\nShe was prescribed a number of medications to help manage her airways disease including tiotropium, seretide 500, salbutamol, prednisolone and most recently oral theophyline (450 mg twice daily), which was started in April 2013 as an adjunct to her current medications.\nShe was admitted in June 2015 following a fall with notable decreased sensation bilaterally in her lower limbs and a tachycardia of 133 beats per minute. Spinal cord compression was an initial concern, however this was excluded with a magnetic resonance imaging (MRI) spine. There was no change to her theophylline prescription on admission to hospital and no new medication was prescribed that would affect theophylline metabolism.\nOver several days additional symptoms were noted, including worsening nausea, blurred vision, increasing fatigue (with stable blood sugars) and worsening speech with slurring of words. Development of vomiting resulted in a decision to measure her theophylline concentration and perform a CT head, as no obvious cause for her symptoms had been elicited. The CT head was reported as clear of any ischaemia, haemorrhage or space-occupying lesion.\nThe trough theophylline concentration was 59.3 mg/l. The last result obtained in July 2014 during an admission for influenza related infective exacerbation of COPD was reported as 20.2 mg/l, marginally above the 10-20 mg/l recommended reference range. The concentration was then not re-checked either in primary care or in hospital; it therefore became apparent this was a case of chronic theophylline toxicity.\nThe medical team at The National Poisons Information Service was contacted for advice on management of this patient. The rationale being abnormally high theophylline concentration, furthermore this was a case of chronic, rather than acute toxicity, for which we felt specialist input was required.\nThe patient was monitored for significant effects of toxicity; these include seizures (reported at trough concentrations of 50 mg/l []), reduced conscious level and tachyarrhythmias. Although Mrs. E suffered a persistent tachycardia, her heart rate remained in sinus rhythm. Cardiac monitoring was started. She was managed conservatively by stopping the theophylline and monitoring symptoms. Due to vomiting, oral activated charcoal was excluded and although charcoal haemoperfusion was discussed, it was not accessible at the RUH and as Mrs. E was haemodynamically stable and not acidotic, it was deemed not to be essential. This point will be discussed further in the conclusion.\nMrs. E developed electrolyte imbalances not long after the vomiting started, suffering hypokalaemia (3.2 mmol/l), hypomagnaesemia (0.56 mmol/l) and hypocalcaemia (1.96 mmol/l). This was postulated to be secondary to both vomiting and theophylline accumulation. This subsequently resulted in a pseudo-obstruction, diagnosed on abdominal x-ray. A nasogastric tube was placed and she was supplemented with appropriate electrolytes via intravenous infusion. An arterial blood gas demonstrated a pH 7.398 (7.34-7.44), pO2 (kPa) 9.24 (11–14), pCO2 (kPa) 6.36 (4.7-6.0), HCO3− (mEq/l) 21.7 (22–26), lactate (mmol/l) 1.5 (0.4-2.2). As no signs of acidosis were present and bicarbonate was at the low end of the normal range, we decided not to prescribe bicarbonate for this patient.\nAfter stopping her theophylline and without any active intervention her serum concentration fell gradually (Table ). By the time her theophylline concentration was within therapeutic range, her electrolytes had been corrected and her pseudo-obstruction had resolved. However, she developed signs of fluid overload, her heart rate also remained in persistent sinus tachycardia. Notably, findings of Congestive Cardiac Failure (CCF) were demonstrated on a previous echocardiogram and were substantiated by results of her computed tomography (CT) abdomen/pelvis and ultrasound sonography scan (USS) liver during her current admission; these both showed abnormal appearances of the liver consistent with cardiac dysfunction. It therefore appeared she had developed acute CCF. Ultimately, this resulted in challenging fluid balance management with serial chest x-rays demonstrating worsening bilateral effusions and renal function continuing to decline until the death of the patient.
A 69-year-old man presented at our hospital with a 5-month history of a rapidly enlarging mass in the right thigh. His past history was pulmonary tuberculosis treated with thoracoplasty and hepatitis C. Physical examination revealed the presence of a large irregular mass with multiple ulcers and necrotic tissues, measuring approximately 10 × 10 cm in the lateral side of the proximal thigh (). Admission laboratory data showed high levels of LDH, GOT, GPT, and ALP, which are 643, 80, 39, and 441, respectively. Typical tumor markers were within normal limits. A roentgenogram of the right proximal thigh showed no apparent expansion to the femur. Chest roentgenogram and computed tomography showed no apparent metastatic lesion and no focus of pulmonary tuberculosis. MR images demonstrated relatively a well defined and heterogeneous mass, which was attached to the fascia lata and expanded across the skin layer (Figures , ). The signal intensity of the mass was isointense to the adjacent skeletal muscles on T1-weighted images and inhomogeneous hyperintense on T2-weighted images. The mass was relatively well defined and enhanced with Gd-DTPA on STIR sequences (). However, partial infiltrating expansions to subcutaneous tissues of the mass made its margins unclear. Despite the mass contacted with the compression to the tensor fascia lata muscle, the gluteus maximus muscle, and the gluteus medius muscle, no apparent signal intensity changes of muscles were seen. Thallium-201 scintigrams showed a robust accumulation accorded for the mass in both of early and delayed phases (). No other abnormal accumulation was detected.\nBased on clinical findings and imaging characteristics, the tumor was diagnosed as a primary soft tissue tumor and an incisional biopsy was performed. Histopathology of the permanent sections showed the nodular or sheets-like proliferation pattern of small round cells with a high nuclear/cytoplasmic ratio in the background of sparse extracellular collagen (). Immunohistochemistry of the sections demonstrated that the staining of BCL2, MIC2, CD56, O-13, neuron specific enolase (NSE), and synaptophysin were positive. They were negative for HMB-45, S-100, broad cytokeratin (AE1/AE3 and MNF116), CD45, CD79a, CD3, and CD20. In addition, the RT-PCR showed the presence of EWS/FLI-1 fusion transcript from tumor specimens. Together with the results of several examinations, we diagnosed this tumor as EES.\nConsidering patient's age, hepatic dysfunction, and patient's disagreement with chemotherapy, we precluded the choice of the neoadjuvant chemotherapy. To define surgical margins especially at subcutaneous tissues and to increase tumor resectability, we determined to treat the patient with pre-operative low-dose RT and additional administration of sanazole before surgery []. As a result of the total dose irradiation of 30 Gy in 15 fractions with sanazole (0.6 g/m2 × 10 days), the tumor volume was reduced to approximately 40% of the pre-treatment condition (). On thallium-201 scintigrams, the accumulation of the tumor markedly decreased in both of early and delayed phases. Subsequently, we could perform the surgical resection with subtotal resection of the tensor fascia lata muscle, the gluteus maximus muscle, the gluteus medius muscle, and adjacent soft tissues to obtain wide surgical margins (). The huge surgical defect in the lateral thigh was repaired with a musculocutaneous flap of rectus abdominis (). There was no major complication in the perioperative period. Histological examination of the resected tissue demonstrated clear surgical margins. Because the tumor cells completely showed necrotic appearances and there were no viable tumor cells in the tumor specimen, we diagnosed the necrosis ratio as 100% (). Based on the histological findings, we could confirm a high efficacy of pre-operative low-dose RT with sanazole in the present case. The patient underwent follow-up without any local recurrence or distant metastasis, and presented without any postoperative functional disturbance for 24 months after surgery.
A 36-year-old Caucasian woman was evaluated with chief complaint of gluteal pain radiating to her leg. Her medical history was remarkable with gunshot injury to the affected leg with multiple pellets dispersed into her pelvis and proximal part of the thigh, as shown in Figs. and . She had gunshot injury 20 years ago. She was previously diagnosed as having lumbar disc herniation at L4–5 level. She underwent a previous discectomy outside our institution 2 years ago. A radiological examination revealed the presence of recurrent disc herniation, as well as multiple shotgun bullets in her pelvis and thigh. One of those bullets was deep into the sciatic nerve inside her quadratus femoris muscle.\nElectromyography (EMG) showed the presence of chronic sciatic nerve injury. Since it was clinically impossible to distinguish lumbar disc herniation from the sciatic injury, we decided to proceed with removal of the foreign object and neurolysis of the sciatic nerve followed by L4–5 discectomy and fusion. We decided to perform those procedures in two different settings. The first surgery included access to the sciatic nerve in the upper portion of her thigh and exposing the nerve fibrotic bands around the nerve. The dissection proceeded deep into the nerve within a muscle, where a bullet was found and removed. The distance from the bullets to the nerve was approximately 2 cm. Muscle tissue around the bullets was excised for analysis. For comparison, another specimen was obtained from the gluteal muscle, superficially away from the nerve and all the bullets. Two weeks later, she underwent scheduled L4–5 discectomy and fusion. Her postoperative course was uneventful. On follow-up examination at 6 months, she was essentially symptom free.\nA scanning acoustic microscope (AMS-50SI) developed by Honda Electronics (Toyohashi, Japan), whose schematic setup is shown in Fig. , was used in AI mode. It has a transducer with quartz lens, a pulser/receiver, an oscilloscope, a computer, and a display monitor. An 80 MHz transducer is installed within the microscope, which generates the signals and collects the reflected acoustic waves. Water is the coupling medium between the quartz lens and the substrate. For two-dimensional scans, an X-Y stage, controlled by a computer, is used. An oscilloscope analyzes the reflected signals from both the reference and target material after being collected by the transducer. As a result, acoustic intensity and impedance maps of the region of interest with 300 × 300 sampling points are obtained.\nThe principle of SAM in AI mode is demonstrated in Fig. . Distilled water is widely used as reference. The signal reflected from the target is\nwhere, S0 is the generated signal by the 80 MHz transducer, Ztarget is tissue’s AI and Zsub is the polystyrene substrate’s AI (2.37 MRayl). The tissue’s AI is calculated by combining the reflected signals from the tissue and the reference. The signal reflected from the reference iswhere Zref is the AI of water (1.50 MRayl). Then, the target’s AI is written aswith a constant signal S0 [] generated by the transducer.\nElectron microscopy-based imaging and chemical analysis studies were performed in a JEOL JIB-4601 focused ion beam scanning electron microscope (FIB-SEM) multi-beam platform coupled with an Oxford X-MaxN EDS system, as shown in Fig. .\nBlood samples were collected in test tubes containing ethylenediamine-tetraacetic acid (EDTA) and no anticoagulant on the day of the first surgery (foreign object removal) prior to the procedure. Then, 2 ml of 20% trichloroacetic acid (TCA) was supplemented into the blood samples to release the red blood cells (RBC) and other ingredients. The supernatant part was received from blood with TCA by centrifugation at 4000 revolutions per minute (rpm) for 20 minutes for the analysis of Pb and cadmium (Cd) within total blood. Coagulation of blood samples enabled serum trace element analysis: chromium (Cr), Fe, Cu, magnesium (Mg), manganese (Mn), selenium (Se), and Zn. The serum specimen was prepared using Hettich Universal centrifuge by centrifugation at 3000 rpm for 15 minutes, separating from cells immediately after and storing at − 20 °C until the analysis [].\nAfter weighing the left sciatic nerve tissue samples, they were digested with 2 ml of 65% nitric acid (HNO3) at 180 °C in the incubator for 1 hour. Then, 2 ml of 65% perchloric acid (HClO4) was added into the cooled mixture. Then, the mixture was digested at 200 °C in the incubator until the volume was halved. Digested materials were vortexed and diluted in water to a total volume of 10 ml. Concentrations were given in micrograms per gram (μg/g) wet tissue weight [].\nAll glassware were maintained at 10% (volume/volume; v/v) HNO3 before use, cleaned with deionized water, and dried in an incubator at 100 °C overnight. Pb, Cd, Cu, Cr, Fe, Mn, Se, and Zn elements were detected by inductively coupled plasma optical emission spectrophotometer (ICP-OES 6000, Thermo, Cambridge, United Kingdom). Measurements for each element were done three times and averaged. The ICP-OES was operated with argon carrier flow rate of 0.5 L/minute, plasma gas flow rate of 15 L/minute, sample flow and elusion rate of 1.51 L/minute, and peristaltic pump speed of 100 rpm, selecting the suitable wavelength for Pb, Cd, Cr, Cu, Fe, Mn, Se, and Zn, which were 220.353 nm, 228.802 nm, 267.716 nm, 324.75 nm, 285.213 nm, 357.610 nm, 196.090 nm, and 206.200 nm, respectively. Transport lines were obtained using 1.25 mm internal diameter polytetrafluoroethylene tubing. Element levels were indicated in micrograms per deciliter for serum (μg/dl) and μg/g for wet tissue. The standard concentrations for standard graph calibration were arranged from standard stock solutions of 1000 μg/ml for each analyzed element [].\nThe tissue samples were investigated by using AI mode of SAM. Figure shows the AI map of the tissue obtained away from the gunshot. The map was constructed by collecting the reflections of acoustic signals, generated by the transducer within SAM, from surfaces of the reference (water) and the tissue sample on the polystyrene substrate. At specific locations within the sample, the AI was calculated to be higher than 2 MRayl, indicating accumulation of elements with different elastic properties. Figure shows the AI map of the tissue obtained close to the gunshot. As can be seen in this image, almost everywhere had an AI of greater than 2 MRayl.\nSEM images of the tissue far away from the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. Similarly, SEM images for the tissue close to the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. The images were acquired at 5 keV energy for both tissue samples.\nThe SEM images show that the tissue far away from the gunshot keeps its original structure, whereas the tissue close to the gunshot seems to be deformed and torn up. These results demonstrate the degree of damage the impact of gunshot causes on soft biological tissues.\nTable represents the EDS measurements in SEM, carried out for determining the elemental distribution differences in the deformed tissues. The measurements show the percentages of the residue elements detected on tissues far away from the gunshot and close to the gunshot. According to the results, among all residue elements, Pb, Cr, Fe, and Mn are found to be higher in weight content in the region close to the gunshot, when compared to distant region. Cd and Cu levels do not differ much; however, Zn level is lower in the tissue close to the gunshot.\nWe determined Pb, Cr, Cd, Cu, Fe, Mn, Zn, and Se levels in both tissue samples. Pb, Cr, Fe, Se, and Mn levels were higher in the tissue close to the gunshot, conversely, Zn level was lower in this sample (Table ). Blood Pb and blood Cd, and serum Cr, Cu, Fe, Mn, Se, and Zn levels of our patient were also analyzed and the results are shown in Table , however, we did not observe significant differences when compared to reference values.
A 62-yr-old female ADPKD patient was admitted to our hospital for further evaluation of intermittent fever, nausea and left flank discomfort on April 26, 2012. The patient had a 15-yr-history of ADPKD that was detected when the patient was diagnosed with hypertension. Her two brothers also were diagnosed as ADPKD. However, the patient had no history of diabetes. The patient had been examined for ADPKD at our outpatient clinic since 2004. Despite a history of recurrent cyst hemorrhage, the patient achieved a full recovery from the hemorrhage with conservative management. In 2009, the patient was admitted for the treatment of suspicious cyst infection versus acute pyelonephritis. She received ciprofloxacin for the infection and had E. coli detected in the urine culture. Her renal function deteriorated progressively thereafter to begin hemodialysis from April 2011. Her daily urine output remained about 200 cc.\nTwo months before the current outpatient visit, she felt generalized weakness, anorexia, nausea and the discomfort in the left flank. She also developed intermittent fever with chilling sense one month prior to the visit. Therefore, the patient visited an emergency care center at the outside hospital to take CT scan which revealed about 8.1-cm large gas-forming cyst at the left kidney lower pole (). Her initial laboratory findings showed WBC counts of 22,190/µL and CRP of 25.5 mg/dL. The urinary sediment contained numerous RBCs and WBCs per high power field. Despite a 1-week course of intravenous antibiotic therapy with ciprofloxacin, her fever and flank discomfort persisted and subsequently resulted in general weakness and anorexia. The patient was referred to our clinic for further treatment plan including left nephrectomy. On admission, the patient had the temperature 38.4℃ and the blood pressure was measured as 150/85 mmHg. On physical examination, the patient showed local tenderness in the left flank. However, there was no definite presence of the costovertebral angle tenderness.\nOn clinical laboratory findings, the patient had WBC counts 12,850/µL, hemoglobin 9.7 g/dL, platelet counts 348,000/µL, blood urea nitrogen 39 mg/dL, creatinine 7.76 mg/dL, albumin 3.3 mg/dL, CRP 6.79 mg/dL. The urinary sediment contained numerous RBCs and WBCs per high power field. Although her laboratory parameters partially improved after a 1-week course of intravenous antibiotic therapy, her symptoms did not improve. Therefore, she was determined to undergo percutaneous cystostomy drainage of a large gas-forming cyst. The cyst was aspirated under ultrasound guidance followed by the percutaneous cystostomy drainage on hospitalization day 2. On clinical laboratory tests, cyst aspiration had RBC counts 290,000/µL and WBC counts 180,000/µL (polymorphonuclear leukocytes 98%, lymphocytes 0% and others 2%). In addition, a gram-stain revealed many ( > 10) WBCs and many ( > 10) G(-) rods. Finally, a culture of the cyst aspirate yielded E. coli, but the blood and urine culture were negative.\nThe patient was started on intravenous ciprofloxacin at a daily dose of 400 mg for 1 week. This was followed by an intracystic irrigation of ciprofloxacin through a percutaneous cystostomy drainage for 6 days. For the intracystic irrigation, 100 mg (50 cc) of ciprofloxacin was mixed with 450 cc of normal saline.Then repeated irrigation with the antibiotics mixture was performed. Following the treatment, the symptoms such as generalized weakness, nausea and discomfort in the left flank improved. This was also accompanied with the normalization of laboratory parameters.\nFollowing intravenous antibiotic therapy and intracystic antibiotic irrigation, the patient had the percutaneous cystostomy drainage removed. Her following CT scans showed decreased size of the infected cyst (8.1 to 4.7 cm) with internal mottled air attenuation (). The patient was discharged with oral ciprofloxacin for 6 more weeks.
A thirty five-year-old gentleman presented to us with gradually progressive difficulty in walking for one and a half years, due to dragging of the right foot on the ground. This difficulty was associated with slippage of footwear from the right foot, with his being aware of it. Despite the weakness, the patient was able to walk with support and had no disability in terms of activities of daily living. On lying down, the patient had spontaneous involuntary painful drawing up of legs, in response to noxious or non-noxious stimuli. He noted these abnormal movements six months after the onset of the illness and since the last two months, these movements were frequent enough to disturb his sleep. The patient also had increased frequency, urgency and incontinence of urine for the past one year. There were no sensory complaints. There was no history of headache, vomiting, seizures, head or back trauma or fever. There was history of low backache for the last two years, which was intermittent, radiating along the back of right thigh, not increasing on straining or with change in posture and which used to respond to analgesics.\nThe patient was of average build, with normal results on general physical examination. Neurological examination revealed normal higher mental functions and cranial nerves. Mental status examination included attention span, memory, fund of acquired information, manipulation of old knowledge, social awareness and judgment, abstract thinking, praxis, right-left orientation and frontal lobe functions, all of which were found to be within normal limits. Fundus examination was normal. Motor system findings were localized to lower limbs. There was grade 3 spasticity (Ashworth scale) in the right lower limb and grade 2 spasticity in the left lower limb. There was MRC grade 0/5 power of dorsiflexors, evertors and invertors and 5/5 of plantiflexors at the right ankle, and normal power elsewhere. Flexor spasms were present in both the lower limbs (spasm score 4). Bilateral knee and ankle jerks were brisk. The right plantar response was extensor while the left plantar was not elicitable. Sensory system examination showed 1 cm difference in two point discrimination on the anterior aspect of the right leg, as compared to the corresponding points on the left side, with normal primary modalities of sensation on either side. No abnormality was detected in other cortical sensations such as stereognosis, graphesthesia, sensory attention and other gnostic or recognition functions. There was high steppage gait on the right side.\nInvestigations revealed a normal hemogram and serum biochemistry. The patient had arrived with a diagnosis of noncompressive myelopathy, as the MRI scans of cervical and thoracic regions were normal. An MRI scan of the lumbosacral spine showed disc prolapse with minimal compression over thecal sac at L4-L5 level. The possibility of an intracranial lesion was considered due to abnormal cortical sensations in the presence of normal primary sensory modalities. A CT scan of the brain showed a large iso- to hypo-dense mass in the left fronto-parietal region, with areas of calcification []. Magnetic resonance imaging of the brain showed a heterogeneous space occupying lesion with areas of calcification, mass effect and minimal contrast enhancement []. Magnetic resonance spectroscopy demonstrated the elevation of the choline peak, with depression of N-acetyl aspartate (NAA) and creatine peaks. Directly observed EEG done on an analog machine at the time of occurrence of flexor spasms did not reveal any electrophysiological correlate. Biopsy of the mass was done through a burr hole in the left parietal bone over the mass, with a punch biopsy by a cruciate incision in the underlying dura. The biopsy revealed a grade II astrocytoma.\nThe patient was initially given intravenous dexamethasone and oral baclofen, with resultant reduction in spasticity and spasm score. Later, he received 60 gray of radiotherapy in 33 fractions. Chemotherapy with temozolomide was given along with radiotherapy. Follow-up examination at two months showed improvement in power at the right ankle joint, from grade 0 to grade II, and only occasional flexor spasms (spasm score 1).
A 42 year-old man with a past medical history of schizoaffective disorder, hyperlipidemia, and gastrointestinal reflux disease was admitted to an adult psychiatric unit secondary to intrusive thoughts that were sexual, homicidal, and suicidal in nature. Similar thoughts with tics were first reported 2 decades prior to the current hospital admission; however symptoms of depression were most prominent then and he was initially diagnosed with only major depressive disorder. During the following years there were documented reports of “paranoia and delusions related to contamination” as well as continued reports of intrusive violent thoughts also believed to be delusions. This led to a diagnosis of schizoaffective disorder; and antipsychotics became the primary modality of treatment over the next decade. Documented antipsychotics trials included aripiprazole, olanzapine, lurasidone, and quetiapine, all of which were associated with a lack of adequate improvement or adverse events. Records indicated that haloperidol combined with fluvoxamine did result in partial improvement. Antidepressants were also utilized intermittently over this 20-year period to target mood symptoms. Historically, the antidepressant trial with the highest documented relative dose was paroxetine 60 mg. It was during this time period with paroxetine that there was documented improvement of the patient's intrusive thoughts. Side effects limited use of the paroxetine 60 mg, and after it was decreased the violent intrusive thoughts worsened.\nInevitably the patient's outpatient provider after years of suboptimal response to antipsychotics initiated clozapine to target what was continued to be described as psychotic symptoms in the medical record. However, as the clozapine was gradually increased to 400 mg the patient noted a severe worsening of intrusive thoughts causing secondary depressed mood, anxiety, and suicidal ideation leading to hospitalization at our facility. It was during this admission that there was exploration of the patient's intrusive thoughts, which were clearly obsessions in that they were persistent, unwanted, egodystonic thoughts. Efforts by the patient to suppress or ignore these thoughts were futile, resulting in significant anxiety and distress. Yet while these obsessions occupied a significant amount of time during the day, impacting his social and work functioning, he denied any ritualistic behaviors or compulsions. Family history was found to be positive for an aunt and mother with hoarding disorder and a cousin with OCD. As the obsession were not attributable to the use of substances, a medication condition, or other mental disorders, the diagnosis of OCD was given for the first time based on criteria from the Diagnostic and Statistical Manual of Mental Disorders, 5th edition []. With the patient now diagnosed with OCD, he was appropriately treated; and there was a plan to initiate fluoxetine with a goal dose of 80 mg. Given the historical nature of the schizoaffective diagnosis, clozapine was continued taking into account there had been improvement during the hospitalization and that it would need to be slowly tapered as an outpatient to avoid adverse effects. The patient was safely discharged with minimal intrusive thoughts and it was recommended to the patient's outpatient provider that clozapine be discontinued after a slow taper. However, in the following months, the patient's outpatient provider had increased clozapine to 700 mg. This was associated again with a subsequent worsening and exacerbation of distressing intrusive thoughts leading to another hospitalization. Serum clozapine and norclozapine levels upon admission were found to be 453 ng/mL and 464 ng/mL, respectively. Clozapine was rapidly discontinued due to the association with OCS, as well as the patient's clinical presentation after each clozapine escalation. Clozapine was tapered over approximately 2 weeks without complications such as cholinergic rebound, catatonia, serotonin syndrome, or withdrawal dyskinesias [–]. More importantly there was no emergence of psychotic symptoms, strengthening the likelihood that the patient did not have schizoaffective disorder based on DSM-5 criteria.\nIn review of records and after discussion with the patient, it was determined that the past fluvoxamine trial did not reach an adequate dose for the treatment of OCD. Fluvoxamine was started and increased to 200 mg after clozapine had been allowed to wash out. Low-dose aripiprazole was also initiated to augment the fluvoxamine given the severity of the presentation, the benefits of this antipsychotic in treatment resistant OCD, and the low risk of inducing OCS []. The patient was discharged with resolution of intrusive thoughts and improvement in mood and still without emergence of psychotic symptoms. Following this hospitalization the patient began to see a therapist who specialized in exposure and response prevention therapy to address his OCD. Longitudinally, the patient struggled with depression and anxiety associated with residual obsessions resulting in fluvoxamine being changed to clomipramine. The last known dose of clomipramine was 150 mg and reported as having reasonable control of OCS as an outpatient. Up to a year following the second reported hospitalization at our facility there were no patient reports or documentation noted of criteria sufficient for a diagnosis of schizoaffective disorder or other psychotic disorders.
A 30-year-old woman (gravida 2, para 0, early abortion 1) at 21 weeks of pregnancy was referred to our hospital for placenta previa with a small amount of vaginal bleeding in April 2019. Three days before hospitalization, the patient experienced dark red vaginal bleeding due to unknown etiology. Ultrasound examination indicated placenta previa. On the day of her hospital admission, ultrasound examination showed that the lower edge of the placenta covered the cervical os, and the cervical length was 2.6–2.7 cm with internal os closed. The estimated thickness of the placenta at the os was 1 cm. A 1.3 × 1.3 × 1.0 cm area with uneven flocculating echo was found between the cervical internal os and the edge of the placenta. The patient's hemoglobin level was 118 g/L. A small amount of vaginal bleeding continued after hospital admission. Two daily intravenous infusion of 25% magnesium sulfate was applied to inhibit uterine smooth muscle contraction and 1.5 g cefuroxime was administered to prevent infection. At 17:40 on April 3, 2019, the patient suffered vaginal bleeding of 380 mL. Her uterine tension was low, and the abdomen was soft. At 18:10, the cumulative vaginal bleeding volume was nearly 800 mL (by weight) with bright red color. Because the severe vaginal bleeding was possibly life threatening, the patient decided to terminate the pregnancy. Her hemoglobin level was 104 g/L and hypersensitive C- reactive protein level was 7.6 mg/L. At 19:20, the blood pressure was dropped to 87/51 mm Hg, the patient was given 3 units of red blood cell suspension and 410 mL of fresh frozen plasma. At 19:30, a cervical ripening balloon was inserted through the placenta and was placed between the fetus and placenta with ultrasound guidance. Vaginal bleeding was rapidly reduced as the external force pulled the cervical ripening balloon to press against the placenta while expanding the cervix. At 22:55, the cervical ripening balloon was slipped out of the vagina. The cervix opening was 2 cm, the fetal head was close to the cervix; contractions were normal. At 23:00, the fetus was delivered via vagina; there was 350 ml of blood loss during the delivery. The placenta and fetal membranes were naturally delivered with placental defects of 4 × 3 × 3 cubic centimeters and fetal membrane defects of 2/3. Intravenous infusion of oxytocin (20 IU) was administered to promote uterine contraction, and curettage was conducted with ultrasound guidance. There was another 380 ml of blood loss during the curettage procedure. The uterus was massaged associated with intramuscular injections of 20IU oxytocin and 250 μg carboprost tromethamine, and intravenous injections of 100 μg carbetocin. At 23:15, the bleeding stopped after using 1 piece of intrauterine iodoform gauze. On the next day, her hemoglobin level was 88 g/L. There was no bleeding after intrauterine iodoform gauze removal. Next, 1.5 g cefuroxime was intravenously infused 2 times daily for 2 days to prevent infection. The patient's temperature was normal. On post-delivery day 5, hypersensitive C- reactive protein level was 25.8 mg/L, and hemoglobin level was 86 g/L. Transabdominal ultrasound showed a 0.8 cm wide echo band in the uterine cavity with no blood flow signal. The patient was successfully discharged at post-delivery day 6.
A 40-year-old male patient came to our hospital in 2014 with multiple intracranial artery dolichoectasia. It was diagnosed as an incidental finding on radiological examination of the patient. The patient had a history of coiling of the left cavernous internal carotid artery (ICA) aneurysm, which was done outside of our hospital. The patient had a history of systemic hypertension, and he was a chronic smoker. The computed tomography (CT) angiography findings in the year 2014 were, dilated and tortuous right supraclinoid ICA, fusiform dilated M1 segment of right and left middle cerebral artery (MCA) [], fusiform dilated segment of the right posterior communicating artery (PCOM), P2 segment of right posterior cerebral artery (PCA) and left vertebral artery []. On the left vertebral artery, two fusiform aneurysm was present, out of which one was located at the level of posterior inferior cerebellar artery origin and the second one just distal to its origin []. The largest size of fusiform aneurysm segment was present in the right M1 MCA segment (size – 7.3 mm), so clipping of an aneurysm was done to prevent future risk of rupture on the year 2014. Then, on the next stage procedure, trapping of left vertebral artery fusiform aneurysm segment was done, as its morphology was not suitable for the endovascular procedure []. The patient was advised to stop smoking and regular antihypertensive medicine.\nOn follow-up CT angiography of the patient in the year 2015, a new fusiform aneurysmal dilated segment was observed on the distal A1 segment of right anterior cerebral artery (ACA) []. The maximum diameter of the fusiform segment of the right ICA, M1 segment of the right MCA, PCOM, and P2 segment of Posterior cerebral artery (PCA) had been increased from previous follow-up image []. In the year 2016, the left cavernous ICA aneurysm refilling was observed, so again repacking of the coil was done. On CT head, no infarction and hemorrhage were observed and patient advice to continue follow-up.\nIn the year 2017, magnetic resonance imaging (MRI) angiography was done, which showed further increase in size of the fusiform dilated segment of right M1 MCA and right PCOM [] as compared to previous cerebral angiography in the year 2015 []. The contrast enhancement was noted in the wall of fusiform aneurysm segment of the right A1 ACA on MRI examination, which may be due to increased dilated vasa vasorum on the wall of an aneurysm [].\nOn the next follow-up CT angiography in the year 2018, size of the fusiform segment of right ICA, PCOM and A1 ACA was further increased slightly from previous cerebral angiography in the year 2017 [, Figures and ]. However, the right MCA fusiform aneurysmal segment size was decreased slightly on CT angiography, which may be due to thrombosis in the aneurysmal sac []. On sequential follow-up cerebral angiography, size of the fusiform segment of left M1 MCA was increased from 3.3 mm in the year 2014 to 5.4 mm in the year 2018 []. The computational fluid dynamic (CFD) study was done in the year 2018 on left M1 MCA fusiform aneurysm segment, which showed wall pressure high and wall shear stress low [Figure and ]. The streamline was showing slow flow on the dome [], and the vector was convergent in proximal fusiform aneurysm segment of left M1 MCA []. As CFD analysis was showing a risk of impending rupture on fusiform aneurysm segment of left M1 MCA, so clipping of an aneurysm was done in the year 2018 with preserving parent artery []. Intraoperatively numerous dilated vasa vasorum was observed in the fusiform segment of left M1 MCA aneurysm []. Till date, no hemorrhage and infarction were observed.
A 45 year old single woman with a non regressing congenital vascular malformation of the right arm and upper chest, presented with a 3 day history of high fever, chills, dysuria, hematuria and right loin discomfort. Her urine output was normal and she had no spontaneous bleeding manifestations or features of uremia. A marked drop in her platelet counts compared to recent baseline levels had been detected by her primary care physician following the onset of symptoms.\nHer past medical history was significant for persistent low baseline platelet counts in the region of 100,000/mm3 with episodic reduction to values as low as 30,000/mm3 following infections. She had been extensively and repeatedly investigated from the time the low baselines platelet counts were detected at the age of 28, however no definite cause was identified. On direct questioning she denied spontaneous mucocutaneous bleeding in the past. However her past medical history was significant for an abandoned plastic surgical procedure after developing a haematoma surrounding the preoperative intravenous cannulation site with a corresponding platelet count of 47,000/mm3. She had also been managed conservatively for uterine adenomyosis with depot provera from the age of 45 due to concerns of high risk of perioperative bleeding.\nDetailed evaluation was negative for symptoms attributable to anemia, connective tissue disease and liver disease. History of medications was negative for agents associated with thrombocytopenia.\nExamination revealed an ill looking lady with a temperature of 100.4 degrees Fahrenheit. She had a giant hemagioma involving the entire right arm, right lateral aspect of the neck and both the anterior and posterior aspect of the chest extending up to the 4th intercostal space (Figure \n). The hemangioma had small areas which were ulcerated and inflamed as well as healed scars. A soft bruit was appreciated on auscultation over the hemangioma. She had no petichiae or ecchymoses and her conjunctivae were pink. No lymphadenopathy was detected.\nCardiovascular examination was remarkable only for a pulse rate of 104/min. The blood pressure was 100/60 mm Hg. Abdominal examination revealed mild right loin tenderness without any organomegaly. Clinical pelvic examination was not performed respecting the patient’s wishes. Respiratory system and nervous system examination were both unremarkable.\nThe urine full report demonstrated 15–20 pus cells and 30–40 red cells per high powered field. Complete blood count on admission showed a hemoglobin of 11 g/dl with a neutrophil leukocytosis (WBC count of 17,000/mm3 – neutrophils 88%, lymphocytes 10%). The platelet count was 27,000/mm3. C reactive protein was elevated at 196 mg/dl (reference upper limit - 6 mg/dl). Serial complete blood counts done on days 4 & 5 of the illness revealed a progressive drop in platelet counts to a nadir of 15,000/mm3. Blood film showed severe thrombocytopenia with toxic neutrophils. Schistocytes suggestive of microangiopathic haemolytic anemia were notably absent. Her serum creatinine was mildly elevated at 1.52 mg/dl (reference upper limit - 1.3 mg/dl). Other biochemical parameters including uric acid, electrolytes and liver profile were all within normal limits. Both blood culture and urine culture yielded gram negative Escherichia coli with identical overlapping antibiotic sensitivity patterns. The wound swab from the ulcerated areas of the hemangioma became positive for Staphylococcus aureus sensitive to both penicillin and cloxacillin. Dengue NS1 antigen was negative. Ultrasonic examination of the abdomen excluded splenomegaly, features of portal hypertension and chronic liver disease. HIV, ANA, antiphospholipid antibodies, antiplatelet antibodies and Coombs test were all negative.\nBackground consumptive coagulopathy was excluded with normal baseline INR, APTT, D-dimers by modified ELISA and fibrinogen levels. There was also no laboratory evidence of acute derangement of the coagulation profile in relation to the worsening of thrombocytopenias triggered by infections.\nIn depth perusal of her previous admission records confirmed repeated episodes of severe thrombocytopenia (<30,000/mm3) precipitated with a variety of infections with return to baseline values of approximately 100,000/mm3 on resolution of the illness (Table \n). Despite extensive investigations no cause or basis had ever been identified in her to explain the phenomenon. The last episode of severe thrombocytopenia was triggered by an upper respiratory tract infection which had occurred 4 months preceding her current illness. Community acquired infections and wound sepsis complicating ulcerated areas over the hemangioma were the commonest precipitants identified.\nBone marrow studies done during a recent episode of severe thrombocytopenia had demonstrated a normal marrow including megakaryopoiesis, whilst the peripheral blood smear had shown evidence of thrombocytopenia and toxic left shift in the neutrophils which were considered to be consistent with a peripheral cause for thrombocytopenia on a background of ongoing infection. Repeated therapeutic trials with steroids had shown no improvement in the thrombocytopenia during episodes of infection and a rise in platelet counts was always temporally related to the resolution of the infection rather than steroid therapy. The baseline thrombocytopenias were not severe enough to warrant continued steroid therapy. However the episodic thrombocytopenias with infections had initially been treated with steroids. Platlet count trends during infections showed that the thrombocytopenia seemed to improve with the resolution of the causative infection rather than steroids. This was observed on many occasions in the patient and after the initial few trials no further steroid therapy was attempted. Thrombocytopenia was seen even on several occasions where the patient presented with untreated infections where she had neither been prescribed or used any medications and hence a drug induced cause was deemed unlikely. Acetaminophen was used by the patient unrelated to infections as an analgesic especially in relation to painful menstruation and no alteration in baseline platelet counts attributable to acetaminophen were seen.\nHer current illnesswas treated with a combination of intravenous levofloxacin and clindamycin which resulted in complete defervescence by the 3rd day of hospitalisation. When she was discharged on the 3rd day post admission, her platelet counts had risen to 79,000/mm3 with improvement in serum creatinine to 1.2 mg/dl and the urine full report was free of any evidence of persistent urinary sepsis. She was discharged on oral co amoxiclav and clindamycin for a further period of one week and advised to continue oral penicillin indefinitely to prevent wound sepsis.
A 42-year-old male was operated for carcinoma parathyroid. He had brittle bones. Despite extreme care during shifting to and from surgery, he developed pathological fracture of femur which produced severe pain in the post operative period. He had a pain score of 10/10 (VAS) which did not subside adequately with intravenous analgesics (Morphine, Diclofenac, Tramadol in maximal allowable doses).\nHence a femoral nerve block was done with an insulated needle passed through the sheath of an ordinary 18G intravenous cannula. Peripheral nerve stimulator was used to elicit movement of the patella. A bolus dose of 25 ml of local anesthetic (0.25% Bupivacaine) provided immediate complete pain relief and provided a hope of cure and the ability to walk normally in the course of time. The bolus injection was repeated every 6 h providing adequate and complete pain relief.\nTo alleviate the patient’s anxiety, we used midazolam 20μg/kg given as an intravenous bolus 1 min before the placement of the needle in both cases.\nThe femoral nerve is situated lateral to the femoral artery and is deep to the iliaca fascia, which in turn is deep to the fascia lata. The femoral artery and vein are in a separate fascial compartment. The femoral nerve is one nerve bundle near the inguinal crease, but a short distance more distally it divides into its two branches (superficial and deep branches).\nThe femoral artery is marked in the groin with a permanent marker. The femoral nerve is situated 1–2 cm lateral to the femoral artery. Mild sedation is all that is required for this block.\nThe equipments required include an 18 gauge PVC cannula, insulated needle which deliver current at the tip with a port to deliver the drug (Stimuplex needle), one ECG electrode, peripheral nerve locator or stimulator (with internal mode).\nThe patient lies supine with a clear view of the patella. The groin is prepared with Betadine and draped with a sterile drape.\nAfter careful skin and subcutaneous tissue infiltration of local anesthetic agent (care must be taken not to block the femoral nerve in the process), the cannula sheath is thread over the stimulating insulated needle and inserted aiming approximately 45 degrees cephalad just inferior to the inguinal crease. The nerve stimulator is connected to wire at the proximal end of the needle.[] After the nerve has been located with the cannula threaded over the needle till the dancing movement of the patella disappears signifying the loss of contact of the bare needle with the nerve due to the cannula. The cannula is withdrawn few millimetres till we can once again elicitate the muscle twitches and patellar movement unchanged in character and intensity. The catheter is now correctly placed near the femoral nerve but will most likely dislodge over time unless secured. At that point, the cannula is fixed to the skin with suture material and proper adhesive plaster.\nThe nerve stimulator is once again stimulated to confirm the position of cannula and the needle. Optimal positioning evoke contractions with 0.5mA or less and the evoked response fades after administration of 1-2ml of local anesthetic drug (Raj test).[] The needle is then removed from the cannula and an extension line of 10 cm attached to the proximal end.\nInitial bolus of 10 ml local anesthetic (1-2% xylocaine) is to be administered.\nBreakthrough pain is rare and patient satisfaction is high in patients when an infusion of 0.1ml/kg/h in children or 5ml/h in adults of 0.25% bupivacaine is used.\nThe entry site of the catheter should be inspected daily for any signs of infection. Since an indwelling cannula is left in situ formal sterile procedures were advised as other indwelling catheter. Sensation is allowed to return to the limb before the catheter is removed and if the patient complained of pain a bolus dose of the drug was administered or the infusion was maintained for few more days. In our cases, the cannula was removed on the fourth day of the catheter placement with no signs of infection.[]\nWe had seen that this method of cannula placement was quite effective with minimal adverse effects and above all cost effective.\nThe cannula should always be withdrawn entirely into the needle before the needle is repositioned. The presence of significant paresthesia during cannula advancement should be carefully evaluated before advancement of the cannula2. Be suspicious of sub-perineural needle or cannula placement if brisk muscle twitches are present with nerve stimulator settings less than 0.2mA (except in children).[] Since an indwelling cannula is left in situ for some time, formal sterile procedures are necessary. Sensation should be allowed to return to the limb before the cannula is removed.
A 64-year-old man with liver cirrhosis, advanced hepatocellular carcinoma (HCC) that invaded the main portal vein, and adrenal metastasis was admitted for the treatment of giant rectal varices due to portal hypertension. During the preceding 6 years he had undergone partial hepatectomy, six sessions of transcatheter arterial chemoembolization, and radiation therapy (39 Gy) for his HCC. He had no history of melena.\nOn admission, his functional reserve of the liver was Child-Pugh grade B. Colonoscopy showed huge tortuous rectal varices. Contrast-enhanced computed tomography (CT) revealed giant varices with tori inside the rectal lumen (Fig. a). Flow from the dilated inferior mesenteric vein was into the rectal varices that drained primarily into the left internal iliac vein. On inspiratory CT the diameter of the inferior mesenteric vein and left internal iliac vein was 10 mm.\nAfter obtaining informed consent from the patient and his family we attempted interventional therapy to prevent rupture of the varices. The plan was to fill the varices with a sclerotic agent via the IVM using balloon assistance. As tumor thrombosis into a main portal vein ruled out the percutaneous transhepatic approach we chose an ileocolic vein approach after laparotomy under general anesthesia. Portal venography via the superior mesenteric vein (SMV) confirmed that the rectal varices were supplied by the inferior mesenteric vein and mainly drained into the left internal iliac vein (Fig. b–d). Inflation of a 6-Fr balloon catheter (Selecon MP Catheter, Terumo Clinical Supply, Gifu, Japan) with a 2-cm diameter balloon in the inferior mesenteric vein failed to produce congestion in the varices. Consequently, we inflated a 6-Fr balloon catheter placed in the main trunk of the left internal iliac vein via the right common iliac vein (CIV) to block the in- and outflow of the rectal varices. However, we could not obtain congestion in the varices and contrast material in the varices drained into the collateral circulation of the left and right internal iliac vein. Thinking that flow in the rectal varices could be reduced by balloon occlusion of the left common iliac vein after coil embolization of the left internal iliac vein, we embolized the main trunk of the left internal iliac vein with nine microcoils. A 3-Fr microcatheter (Renegade, Boston Scientific, Natick, MA, USA) was advanced through the inflated 6-Fr balloon catheter placed into the orifice of the left internal iliac vein to prevent coil migration. First, one interlocking detachable microcoil (diameter 12 mm, length 30 mm) (InterlockTM, Boston Scientific, Cork, Ireland) was introduced as an anchor coil. Then we intertwined six pushable microcoils (diameter 8 mm, length 14 cm) (Micronester, Cook, Bloomington, IN, USA) with the anchor coil. Lastly two InterlockTM coils (diameter 10 mm and 12 mm, length 30 cm) was placed to hold the other seven coils and to obtain embolization.\nPostprocedure portal venography obtained under double balloon inflation in the inferior mesenteric vein and left common iliac vein revealed flow reduction in the rectal varices. However, as drainage via collateral vessels in the pelvis persisted 5 mL of absolute ethanol and 40 mL of a glucose solution were injected via the inflated balloon catheter in the IMV to embolize these drainers. This also failed to obtain complete congestion in the varices. As filling the rectal varices with a sclerotic agent was difficult under the existing conditions we attempted to embolize the varices with liquid glue. A 3-Fr microcatheter was advanced through the inflated balloon catheter in the internal mesenteric vein into the left superior rectal vein as close as possible to the rectal varices. Then 5.5 mL of a 1:10 mixture of N-butyl cyanoacrylate (NBCA) (Histoacryl, Aesculap, Tuttlingen, Germany) – lipiodol (André Guerbet, Aulnay-sous-Bois, France) was injected via the microcatheter under inflation of the balloons in the IMV and left CIV. As portal venography revealed that the right superior rectal vein flowed into residual rectal varices we injected 40 mL of 50% glucose and 12.5 mL of 5% ethanolamine oleate (Oldamin, Takeda Pharmaceutical, Osaka, Japan) with iopamidol (Iopamiron 300, Bayer HealthCare, Osaka, Japan) as a sclerotic agent through the microcatheter in the right superior rectal vein. Then eight pushable microcoils (diameter 8 mm) (Micronester) were placed in the internal mesenteric vein under double balloon inflation. As portal venography confirmed the complete obliteration of the rectal varices we removed the catheters (Fig. e, f). A plain radiograph obtained 30 min later confirmed that the coils remained in the left internal iliac vein. Throughout these procedures the patient's condition was stable and he was under continuous observation by anesthesiologists.\nDuring extubation the patient suffered a paroxysm of coughing and immediately after extubation he developed dyspnea and shivering. His partial pressure of arterial oxygen and percutaneous oxygen saturation slightly fell to 87 mmHg and 95% under oxygen inhalation of 5 L/min. Under continuous oxygen inhalation his symptoms abated somewhat and he was placed under observation with oxygen inhalation.\nOn the first postoperative day his dyspnea disappeared and the percutaneous oxygen saturation was 98% in room air. There were no respiratory symptoms. Although the liver function deteriorated transiently he developed no acute complications. A CT study performed on the fifth postoperative day confirmed complete obliteration of the rectal varices (Fig. a). However, all nine metallic coils placed in the left internal iliac vein had migrated into a lower branch of the right pulmonary artery (Fig. b and c). As this elicited no respiratory symptoms and as we considered the removal of the coils by interventional procedures inadvisable at that time, he was discharged 10 days after undergoing the procedures.\nHe subsequently received hepatic arterial infusion chemotherapy for HCC and endoscopic treatment for aggravated esophageal varices. Although colonoscopy showed shrinkage of the rectal varices, CT obtained 3 months later revealed progression of his intrahepatic HCC, enlargement of the adrenal metastasis, and newly developed lung metastasis. He died of HCC 5 months after the obliteration of the rectal varices.
A 39-year-old male patient, a known case of non-ischemic dilated cardiomyopathy (Left Ventricular Ejection Fraction 30%) who was in NYHA functional class II on drug therapy (Frusemide 20 mg, Aldactone 50 mg, Ramipril 5 mg and Carvedilol 6.25 mg twice daily) presented with history of recurrent palpitation and pre-syncope of 24-hour duration. His ECG revealed polymorphic ventricular tachycardia (PVT) []. On further enquiry, it was found that the patient is taking amiodarone 600 mg daily for last 3 months. Amiodarone was prescribed to him for one episode of sustained monomorphic ventricular tachycardia (VT) requiring cardioversion and he was continuing with the loading dosage of amiodarone by mistake for 3 months. There was no history of any other drug intake. Serum electrolytes were within normal limits. As there was association with no other offending agent other than high dosage of amiodarone, we kept the possibility of amiodarone induced PVT. The patient was treated with intravenous magnesium (2 g) along with intravenous lidocaine (1 mg/kg IV bolus) with out any effect. Intravenous Atropine 1.2 mg was also given to accelerate the sinus rate but failed to suppress the arrhythmia. Two more boluses of lidocaine at five minute intervals along with infusion at a rate of 1 mg/min were administered but the patient continued to have PVT. As the arrhythmia failed to respond to intravenous magnesium, atropine and lidocaine, we thought to try phenytoin to suppress the arrhythmia (a class IB antiarrhythmic drug that suppresses early after depolarization (EAD)) responsible for initiation of PVT secondary to amiodarone overdose).[] Phenytoin was administered in a dosage of 100 mg slowly every 5 minutes. After administration of 300 mg of phenytoin, significant reduction in episodes of PVT was noted and sustained sinus rhythm with corrected QTc of 490 milliseconds was restored within 30 minutes []. The patient was administered intravenous phenytoin 300 mg/day for 2 more days followed by oral phenytoin 300 mg/day. He was discharged after 1 week on oral phenytoin. Predischarge holter monitoring showed normal sinus rhythm with infrequent monomorphic VPCs and no evidence of nonsustained or sustained VT. As the patient had developed amiodarone-induced PVT due to overdose, we also assessed the patient for extra cardiac toxic effects of amiodarone. The patient did not have any history of dry nonproductive cough and lung function test was normal. His liver function test was normal but thyroid function test showed evidence of hypothyroidism (TSH>10 m IU/ml with decrease in T3 and T4 levels). The patient, however, did not have any symptoms of hypothyroidism which can be subtle or even absent.[] Ophthalmological examination revealed evidence of corneal deposit but no visual impairment. In view of previous history of ventricular tachycardia requiring cardioversion with LV dysfunction, patient was advised Implantable Cardioverter-Defibrillator, but he refused due to non-affordability. He is at present asymptomatic and on medical follow-up with diuretic (Frusemide 20 mg+Aldactone 50 mg), ACE inhibitor (Ramipril 5 mg), β-blocker (carvedilol 50 mg/day), L-thyroxine 25 μg and oral phenytoin (300 mg/day) for last 2 months.
The patient was a 2-year-old boy from Russian republic of Ingushetia. Both parents and younger sibling (female) were asymptomatic. Family history is not burdened. Parents may be related, as the mother of the child and the grandmother (father's line) are from neighboring villages of Malgobek region of Ingushetia.\nThe patient is the first child of healthy parents. Pregnancy proceeded against the background of gestosis in the first and second trimester, threats of interruption for a period of 12 weeks, anemia, and lack of hydration. The child was born as a result of fast unassisted childbirth. His birth weight was 3570 g and height was 55 cm. Apgar scores were 6/7. Early motor development slightly delays: He attained head holding at 3 months, sitting without support at 6 months, by the year the boy got up and walked with support. From the age of 8 months, the baby had a weight loss of 2 kg for 2 months with episodes of repeated vomiting. At the age of 1 year 2 months against a background of acute respiratory viral infection, there was a regress in development—the boy stopped walking. Subsequently, rachitic skeletal changes, an increase in the volume of the abdomen appeared, and a loss of motor skills progressed. Magnetic resonance imaging (MRI) of brain revealed a mildly expressed leukopathy in white matter of parietal lobes. Proteinuria and glucosuria appeared at the age of 1 year 8 months.\nAt the age of 2 years 2 months, he was admitted to Russian children's clinical hospital with psychomotor retardation and treatment resistance. On physical examination, he was observed to have a pronounced delay in psychomotor development (he did not get up, walk, crawl; he could only keep his head and turn over). Neurological examination revealed muscular hypotrophy, tendency to hypotension in axial musculature, and uniform decrease in tendon reflexes from hands and feet. MRI of brain revealed subatrophic changes, moderate expansion of the lateral ventricles within the subatrophy with compensated liquorodynamics, and the presence of hypomyelinization zones in the periventricular white matter of the posterolateral regions.\nClinical exome sequencing was carried out by Genotek Ltd. Genomic DNA from peripheral blood sample was extracted using QIAamp DNA Mini Kit (Qiagen, Hilden, Germany). DNA libraries were prepared using NEBNext Ultra DNA Library Prep Kit for Illumina (New England Biolabs, Ipswich, Massachusetts, USA) with adapters for sequencing on Illumina platform. Double barcoding was performed by PCR with NEBNext Multiplex Oligos for Illumina kit. Quality control of DNA libraries was carried out using Bioanalyzer 2100 (Agilent Technologies, Santa Clara, California, USA). We used SureSelect XT2 (Agilent Technologies, Santa Clara, California, USA) for target enrichment. Enriched samples were sequenced on Illumina HiSeq 2500 using pair-end 100 base pairs reads. After sequencing, we trimmed 3′-nucleotides with read quality below 10 using Cutadapt. Raw reads were aligned to reference genome hg19 (GRCh37.p13) using BWA MEM. Deduplication of reads was carried out using SAMtools rmdup. FastQC was used for data quality control. We called short variants using GATK HaplotypeCaller according to GATK Best Practices DNA-seq. The effect of each mutation was assessed using snpEff. To assess pathogenicity and conservatism, the data were extracted from the dbNSFP, ClinVar, OMIM database, and HGMD, as well as using the SIFT and PolyPhen-2 utilities to predict pathogenicity of the mutation. Information on the frequency of mutations was taken from 1000 Genomes project, ExAC, and Genotek frequency data. Description of mutations and their pathogenicity was predicted according to the Standards and Guidelines developed by ACMG (American College of Medical Genetics and Genomics), AMP (Association for Molecular Pathology), and CAP (College of American Pathologists). Copy number alterations were determined using CNVkit. CTNS variant identified by exome sequencing was confirmed by Sanger sequencing of patient, parental, and sibling DNA samples.\nAfter exome sequencing, on admission in Veltischchev Research and Clinical Institute for Pediatrics, patient was 9 kg in weight and 80 cm in height, with low weight-for-height proportion and disharmonious physical development. Deficiency of subcutaneous fat was noted. Attention was paid to rachitic changes in skeleton: expansion of metaphyses, keel-like deformation of thorax, rachitic “rosary,” varus deformity of lower limbs. Hepatosplenomegaly was noted: liver and spleen protruded from under edge of the costal arch by 2.5 cm. Clinical signs include polyuria and polydipsia (consumed liquid per day 1300 mL, daily diuresis—1300 mL). Blood tests showed mild anemia, hypokalemia, decrease in uric acid, increase in alkaline phosphatase activity, hypophosphatemia, increase in triglycerides, and creatinine level at the lower limit of the norm. The study of equilibrium of acids and bases of blood revealed metabolic acidosis. Test of thyroid gland hormonal profile showed subclinical hypothyroidism. Level of cystine in leukocytes was normal. Glomerular filtration rate (GFR) (97 mL/min) was within the normal range (80-120 mL/min). Urine test showed phosphaturia, glucosuria, and low molecular weight proteinuria. Cystine was detected in urine test. Ultrasound examination showed hepatosplenomegaly, multiple foci of increased echogenicity in liver, nephromegaly with thickening, and diffuse changes in renal parenchyma. Radiologic examination revealed pronounced osteoporosis, lagging behind bone age. Ophthalmoscopy revealed cystine crystals in the cornea. Thus, the child has signs of infantile form of nephrotic cystinosis with preserved kidney function.\nThe patient was commenced on cysteamine (Cystagon), initially 50 mg orally four times daily, to increase gradually to 100 mg four times daily (0.8 g/m2/d). Also he was commenced on ophthalmic solution Cystadrops (one drop six times daily) and potassium, phosphorus, vitamin D, energotropic agents. After 6-month therapy, the patient's condition remains stable and serious with slight positive dynamics. The child gained 1 kg in weight and grew by 0.5 cm. Polydipsia/polyuria decreased to 1000-1200 mL. Hemoglobin and red blood cell levels, and thyroid-stimulating hormone and T4 levels were normalized. Minimal phenomena of metabolic acidosis persist. Also, filtration function of the kidneys was preserved, and no signs of nephrocalcinosis were detected.\nAt the last examination, patient had elevated cystine level in the granulocytes (2.32 nmol) and nephrocalcinosis of both kidneys. Cysteamine dose was increased to 1.25 g/m2/d.
A 56-year-old male presented to the emergency department (ED) with significant substernal chest pain starting 30 minutes prior to arrival. The patient was immediately brought back to an exam room after an electrocardiogram (ECG) was performed and was seen by a provider within 10 minutes of registration (Figure ).\nThe initial ECG revealed atrial fibrillation with a rapid ventricular response, rate of 147, with minimal ST depression within the lateral leads but was without apparent ST-segment elevation. On initial assessment, the patient had point-of-care labs immediately drawn (a basic metabolic panel and troponin), and a chest X-ray performed to evaluate for a possible aortic dissection which revealed no gross abnormalities (Figure ).\nWith a non-diagnostic chest X-ray alternative diagnoses were pursued. The ultrasound fellow in the department was consulted for an immediate cardiac ultrasound for evaluation of right heart strain secondary to pulmonary embolism. During the bedside cardiac ultrasound, the patient experienced ventricular fibrillation (Video ), and CPR was immediately started.\nThe ACLS algorithm was followed for pulseless ventricular fibrillation, and the patient received multiple rounds of epinephrine, 450 mg of amiodarone (300 mg and then 150 mg), and three conventional defibrillations with increasing joules at 150 J, 200 J, and 200 J (the departmental defibrillators are biphasic and have a maximum output of 200 J). The patient continued with ventricular fibrillation throughout the ACLS algorithm, and the decision was made to attempt DSD. The patient had a second set of pads applied in the anterior-posterior orientation in addition to the conventional right upper chest and left lateral chest with successful conversion of the ventricular fibrillation. The patient was additionally given Lidocaine, 100 mg, due to a wide-complex tachycardia and apparent non-responsiveness to the previously given amiodarone (Figure ). An improvement was noted after the use of Lidocaine, and a Lidocaine drip was started.\nOnce the patient was stabilized, he was taken for computed tomography (CT) imaging to further evaluate for the possibility of a pulmonary embolism. In the CT room, he developed bradycardia and subsequently lost his pulse. CPR was again started, the patient was given atropine, and return of spontaneous circulation (ROSC) was achieved shortly after. The CT scan did not reveal any evidence of aortic dissection or pulmonary embolism and a repeat ECG was performed which showed a persistent wide complex tachycardia with no obvious ST-segment changes. Due to the morphology of the QRS complexes and length of resuscitation time from initial arrest (nearing 90 minutes), tissue plasminogen activator (tPA) was used as a thrombolytic for what was presumed to be a large vessel occlusion myocardial infarction. Hundred milligram of tPA was administered (50 mg as a bolus and 50 mg as a drip given over 60 minutes) with an apparent reperfusion rhythm followed by a "normal" appearing sinus tachycardia. Another ECG was repeated which revealed sinus tachycardia, at a rate of 114, now with ST-segment elevations present in aVR, V1, V2, V3, and V4 with depressions in leads II, III, and aVF (Figure ).\nGiven the patient's persistent elevations despite thrombolytic therapy, interventional cardiology was consulted, and the patient was transferred to a tertiary care facility for cardiac catheterization revealing a thrombotic occlusion in the proximal left anterior descending coronary artery.\nAfter transfer to the tertiary care facility for cardiac catheterization, the patient developed cardiogenic shock. The patient was started on ionotropic medications with no improvement, and he was placed on venous-arterial extracorporeal membrane oxygenation (ECMO) therapy (~20 hours after his cardiac arrest). Before initiation of ECMO, the patient was awake, alert, and following simple commands (although still intubated). Unfortunately, despite ECMO support, his cardiac function did not improve, and the patient was not a candidate for cardiac transplantation. Seventeen days after the patient's initial presentation to the ED, the family decided to withdraw care. The patient was extubated, had ECMO discontinued, and time of death was documented shortly after.
An 86-year-old female was referred to our hospital for treatment of a hilar biliary stricture lesion on abdominal CT initially performed to evaluate jaundice and vague abdominal pain. She complained of yellow skin and eyes that began 2 weeks prior. Around the same time, she also began experiencing abdominal pain that was dull and intermittent. The pain did not radiate nor was it related to meals; it was sometimes accompanied by anorexia and nausea. She was constipated but reported no changes in the color of stool. The patient also did not notice weight changes. She had a past medical history of diabetes mellitus controlled by metformin for the past 2 years and had no history of surgery. Her family history was non-contributory. Initial physical examination revealed no specific findings. Contrast-enhanced CT showed a distended intrahepatic bile duct with a stricture at the common hepatic duct that included the bifurcation (Fig. ). This finding was suspicious for cholangiocarcinoma of the bile duct. Direct invasion of nearby vascular structures and distant metastases were not detected. The initial laboratory data suggested cholestasis, with a total bilirubin of 8.6 mg/dL, aspartate amino transferase 178 U/L, and alanine transferase 105 U/L. Furthermore, the level of carbohydrate antigen 19-9 as a tumor marker was elevated at 167 U/mL. The viral markers were HBsAg (−), HBsAb (−), and anti-HCV (−). She was negative for HIV. As the patient had fever and leukocytosis, intravenous antibiotics were started out of suspicion for concurrent cholangitis. Immediately after administration of antibiotics, the bilirubin level dramatically decreased and was within normal range a week later. The lesion was not thought to have completely blocked biliary outflow. Preoperative evaluation of the patient’s cardiac and pulmonary function was unremarkable, with echocardiogram showing no definite cardiac dysfunction. The patient’s hemoglobin and hematocrit levels were mildly decreased to 11.7 g/dL and 34.2 %, respectively. The serum blood urea nitrogen and creatinine were 8.5 and 0.6 mg/dL, respectively. There was no serum electrolyte imbalance or coagulopathy found. Her performance status was appropriate. Due to suspected hilar cholangiocarcinoma, extrahepatic bile duct resection and biliary reconstruction (Roux-en-Y hepaticojejunostomy) were planned. After careful exploration of the peritoneal cavity, hepatoduodenal dissection was performed, following cholecystectomy. On manual examination of the common bile duct (CBD), a mass was palpated. However, this mass was soft and had not invaded any neighboring tissue, including the hepatic artery and portal vein. The portion of the CBD containing the mass was easily separated from the surrounding hepatoduodenal connective tissues. The proximal resection margin was the nearby bifurcation of the hepatic duct, and the distal margin was just above the intra-pancreatic portion (Fig. ). The light microscopic examination revealed diffuse infiltration of atypical lymphocytes in the common bile duct. They formed lymphoid follicles and occasionally infiltrated into glandular epithelium resulting in the so-called lymphoepithelial lesion (Fig. ). On immunohistochemical staining, CD20 was diffusely positive in neoplastic cells and CD3 was expressed in scattered T cells. The neoplastic cells were BCL2 positive but germinal center B cells were negative. Also, the neoplastic cells were negative for cyclin D1 (Fig. ). The final diagnosis was extranodal marginal zone lymphoma of MALT lymphoma arising in the common bile duct. Postoperatively, the patient had no serious complications. By postoperative day 2, she was tolerating a liquid diet. She was discharged on the 12th postoperative day. No additional chemotherapy was considered, as complete resection of the tumor had been achieved. At 1-year post-operation, her recovery had been completely uneventful, without recurrence of any symptoms of disease.\nAn extranodal marginal zone B cell lymphoma, also called low-grade B cell lymphoma of MALT, arises in a number of epithelial tissues, including the stomach, salivary gland, lung, small intestine, and thyroid. It has a tendency to remain localized to the tissue of origin over time, but does recur frequently, with potential for systemic spread and transformation to a high-grade B cell lymphoma. The pathogenesis of MALT lymphoma owes to chronic inflammation of the tissue, which leads to the local accumulation and proliferation of antigen-dependent B cells and T cells. With time, B cell clones emerge, still dependent on antigens for growth and survival. At the stage of monoclonal proliferation, these cells are not able to spread beyond the site of inflammation. With acquisition of additional mutations, however, the tumor becomes antigen-independent and capable of systemic spread []. This patient had no known history of prior cholangitis or any evidence of hepatitis, so it is hard to be suspicious of any chronic antigenic stimulation which carries etiologic specificity. The underlying etiologic mechanism of bile duct involvement by this type of lymphoma should be further elucidated.\nPrimary non-Hodgkin’s lymphoma of the extrahepatic bile duct presenting as obstructive jaundice is extremely rare, with lymphoma occupying the perihilar bile duct being even more uncommon. In 2005, the first case was reported of primary MALT lymphoma arising from the perihilar bile ducts. Primary MALT lymphoma is such a rare finding at this site that only four cases have been reported thus far, making it difficult to suspect MALT lymphoma in such cases []. It is challenging to differentiate MALT lymphoma of the perihilar bile ducts from hilar cholangiocarcinoma, the most common form of bile duct cancer. The two types of malignancies are indistinguishable both clinically and radiologically. We reviewed three cases of patients whose diagnoses could not be confirmed as primary MALT lymphoma until radical resection of the bile duct was performed; we further included one patient who had been confirmed by biopsy and had undergone chemotherapy [–] (Table ).\nThe three patients who had undergone surgery were males aged 59 to 71 years (mean 64 years). They had been admitted to the hospital for jaundice with mean total and direct bilirubin levels of 11.15 and 7.85 mg/dL. Radiological studies revealed that the perihilar bile ducts were diffusely thickened, with irregular, incomplete stenosis or long circumferential wall thickening shaped. All patients who underwent surgery had received a preoperative diagnosis of Klatskin tumor. On postoperative immunohistochemical studies, however, bile duct narrowing turned out instead to be related to MALT lymphoma. In three cases, partial hepatectomy combined with caudate lobectomy were also performed. In our case, a negative resection margin was secured without the need for further hepatic resection, as the proximal margin was preserved just below perihilar duct bifurcation.\nTo avoid misdiagnosing and inappropriately treating cases of MALT lymphoma resembling Klatskin tumor, several suggestions have been made. According to Shimura et al., evaluation of incomplete stenosis of the bile duct by 18-F fluoro-2-deoxyglucose positron emission tomography can distinguish MALT lymphoma from Klatskin tumor []. Yoon et al. propose that it is necessary to consider MALT lymphoma when cholangiography shows smooth luminal narrowing of the extrahepatic bile duct without mucosal irregularity, despite diffuse thickening of the ductal wall on CT and magnetic resonance imaging (MRI) []. Still, it remains difficult to diagnose primary MALT lymphoma in this region on either CT or MRI. MiKail et al. reported a unique case that was diagnosed without the need for surgical specimens []: a 58-year-old female patient was thought to have a Klatskin tumor and was prepared for major surgery; however, a type of lymphoma was suspected based on concerning biological behavior of the tumor (rapid growth resulting in doubling of size within a month), and the patient underwent a tru-cut biopsy via percutaneous transhepatic cholangiography (PTC), which led to the diagnosis of low-grade B cell lymphoma at the hepatic hilum without the need for surgery.\nAt the time of diagnosis, only 20–30 % of patients with Klatskin tumors are operable, leaving the remainder to follow a course of palliative treatment []. Biliary stents in both the right and left hepatic ducts help relieve jaundice and minimize the risk of cholangitis. With complications of stents, such as occlusion and migration, stent patency lasts only a few months before replacement becomes necessary. However, palliative stenting is actually inappropriate for patients diagnosed with primary MALT lymphoma mimicking Klatskin tumor. Whereas patients with inoperable Klatskin tumors can expect less than 1 year of survival, non-gastric MALT lymphomas have an indolent course, resulting in a 5-year survival rate of 93 % []. For the latter, chemotherapy or resection of space-occupying lesions can improve patient quality of life and long-term survival.\nThe greatest number of patients with extrahepatic bile duct cancer lies within the 75–84 year age range at diagnosis []. Patients 70 years or older are referred to as the elderly, and advanced age has been considered to be a contraindication for surgery, out of fear that extended resection of hepatobiliary tumors is too risky. In addition, life expectancy for the elderly is usually underestimated []. In one study, 14 % of inoperable Klatskin tumors were due to advanced age and coexisting medical conditions []. The 86-year-old female patient in our case had previously been considered inoperable due to her advanced age but was in fact well on the way to the recovery after surgery. There has been a recent trend to change this preconception and allow elderly patients more opportunities to undergo surgery. Resection of hepatobiliary cancer can be offered to the elderly as well as to patients with few co-morbidities and good functional status []. A study of Veterans Administration (VA) patients aged 80 years and over showed that the 30-day mortality rate was better predicted by functional status than by chronological age []. If preoperative performance status is favorable, a forward-looking approach to major biliary surgery in elderly patients can provide them a chance for cure.
A 28-year-old male first presented with stuttering at the age of 7 years. No management was carried out at that time because his symptoms were mild. By the age of 20 years, he was being ridiculed and humiliated by his college classmates. He became more introverted and did not want to communicate with others. Since then, his stuttering had worsened. He experienced multiple blocks in expressing words, including sound and syllable repetitions, circumlocutions, and monosyllabic repetitions, that made it difficult to conduct the interview.\nIn addition to deterioration of his PDS, he developed depression and anxiety and was afraid to communicate with others. He also had physical concomitants of involuntary jerking of his head and upper extremities and muscle spasm of his neck and lower jaw while stuttering. He was diagnosed with depression and anxiety disorder by the psychiatrists at the age of 24 years. He was managed medically with multiple antipsychotics in conjunction with cognitive behavioral therapy. Significant improvement in his psychiatric disorder but not his PDS was achieved at that time.\nOne year earlier, because of his local culture, he had refused to take any medicine, which led to a relapse of his psychiatric symptoms. Subsequent antipsychotics and cognitive behavioral therapy were of limited help in alleviating his psychiatric symptoms. We carefully addressed his treatment options, which included further medical treatment, deep brain stimulation, or capsulotomy. Both the patient and his family opted for capsulotomy for financial reasons and in the hope of being able to avoid using antipsychotics. The surgery was approved by the ethics committee at our hospital.\nPreoperative magnetic resonance imaging was done to rule out an intracranial tumor, hemorrhage, infarction, and infection. No family history of a speech disorder was reported. The preoperative neuropsychological evaluations were carried out using the 24-item Hamilton Depression Rating Scale, the Hamilton Anxiety Rating Scale, the Mini-Mental State Examination, the similarities and block design subtests of the Wechsler Adult Intelligence scale, the logical memory and visual reproduction subtests of the Wechsler Memory Scale, and the Wisconsin Card Sorting Test-Simplified (WCST-S). Both the logical memory and visual reproduction tests were carried out immediately and after a 30-minute delay. The WCST-S included 48 cards, and his performance was assessed by the number of correct answers, number of errors, number of perseverative errors, number of nonperseverative errors, and number of categories completed. Neuropsychological and PDS evaluations were done before surgery and at 6-month follow-up by the same psychiatrist and speech therapist, who were both aware that the patient had undergone neurosurgery.\nTo better identify the magnetic resonance images without fixation of the head frame were obtained the day before surgery. A 3 T Trio Unite magnetic resonance imaging system (Siemens AG, Muenchen, Germany) was used with T1-weighted and T2-weighted spin-echo sequences in the axial and coronal planes. The slice thickness was 2 mm with an interval gap of 0.02 mm. The preplanning procedure was completed using the Surgi-plan workstation (version 2.1; Elekta Instruments AB, Stockholm, Sweden). On the day of surgery, a Leksell model G head frame (Elekta Instruments AB) was positioned on the skull parallel to the anterior commissure–posterior commissure line under local anesthesia. A repeat magnetic resonance scan with the same parameters as the preplanning scanning was performed using a 1.5 T Sonata Unite magnetic resonance imaging system (Siemens AG). With the help of the Surgi-plan workstation, the prescanned images were then coregistered with the images using location markers. The coordinates of the target and the angles of electrode penetration were calculated. The lesion targets were located 14 mm anterior and 18 mm lateral to the anterior commissure and 5 mm below the anterior commissure–posterior commissure plane. Under the guidance of the Leksell multifunctional stereotactic operation system (Elekta Instruments AB), the lesion electrode was then inserted into the lesion target according to the calculated coordinates. A test stimulation generated by the Elekta neurostimulator at both high frequency (130 Hz) and low frequency (5 Hz) was then carried out to verify the target of the electrode. Next, multiple lesions were produced using the Elekta neurostimulator at 75°C for 70 seconds each. The length of the lesions on both sides was 14 mm ( and ).
A 54-year-old male was admitted to the Accident and Emergency Department at King Saud Medical City, Riyadh, Kingdom of Saudi Arabia, with a huge ventral hernia on June 14, 2013 at 11 PM. The hernia had markedly increased in size within the previous month, and for the past week it had become stony, hard, painful, and irreducible, as in . These changes were also associated with vomiting and absolute constipation. The patient’s detailed history also depicts that he had experienced a road-traffic accident 20 years earlier, which resulted in dysarthria and rendered him socially inactive. No other significant past surgical and medical history was available. He was not on any specific medications.\nThe physical examination revealed a dehydrated patient with a pulse rate of 104 beats/minute, but his blood pressure was within normal range. There was a huge ventral hernia, around 25 × 30 cm, almost equal to the size of a football, which was tense and tender, bulging more on the right side of the abdomen. Skin discoloration overlying the hernia was also noted. Bowel sounds were not audible and a rectal examination was unremarkable. A laboratory investigation revealed leukocytosis. Chest x-ray showed no air under the diaphragm. Abdominal x-ray showed dilated bowel loops with few air fluid levels. A diagnosis of strangulated ventral hernia was made. He was resuscitated with isotonic fluids. Intravenous cefuroxime and metronidazole were also given before surgery. After resuscitation, and once he provided consent and an intensive care unit (ICU) bed arrangement was made, he was shifted directly for operation from the emergency department without any further investigations or delays within 2 hours from presentation to the emergency room. Laparotomy revealed that almost the entire small bowel and some part of the large bowel were protruding out of the abdomen in the hernial sac, featuring some hemorrhagic fluid. There was a constriction at the abdominal wall that was shelf-like, and there was pressure over the mesentery due to the tight neck of the sac, with a size of around 5 × 5 cm. The huge hernial sac was opened, containing offensive fluid and gangrenous bowel, mesentery, and omentum. The pressure within the hernial sac was so high that upon opening of the sac, hemorrhagic fluid came out like a shower. The gangrenous area included most of the jejunum, as well as the entire ileum, cecum, and the ascending and proximal half of the transverse colon, as in . The constriction was divided and full trial was given (100% oxygen and warm sponging of gut) to regain the viability of the gut. A clear demarcation line was established. The gut was totally black, foul smelling, and had lost its sheen and luster; there was no peristalsis. Even after full trial, viability of the gut was not regained. Secondly, the mesentery was also black, and the vessels in the mesentery were gangrenous due to continuous pressure of hard shelf-like sac. In addition, the general condition of the patient was not good during either anesthesia or surgery due to the septic, gangrenous gut. While keeping of all of these things in mind, the decision was made to resect the gangrenous bowel and omentum. The gangrenous sac was also excised. After resection, only 60 cm of proximal jejunum remained, and this portion was clearly viable with all of its features, which was anastomosed to the remaining parts of the healthy transverse colon. There were no doubts concerning the remaining portion of the gut and mesentery, so a decision was made to perform primary anastomosis. No healthy parts of the gut were excised; however, clear cuts of healthy, safe margins for anastomosis were preserved. The patient subsequently ended up with a short bowel. Direct hernia repair was performed without prosthetic mesh. Histopathology showed a gangrenous bowel with clear margins. The postoperative course was uneventful. Initially, total parenteral nutrition (TPN) was started, and it was gradually overlapped with enteral feeding later on. There were a few episodes of diarrhea that were managed conservatively. He tolerated the gradual increase of enteral feed and was discharged from the hospital after 30 days, when he went completely off TPN, tolerating full enteral feeds. He was regularly followed in the outpatient surgical clinic with short bowel syndrome. He is currently on a combination of an enteral and normal diet. He is passing mixed-consistency stool 4 to 5 times a day. He lost approximately 30 kg of weight during the last 3 months. His dietary issues were managed with the help of a dietitian.
A 56-year-old woman presented to our oncology department in May 2018 with a painful erythematous rash with multiple blisters covering the upper part of her back toward the neck. In January 2018 she had been diagnosed with breast cancer and was in the middle of her adjuvant chemotherapy after partial mastectomy. She was on day 8 after the first course of docetaxel treatment (90 mg/m2 in a three-week regimen), which was given after three courses of prior treatments with epirubicin and cyclophosphamide. Earlier on day 3, she started to feel irritation and pain over her upper back and neck. An extensive rash over the area was noticed on day 5 with increasing pain. She visited the emergency room on day 6 and was found neutropenic. Blisters appeared over the erythematous area later that day. The patient suffered from an intensive pain despite maximal dosages of paracetamol and ibruprofen day 8, she showed up in our clinic and presented a confluent, elevated, burning erythema distributed on previously sunburned areas, the neck and the upper back, strictly excluding areas previously covered with clothes and hair. The appearance reminded of an acute sunburn. However, she had not been exposed to the sun for the last weeks. The patient was admitted to the infectious diseases ward for assessment because herpes infection was suspected. One of the blisters was punctured and PCR tests for herpes simplex virus type I (HSV I), herpes simplex virus type II (HSV II) and varicella zoster virus (VZV) were performed. The patient had fever up to 38.3 degree Celsius and the treatment with valaciclovir was initiated. However, the clinical picture was atypical since the erythema was covering several dermatomes while distinctly sparing the parts under her bra straps, rising suspicion of photo toxicity. The patient had not been sun exposed during the period of chemotherapy, but she had undertaken a trip to Vietnam for two weeks 5 months before the docetaxel treatment. Her upper back and neck was sunburned at that time, but to a much lighter degree without skin bullae. The rash progressed with a vesicular erythema and some large blisters followed by shallow erosions. At day 9, the patient was sent for dermatological consultation. A skin biopsy from the back was taken and it revealed a blistering dermatitis with interface changes including basal vacuolar degeneration, apoptotic keratinocytes at all levels, engagement of a sweat gland duct, intraepithelial vesiculation and partial epidermal necrosis. A subepithelial lymphocytic infiltrate with few eosinophils and neutrophils was seen. These findings were similar to erythema multiforme. Immunohistochemistry with antibodies against HSV1/2 and VZV were negative, which were also confirmed by the negative PCR results by serology. Thus, we diagnosed a severe docetaxel-induced photo toxicity which was recalled 5 months after the initial sunburn. The medication of valaciclovir was stopped after two days of administration.\nThe recall eruption was much more intense than the initial sunburn. Both the clinical (Fig. ) and pathological (Fig. ) presentations showed similarities to HSV/VZV infections. Although the erythema was clinically assessed as grade 4, it resolved relatively quickly after 3 days of application with topical corticosteroids (betametasone cream once a day). The erythema faded with a superficial desquamation leaving a hypo- and hyperpigmentation on day 11. Due to this severe skin toxicity, the patient was not continued with docetaxel treatment. Instead, weekly paclitaxel was administrated. Six doses of paclitaxel were given successfully, and no more skin toxicity was observed. The adjuvant chemotherapy was thus completed according to the planed time schedule, without any delay.\nPhoto-recall phenomenon is a phototoxic eruption occurring on areas of previous ultraviolet-induced solar erythema following a systemic administration of a chemotherapeutic drug. It has been mostly described with methotrexate but remains rare with other antineoplastic drugs. Few cases have been documented with gemcitabine, etoposide, and cyclophosphamide [, , ], but even more rarely cases have been reported with taxane based regimens [, , ]. Droitcourt et al described a case of docetaxel-induced photo-recall skin rash on a woman treated for a non-small-cell lung cancer []. He et al. reported a case induced by paclitaxel [].\nApparently, docetaxel seems to be more potent to recall the photo toxicity than other cytotoxic drugs the patient has received. Prior to docetaxel, our patient had already received three repeated courses of epirubicin in combination with cyclophosphamide relatively shortly after the initial sunburn, but no skin toxicity was developed. In addition, she has also received 6 courses of paclitaxel treatment after the docetaxel course, without any occurrence of skin toxicity. Furthermore, the recall occurred 5 months after the initial sun damage, which was far longer than that in other reports (1–8 days), but within the time period of 2–7 days after administration of the triggering drug.\nThe physiopathology of photo-recall phenomenon is still unclear. One hypothesis was that after a sub-erythemal exposure to UV light, the UV-dependent pro-inflammatory factors might be upregulated by the triggering drug to reach the erythemal threshold. However, this theory can hardly explain the recall reported in our patient which occurred 5 months after the initial sun damage. Another theory is that some skin-resident cells remember previous damage, and memory T cells that were primed during the original inflammation may persist at high levels during the asymptomatic phase and evolve into long-lived memory cells that can cross-react with unrelated drug []. This theory may explain our case. However, these hypotheses need to be further confirmed in future research.\nTo our knowledge, this is the first reported case of a breast cancer patient with a rare but severe photo toxicity recalled by an adjuvant docetaxel treatment 5 month after the initial sunburn. We believe this rare and peculiar type of skin eruption induced by docetaxel is worth being recognized by the oncologists as well as dermatologists and pathologists. Paclitaxel can be the drug of option after docetaxel recalled photo toxicity.
A 41-year-old man with known metastasis to the adrenal glands and the second part of the duodenum close to the ampulla of Vater from clear cell renal cell carcinoma (ccRCC) was admitted to our department due to massive gastrointestinal bleeding.\nThe patient had undergone left nephrectomy one year ago for ccRCC (Fuhrman Grade 4, with vascular invasion). Six months after the nephrectomy, the patient showed progression of disease, with adrenal metastasis bilaterally and to the midthoracic lymph nodes. The patient underwent 4 cycles of pazopanib and a computed tomography (CT) scan showed good response. Unfortunately a CT after six cycles of pazopanib showed progression of disease with an increase of the size of the known metastasis and also a duodenal metastasis confirmed with biopsy.\nFollowing emergency admission, endoscopy revealed the site of haemorrhage to be the known metastasis at the second part of the duodenum near the ampulla of Vater. Endoscopic haemostasis was not possible and the patient underwent angiography with a plan for embolization. The angiography revealed the presence of stenosis of the coeliac artery and also hypertrophic inferior pancreaticoduodenal arteries supplying through the GDA the proper hepatic artery (). The embolism of the bleeding vessels (superior and inferior posterior pancreatoduodenal arteries) controlled only temporarily the haemorrhage with the patient deteriorating 24 hours later.\nIn this interval the patient had a CT of chest/abdomen/pelvis for staging of the disease, which confirmed the findings of the angiography in regard to the stenosis of the celiac artery and a dilatation distal to the stenosis (). The CT also showed extended progression of the disease with an increase of size of the adrenal metastasis (the right one from 3.2 cm to 5.2 cm and the left one from 7.1 cm to 7.9 cm), an increase of size of the known duodenal metastasis (from 2.5 cm to 4.5 cm), and also five new liver metastases with the bigger one in segment VI measuring 12 mm, while there was progression of disease in the thorax with an increase of the midthoracic lymph nodes and development of lung metastasis with extensive pleural effusions.\nThe deterioration of the haemorrhage led to a haemodynamically unstable patient who was taken to theatre. The extent of the metastatic disease necessitated a palliative surgical intervention in order to control the bleeding and stabilize the patient, with a plan for second line chemotherapy or participation in a clinical trial.\nThe fact that the bleeding duodenal tumour was very close to the ampulla of Vater, reconfirmed with intraoperative endoscopy prior to laparotomy, made a PPPD the only choice.\nDuring laparotomy, the presence of sizeable adrenal metastasis bilaterally was confirmed, as well as the metastasis in the duodenum, in the para-aortic lymph nodes, while the intraoperative ultrasound demonstrated five metastatic sites in the liver. Following mobilization of the duodenum and the formation of a tunnel between the pancreas and the SMV/portal vein, the GDA was dissected. The occlusion of the GDA led to the vanishing of pulse in the common, right, and left hepatic artery, confirming the findings of the angiography with the arterial flow to the liver based on a collateral network between the SMA and the hepatic artery via the GDA. This led to the decision for a PPPD with preservation of the GDA. The posterior superior pancreatoduodenal artery, a branch of the gastroduodenal artery, and the posterior inferior pancreatoduodenal artery, a branch of the SMA, as shown on the angiography that they gave blood supply to the duodenal metastasis, were dissected and ligated with established presence of pulse in the hepatic artery following this. On the contrary the branch of the gastroduodenal artery and the arterial network between superior and inferior anterior pancreatoduodenal artery were preserved (). The PPPD was completed without further modifications and with three anastomoses in the same loop of jejunum, an end-to-side pancreaticojejunal (duct to mucosa), end-to-side hepaticojejunal in a retrocolic fashion, and end-to-side duodenal-jejunal in antecolic fashion. The reconfirmation of presence of pulse in the hepatic artery after the end of the operation confirmed the blood flow from the SMA to the hepatic artery via the GDA.\nThe patient was transferred to the intensive care unit. The patient's recovery was complicated by a surgical wound infection requiring the use of a vaccum system which led to longer in-hospital stay. There was no bleeding following the surgical intervention and also the LFTs during the in-hospital stay were normal showing an adequate blood flow to the liver.\nThe histopathology test of the specimen confirmed the duodenal tumour to be a metastasis from the known ccRCC (immunohistochemistry showed that the tumour cells strongly express PAX8, RCCAg, AMACR, and CD10 with more focal expression of E-cadherin, C2M5.2 vimentin, and EMA). There was no tumour expression of CD117 or CK7. This immunoprofile which was consistent with a metastatic clear cell renal cell carcinoma was similar to that seen in the original kidney tumour. Twenty-four out of twenty-eight resected lymph nodes were infiltrated with metastasis. The tumour was completely excised (R0).\nThe patient was discharged twenty-six days after the operation; however, further systematic therapy was not possible as the patient died two months later due to a generalized extensive disease to the lungs, the brain, the liver, and the bones.
A 30-year-old woman was admitted to the emergency room because of persistent abdominal pain in the right side, appeared some day before, during a shift work as agricultural laborer. An accurate medical history ascertained that she had an increased steady weight (109 kg; BMI = 40 Kg/m2), but she was in apparently good health also showing customary appetite and regular menses, before the appearance of pain. Physical examination revealed the presence of a large mass, palpable in hypogastric region and in right lower abdominal quadrant. Ultrasonography confirmed an abdominal mass adjacent to the right ovary. To better define the diagnosis, the physician required blood sampling for routine tests, gynecological counseling and an abdominal computer tomography (CT). The abdominal CT confirmed the presence of a mass measuring 10 x 6 cm, in the right ovary. The patient underwent right unilateral salpingo-oophorectomy. In particular, an ovariian cyst (7, 3 x 3 cm) along with a portion of the fallopian tubes of 4 cm and the vermiform appendix were removed. Peritoneal washing and multiple peritoneal biopsies in nearby areas (Douglas, bladder, bilateral paracolic gutter, and diaphragm) were also performed. Histological examination revealed the presence of a follicular variant of papillary thyroid carcinoma (0.9 cm) harbored in a mature teratoma. The cancer was localized and the right ovary capsule was not infiltrated by the tumor. Based on Union for International Cancer Control (UICC) 2002, classification tumor received a pT1a grading. Appendix showed the features of chronic follicular inflammation and the remaining tissues were disease-free. After surgery, functional examination of thyroid gland revealed serum iodothyronines levels in the normal range, thyroid ultrasonography showed a normal glandular volume with patchy echo-structure and, in the left thyroid lobe, the presence of a 23, 4 x 14 x 9 mm hypoechogenic nodular lesion. An ultrasound assisted fine needle aspiration of this thyroid lesion was performed and cytological examination showed the presence of dense colloid in particles and small groups of thyrocytes with notes of oxyphilic metaplasia and anisonucleosis; this sample was classified as follicular lesion with oxyphilic cells of undetermined significance (category III according to the Bethesda system, 2009) (). So far, in presence of follicular lesion and for an appropriate follow-up of papillary carcinoma arising in struma ovarii, the patient underwent total thyroidectomy (, ). Histological examination revealed that the nodule examined in the left lobe was benign, but showed a papillary micro-carcinoma in the right lobe (diameter = 0.2 cm; pT1a) within a hyperplastic thyroid characterized by abundant oxyphil cells. The ablation of residual thyroid tissue was performed by a dose of 30 mCi of 131I according to Meas et al. (). Whole body scan after therapeutic dose showed two focal areas of strong uptake in the anterior region of the neck, in right superior paramedian and left inferior paramedian areas, due to minimal glandular remnants; no area of uptake in the pelvis or elsewhere was notice. The thyroid-stimulating hormone (TSH) value before radioiodine therapy was 64.8 mU/L and serum thyroglobulin (Tg) was 1.24 mg/dL, in the absence of anti-thyroglobulin antibodies (anti-TgAb). Levothyroxine treatment was started (1.6 μg/Kg/day) and thyroid hormones and TSH were evaluated after four months. Levothyroxine dose had been increased until an appropriate TSH value (0.24 mU/L) had reached at 1.92 μg/Kg/day. Six months after total thyroidectomy, patient underwent neck ultrasonography and Tg evaluation, which showed no evidence of recurrences. Patient is up to now in follow-up for both struma ovarii and differentiated thyroid cancer according to European Thyroid Association guidelines ().
A 56-year-old male visited his primary care physician to be evaluated for abdominal pain, and the subsequent workup revealed a large retroperitoneal mass measuring 21.8 × 17.5 × 11 cm. A diagnostic workup revealed a poorly differentiated germ cell neoplasm, most likely an embryonal carcinoma. Two months after his initial presentation he began chemotherapy (bleomycin, etoposide and cisplatin). By day 10 of cycle 3 (approximately 4 months after initial presentation), imaging showed a drastic decrease in tumor size.\nSix days after his most recent dose of chemotherapy, he presented to the emergency department noting intermittent fatigue, nausea, vomiting and diarrhea since his last chemotherapy treatment. He also noted headache and fever over the past day. In the emergency department (hospital day 0), he was found to be neutropenic with a total white blood cell (WBC) count of 0.6 × 109 cells/l and an absolute neutrophil count (ANC) of 0.4 × 109 cells/l. He appeared to be in no acute distress but febrile with a temperature of 38.7°C. No obvious signs of an infectious source were noted, including examination of his port-a-cath. The patient was started on cefepime and admitted to the inpatient oncology service for treatment of neutropenic fever.\nThe admitting physician noted tachycardia, but the patient's blood pressure was within normal limits. The only possible localizing sign of infection was a questionable cellulitis of the right lower leg. As the patient had previously been on prophylactic levofloxacin and had frequently been to infusion clinics, vancomycin was added for methicillin-resistant Staphylococcus aureus coverage. The patient was also started on G-CSF (480 μg/day) for neutropenia. Over the subsequent 2 days, his fever and tachycardia continued, and on hospital day 3 the patient became lethargic and confused. A chest radiograph (which showed no sign of pneumonia), blood and urine cultures and a CT scan of the head without contrast showed no acute changes. Throughout this period his ANC remained <1.0 × 109 cells/l.\nBy hospital day 4, the patient was unable to respond to commands and was transferred to our institution for further care; his WBC count had risen to 1.8 × 109 cells/l (ANC not measured) and he remained febrile. His vancomycin was continued while cefepime was changed to ceftazidime out of concern for cefepime-induced encephalopathy []; G-CSF was continued. Shortly after arrival, the patient had 2 tonic clonic seizures, and lorazepam and fosphenytoin were given. He became more lethargic, with periods of apnea, and was transferred to the intensive care unit for endotracheal intubation. An MRI of the brain and MRA of the brain and neck with and without contrast showed an acute punctate infarct in the right hippocampus and no major vascular findings. Continuous EEG monitoring was started. His WBC count shortly after intubation was 11.4 × 109 cells/l with an ANC of 7.0 × 109 cells/l. Fig. shows ANC, WBC count, G-CSF dosing and the patient's symptoms over time.\nA lumbar puncture was performed on hospital day 5 and revealed 64 WBC/μl, 30% neutrophils, 39% lymphocytes, glucose 37 mg/dl and protein 99 mg/dl. No organisms were seen on Gram staining. No cerebrospinal fluid opening pressure was measured. A thorough cerebrospinal fluid workup for infection was sent for evaluation, and empiric acyclovir was started. Cerebrospinal fluid India ink stain was completed in the early evening of hospital day 5 and showed rare encapsulated yeast, and the cerebrospinal fluid cryptococcal antigen (Latex Agglutination, IMMY, Inc., Norman, Okla., USA) was positive at a dilution of 1:32.\nThe infectious disease service was informed of the result, amphotericin B lipid complex was started at a dose of 5 mg/kg, and flucytosine 100 mg/kg/day was also ordered but was not but available until hospital day 7. On hospital day 6, the WBC count rose to 28.0 × 109 cells/l and G-CSF, acyclovir, ceftazidime and vancomycin were stopped.\nThroughout hospital days 6 and 7, the patient was minimally responsive despite no sedation since intubation; however, he did withdraw from painful stimuli. No additional seizures occurred. On hospital day 8, the patient moved his lower extremities on command, and his neurological function rapidly improved. Repeat lumbar puncture was performed on hospital day 9 with an opening pressure of 16 cm H20. By hospital day 10, the patient's neurologic status had improved, and he was extubated. His culture showed Cryptococcus neoformans resistant to flucytosine, and so flucytosine was replaced with fluconazole 800 mg daily.\nAfter 14 days total of amphotericin-based combination therapy, the patient continued on fluconazole 800 mg daily consolidation monotherapy. Repeat lumbar puncture at the time of stopping amphotericin showed a decreased cryptococcal antigen titer of 1:4 and an opening pressure of 22 cm H20. On hospital day 21, the patient was discharged to a transitional care unit. Since hospital discharge, the patient has continued to do well in terms of his CM, although he has required hospitalization for pericarditis. He is being maintained on fluconazole 200 mg daily for secondary prophylaxis, and further chemotherapy has been postponed pending improvement of his other health issues.
A 70-year-old woman was referred to our institution in July 2012 for treatment of left ocular pain. She complained of ocular discomfort and decreased visual acuity (12/20) at the first visit. She underwent nasal pterygium excision with topical MMC in the left eye six years previously at another facility. A few years after surgery, symptoms of intermittent ocular discomfort and mild ocular pain developed, so she went for treatment to a local medical center. She had neither medical history or family history of ophthalmic disease.\nOn slit-lamp examination, the underlying sclera at the site of the prior pterygium excision was necrotic and avascular, and showed marked thinning in the nasal portion of the left eye (Fig. ). The scleral bed appeared conjunctivalization over the exposed ciliary body with no evidence of scleral perforation sign by Seidel test. The adjacent corneal epithelium was intact and there was no anterior chamber inflammatory reaction. We thought that the scleral thinning was a possible MMC-associated thinning. Microbial smears and cultures of the scleral bed were obtained at the first visit, but were negative.\nThe patient was started on topical 0.1 % fluorometholone and 0.5 % levofloxacin eye drops. Two weeks later, the patient’s ocular pain and discomfort was gone. After informed consent, the patient underwent an OCM graft with CAU at the scleral thinning area. The surgical technique is described in Fig. . Proparacaine hydrochloride eye drop (0.5 %, Alcaine; Alcon, Fort Worth, TX) was used as topical anesthesia before surgery. First, the necrotic soft tissues and devitalized sclera were surgically debrided with a diamond burr, taking care not to damage the exposed ciliary body and the unaffected adjacent conjunctiva. Gentle polishing with a diamond burr instead of a knife can produce a flat, regular surface without damaging the already much thinned scleral bed. All necrotic scleral tissue was dissected away until the scleral surface was clean, smooth, and even. Conjunctival hemorrhage during debridement was controlled by ocular bovie and cotton swab compression. To determine the boundary for the conjunctivectomy, the size of the scleral defect was measured and its margin was marked with a 3-mm diameter biopsy punch (Fig. ). The margin of scleral thinning area was trimmed by Vannas scissors and the OCM was cut with a circular-shaped biopsy punch of the same size (3-mm diameter). The OCM was trimmed and fitted to cover the scleral defect (Fig. ). The OCM was sutured with a recipient scleral wall using six stitches of 10–0 nylon interrupted sutures. The color of the OCM changed from white to red due to blood accumulation (Fig. ). Once the scleral defect was repaired, a 4-mm diameter, circular, free CAU was harvested from the superonasal bulbar conjunctiva with a punch biopsy 1 mm larger in diameter than that of the piece of OCM. A conjunctival graft that is larger than the scleral defect can achieve a stable, tension-free graft to avoid a wound dehiscence (Fig. ). The CAU was carefully positioned over the previously sutured OCM bed and anchored to the scleral wall and the healthy conjunctival margin through the OCM bed with 11 stitches of interrupted sutures of 10–0 nylon (Fig. ).\nAfter surgery, the patient was given a patch dressing with a topical antibiotic ointment (Erythromycin, Ecolicin®, Taejoon Pharm., Seoul, Korea) and a steroid ointment (Dexamethasone, Maxitrol®, Alcon Laboratories Inc., Fort Worth, TX, USA) to be taken four times daily. The patient was examined daily for the first five postoperative days. After five days, the ointment patch dressing was replaced by topical antibiotics (0.5 % levofloxacin, Cravit®, Taejoon Pharm, Seoul, Korea) and steroid eye drops (0.1 % fluorometholone, Flarex®, Alcon Laboratories Inc., Fort Worth, TX, USA) to be taken four times a day. Inflammation of the scleral bed subsided and the patient was comfortable. The ocular surface was re-epithelialized six days after surgery. The conjunctival sutures were removed at intervals of one week to one month over the postoperative course. Starting about one month after surgery, the topical 0.1 % fluorometholone and 0.5 % levofloxacin eye drops were tapered off over three months. Artificial tear substitutes were used continuously after surgery. The patient was examined weekly for the first two months and at three, four, six, nine, 12, 16, 20, and 24 months postoperative.\nOver the first postoperative month, the graft site vascularized and was taken up well (Fig. ). The conjunctival surface was stable at six months and 12 months postoperative. When reviewed 24 months later, the conjunctival surface was still stable with no recurrence of scleral thinning and scleromalacia (Fig. ).
A 70-year-old Sri Lankan Tamil male with a history of well-controlled type 2 diabetes mellitus and a goitre of 30 years presented with a painful enlargement of goitre on the left side for one month. He had progressively worsening difficulty in breathing with intermittent dysphagia for solids. He did not have any symptoms of local infiltration and was clinically euthyroid. Examination revealed a hard mass arising from the left thyroid lobe measuring 8 cm × 7 cm in size with gross tracheal deviation to the right side. In addition, there were two mobile lumps anterior to the mass in the subcutaneous tissue plane (). There was no retrosternal or retroclavicular extension on the left side. The right thyroid lobe was moderately enlarged and had multiple palpable nodules. There was no cervical lymphadenopathy. Ultrasound scan showed a large calcified left thyroid nodule and few superficial nodules. The outer surfaces of the nodules were delineated by an echogenic line suggestive of surface calcification. The internal echotexture of the nodules was not clearly appreciated due to artefacts from the surface calcifications. Right thyroid lobe showed only benign characteristics. Neck X-ray radiography showed a calcified left lobe with significant tracheal deviation to the opposite side (Figures and ). Thyroid stimulating hormone (TSH) and free thyroxine (T4) levels were within normal limits. Ultrasound-guided fine needle aspiration cytology (FNAC) showed scattered cyst macrophages, lymphocytes, and multinucleated giant cells in an eosinophilic background with scanty colloid. The features were compatible with a benign cyst (Thy 2).\nThe patient underwent a total thyroidectomy. Two confluent nodules were noted in the subcutaneous tissue plane extending through the deep fascia between the strap muscles to the calcified left lobe nodule. The deep fascia and strap muscles were thinned out and were adhered to the calcified left lobe (). Division of strap muscles on the left side was required to mobilize and deliver the left lobe containing the calcified nodule.\nMacroscopic assessment of the specimen consisted of the thyroid gland with the right lobe measuring 45 × 25 × 20 mm, the isthmus measuring 65 × 15 × 4 mm, and the enlarged left lobe measuring 80 × 75 × 55 mm. The outer surface of the gland was smooth. There were two confluent nodules over the anterior surface of the left lobe measuring 12 × 8 × 8 mm and 10 × 8 × 6 mm with a smooth outer surface.\nHistology of the thyroid revealed an encapsulated left lobe lesion composed of a thick fibrous wall with foci of calcification. A dense inflammatory reaction comprising lymphocytes, foamy histiocytes, and scattered multinuclear giant cells was present within the capsule. The lumen was filled with amorphous, eosinophilic material with cholesterol clefts. A thin rim of compressed thyroid tissue was noted outside the fibrous wall. Sections from the confluent nodules revealed similar histopathological features and showed encapsulated lesion surrounded by a thin fibrous capsule. They were filled with numerous foreign body type giant cells and foamy histiocytes admixed with amorphous eosinophilic material and cholesterol clefts. No thyroid or lymphoid tissue was seen. The right lobe and isthmus showed features of a colloid storing goitre. There was no evidence of malignancy in the entire specimen. Overall, features of the main calcified nodule of the left lobe and the two smaller confluent nodules were compatible with a colloid cyst with secondary changes including calcification and chronic inflammation. The patient had an uneventful postoperative recovery with the alleviation of the obstructive symptoms. He was discharged on the first postoperative day on thyroxine 100 mg daily and remained healthy without any obstructive symptoms during a three-month routine outpatient clinic review.
A 53-year-old man manual worker with a 13-year history of gout in his right hallux presented to his general practitioner with right knee pain, stiffness, and giving way with no history of trauma. He had been taking allopurinol for 11 years. He drank 10 to 12 units of alcohol per week and his body mass index was 24.4. On examination, he had anterior knee pain and crepitus was felt from the patellofemoral joint. He had a range of motion from 0 to 90 degrees.\nA magnetic resonance imaging (MRI) was performed and reported a grossly abnormal patella tendon showing heterogenous characteristics with areas of architectural distortion and altered signal in all sequences. The appearances were not typical for a tendinosis but more in keeping with findings seen in gout (\n).\nThe patient was commenced on anti-inflammatory medication in addition to his regular allopurinol and referred to an orthopaedic knee surgeon. At assessment, he was found to be significantly compromised by his knee. He was unable to ride a pushbike, walk with his dog, or even get out of a chair. Plain films showed calcification within his patellar tendon (\n).\nA multidisciplinary discussion with rheumatology and radiology consultants confirmed that the likely diagnosis was a tophaceous gouty deposit within the patella tendon. His uric acid level was 560 μmol/L (above the target of <300 μmol/L set by the British Society for Rheumatology [BSR])\nand estimated glomerular filtration rate was 52 mL/min. He was referred to rheumatology who advised increasing the dose of allopurinol. A subsequent ultrasound (US) scan showed the superficial fibers of the patellar tendon relatively intact, but within the deep fibers, there were multiple hyperechoic areas with distortion of the tendon architecture. There was no significant cyst. A computed tomography (CT) scan demonstrated a markedly thickened patellar tendon with areas of mineralization within the tendon itself (\n). He was seen in a complex knee clinic with three consultant orthopaedic knee surgeons present. With the patient being fully informed about the risk of surgery and patellar tendon weakening and disruption, he was added to the list for open surgical excision of gouty tophus 18 months from initial presentation. His medical management of 200 mg/d of allopurinol had brought his uric acid down to 366 μmol/L with no side effects, but this was still above the BSR guidance. Subsequently, his allopurinol was increased to a daily dose of 300 mg.\nSurgery was performed with the tourniquet inflated at 300 mm Hg for 20 minutes. A midline skin incision was utilized, and the paratenon visualized and incised longitudinally. It was then developed as a definite layer following which the patellar tendon was encountered. The patellar tendon was incised longitudinally and stay sutures placed on either side of the tendon. With gentle traction, the deeper diseased tendon along the lower half of the patellar tendon could be exposed and excised with sharp dissection (\n). The excised tissue was sent for histology (\n). Finally, a bony spur was encountered, and this was excised with a nibbler. Postoperatively, the patient was allowed to fully weight bear, at his level of comfort. At 6-week review, the wound had healed with no postoperative complications .The patient was able to perform a straight leg raise and manage a full range of pain-free knee movement. His Oxford Knee score was 45.
A 48-year-old man with ESRD secondary to diabetic nephropathy was admitted for initiation of renal replacement therapy. He had undergone 36 cycles (36 hours of 1 L/h cycle) of acute PD, through a temporary PD catheter inserted through the infra-umbilical approach, at another center 10 days prior and presented to us for maintenance dialysis. After discussing the options, he was started on hemodialysis through a temporary catheter and was planned to start on CAPD. He had a history of diabetic retinopathy and neuropathy. He did not have any history or symptoms suggestive of neurogenic bladder.\nThe patient underwent 3 sessions of hemodialysis and was planned for CAPD catheter insertion with vancomycin prophylaxis. As per protocol, he was advised to void his bladder completely and to pass stools before insertion. An infraumbilical midline incision was made under local anesthesia and IV sedation, an 18-G introducer needle was inserted through the rectus sheath until it reached the peritoneal cavity, and 1.5 L of peritoneal dialysate was instilled intraperitoneally. After instillation of fluid, a guidewire was passed through the needle, with the tip directed towards the suprapubic region. The needle was removed, the tract was dilated using a 16 Fr dilator followed by insertion of a 16 Fr peel away sheath/dilator. The guidewire was removed followed by the dilator, and Tenckhoff swan-neck double-cuff straight catheter was inserted through the peel away sheath. The sheath was peeled away, and a subcutaneous tunnel was created with the exit site facing down and out towards the left iliac fossa. The deep cuff was positioned over the peritoneum, and the superficial cuff was placed in the tunnel. The incision site was sutured in layers. Postprocedure, the inflow and outflow were found to flow without resistance, and the catheter was brought out through a left-sided tunnel. A 500-mL flush was given, and good inflow and outflow were noted. The outflow was initially blood-mixed, followed by drainage of clear fluid. There were no complications during the immediate postoperative period. With a plain abdominal radiograph, the position of the catheter tip was confirmed to be in pelvis (). Ultrasonography was not used during the procedure due to logistic reasons.\nOn the 3rd day after insertion, after instillation of 500 mL of PD fluid, the patient complained of urinary urgency with an increase in urinary volume to 700 – 800 mL/day from his baseline of 300 – 400 mL/day. Also, there was decrease in the outflow volume with drainage of only 100 mL following instillation of 500 mL of PD fluid. With the increase in the inflow volume, there was a significant increase in his urine volume. Urine analysis was done to look for glycosuria, which showed a 3(+) on dipstick, from his baseline glycosuria of 1(+). He was asked to void after instilling 500 mL PD fluid through the PD catheter, and urinary creatinine testing was done, which was found to be absent. A possibility of peritoneovesical fistula was considered, and a cystogram was planned. The instilled fluid presumably entered predominantly into the urinary bladder through the side holes and led us to presume that the PD catheter could be in the preperitoneal location. Urine culture and PD fluid cultures were sent, which revealed growth of enterococcus (> 105 colony forming units/mL), and he was treated with vancomycin intraperitoneally (IP) and broad-spectrum antibiotics intravenously.\nThe cystogram revealed a smooth-walled bladder, with no intraperitoneal leak of the contrast, and the catheter abutting the bladder wall (). A plain abdominal computed tomography (CT) was performed to confirm the position (). The catheter was seen to be piercing the anterior abdominal wall and entering the bladder anteriorly, perforating and coursing through the bladder before exiting through the posterior wall, with the tip in the rectovesical fossa. PD was discontinued, and he was continued on hemodialysis through a temporary femoral access catheter. After discussion with the urologists, it was planned to remove the catheter, followed by reinsertion at a later date, with conservative management for the bladder perforation. With cessation of PD, there was decrease in urine output to ~ 50 – 100 mL/day, and a repeat urine routine performed 3 days later showed reduction in pyuria (from plenty of white blood cells/high power field to 6 – 8 WBC/HPF).\nThe options were discussed with the patient, and the need for a vascular access was explained. However, due to logistic reasons, an attempt was planned towards catheter salvage. He was planned for catheter repositioning using fluoroscopic guidance under the cover of intravenous and intraperitoneal broad-spectrum antibiotics.\nUsing fluoroscopy with contrast (urograffin), first the bladder perforation was confirmed. The infraumbilical incision was re-explored under local anesthesia, while leaving the tunnel in situ. The deep cuff was identified, and the PD catheter was pulled back by ~ 6 cm (as measured by the prior abdominal CT image). Contrast was injected through the catheter, and it was confirmed to be intraperitoneal with no filling up of the urinary bladder. Using a guide wire, the catheter was redirected towards the right iliac fossa, and the position confirmed. On subsequent contrast injection through the PD catheter, the intraperitoneal nature and absence of bladder filling was confirmed. The bladder was later filled up with contrast, and there was no presence of any leaks (). The initial bladder perforation could have been an intraperitoneal perforation since pulling back and re-positioning the catheter, without any new entry into the peritoneum, confirmed it to be in the peritoneal cavity.\nThe patient was continued on IV antibiotics for 5 days, IP ceftazidime for a week, and IP vancomycin for 21 days, and was continued on hemodialysis. IP vancomycin was given in a single dwell (1,000 mL) for 6 hours every day. One week post repositioning, he was started on low-volume CAPD and discharged, and a repeat urine and peritoneal fluid culture were sterile. He was continued on Foley’s catheter for 4 weeks, with a residual urine output of ~ 200 – 300 mL/day. At 9-month follow-up, the patient is comfortable with CAPD 3 exchanges/day with good ultrafiltration (~ 1.4 L/day), and no change in urinary volume, with no further episodes of peritonitis.
A 50-year-old female Asian patient reported to the Department of Oral Medicine and Radiology with a two month history of multiple painful oral ulcers with difficulty in eating and swallowing solid food . Initially she noticed a bullae in her left buccal mucosa which was initially small and later involved the entire oral cavity. The patient had medical consultation and was prescribed topical medication and vitamin supplements with no relief. Her medical and family history was not significant. The patient showed no signs of skin and other mucosal involvement. Intraoral examination revealed a large shallow irregular ulcer, about 4 cm x 4 cm in diameter on the left buccal mucosa. The ulcer was round in shape with irregular margins and an erythematous halo. The base of the ulcer was covered with yellowish white pseudomembrane interspersed with erythematous area. Multiple irregular shallow ulcers were observed in the right buccal mucosa, lower labial mucosa, ventral surface, and the right and left lateral border of the tongue. Multiple pin-point isolated ulcers were evident in the soft palate (Figures -).\nThe ulcers were tender, with a yellowish white pseudomembrane on the floor that peeled off leaving an erythematous area that bled on palpation. Correlating the history of bullae and the clinical features of multiple chronic oral ulcers, the fragility of the oral mucosa (Nikolsky’s sign), the provisional diagnosis of oral PV was made. Since chronic oral ulcers are common in conditions of erosive lichen planus, mucous membrane pemphigoid, bullous pemphigoid, and major variants of pemphigus paraneoplastic pemphigus and pemphigus foliaceous were considered in the clinical differential diagnosis. The erosive form of lichen planus (LP) frequently presents with the characteristic feature called Wickham’s striae, along with erosions. Mucous membrane pemphigoid is more common in females with intact vesicles of the gingival or other mucosal surfaces. The erosions spread more slowly than PV. Bullous pemphigoid occurs chiefly in adults over the age of 60, oral lesions are smaller, form more slowly, and are less painful than those seen in PV, and the extensive labial involvement seen in pemphigus is not present. Paraneoplastic pemphigus is a severe variant of pemphigus that is associated with erosions in the mucous membrane and the skin associated with an underlying neoplasm. Mucosal involvement is uncommon in foliaceus and erythematous forms of the disease []. The smear was taken from the right buccal mucosa and subjected for exfoliative cytology and the smear showed acantholytic cells, a few normal squamous epithelial cells along with mixed inflammatory cells chiefly lymphocytes and neutrophils suggestive of pemphigus (Figure ). Incisional biopsy was performed on the perilesional site of the right buccal mucosa and was subjected for histopathological investigation.\nHistopathology in the present case was characterized by stratified squamous epithelium with intraepithelial bullae containing acantholytic cells and characteristic tombstone appearance, connective tissue with mixed inflammatory cells chiefly plasma cells and lymphocytes and fibro adipose tissue in the deeper section, suggestive of PV with secondary infection (Figure ).\nCorrelating the clinical and the histopathological features, a final diagnosis of oral PV was made. The patient was started on oral prednisolone at an initial dose of 20 mg, in divided doses of 5 mg, four times a day, along with topical application of triamcinolone acetonide oral paste 0.1%, three times a day after meals for about one hour. In addition, the patient was given hexidine mouth rinse for oral prophylaxis and clotrimazole oral paint 1% twice a day after topical application of steroids for an hour to prevent secondary fungal infection for one week. Treatment was continued for another week with the same dosage since there were few lesions present. After two weeks the dosage was tapered to 10 mg, in divided doses of 5 mg, two times daily for another two weeks. At the end of the fourth week, prednisolone was decreased to 5 mg single dose for two weeks. The patient got recovered. She was periodically reviewed once a month, over a period of seven months, with no recurrence of oral lesions (Figures -).
A 26-year-old male presented to Accident and Emergency following assault with a baseball bat. His past medical history consisted of longstanding Hepatitis C infection only, he was on no regular medications nor had any known allergies.\nOn admission, the patient had complained of bilateral pleuritic pain on inspiration with visible, tender bruising to the posterior thoracic region bilaterally. There were no urinary, bowel or neurological symptoms reported and no open wounds were visible on examination. The anterior abdomen was soft and non-tender to palpation.\nA CT scan of chest (performed at 30s post i.v. injection) showed acute fractures of left 10th/11thand right 8th/9th ribs, but no evidence of pneumothorax or lung contusion. A splenic laceration was not suspected clinically and the timing of the scan, optimized for the chest, only showed subtle splenic changes which were not identified by the original reporter ().\nLater that day, the patient complained that their pain had spread from the chest down to the right and left flanks. This prompted a CT scan of abdomen/pelvis (performed at 70 s), which demonstrated a contained splenic laceration with no evidence of capsular breach (). Both the rib fractures and splenic injury were treated conservatively with bed rest and analgesia.\nThe patient abruptly deteriorated becoming hypotensive and tachycardic in keeping with hypovolaemic shock. A further CT scan showed splenic capsular rupture, active bleeding and large-volume intraperitoneal haemorrhage ().\nThis was managed urgently with endovascular embolisation. While the patient gave informed consent for the procedure he indicated that he was unlikely to be compliant with some aspects of his aftercare and likely to self-discharge very soon after completion of the procedure.\nFollowing local anaesthesia an ultrasound guided puncture of the right common femoral artery was performed and the splenic artery selectively catheterized with a Sim 1 catheter (a reverse angled catheter). Angiography revealed three pseudoaneurysms, two arising from a second order upper pole branch and one from a third order equatorial branch (). These were then superselectively catheterized and embolized with a series of 3, 4 and 5 mm microcoils (Nester & Vortex, Nester = Cook Medical, Bloomington, IN, USA, Vortex = Boston Scientific, Watertown, MA, USA), delivered through a microcatheter. This abolished filling of the pseudoaneurysms but there was more devascularisation of the upper half of the spleen than envisaged when the decision to perform superselective embolisation was made. The right common femoral artery was then closed with a Starclose vascular closure device (Abbott Medical).\nThe decision to perform superselective embolisation was made with the intention of achieving definitive haemostasis in a patient who was likely to leave hospital at the earliest possible opportunity and not comply well with the procedural aftercare (). There was no further bleeding.\nDay 1 post-procedure, the patient developed a post-embolisation syndrome (PES) characterized by fever, nausea and left abdominal pain. A fourth CT scan was performed. This showed splenic necrosis, parenchymal and intravascular gas, which is an expected appearance post-embolisation. No fluid collection was demonstrated. Conservative resuscitation was initiated with i.v. fluid and antibiotic administration.\nThere was no indication for surgical drainage at this point as the CT scan showed only splenic necrosis, with no evidence of abscess formation. The patient’s temperature and clinical features were also settling, further reassuring stabilisation of his condition ().\nThe patient recovered from this episode a few days later, and deemed medically fit, was subsequently discharged home.\n3 weeks later, the patient presented to Accident & Emergency once again; this time with episodes of frank haemoptysis. He had an associated fever, and left upper quadrant (LUQ) pain.\nBlood tests showed elevated inflammatory markers, suggesting an infection, thought to be of likely respiratory origin at this time.
We report the case of a 20-year-old Caucasian, nonsmoking female student, height 185 cm, weight 66 kg, who developed pain of the left pinna which is why she attended the otolaryngological ambulatory unit two days later (). The left pinna was found to be sore and reddish but without vesicles, which is why chondritis was diagnosed and amoxicillin and clavulanic acid prescribed. One day later she awaked with a peripheral facial nerve palsy on the left side and hypoesthesia of the left upper lip. The weeks before onset she had a lot of stress with her studies at the university but there was no fever, no vaccination, or infection. A few days before onset of the palsy she recognised dysesthesias of the parietal skull on the left side. The night before onset of the palsy she was exposed against the ventilator of an air condition for more than an hour. She was regularly taking loratadine.\nClinical neurologic examination on admission revealed slight hypoesthesia of the left upper lip, moderate peripheral facial nerve palsy, and generally reduced tendon reflexes. The left pinna showed an erythematous rash but no vesicles. Hearing and taste were normal and there was no vertigo, double vision, skew deviation, nystagmus, headache, hyposmia, or ataxia []. Blood chemical investigations revealed slight leucopenia but normal C-reactive protein. Ophthalmologic examinations excluded a zoster of the eye. Immediately after admission, she was treated with dexamethasone (40 mg intramuscularly) during two days followed by dexamethasone orally during another six days. Additionally, she received 750 mg acyclovir three times a day intravenously during eight days. For continuous pain periauricularly she received mefenamic acid and paracetamol with success.\nAt followup three days later she reported some transient vesicles at the left pinna, no longer visible at the visit, lid closure had improved, and there was recovery from the pinna's rash but only marginal voluntary innervation of the frontalis and lower facial muscles. Audiometry was normal. Followup six days after onset of the palsy showed complete lid closure but unchanged innervation of the other facial muscles. She reported neuralgiform pain in the left ear which is why gabapentin was prescribed with success. Eleven days after onset of facial palsy lid closure on the left side was complete. There were slight innervation of the frontal branch and only slight innervation of the left mouth corner. Nerve conduction studies 12 days after onset showed almost equal compound muscle action potential (CMAP) amplitude when recording from the orbicularis oculi muscle but >50% amplitude reduction on the left side when recording from the orbicularis oris muscle. Antibodies against the varicella zoster virus (VZV) were determined three times by means of an ELISA test. IgG antibodies against VZV were positive at all three determinations and IgM antibodies against VZV were negative at the first and positive at the second and third determination, which is why valacyclovir orally was given for seven days after discontinuation of acyclovir. Antibodies against other viruses and Borrelia burgdorferi were normal. Follow-up 6 weeks after onset showed further recovery of the palsy, such that it was no longer visible in the absence of emotional reactions. Nerve conduction studies confirmed the improvement such that the CMAP amplitude difference was only reduced to 30%.
A 24-year-old pregnant woman (G2P1) was referred to us due to suspected bilateral ovarian cysts at 8 weeks of gestation. She had undergone ovarian cystectomy twice under open surgery: left and right ovarian cystectomy for mature cystic teratoma and mucinous cystadenoma, respectively. She had no additional medical history or familial medical history. Transvaginal ultrasound and magnetic resonance imaging (MRI) (Figures and ) revealed two pelvic cysts. The left-sided unilocular cyst was 9 cm in diameter. The right-sided multilocular cyst was 5 cm in diameter. We diagnosed this condition as bilateral ovarian cysts.\nAlthough the serum levels of tumor markers (CA125, CA19-9, and CEA) were normal for a pregnant woman, considering the large size of the cyst, cyst resection was attempted at 14 weeks; however, it was converted to probe laparotomy. Marked adhesion around the cysts, posterior uterus, and Douglas' pouch made cyst resection impossible as extensive adhesiolysis may cause uterine damage and also uterine contractions after surgery. Gross examinations revealed no metastatic lesions or lymph node swelling. Abdominal fluid cytology revealed no malignant cells.\nAt 32 weeks of gestation, MRI revealed that the left-sided cyst size had increased to 27 cm in diameter (Figures and ), although she was asymptomatic. As shown in , the right-sided multilocular cyst became very close to the left monocytic cyst. At this stage, the left large monocytic cyst appeared to merge with the smaller right multilocular cyst, forming a large cyst occupying the entire pelvic cavity, which was later confirmed by laparoscopic findings.\nThis large cyst showed no solid-part or papillary growth. The serum levels of tumor markers remained normal. Malignant ovarian tumor could not be ruled out but was considered less likely. We weighed merits and demerits between relaparotomy for tumor resection during pregnancy and a wait-and-see approach for several weeks; the former is likely to require extensive adhesiolysis and may cause preterm delivery. We decided on the latter strategy, since resection should be performed in the event of a size increase or images indicative of malignancy. The fetus normally developed without fetal growth restriction.\nCesarean section and tumor resection were performed at 37+4 weeks of gestation, yielding 3,012-g male infant with Apgar score 8/9 at 1/5 minutes, respectively. The infant did not have congenital abnormalities. After the completion of cesarean section, we ruptured the wall of this large cyst, with care to avoid the cyst content entering into the abdominal cavity. A large amount of serous fluid was drained. This large cyst was a multicystic cyst (5 cm), considered to be the right multicystic ovarian cyst that had been observed from the first trimester. The wall of the large cyst showed marked adhesion to the peripheral peritoneal cavity. We resected it as widely as possible together with right salpingo-oophorectomy (Figures and ). The left ovary was macroscopically normal, and thus there was no evidence of the left ovarian tumor. The resected tumor consisted of a large unilocular cyst with serous fluid and a mucinous cystadenoma (Figures and ). In the former, lining epithelium was absent in many parts () and mucinous epithelium was occasionally found in continuity with the cyst wall of the latter (right ovarian cystadenoma). No malignant cells were found in the resected specimen. Immunohistochemistry revealed focally positive staining for estrogen and progesterone receptors on the resected cyst wall (Figures and ). At 12 months after the delivery, left ovary remained normal and the retention cyst did not recur. An informed consent for this reporting was obtained from this patient.
A 6-year-old, previously healthy boy, presented to the Pediatric department because of a strongly positive tuberculin skin test (34 mm) and mild chronic swelling with a skin fistula in the left submandibular area, which developed 3 months prior to admission. He was initially treated with oral and then intravenous broad spectrum antibiotics for 3 weeks with the presumptive diagnosis of cervical adenitis with no response at another hospital. After evaluation by an oral and maxillofacial surgeon, the diagnosis of a submandibular abscess originating from the decayed primary second lower left molar was made (Fig. ). The patient underwent incision and drainage of the abscess extraorally and extraction of the involved tooth. Postoperatively, the symptoms improved, and he was discharged. Routine bacterial cultures were negative. The following 2 months, he had persistent moderate swelling, and a fistula developed at the incision site.\nThe patient had no history of contact with a case of active tuberculosis, and investigation of family contacts was negative. He lived in an urban area and had no contact with cattle or history of consumption of unpasteurized dairy products. The child was born in Greece and never travelled abroad. Both grandparents and parents were born in the former Soviet Union and had immigrated to Greece 20 years ago. The patient was not immunized with Bacillus Calmette–Guérin vaccine.\nPhysical examination on admission revealed a well-nourished boy in good clinical condition. A left submandibular firm, erythematous swelling measuring 3 × 3 cm, was palpated with a draining sinus tract. Laboratory studies were unremarkable. Blood tests revealed a white blood cell count of 15,200/mm3 with 74% polymorphonuclears, 18% lymphocytes, and 8% monocytes; erythrocyte sedimentation rate was 50 mm/h, and C-reactive protein was 77 mg/L. Measurement of serum immunoglobulins, determination of lymphocyte subpopulations by flow cytometry, and nitroblue tetrazolium test were performed and were normal. Serologic testing ruled out human immunodeficiency virus (HIV) 1 and 2 (enzyme-linked immunosorbent assay method), toxoplasmosis, cytomegalovirus, Epstein–Barr virus, and Bartonella henselae infection. Furthermore, chest radiography and abdominal ultrasound were normal, while culture of gastric aspirate was negative. QuantiFERON-TB Gold In-tube (QFT-IT) test was positive. A computed tomography of the region revealed the presence of chronic osteomyelitis of the mandibular body adjacent to the extraction site with osteoblastic subperiosteal activity and thickening around the lower mandibular border (Fig. ).\nAntituberculous treatment was initiated with isoniazid, rifampicin, and pyrazinamide (PZA), and the patient underwent surgical excision of the submandibular mass and the draining sinus. The histopathological examination revealed no lymphoid but only fatty and fibrous tissue with granulomatous caseating infection. Acid fast bacilli were visualized by Ziehl–Neelsen stain. Two months later, mycobacterial growth was evident on Lowenstein–Jensen medium. The culture isolate was identified as M. bovis subsp. bovis by means of the GenoType Mycobacterium tuberculosis complex (MTBC) assay (Hain Lifescience, Germany). This assay is based on the DNA-STRIP technology and permits the genetic differentiation of species belonging to the Mycobacterium complex on the basis of gyrase B polymorphisms []. Our finding was subsequently verified in the central laboratories of the kit manufacturer. Susceptibility test showed resistance to PZA, as expected. Based on these results, PZA was discontinued, and ethambutol was added to the therapeutic regimen. By the end of a 6-month therapy, the lesion had healed and showed complete resolution. The patient has been followed for 2 years and had no recurrence.
A 78-year-old male with multiple comorbidities, including hypertension, hyperlipidemia, and type II diabetes mellitus, presented to our emergency setting with complaints of recurrent bouts of abdominal pain and fluctuating fevers for the previous two weeks. The patient reported that the pain is a new manifestation of a previously dull aching pain that had waxed and waned over the last decade. His description alluded to a pain that was sharp and intermittent with localization in the right upper quadrant. He could not attribute the intermittent nature of his predicament to any aggravating or relieving influences. The pain was associated with fluctuating low-grade fevers (99°F-100°F), anorexia, and an associated 13-pound weight loss, which culminated in a visit to our clinical setup.\nFurther interrogation disclosed that the patient underwent a laparoscopic cholecystectomy in 2003. The ensuing year was relatively pain-free but was followed by recurrent bouts of right upper quadrant pain, albeit less upsetting than his current presentation. He was subsequently diagnosed in 2005 with gallstone spillage. The patient chose conservative treatment for his abdominal pain, rather than invasive interventions, which included the administration of acetaminophen and non-steroidal anti-inflammatory drugs (NSAIDs). This treatment modality was sufficient for the duration of a decade. He presented to another medical facility with similar complaints of fever and abdominal pain in 2016. A computed tomography (CT) scan of his abdomen disclosed the presence of a necrotic phlegmon, which was subjected to aspiration. Its composition included a combination of fibrous material, granulation tissue, and inflammatory infiltrate. The aspiration provided considerable relief of symptoms and he was discharged on a gabapentin prescription that was well-tolerated and produced sustained amelioration of his pain, with only occasional wavering with respect to his baseline.\nThe initial assessment showed an elderly gentleman, who was alert and well-orientated but under considerable distress due to the abdominal pain and accompanying chills. He had a fever of 104°F, a heart rate of 120 beats per minute, a blood pressure of 95/70 mm Hg, and a respiratory rate of 19 per minute. An abdominal exam revealed a non-protuberant, soft, and tender abdomen with marked sensitivity in the right upper quadrant. He had perceptible bowel sounds, an absence of dullness on percussion, and a clear rectal vault. Pertinent initial laboratory investigations included an elevated white blood cell (WBC) count of 16,900/µL, C-reactive protein (CRP) of 6.5 mg/dL, and a random blood glucose (RBG) of 280 mg/dL. The patient subsequently underwent a whole body CT scan, which revealed a large 19-cm sub-diaphragmatic, right retroperitoneal abscess, which was inferior and posterior to the right hepatic lobe (Figure ).\nIn lieu of these findings, the patient was admitted and started on a combination of intravenous fluids, intravenous (IV) vancomycin and piperacillin-tazobactam due to an underlying suspicion of sepsis secondary to a hepatic and/or perihepatic liver abscess caused by a lack of gallstone retrieval following his cholecystectomy. He was subsequently subjected to a percutaneous CT-guided drain placement, which allowed for the evacuation of approximately 700 mL of grossly purulent material and provided prompt pain relief. The fluid sample was sent for a gram stain, culture, and a bilirubin assay. The gram stain of the abscess fluid revealed branching gram-positive rods concerning for Actinomyces, Nocardia, and Streptomyces; therefore, the patient remained on IV vancomycin and piperacillin-tazobactam, with the addition of trimethoprim-sulfamethoxazole (TMP-SMX) to the antibiotic regimen. Remarkably, the culture growth was positive for Propionibacterium and ampicillin-sulbactam was added to the list of antibiotics. Two days later, the patient was shifted to the intensive care unit (ICU) following unrelenting fever and persistent tachycardia, even after his abdominal drain placement was supplemented with vigorous antibiotic and antipyretic therapy. His ICU stay was complicated by episodes of dyspnea, tachypnea, and pleuritic chest pain, while a chest auscultation unveiled a decrease in breath sounds at the right basal region. A repeat CT scan confirmed the reduction in the size of the abscess and showed a new right-sided pleural effusion, which explained the patient’s breathing difficulties (Figure ).\nThe patient underwent an ultrasound-guided chest tube placement, which evacuated 10 mL of purulent fluid, followed by two doses of lytic therapy with tissue plasminogen activator (tPA) via the catheter. The following morning, he described an aggravation in his pleuritic chest pain, and an episode of shortness of breath, whereby, the oxygen saturation dipped to 90%. A new chest CT scan was ordered, which showed an interval increase in the right basilar opacity with a moderately sized pleural effusion (Figure ).\nHe was subsequently upgraded to a larger catheter via a CT-guided approach that evacuated a total of 900 mL of purulosanguinos fluid. The patient tolerated subsequent lytic therapies well and showed vast clinical improvement following this procedure, with a reduction in dyspnea, pleuritic chest pain, and fever, as well as a down-trending of his inflammatory markers.\nA repeat CT scan was performed on the 10th day of admission, which revealed a near-complete resolution of his right-sided pleural effusion, and a decrease in his right-sided retrohepatic intrabdominal abscess (Figure ).\nThe chest drain was removed and the patient was subsequently discharged with a transhepatic drainage catheter, daily intravenous ampicillin-sulbactam, and acetaminophen, as needed for pain control. The patient was shifted to oral amoxicillin-clavulanate a month after the resolution of his pain, fever, and stabilization of inflammatory markers. The transhepatic percutaneous drain was left in place in order to allow for the formation of a chronic drainage tract, with hopes of localizing the perpetrating gallstones at its base.\nTwo months after discharge, the patient underwent a procedure for upsizing the drain in order to accommodate for a choledochoscope into the abscess cavity and the maturing sinus tract. He subsequently underwent an abscess cavity endoscopy, whereby a catheter and scope were advanced into the cavity for exploration. An abscess catheter study was performed through the existing catheter and showed no filling defects, after which the catheter was removed and an endoscope was inserted for direct visualization of the main cavity, as well as the two “fingers” branching out of the abscess cavity. Two small stones were expelled after flushing the main cavity multiple times. Following the expulsion of the gallstones, a replacement catheter was advanced into the cavity and a second catheter study was performed to rule out the possibility of any other stones. The patient tolerated this intervention well and had no further drainage from the intra-abdominal drain.\nFollowing a lack of drain output and resolution of symptoms after his last endoscopic procedure a month ago, the patient's intra-abdominal drain was removed. The patient is currently stable and is being followed as an outpatient to date.
A 22-year-old female with a pertinent medical history of DBA and chronic hepatic thrombosis (on anticoagulation with Eliquis) was admitted to the hospital for left upper back cellulitis. Unfortunately due to her anemia (baseline hemoglobin: 9 g/dl), she had required transfusions with packed red blood cells every three to four weeks and was also on daily chronic chelation therapy with deferasirox; her last transfusion had been four days before the admission. Her history was further complicated by multiple endocrinopathies including hypothyroidism, hypogonadism, growth hormone deficiency, and insulin-dependent diabetes mellitus. On admission, the patient's vital signs were normal. Her initial blood work included a metabolic panel (Table ) revealing hyperglycemia, hypocalcemia, high phosphorus, and transaminitis. Findings on a CT scan of the chest correlated with cellulitis overlying the left trapezius muscle for which empiric treatment with broad-spectrum IV antibiotics was initiated. She was subsequently started on subcutaneous weight-based insulin for hyperglycemia, calcium supplementation through calcium drip at 50 cc/hour of calcium gluconate for 24 hours; an electrocardiogram was done, which revealed normal sinus rhythm, and an ultrasound of the liver was performed, but it revealed no obvious abnormalities, deeming her transaminitis likely a result of chronic hepatic thrombosis combined with hepatic iron deposits from chronic transfusions.\nOn day two of her hospitalization, a rapid response was called on the patient as she fell backward from a sitting position and became unresponsive. She was placed on a monitor, which revealed sinus bradycardia with a heart rate of 36 bpm and hypotension with a blood pressure of 68/40 mmHg. Her pulse oximeter was 100% on a non-rebreather. TPA was held due to her being on anticoagulation. Within a few minutes, she became responsive but revealed poor motor response on physical exam with diminished reflexes. She was taken for a CT scan of the head along with a CT perfusion study of the head, but both revealed no acute findings. Blood work was performed, which included tests for thyroid-stimulating hormone (TSH), free thyroxine (FT4), T3, complete blood count (CBC), blood cultures, an 8-am cortisol level, and a metabolic profile. On the morning of hospital day three, the patient became unresponsive again. Vital signs were again significant for bradycardia and hypotension. She remained unresponsive for a few minutes and then quickly returned to baseline. Her physical exam persistently revealed diminished reflexes bilaterally. The patient was managed for possible seizure activity and was loaded with levetiracetam, and an electroencephalogram (EEG) was performed, which revealed normal results. Later in the day, her laboratory results for thyroid function came back and were significant for an elevated TSH and a suppressed FT4 (Table ).\nThe patient's history was taken again by the covering physician, and she revealed a history of hypothyroidism along with non-compliance with her home dose of 100 mcg of levothyroxine. On further review of her medical records, it was found that she was negative for anti-thyroid peroxidase antibody, and an ultrasound of the thyroid gland from last year was non-revealing of any thyroid nodules. She was seen by endocrinology, who attributed her condition to myxedema coma. The patient did not have a baseline 8-am cortisol level result, and hence she was given a stress dose of 100 mg of IV hydrocortisone and started on a standing dose of 100 mg hydrocortisone every eight hours. She was then given a bolus dose of 400 mcg IV levothyroxine, put back on her home dose of oral levothyroxine, and started on triiodothyronine 5 mcg twice daily. She improved clinically in the next three days and her morning cortisol drawn before treatment was initiated came out to be normal (16 mcg/dl). Her triiodothyronine, antiepileptic medication, and hydrocortisone were gradually tapered off. Her blood cultures came out positive for methicillin-sensitive Staphylococcus aureus and she completed a course of antibiotics for the underlying cellulitis. Given her studies were significant for hypocalcemia and elevated phosphorus, parathyroid hormone (PTH) was checked during her hospitalization. The PTH results showed a suppressed intact PTH (Table ) and confirmed our suspicion of hypoparathyroidism in her. After initially being placed on a drip of calcium gluconate, she was started on daily oral calcium carbonate, which was asked to continue on discharge. She was also started on calcitriol 0.5 mcg daily. She was advised on the importance of compliance with her medications and to have a close follow-up with her endocrinologist on discharge.
A 70-year-old man with antecedent of follicular lymphoma in complete remission presented at the Timone University Hospital (Marseille, France) in 2016 for a squamous cell carcinoma of the hypopharyngeal region. The patient categorically refused any treatment, including preservative surgery, radiotherapy, chemotherapy or supportive care.\nOne year later, he was addressed to our palliative care unit by the hand-surgery department after attempting suicide. The patient explained his action by the fear of suffering. No depressive state was diagnosed by our psychiatrists. Despite persistence fear of suffering, the patient rejected the idea of suicide because of his family, but still wanted to die and asks for assistance. Information on Claeys-Leonetti law was given, especially on assisted-suicide banishment and on the possibility to relieve suffering with adapted treatments.\nOne week after discharge, the patient was readmitted to our department for dyspnea and anxiety. Symptoms were managed by appropriate treatments (oxygen and low dose of midazolam in an anxiolytic purpose). Despite stabilisation, the patient was afraid of dying suffocated and asked for deep and continuous palliative sedation until death. Apart from the fear he expresses, the patient has no symptoms of anxiety, depression or pain after the introduction of appropriate treatments. On the other hand, he clearly states that he refuses to live again knowing that his death is approaching and that he is apprehensive of suffering. He says he wants to rush his death. For us, this is a request for assisted-suicide (active help from a third party for the administration of a lethal product) or euthanasia (act of a third party which intentionally provokes the death of another to put an end to his sufferings), rather than a real demand for deep and continuous sedation. It seems important to note that patient’s requests for deep and continuous sedation until death are not registered officially. The law does not even impose a written request. Thus, the request is most often made orally in the presence of several doctors and clinicians.\nIn order to try to objectify this request and therefore our answer, the patient’s request was examined and denied by palliative multidisciplinary board, in accordance with by the French Oncology Coordination Centre guidelines. This situation did not fulfil the criteria requested by Claeys-Leonetti law. Indeed, prognosis appeared not short term committed (no visible clinical progression of the disease, which commits for sure the short-term vital prognosis), symptoms were managed with appropriate treatments and no life-sustaining treatment arrest could lead to potential unbearable sufferings. Regarding the short-term criterion of life-threatening prognosis, the patient was offered to have a Computed Tomography (CT) scan to measure the progression of the disease. Indeed, no imaging had been performed for one year (time of diagnosis of recurrence). The patient refuses this proposal. The request for deep and continuous sedation was reiterated several times by the patient, who was still refusing any investigations to define the progression of his cancer and wanted parenteral hydration to be maintained. Daily, he questioned each caregiver about the rationale for the refusal of his request. How can the medical staff be sure that his prognosis is not short-term compromise? Why his psychological distress could not be considered as refractory? One week after refusing further investigation, the patient finally agrees to undergo a CT scan. Three days after the exam he dies peacefully, according to our team (no specific questionnaires or objective elements to judge the quality of death exists), of a not predictable respiratory distress certainly linked to the evolution of his cancer of the hypopharyngeal region without introduction of deep and continuous sedation, but with introduction of midazolam for anxiety. Opiates were not introduced because the patient was saying not being painful. The CT scan results, unknown at the time of death, reveal nothing conclusive (pulmonary metastases, but no lymph node involvement) and would have required additional analyzes.
An 80 year old man from the Tarai region of Nepal was brought to emergency with the chief complaint of falling from a swing after being pushed by his grandson 2 days prior. He complained of pain at the nape of his neck. The patient was neurologically intact. He was placed in a cervical collar and an urgent X-ray of the cervical spine revealed presence of spondylolysthesis of the axis with significant translation and angulation (\n). Magnetic resonance imaging (MRI) of the cervical spine revealed a type III hangman’s fracture with presence of pinching effect on the cord without any significant signal changes (\n).\nThe patient was an ex-army serviceman and was in good health with good Karnofsky performance score\n. There was no significant past medical or surgical illnesses. He had a habit of smoking marijuana previously. However, routine screening echocardiography revealed a cardiac ejection fraction of only 33%. Because the fracture was an unstable type III variant, the decision of surgical fixation was taken. The best option in such a situation would have been anterior cervical disectomy and Casper plate fixation thereby avoiding prolonged prone positioning. However such armamentarium for the procedure was not available with us. After explaining the disease condition, treatment options available and the risks involved, the patient was placed on minimal cervical traction so as to avoid the risk of iatrogenic hanging. We looked for the level of realignment that was possible with the guarded traction. Stringent care was taken to observe for features of over distraction. Repeat imaging showed good realignment and normal canal diameter. Therefore we decided to go for occipito-cervical fusion so as to minimize the anesthetic risk imposed to the patient from both anterior and posterior approaches. A DEXA scan for assessing bone density would have been justified prior to occipito-cervical fusion since such procedure would further lead to severe motion restriction in such an elderly spine. However such facility was not available to us. Intra-operatively there was fracture of the pars and the lamina of C2. Since there was no atlantoaxial dislocation, we opted for occipital and C1 and C3 lateral mass fixation. There is evidence of good results with short fixation of C1 and C3 only, but keeping in mind the risk of osteoporosis in this case, we wanted further anchorage from occipital fusion as well. Since there was good posterior realignment of the spinal lines after traction (\n) and intra-operatively, we choose the posterior approach only to minimize the added risk of the anterior approach. Lateral mass screws were placed in C1 and C3 (\n and\n). Bone graft harvested from iliac bone was placed in the C1 and C2 inter-space to further enhance the fusion process. The patient was started on dexamethasone (8 mg intravenously and then rapidly tapered off in the following 2 days). The patient was safely extubated. Neurological examination was normal. The patient was in complete bed rest for a week and then mobilized with support. A CT spine check after one week revealed good screw purchase (\n) and good reduction of fracture segment (\n). The patient was restricted to light weight bearing and was advised to keep the cervical collar for at least 6 weeks. The patient was started on calcium supplementation (tablet calcium 500 mg orally every 12 hours. The patient followed up in the outpatient department after 1.5 months walking on his own without any deficits.
A 62 years old man had been referred to our outpatient clinic because of recently diagnosed Lynch syndrome due to an MSH2 mutation. His family history was negative for any kind of cancer. He had been treated with curative intent for pancreatic cancer four years ago. The cancer was located in the pancreatic tail and histology showed a poorly differentiated adenocarcinoma of the pancreaticobiliary type, 6 centimetres in size, that extended into the spleen. The tumour could be radically resected; there were no positive lymph nodes. Two years later, he underwent a left-sided nephrectomy because of a low grade (grade I) urothelial cell carcinoma of the pyelum of the left kidney. Both malignancies showed loss of expression of MSH2, and subsequent genetic testing revealed a germ line mutation in the MSH2 gene (c.2090G>A p.Cys697Tyr in exon 13). In a functional test, this missense mutation shows mismatch repair deficiency and is therefore classified as a pathogenic mutation []. At his index colonoscopy, a small but suspect lesion was found in the ascending colon. There was a slight ulceration of the surface of a 7 × 7 mm Paris Is lesion and careful inspection using a Fujinon® Slim zoom video colonoscope (Eluxeo 700 series; 135 × maximum magnification) showed a Kudo Vn pit pattern, suggestive of an early invasive cancer (Fig. a, b). The colonoscopy was aborted and the different therapeutic options, as well as the pros and cons of each option, were discussed with the patient and his son. Besides the possibility of a segmental colectomy or subtotal colectomy, we also discussed the option of removing the lesion by eFTR. The patient consented with the option of endoscopic en bloc removal of the lesion and a colonoscopy under propofol sedation was scheduled to remove the lesion endoscopically. This procedure was carried out as follows: first the margins of the lesion were marked with a marking probe. Then the colonoscope was withdrawn and the Full-Thickness Resection Device (FTRD, Ovesco®) was mounted on the colonoscope. The colonoscope was re-inserted into the caecum and an FTRD® Grasper was used to draw the lesion into the cap of the eFTR system. When all circumferential markings were visible inside the cap, the over-the-scope clip (OTSC) was released and immediately afterwards the tissue within the OTSC was resected using the pre-mounted snare within the cap and the pure cut setting of the Erbe® coagulation system. The endoscope was withdrawn with the specimen in the cap and the specimen was subsequently pinned on a cork board for optimal pathological evaluation. After re-introducing the endoscope a nice full-thickness wound was seen with the OTSC in good position (Fig. c). Pathological examination showed a pT1 moderate to well-differentiated adenocarcinoma of the ascending colon with invasion into the submucosa, Kikuchi level sm1, with an invasive component of 0.3 cm, no lymphovascular invasion and a free resection margin of at least 2 mm (Fig. a H&E, Fig. b desmin immunohistochemistry). There was grade I tumour budding and loss of MSH2 staining. After discussion in the multidisciplinary team and shared decision making with the patient, we agreed not to opt for additional surgical resection, but for close follow-up by regular colonoscopy. Colonoscopy 12 months after the procedure showed no sign of residual or recurrent cancer and a CT scan, that was carried out in the follow-up of his urothelial call carcinoma, showed no sign of distant metastases 12 months after the endoscopic resection.
A 71-year-old man with progressive angina was found to have severe stenosis in the circumflex artery and complex CTO of the left anterior descending artery (, Supplementary ). Coronary artery bypass surgery was offered to the patient but declined. He underwent uncomplicated stenting of the circumflex artery, followed by staged CTO recanalization of the LAD. A guidewire was passed relatively easily across the occlusion into the true lumen of the distal LAD beyond the bifurcation. Antegrade wiring of the diagonal artery was difficult. An epicardial collateral from the distal right coronary artery was used to access the diagonal branch retrograde, and this wire was steered into the antegrade guide catheter and externalized (). Both the LAD and diagonal were dilated with 3.0 mm noncompliant balloons. The diagonal was stented into the proximal LAD, and the distal LAD was rewired. The distal LAD was dilated through the stent struts to allow passage of stents into the distal LAD (Culotte technique). The distal LAD was stented using four everolimus drug-eluting stents. The stents were postdilated with 3.0 mm noncompliant balloons in the diagonal and distal LAD and 4.0 mm noncompliant balloon in the proximal LAD. Within the distal-most stent in the distal LAD, the balloon had a persistent waist until an inflation pressure of 12 ATM. At that point, the balloon suddenly expanded. Angiography revealed contrast filling of the right ventricle (RV), with the appearance of one or two focal jets of contrast extravasation at the location of the rigid lesion in the distal LAD, consistent with iatrogenic LAD-RV fistula (, Supplementary ). Of note, the flow beyond the stents in the distal LAD was not seen, likely due to shunt flow and “coronary steal.” The patient remained hemodynamically stable and was asymptomatic, and it was elected to manage the fistula conservatively. Serial echocardiograms revealed only trace pericardial effusion. He was discharged in stable condition.\nApproximately three weeks after the CTO procedure, the patient was presented to the emergency department with chest pain and dyspnea. He described several episodes of “tearing” sensation in the chest. Blood pressure and heart rate were 123/69 mmHg and 64 beats per minute, respectively. A 12-lead ECG revealed inferior T wave inversion. Troponin I was elevated to 0.06 ng/mL. Urgent coronary angiography was performed, which revealed patent stents in the proximal LAD and diagonal branch. LAD-RV fistula appeared relatively unchanged compared to during the CTO procedure (). The right coronary artery was normal and provided a very faint collateral to the apical LAD. Left ventriculography in the left anterior oblique projection revealed no evidence of a ventricular septal defect. The patient developed profound hypotension of unclear etiology during angiography. A right heart catheterization revealed normal filling pressures, normal cardiac output and ratio of pulmonic to systemic flow (Qp:Qs) 1.7. A transthoracic echocardiogram revealed preserved ejection fraction and normal left ventricular wall motion with turbulent flow signals at the LV and RV apex throughout the cardiac cycle (Supplementary ). There was no pericardial effusion. The patient was transferred to the cardiac intensive care unit in stable condition.\nHeart team evaluation was undertaken, and it was decided to perform coil embolization of the distal LAD. A standard 6 French left coronary guide catheter was used to advance a ProGreat (Terumo Medical Corp., Somerset, New Jersey) guidewire and 2.8 French catheter into the distal LAD. This was used to deploy two Ruby (Penumbra, Inc., Alameda, California) coils at the distal end of the stent.\nThere was still persistent shunt flow, so two Tornado (Cook Medical, Bloomington, Indiana) coils were subsequently deployed. Angiogram of the LAD confirmed complete cessation of flow into the distal LAD and absence of shunt flow (, Supplementary ). Hemodynamics postprocedure demonstrated a 20 mmHg increase in systolic blood pressure and normalization of Qp:Qs. A follow-up echocardiogram revealed the obliteration of apical shunt flow, normal left ventricular ejection fraction, and no left ventricular wall motion abnormalities. Creatine kinase eight-hour postprocedure was normal. The patient was discharged home in stable condition. At follow-up, one month later, the patient remained asymptomatic.
A 32-year-old male patient reported at the dental office with a chief complaint of receding gums and visibility of root portion of teeth with generalized sensitivity to hot and cold food stuff. The patient was healthy with no systemic disease and no adverse habit such as smoking. The patient had Class II recession with cervical abrasions in relation to (irt) 14.15 and 16 and Grade II furcation irt 16. Recession irt 14 and 15 was 5 mm, and irt 16, it was 7 mm, there were no pockets, all the measurements were done with UNC 15 probe. After 1 month of completion of phase I therapy, abrasion was filled with glass ionomer cement irt 14.15 and 16. The use of the pedicled buccal fat pad flap was planned to cover the recession and augment the final width of the keratinized mucosa coronal to recession.\nBefore starting with the surgical procedure, surgical area was anesthetized, glass ionomer cement (GIC) restoration was planed [], and a full-thickness flap was raised []. There are three approaches to harvest BFP. A horizontal incision through the mucosa on the buccal aspect of the vestibule in the molar region will readily expose the BFP []; second approach is to give a vertical mucosal incision slightly lateral to the anterior margin of the ascending ramus, this will result in a forward bulging of the fat pad []; a third approach to the fat pad is elevation of a mucoperiosteal flap in the molar region on the lateral aspect of the maxillary alveolar process and then a horizontal incision of the periosteum at the level of the buccal sulcus [].[] The choice of exposure depends on the requirements of the specific situation in which the flap is used. For this clinical procedure, third approach was used. Using blunt dissection through the buccinator and loose surrounding fascia, BFP was exposed into the mouth. The body of the BFP and the buccal extension were gently mobilized by blunt dissection, taking care not to disrupt the delicate capsule and vascular plexus and to preserve as wide a base as possible []. BFP was easily spread over the maxillary roots of 14, 15, and 16, 1–2 mm apical to cementoenamel junction (CEJ), acknowledging possible wound contraction. BFP was first stabilized using horizontal mattress suture, over which mucogingival flap was placed which was not covering BFP completely and was sutured with Vicryl 3-0 [] (Polyglactin 910, Ethicon, Johnson and Johnson).\nPatient was prescribed with capsule amoxicillin 500 mg TDS for 5 days, tablet ibuprofen 300 mg + paracetamol 325 mg SOS, chimeral forte, chlorhexidine (0.12%) mouthwash twice daily for 2 weeks. The patient was instructed to eat a soft diet. Among other oral hygiene instructions and methods, the patient was asked to use a soft brush and brush very gently over the surgical area even after suture removal and not to exert too much pressure during brushing. His brushing habit was changed to modified bass method, and every time, he would come for check he was asked to bring his brush along so that his method of brushing could be checked. He was asked to use floss and rinse mouth with warm saline rinse 2–3 times daily. Furthermore, patient's scaling was done at every 6 months.
A 41-year-old man was accidently detected with a mass without tenderness on the right lobe of the thyroid. B-ultrasound showed that the morphology and echo of thyroid were abnormal though without nodules. Magnetic resonance imaging (MRI) showed significantly enlarged right lobe of the thyroid with a clear boundary and uneven density of mass, partly involving the isthmus of the thyroid, which was suspected as being thyroid adenoma (Fig. ). The thyroid function testing and emission computed tomography (ECT) scan were normal. Physical examination showed the following: right lobe of the thyroid showed no-fixed II degree swelling with no tender and obvious nodules. The liver and spleen were normal. Bone marrow biopsy was normal and the percentage of eosinophils in peripheral blood was 2.97% (normal range, 0.5%–5%). The patient had a history of hypertension for 7 years and hepatitis for 10 years. There was no family history of thyroid diseases and radiation ray contact. The patient underwent a right thyroid resection and dissection considered the possibility of thyroid adenoma. However, the pathology after surgery indicated LCH because immunohistochemistry staining confirmed CD1a (+), S100 (+), CD68 (+), Valentine (+), and thyroglobulin (+). The thyroid lesion gradually improved after the patient further received radiotherapy of 16 regimens combined with interleukin-2 therapy. The patient regularly monitored the function of thyroid and began to take 50 μg Euthyrox (levothyroxine) per day since being diagnosed with hypothyroidism 3 years after subtotal thyroidectomy. In 2007, the patient was again admitted to our hospital with the complaint of thirst and polyuria for several months. Urine osmolality was obviously lower than blood osmolality and urine specific gravity was less than 1.005. Further, fluid restriction test was positive and MRI of the pituitary revealed loss of the pituitary posterior lobe signal and the pituitary stalk was slightly thicker (Fig. ). We considered the diagnosis of central diabetes insipidus (DI) and the patient received 3 tablets of desmopressin acetate (0.3 mg/day) with improvement of the symptoms. However, the thyroid and total bone ECT scan were both normal.\nIn 2008, the patient felt pain and distension in the upper abdominal area and was admitted to the hospital. Abdominal computed tomography (CT) showed diffuse fine nodules on the right hepatic with partly fusion. Immunohistochemical staining showed there were Langerhans cells with ovoid to reniform nuclei with grooves by percutaneous liver biopsy (Fig. ), meanwhile in which the histiocyte-like cells S-100 (+) and CD1a (+) diagnosed as liver Langerhans cell. Liver function testing revealed that alkaline phosphatase (ALP) was 233 U/L (normal range, 40–160 U/L), gamma-glutamyl transaminase was 220 U/L (normal range, <50 U/L) and total bilirubin, liver transaminase and alpha-fetoprotein were in the normal range. Further, the patient received chemotherapy of CHOP regime (CTX1350 mg, VCR2 mg, prednisone 100 mg for 5 days,) and radiation therapy of 11 regimens including 16.5 Gy dose. Surprisingly, we found that the hepatic local region in the patient had remarkably improved and finally was normal during 5 years of follow-up. There was no lesion found in the right lobe during the T1-weighted MR images in 2012 and enhanced MR images of the liver in 2017 (Fig. ). We considered the patient in clinical remission while the examination of thyroid and pituitary was favorable.
A 56-year-old male presented to a medical emergency department with complaints of high-grade fever, altered behavior in the form of irrelevant talk, agitation, and irritability with fluctuating orientation for 2 days.\nThe patient was a known case of undifferentiated schizophrenia as per International Classification of Diseases, 10th edition since past 24 years and was on regular treatment with good compliance since the start of treatment. Initial symptoms of the patient included suspicion against family members that they will harm him, muttering and gesticulating to self, aggressive and abusive behavior, reduced sleep, and disinhibited behavior for which he was treated with electroconvulsive therapy and psychotropic medication during initial period. The patient improved in a few weeks and continued the treatment as advised by the psychiatrist. The patient had aggravation of symptoms while on medication multiple times mostly without any precipitating factors during the course of illness. Since 24 years, the patient had been prescribed various antipsychotic medications including trifluperazine, risperidone, and olanzapine in adequate doses and for adequate duration. Quetiapine was prescribed in low doses for agitation whenever required as per the response. In spite of being compliant to the treatment, the patient never improved to a premorbid level and some residual symptoms would always remain. The patient had been admitted under psychiatrist's care for three times due to aggravation of symptoms despite being compliant in the past few years before he was considered to be a case of resistant schizophrenia and prescribed tablet clozapine with normal baseline CBC and weekly counts were advised. Dose was gradually increased to reach up to 200 mg in 2 weeks along with continuation of risperidone 4 mg in divided doses. Patient showed partial improvement in symptoms after 2 weeks of treatment and improvement was increasing slowly.\nWithin 30 days of starting clozapine, the patient presented to the casualty with above symptoms suggestive of some organicity. The patient also had urinary complaints (urgency and frequency). Neuroimaging revealed no abnormality, but urine examination showed plenty of pus cells. Urine and blood culture revealed infection with Klebsiella pneumonia with signs suggestive of cystitis on ultrasonography. His serum potassium levels were low (2.57 mEq/L) with deranged kidney and liver functions. Patients' CBC revealed low hemoglobin of 7.6 g% and TLC of 400/mm3 (differential count could not be done due to low leukocyte count) with 3.47 mil/ul RBCs. His platelet count was normal (3.42 lakh/mm3). Bone marrow biopsy was suggestive of “depressed granulopoiesis and erythropoiesis with hypoplastic anemia.” Patient was negative for antinuclear antibodies and his cardiac functioning in echocardiography was within normal limits. There were no abnormalities in other blood investigations. The patient was treated in the Intensive Care Unit (ICU) with antibiotic drugs, adequate hydration, supportive treatment, and other measures. Clozapine was stopped immediately, risperidone dose was increased to 6 mg (divided doses), and aripiprazole was added in 5 mg dose that was later increased. Patients' blood investigation charting was done, and there was improving trend in blood counts. Slowly patients' hemoglobin increased to 9.4 g/dl at the end of 3 weeks. TLC found increased in subsequent testing, and at the end of 3 weeks, it was 6900/mm3 with absolute neutrophil count (ANC) 5796/mm3. RBC count increased to 4.3 mil/ul at the time of discharge. Serum potassium level became normal within few days of admission. There was no growth on urine and blood culture on repeat testing.\nWhen the patient was discharged, dose of aripiprazole was increased to 30 mg in the next few days along with risperidone 6 mg. The patient showed aggravation of symptoms within a week of stopping clozapine but again reported improvement after increasing the dose of aripiprazole.
A 50-year-old Thai male presented with three episodes of generalized seizures and right-sided hemiparesis for 6 h before arrival. He had no previous seizures. He had a history of well-controlled diabetes mellitus and hypertension for 20 years and took metformin 1000 mg/day and diltiazem 60 mg/day. His past medical history revealed progressive slowness in thinking and walking, memory impairment, sleep-wake disturbance and mood disorder, which had slowly progressed for the past 20 years; however, it had rapidly worsened during last year of his life. He was diagnosed with organic mood disorder 5 years before this presentation and treated with risperidone 0.5 mg/day, sertraline 50 mg/day, and trihexyphenidyl 1 mg/day. Even with this treatment, his symptoms had been progressively worsening for the past 1 year to the point that he could not perform daily living activities, such as taking the correct medications. He was the fifth of seven children. His sister had a history of unexplained hearing loss, cognitive decline, and slowness of movement starting at the age of 20. When she was 40 years old, she developed visual and auditory hallucinations as well as recurrent transient ischaemic attacks with full recovery. The patient’s father and mother died at the ages of 70 and 78, respectively, and had no history of cognitive impairment or stroke. A mental status examination showed a good level of consciousness; however, he was mute and slow to respond to commands. A motor examination showed right-sided weakness (grade 2/5 for arm and grade 0/5 for leg) and generalized hyperreflexia except for right leg hyporeflexia and no sensory impairment. There was also mild right facial weakness. A CT of the brain showed diffuse white matter abnormalities, old multiple lacunar infarctions in the bilateral basal ganglia, thalamus, and left pons. The initial diagnosis was acute ischaemic stroke with seizures. He was prescribed 300 mg/day of aspirin and usual stroke care. Phenytoin was prescribed for seizure control. An MRI of the brain was performed on day 12 after admission. The results showed acute infarction of the left pons (Fig. ), several old lacunar infarcts surrounded by minimal gliosis in the bilateral putamen and thalamus as well as few scattered small, old infarcts surrounded by minimal gliosis in the bilateral frontal-parietal periventricular white matter without anterior temporal lobe lesion (Fig. a, b, and c). Surprisingly, a large number of microbleeds were found throughout the brain (Fig. d, e and f). The total numbers of cerebral microbleeds was 214 and 136 in lobar areas according to the Microbleeds Anatomical Rating Scale (MARS) []. After a comprehensive review of vascular risk factors, his HbA1C was 5.5%, and his serum LDL was 79 mg/dL. His blood pressure was well-controlled. None of these risk factors explained his symptoms and MRI findings. He had a history of unexplained cognitive impairment and mood disorder. In addition, his sister had a history of cognitive decline, psychiatric symptoms and transient ischaemic attack; therefore, a genetic condition, such as CADASIL, was suspected and confirmed by molecular genetic testing, which revealed a homozygous known pathologic variant, c.1672C > T (p. Arg558Cys), in the NOTCH3 gene. His clinical symptoms deteriorated, and he died of tracheobronchitis with secretion obstruction.
The patient was a 25-year-old male mine worker, who suffered a multiorgan injury when he was buried by falling rocks. On admission to the Intensive Care Unit, he was in a severe condition and exhibited symptoms of cardiopulmonary failure. The patient was intubated – mechanical ventilation was introduced along with pharmacological therapy with catecholamines and antibiotics. After a chest radiogram revealed right-sided pneumothorax and costal fractures (ribs II–V), a drain was introduced into the right pleura. The patient’s crushed right foot was treated surgically (amputation at Chopart’s joint), while the fractured medial condyle of the femur was treated conservatively. Due to the observed incidents of reduced respiratory fitness parameters, computed tomography of the chest was performed on the 3rd day after the trauma. The examination visualized irregularities in the outline of the left main bronchus and a consolidation of gas bubbles in this area, which prompted the suspicion of bronchial injury (, present a tomographic reconstruction of the respiratory tract).\nDiagnostic investigation was supplemented with bronchofiberoscopic examination, revealing hyperemia and massive edema of the mucosa in the proximal segment of the left main bronchus, immediately next to the tracheal bifurcation. Consequently, the attempt to reach the left main bronchus with the endoscope was unsuccessful.\nThe patient was referred for further treatment at the Thoracic Surgery Center in Wrocław with a suspicion of airway disruption. A decision was made to perform urgent surgical treatment. Left posterolateral thoracotomy was performed. Typical post-traumatic lesions were found intraoperatively: a pleural hematoma (approx. 500 ml in volume) and extensive hematomas in the tissues of the thoracic integuments. A small amount of air was found in the mediastinum. Prepare of the mediastinal tissues revealed the completely amputated left main bronchus; the whole length of the membranous part of the bronchus was ruptured. The bronchial membrane was sutured longitudinally. Subsequently the detached bronchus was anastomosed with the trachea, and the anastomosis was wrapped with an intercostal muscle flap.\nThe patient’s hospitalization continued at the Intensive Care Unit of the Lower Silesian Lung Diseases Center. The dosage of catecholamines and sedatives was gradually reduced. On the 3rd day, the drain from the left pleura was removed; 3 days later, the right pleural drain was removed as well. On the 6th day after the thoracotomy, the airway was reevaluated with an endoscope, revealing stenosis at the tracheobronchial anastomosis; the endoscope could not pass through this location. Additionally, a large amount of mucous secretion, which filled the whole bronchial tree, was evacuated. A decision was made to wake and extubate the patient. Consequently, the secretion accumulated again, resulting in a repeated bronchoaspiration after less than 20 h. It was deemed necessary to perform tracheotomy. After the patient’s cardiopulmonary parameters stabilized on the 14th postoperative day, the patient was referred for further treatment on the Thoracic Surgery Department. The treatment consisted in rehabilitation and fitness improvement. The patient was discharged from the Center on the 18th postoperative day. After less than 6 weeks, the patient was readmitted with symptoms of left main bronchus occlusion. For the past several days, the patient had manifested symptoms of airway infection: he was fever and he had dyspnea. An out-patient computed tomography (CT) scan demonstrated anastomotic stenosis (), but lung aeration was maintained ().\nA chest radiogram () taken on the day of readmission (7 days after the CT exam) revealed complete atelectasis of the left lung.\nBronchofiberoscopic examination showed that the lumen of the left main bronchus was very narrow: its diameter did not exceed 3 mm. An attempt was made to perform mechanical dilatation, but without success.\nThe diagnostic investigation was supplemented with chest CT, revealing obturation of the left bronchial tree () and a complete lung atelectasis (–).\nA decision was made to perform surgical intervention. Thoracotomy was repeated with sleeve resection of the stenosed left main bronchus (length: 1–1.5 cm), and the bronchus was anastomosed with the trachea (the posterior wall with single sutures, and the anterior wall with a continuous suture). shows the chest radiogram obtained on the 1st postoperative day, while presents a radiogram from the 6th postoperative day.\nFrom the day of readmission, the patient was administered broad-spectrum antibiotic treatment. On the third postoperative day, the drain placed in the left pleura was removed. The patient was discharged on the 7th postoperative day. He continued to be monitored as an outpatient – he attended follow-up visits, during which the site of anastomosis was evaluated with an endoscope. The first such examination took place 2 weeks after discharge, demonstrating permeability of the anastomotic site and no signs of inflammation. Subsequent follow-up examinations took place every three months until the end of February, 2015. The final examination showed a stable endoscopic image with satisfactory width of the left main bronchial lumen.
A 33-year-old male with human immunodeficiency virus (HIV), not on highly active antiretroviral therapy (HAART) for the last two years, presented to an outside emergency department with a three-week history of subjective fever, arthralgias, myalgias, and a diffuse painful papular upper body rash. The rash had progressed from his trunk to involve his face, arms, and upper legs. He reported painless genital lesions preceding the diffuse rash, as well as two unprotected sexual encounters approximately one month prior. The patient took no home medications and had no known medication allergies. In the emergency department, he was found to have a temperature of 100.9°F, a heart rate of 130 beats per minute, and a respiratory rate of 22 breaths per minute. Urinalysis was not suggestive of infection, and chest radiograph demonstrated no infiltrate. Blood and urine cultures were obtained. He was given 1 L bolus of intravenous fluids and 1 g of ceftriaxone intravenously for presumed sepsis. He was subsequently transferred to our institution for further workup and management.\nUpon arrival to our emergency department six hours later, the patient was febrile to 102.8°F with a heart rate of 161 beats per minute and systolic blood pressure initially 89 mmHg. Physical exam was remarkable for normal mentation, tachycardia but no murmurs, and clear lung fields bilaterally. There were erythematous scaly papular and plaque-like lesions over the face, back, chest, arms, abdomen, and upper legs (Figures , ). The rash spared the palms and soles. He also had an erythematous papule on the mucosa of the lower lip. The patient remained hypotensive, with a blood pressure of 82/53 mmHg, despite 3 L of normal saline. A central venous catheter was placed, and a norepinephrine drip was started. The patient also received empiric antibiotic treatment with vancomycin and cefepime for presumed septic shock. The patient was admitted to the medical intensive care unit, where he required less than 24 hours of vasopressor administration with norepinephrine to maintain adequate blood pressure.\nBlood and urine cultures obtained at the outside hospital prior to the first antibiotic dose were negative for bacterial growth. Broad-spectrum antibiotics were discontinued once cultures showed no growth for over 48 hours and the patient remained clinically stable. Blood cultures, fungal cultures, acid-fast bacilli smear and cultures, cerebrospinal fluid (CSF) cultures, cryptococcal serum and CSF antigens, Histoplasma galactomannan urine antigen, Neisseria gonorrhea polymerase chain reaction (PCR), and Chlamydia trachomatis PCR obtained at our facility were all negative. CSF studies demonstrated no red blood cells, no white blood cells, glucose 53 mg/dL (reference range 40-70 mg/dL), and protein 49.5 mg/dL (reference range 10-45 mg/dL), which was not thought to be consistent with aseptic meningitis. Rapid plasma reagin (RPR) was positive, and syphilis serum titer was 1:256 dL. CSF RPR was negative, and CSF Treponema pallidum IgG by immunofluorescence assay was nonreactive. The patient was also noted to have a CD4 T-cell count of 162/mm3 (reference range 338 to 1448 per mm3) and HIV-1 ribonucleic acid (RNA) of 456,000 copies/mL. Dermatology was consulted and deferred skin biopsy as exam findings were clinically consistent with secondary syphilis. The patient was treated with 2.4 million units of intramuscular benzathine penicillin. The remainder of his hospital course was uncomplicated, and he was discharged home after three days with close follow-up with Infectious Diseases with plans to resume HAART as an outpatient.
A 76-year-old man with a medical history of hypertension, atrial fibrillation, type 2 diabetes mellitus, and mild chronic renal impairment with a single functional kidney was referred to our hospital because of a Crawford type 3 thoracoabdominal aneurysm with a diameter of 6.4 cm. There was a preexisting occlusion of the left renal artery. Baseline serum creatinine was 1.81 mg/dL. The estimated glomerular filtration rate (eGFR) using the modified diet and renal disease study equation (MDRD) was 37 mL/min/1.73 m2.\nEndovascular repair was performed with an aortic branch graft including side branches for the celiac trunk, superior mesenteric artery (SMA), and the right renal artery. Covered stents were placed through the side branches in the target vessels and these were relined with nitinol self-expanding stents. The procedure was complicated by diplopia caused by occipital infarction, despite intraoperative heparinisation and immediate postoperative readministration of anticoagulant therapy (coumarin and clopidogrel). Postoperative serum creatinine remained unchanged. Three weeks after surgery, clopidogrel was discontinued because of side effects. Follow-up CT-angiography 6 weeks after discharge showed adequate position of the aortic branch graft (), patency of the visceral side branches, and target vessels without kinking of the (covered) stents. However, a mass in the left mamma was found. Additional diagnostic tests showed an invasive ductal carcinoma. Subsequently the patient was planned for mastectomy with sentinel node procedure. Anticoagulant therapy was discontinued 3 days before surgery. Upon admission for the planned surgery, the patient reported anuria for approximately 12 hours and right flank pain that had started early that morning. Blood test showed an increase in serum creatinine to 5.37 mg/dL. Ultrasound investigation showed an occlusion of the stents in the right renal artery side branch and minimal venous flow suggesting acute ischemic kidney injury. The side branches to the celiac trunk and SMA were patent without presence of thrombus. Systemic heparinisation was started immediately and the mastectomy was cancelled. Angiography was performed via left brachial access and occlusion of the right renal artery side branch was confirmed (). Via direct transcatheter injection, a single bolus of 250.000 IE urokinase was administered into the right renal side branch and an EKOS thrombolysis catheter (EKOS Endowave system; EKOS Corporation, Bothell, WA, USA) with a working length of 6 cm was placed in the occluded renal side branch (). Intra-arterial infusion with 100.000 IU/h of urokinase was started together with 10.000 IU/24 h of heparin via the side port of the sheath. Thrombolysis was started 19 hours after the beginning of anuria. Angiographic control 4 hours later showed some dissolution of the thrombus and thrombolysis was continued. Approximately 8 hours after the start of thrombolysis, diuresis recovered and right flank pain diminished. Second angiographic control 18 hours after beginning of therapy demonstrated complete lysis of the thrombus with a patent side branch of the aortic stent graft and adequate flow through the renal artery (), after which thrombolysis was stopped. Subsequently, anticoagulation with low molecular weight heparin was started. The serum creatinine increased to a maximum of 10.44 mg/dL on day 3 after procedure, requiring dialysis on days 3 and 5. Hereafter, there was partial renal function recovery (eGFR (MDRD) of 10 mL/min/1.73 m2), after which mastectomy was performed without cessation of anticoagulant therapy. Postoperative coumarin was started and the patient was discharged on day 9, after an uneventful postoperative course. Renal function continued to improve, and one month after thrombolysis, renal function approached baseline function (creatinine 2.33 mg/dL; eGFR (MDRD) 27 mL/min/1.73 m2). One year after surgery, the patient was doing well and renal function was stable and the same as before the aneurysm repair. All side branches of the aortic branch graft were patent and the aneurysm was shrinking.
An 83-year-old female was directed to the emergency department by her primary care physician three weeks after falling on her left flank for a CT of her abdomen. This CT showed concern for bile duct dilatation, but as she was asymptomatic, she was discharged home with followup. Several days later an endoscopic retrograde cholangiopancreatography (ERCP) was performed, and a large hypopharyngeal mass was incidentally noted. A biopsy was taken at the time of her GI procedure. The patient was subsequently admitted, and ENT was consulted the following day. Her past medical history includes hypertension, congestive heart failure, stroke, and history of cervical metastasis from unknown primary that was treated with radiation therapy 21 years ago.\nThis patient was originally diagnosed with a T0N1M0 cancer of unknown primary in 1987. A primary site was not identified on CT of the head, neck, chest, abdomen, and pelvis as well as quadruple endoscopy. Fine-needle aspiration of the neck mass was positive for a poorly differentiated carcinoma. The lesion was 1–1.5 cm in size in the right jugular digastric region just below the angle of the mandible. She received definitive radiation therapy to the head and neck region at Gershenson Radiation Oncology Center, Detroit, MI, from February 11th, 1987 to May 5th, 1987. She received treatment to the neck node and potential sites of primary via left and right lateral opposing ports to 3600 rads by means of Cobalt 60. After this dose, off-cord treatment was delivered to the left and right anterior neck to an additional 1440 rads. Finally, the site of cervical adenopathy was given an additional boost of 900 rads with 10 MV electrons. The lateral supraclavicular field and posterior neck were also treated. On review of the treatment portals and prescribed therapy, the patient received a cumulative dose of 5940 rads to the anterior neck 20 years prior. The anterior neck irradiated included epiglottis and larynx.\nOn further questioning, patient admitted to one-month history of dysphagia to solids and liquids. She reported a globus-type sensation as well as discomfort when eating. She also had intermittent hot potato voice and coughing episodes. She has had a twenty-pound weight loss and was more easily fatigued. However, she denied any fevers, night sweats, shortness of breath, or otalgia. The patient is a former heavy tobacco user, about a pack a day to a third of a pack a day. She denied any alcohol or drug use. On physical examination, a large and fungating epiglottic mass extending into the base of the tongue was noted on fiberoptic nasopharyngoscopy. Neck examination revealed no obvious lymphadenopathy.\nCT scan of the neck and thorax with IV contrast performed the same day demonstrated a heterogeneous enhancing epiglottic mass which involves the preepiglottic space and extends inferiorly along the mucosal pharyngeal space more on the right involving the laryngeal surface of the aryepiglottic folds. This mass measures 2 × 1.8 × 1.6 cm. The mass extends to involve the false cords and possibly the superior aspect of the right true vocal cord. There is no cartilage invasion, retrocricoid invasion, or subglottic extension. No significant lymphadenopathy.\nThe original biopsy at the time of ERCP returned necrotic debris with focal squamous features and cellular atypia. Numerous clusters of bacterial and fungal elements were consistent with candida. The patient was then taken back to the operating room for triple endoscopy, tracheostomy, and biopsies. Direct laryngoscopy revealed a friable, exophytic mass involving the base of tongue, vallecula, and the oral surface of the epiglottis as well as the laryngeal surface of the epiglottis down to the aryepiglottic fold but does not involve the false vocal cord, arytenoid, piriform sinus, or the postcricoid space. The mass was biopsied. Direct bronchoscopy was negative. Direct esophagoscopy revealed a duodenal mass which was biopsied. A PEG tube was placed. The pathology returned predominantly fibrinopurulent exudates and necrotic debris with bacterial and fungal organisms. Because of the negative biopsy, the patient was once again taken to the operating room for biopsy. This was biopsied several times and sent off for frozen section. Frozen section was negative. We then sent off for another set of biopsy specimens. Again this was negative. We sent a third set of biopsy specimens, and this was deferred for permanent. Pathology returned benign squamous mucosa with marked subepithelial inflammation, florid granulation tissue with prominent endothelial proliferation and extensive necrosis. Sections show extensive necrosis with prominent exuberant granulation tissue with spindle-cell proliferation and a chronic inflammatory infiltrate. The spindle cells do not show much cytologic atypia, and no mitotic figures were noted. Some of the spindle cells appear to form vascular channels. The differential diagnosis included florid granulation tissue formation, a poorly differentiated spindle-celled carcinoma or a spindle-cell sarcoma. Immunohistochemical stains were performed and included actin (smooth muscle marker), CD31 (endothelial marker), P63, CAM5.2, and AE1/AE3 epithelial markers. Sections showed the spindle cells to stain strongly with the CD31, while the other stains were essentially negative in the spindle cell area. This is consistent with a florid endothelial proliferation or granulation tissue formation. No unequivocal malignancy is seen in this specimen.\nAfter three negative and inconclusive biopsies, patient's case was discussed in the Multidisciplinary Head and Neck Tumor Board, and a recommendation for a larger and more aggressive biopsy was recommended. Infectious disease has also been consulted regarding this patient, and they requested tissue for cultures. After extensive discussion with the patient and her family, patient was taken back to OR for extensive debulking of the epiglottic mass. The final pathologic diagnosis was angiosarcoma. The biopsy shows a highly cellular neoplasm with necrosis. Tumor cells are positive for CD31, CD34, and negative for Factor VIII, cytokeratin AE1/AE3 and CK903. The morphology and immunoprofile are diagnostic of epithelioid angiosarcoma. Outside consultation from the University of Michigan ultimately agreed with the above diagnosis. The patient then underwent a total laryngectomy and cricopharyngeal myotomy with tracheoesophageal puncture. During the surgery, 2 primary foci involving the epiglottis and base of the tongue were noted (). The final pathology confirmed the presence of multifocality with a 2.2 cm lesion in the epiglottis and a 1.5 cm lesion in the base of tongue. All margins were negative, and the pathology showed high-grade epitheliod angiosarcoma of the epiglottis (Figures and ). Unfortunately, the patient developed several complications, including fistula, syncope, seizure, and pneumonia, following the surgery, and discussion of adjuvant chemoradiation therapy was delayed. Patient was doing well until approximately 10 months after the surgery when she was noted to have a purplish lesion in the right tonsil area. CT scan of neck and chest was negative. Examination under anesthesia revealed 2 foci of lesion, one in the right tonsil and one in the left pharynx. Biopsy showed angiosarcoma. Patient was taken back to the OR for right radical tonsillectomy and L partial pharyngectomy. Patient did well postoperatively and was started on adjuvant chemotherapy consisting of taxotere. Approximately 6 months after the last surgery, patient was again noted to have purplish lesion in the left base of tongue region. She was experiencing significant side effects from the chemotherapy, and family decided to put her in hospice. She died 2 months later.
A 64-year-old woman presented to a local doctor in 2000 with blurred vision in her right eye. She was diagnosed with chronic iridocyclitis and treated with topical corticosteroids with limited improvements. More detailed examinations were not performed at that time. In 2003, she consulted a neurologist for depression, and thereafter was diagnosed with CNS lymphoma by brain MRI. On presentation, best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/20 in the left eye, and intraocular pressure was normal. A slit-lamp examination showed KPs and inflammatory cells (2+) in the anterior chamber of the right eye. A fundus examination of the right eye revealed vitreous opacities (3+). Slit-lamp and fundus examinations of the left eye showed no abnormalities. Since the identification of lymphoma cells in the vitreous is required for a diagnosis of PVRL, we performed diagnostic vitrectomy. A vitreous biopsy sample indicated increased IL-10 levels (379 ng/mL) and an elevated IL-10 to IL-6 (12.6 pg/mL) ratio. A cytological analysis also showed large atypical lymphoid cells, resulting in DLBCL. She was diagnosed with PVRL with CNS involvement and started treatment. She underwent one course of intravenous HD MTX chemotherapy (3.5 g/m2) and radiotherapy to the right eye of 40Gy. Brain recurrence has not been observed since then.\nBilateral involvement was detected during the observation period. KPs and anterior chamber cells are also observed in the left eye. She had been repeatedly treated with a dose of 400 μg of intravitreal MTX and a dose of 1 mg of intravitreal rituximab for recurrent intraocular lesions between 2004 and 2011: intravitreal MTX; 7 times for the right eye/3 times for the left eye: intravitreal rituximab; 7 times for the right eye. The MTX injections were administered using a 30-gauge needle after application of a topical anesthesia and 5 % povidone iodine disinfection in the superior temporal quadrant 3.5 mm posterior to the limbus. Intravitreal rituximab was also administered to the recurrence from 2008 to 2009 and was temporally effective; however, the iridocyclitis as the side effects of injection was developed each time. Therefore, intravitreal rituximab was discontinued. In 2012, she presented at our hospital with blurred vision in her right eye. BCVA in her right eye was 20/250. A slit-lamp examination of the right eye showed a 2-mm high viscous pseudohypopyon, anterior chamber cells (3+), and flare (2+) (Fig. ). KPs were not observed. A fundus examination of the right eye showed (3+) strong vitreous opacities (Fig. ); however, subretinal invasion was not observed. There was no abnormal finding in her left eye at the time of right eye recurrence. Anterior chamber biopsy revealed the presence of atypical lymphocytes, indicating the recurrence of DLBCL (Fig. ). Intravitreal MTX was initiated. One week later, pseudohypopyon completely disappeared, while anterior chamber cells (1+) remained (Fig. ). Vitreous opacities also disappeared. Symptoms resolved completely within a few months by the sequential treatments.\nThe recurrence of PVRL with pseudohypopyon appeared 14, 15, and 18 months after the first appearance of pseudohypopyon (Fig. ). An intravitreal MTX was indicated for the management of each recurrence, resulting in the resolution of symptoms. Moreover, the recurrence of PVRL with KPs (Fig. ) and subretinal invasion (Fig. ) were also noted (5 times for KPs/1 time for subretinal invasion). Intravitreal MTX was also an effective treatment for recurrence (Fig. ). Recurrence with pseudohypopyon was not simultaneously observed with KPs, vitreous opacities, or subretinal invasion. In May 2015, there had been no recurrence for 6 months. Visual acuity remained at 20/50 in her right eye and 20/20 in her left eye. The subsequent clinical course of left eye was uneventful. No CNS involvement was noted during the observation period.
A 22-year-old male patient came to the orthopedic and rehabilitation clinics at our hospital with right equinovarus and gait disturbance. He had a history of hypoxic brain damage caused by the umbilical cord wrapping around fetal neck at birth, and was diagnosed with CP at the age of 1 year. He was experiencing severe dystonia in his neck and bilateral upper extremities; the Burke-Fahn-Marsden dystonia rating scale (DRS) showed 55 points on the dystonia movement scale, and 10 points on the disability scale.\nOn manual muscle test, the left extremities were within the normal limit, but the right upper and lower extremities only showed grade 3 (Fair). He showed right hip joint flexion contracture and right ankle plantar flexion contracture on the range of motion test. In terms of spasticity, his right upper and lower extremities scored grade 2 on the modified Ashworth scale (MAS). He had 78 points on the modified Barthel index (MBI), and 105 points on the functional independence measure (FIM). He showed asymmetry in the lower extremities and equinovarus in the right ankle on a plain X-ray, and 60 degrees right femoral anteversion, 15.3 degrees left femoral ante version, 38.9 degrees right tibial torsion and 37.1 degrees left tibial torsion on CT.\nIn gait analysis, he showed no dissociated movement between the pelvis and lower limbs, so we were able to see the bump sign, and the right pelvis were positioned superiorly to the left pelvis in the coronal plane during the whole gait cycle. Internal rotation of the right pelvis and external rotation of the left pelvis were observed in the transverse plane, and both hip joints showed internal rotation in the sagittal plane. Due to tightness in the hamstring muscle, both knee flexion posture and right ankle planter flexion posture were displayed during the whole gait cycle ().\nTo differentiate from hereditary torsion dystonia, when fragment analysis was performed on the DYT1 gene, the GAG deletion of exon 5 was not observed. Likewise, there were no symptoms, and no findings were observed in blood tests and urinalyses after suspicion of Wilson's disease. In addition, no notable findings were observed on the brain MRI scan, but it was found that uptake of fluodeoxyglucose (FDG) decreased in the bilateral parietal lobe, thalamus and cerebellum on a positron emission tomography (PET) of the brain.\nA plan was made for this patient to use DBS in consultation with the department of neurosurgery. After electrode insertion was performed on the globus pallidus interna (GPi), muscle tone decreased. Approximately six months later, right adductor tenotomy and bilateral medial hamstring release were performed in addition to rectus femoris transfer on the right sartorius, tibialis posterior transfer on the right peroneous brevis and derotational osteotomy on the right femur in the department of orthopedic surgery.\nThe patient was then transferred to the department of rehabilitation medicine and treated with comprehensive rehabilitation, including physical therapy and occupational therapy. The therapeutic outcomes were evaluated through DRS and gait analysis. On the dystonia movement scale, the patient scored 55 before DBS, but 3 months later, he scored 26.5, which was an improvement of 51.9%. One year later, the patient scored 24, which was an improvement of 56.4%. On the disability scale, the patient scored 10 before surgery and 9 one year after surgery (). On the gait analysis performed three months after surgery, the anterior tilt of the pelvis decreased in the sagittal plane. In the coronal plane, pelvic obliquity almost disappeared. In the transverse plane, pelvic asymmetry and femoral internal rotation were also attenuated. In addition, right ankle plantar flexion was remarkably ameliorated in the sagittal plane ().
We present an 18-year-old man with a history of transposition of the great arteries for which an arterial switch operation and a transpulmonary closure of an associated ventricular septal defect were performed shortly after birth. One year later, a pacemaker and epicardial leads were implanted because of a third-degree atrioventricular block. Despite not present in the first years after surgery, he developed progressive regurgitation of the neoaortic valve and consequently left ventricular dilatation. Aortic valve replacement with a decellularized homograft at the age of 17 years was performed. Follow-up transthoracic echocardiography at 5 months after the procedure detected an additional structure at the aortic root, raising the suspicion of a post-operative pseudoaneurysm. Computed tomography (CT) angiography confirmed the presence of a large pseudoaneurysm, measuring 4.5 × 1.8 × 2.5 cm, arising from the right side of the aortic root (Figure ). The neck of the entry at the aortic site was measured 4 × 4 mm on CT.\nThis asymptomatic pseudoaneurysm was considered to be a post-operative sequelae and after revision of the post-operative echocardiogram at discharge retained as a pure incidental finding. Endocarditis was excluded by laboratory analyses, negative blood cultures, and exclusion of vegetations on transoesophageal echocardiography. It was hypothesized that the orifice of the pseudoaneurysm occurred first in the ascending aorta and that in a second time the pseudoaneurysm re-entered in the left ventricular outflow tract (LVOT).\nGiven its size, it was decided that repair was warranted. Because surgical reintervention was not preferred, transcatheter closure under general anaesthesia and fluoroscopic guidance was attempted. During the procedure, the patient was anticoagulated with intravenous heparin aimed at an activated clotting time above 250 ms.\nAngiography in the ascending aorta revealed the ostium of a sizable pseudoaneurysm with an additional fistula of 4.6 × 3.4 mm in diameter between the pseudoaneurysm and the LVOT (Figure ). In a next step, the aortic orifice of the pseudoaneurysm was reached through a right guiding coronary catheter and subsequently, this fistula to the LVOT was approached via the pseudoaneurysm with a Progreat micro-catheter (Progreat® Microcatheter, Terumo). Then, the fistula between the pseudoaneurysm and the LVOT was closed by a duct occluder (Amplatzer Duct Occluder II 9-PDA2-05-06, Abbott) with a disc size of 11 mm and waist of 5 mm. Subsequently, the aortic orifice of the pseudoaneurysm was closed using a 6 mm (waist) atrial septal defect (ASD) device (Figulla Flex II ASD device 29ASD06, Occlutech). We intended to close both entrances to be sure that no reintervention would be needed, as in our experience in previous cases it was noted that when only one orifice is closed, the residual pseudoaneurysm is continued to be fed by the second orifice. Control angiography demonstrated two well-positioned devices without contrast extravasation. Furthermore, the coronary arteries were free from compression, considering the close proximity of the ASD occluder to the right coronary artery (Figure ).\nThe post-procedural course was uneventful, the patient was discharged the day after. No oral anticoagulation nor antiplatelet therapy was initiated as this might interfere with thrombus formation within the occluded pseudoaneurysm; also, the high-flow state over the LVOT and the ascending aorta contains no indication for systematic antiplatelet therapy. A CT scan after 1 month showed no residual contrast flow in the pseudoaneurysm (Figure ).\nAt follow after 6 months, patient revealed no symptoms and could undertake all normal daily activities. Transthoracic echocardiography showed a good function of the aortic homograft and a complete occlusion of the pseudoaneurysm. The left ventricular cavity remained only slightly dilated however with preserved ejection fraction and without any clinical nor echocardiographic signs of heart failure. Treatment with a low-dose beta-blocker and angiotensin-converting enzyme-inhibitor was continued.
A 10-year-old girl presented with osteosarcoma of her right distal femur, identified after a pathological fracture. Contrast-enhanced magnetic resonance imaging showed a large tumor arising from the distal femur and infiltrating the wall of the superficial femoral artery (Fig. ). She was otherwise healthy. She underwent neoadjuvant chemotherapy with adriamycin, cisplatin, and methotrexate. Then, intercalary wide resection of the femur, including the femoral artery and vein, was performed. The bony defect was 15 cm long and the vascular defect was 10 cm long. The sciatic nerve and the saphenous nerve were preserved.\nThe ipsilateral great saphenous vein (GSV) was harvested, and the superficial femoral artery and vein were reconstructed with interpositional vein grafts (Fig. ). The bony defect was bridged with an intramedullary nail, and bilateral fibular grafts were transferred. Both fibular grafts were harvested with a monitoring flap; however, circulation of the left monitoring flap was found to be poor and the flap was sacrificed. The left fibula was hooked up to the superficial femoral artery and vein with an end-to-side anastomosis. The right fibula was hooked up to the descending branch of the lateral circumflex femoral artery and its comitant vein (Fig. ). The monitoring flap of the right fibula was externalized for postoperative monitoring. We did not perform tibiofibular metaphyseal synostosis for either leg.\nThe wounds healed uneventfully. The circulation of the left fibula was confirmed using color doppler ultrasonography. The rehabilitation started from the fifth postoperative day. The patient underwent adjuvant chemotherapy with the same drugs from the 15th postoperative day for 3 months. At 7 months after the operation, the patient was allowed to walk with full weight bearing. Contrast-enhanced computed tomography obtained 14 months after the operation showed patency of the femoral artery and vein. At 20 months after the operation, the patient was able to walk without any assistance, despite a slight leg length discrepancy (Fig. ). No donor-site morbidity including valgus ankle deformity developed. A plain radiograph obtained 20 months after the operation showed complete bone union (Fig. ).\nWe successfully achieved limb salvage for a complex femoral defect after osteosarcoma resection. The defects of the femur and the femoral vessels were reconstructed with bilateral FVFG and autologous vein grafts, respectively.\nSeveral methods have been reported to date for intercalary reconstruction of femoral defects [–, ]. Among them, the most reasonable method for this case was the Capanna method, which combines FVFG with a massive allograft []. In this method, the allograft allows for immediate structural strength and FVFG provides excellent bone-healing potential []. However, a bone allograft is not readily available in Japan because of socioreligious reasons []. Instead, recycling of devitalized autograft is more widely used [, , ]. Promising results have been reported with its combined use with FVFG for intercalary femur reconstruction [], but this method was not indicated for this patient because she had a pathological fracture.\nThe FVFG was the only biological reconstructive method for this patient. The major problem with FVFG for femoral reconstruction is that single-strut FVFG cannot provide sufficient primary stability for femoral reconstruction []. The use of folded FVFG is reported to increase initial strength, but the available length is limited to 13 cm []. In this patient, the bone defect was 15 cm long, which exceeds the maximum available length of folded FVFG. Therefore, bilateral FVFG became necessary.\nThe use of bilateral FVFG for intercalary reconstruction of extensive femoral defects has been reported by several authors [, , –]. High rates of bone union with shorter nonweight-bearing duration are reported with this method. Tomita et al. performed bilateral FVFG for 18 femoral pseudarthrosis patients with large bony defects and achieved bone union in 15 of them (83.3%) []. Liang et al. performed bilateral FVFG for 16 patients with massive juxta-articular defects of the distal femur. They reported that primary bone union was achieved in 15 patients (93.8%) and eventual union in all patients (100%) []. Niethard et al. performed bilateral FVFG for five patients with an oncologic femoral defect. They reported that bone union was achieved in all patients (100%), including two patients with delayed union []. Despite these successes, bilateral FVFG are time consuming and technically demanding compared with other alternatives. This technical hurdle becomes even harder after resecting the femoral vessels because bilateral FVFG require two sets of recipient vessels.\nEfficacy of vascular reconstruction in the treatment of extremity sarcoma with vascular involvement is well established [, ]. Defects of the femoral artery are most commonly reconstructed using an interpositional GSV graft []. Synthetic grafts have a similar patency rate to autologous vein grafts [], but the need for anticoagulation therapy is a major disadvantage in young patients. Autologous vein grafts, therefore, are preferred for young patients. Although the need for simultaneous venous reconstruction is controversial [], we performed venous reconstruction with a GSV graft in this patient. No postoperative edema of the distal limb occurred, and the reconstructed vein remained patent through the final follow-up at 20 months.\nDonor-site morbidity after fibula harvest is not negligible in children. Some authors have reported progressive valgus ankle deformity after fibula harvest in children [, ]. To prevent valgus deformity, tibiofibular metaphyseal synostosis has been recommended in children under the age of ten []. On the other hand, Kanaya et al. reported that valgus deformity is inevitable even if tibiofibular metaphyseal synostosis is performed []. The indication of tibiofibular metaphyseal synostosis in our patient is controversial because she was just 10 years old at the time of surgery. We did not perform tibiofibular metaphyseal synostosis in this patient, and no ankle deformity developed.\nTo the best of our knowledge, there has been no previous report of the simultaneous reconstruction of the femoral artery and femur. This paucity can be explained by the suggestion that patients with femoral osteosarcoma with vascular involvement are commonly stratified to above-knee amputation or knee rotationplasty. Several studies demonstrated that, if the tumor infiltrates or surrounds the femoral artery, there is no difference in local tumor control and overall survival between amputation and limb salvage with vascular reconstruction [–]. Patients with knee rotationplasty are expected to have almost the same function as those who undergo below-knee amputation [, ]. Despite the high functionality, the resultant disfigurement is difficult for girls to accept.
A 62-year-old man with a past medical history significant for iron deficiency anemia, coronary artery disease, benign essential hypertension, gastroesophageal reflux disease, treated hepatitis C, alcoholic liver cirrhosis with history of esophageal varices, and internal hemorrhoids was a well-known patient for our hepatology team for history of liver cirrhosis in the past. Four months prior to establishing care in our clinic, he had presented with epigastric pain to the emergency department. During that emergency department visit, he underwent a CT of the abdomen and pelvis with intravenous contrast, which was notable for peri-pancreatic stranding and multiple small fluid collections around the entire pancreas, the largest of which was along the superior aspect of the pancreatic tail measuring 4.7 x 3.4 cm (Figure ).\nEvaluation done at an other center showed a hemoglobin level of 5.9 and was admitted to the emergency department for workup. The patient gave a history of having intermittent melena and one episode of emesis of blood clot. The patient had a full evaluation with esophagogastroduodenoscopy (EGD) and colonoscopy to rule out common causes or expected causes of GI bleed. EGD showed small isolated gastric varices (IGV 1 in the fundus) without bleeding, and otherwise normal stomach mucosa and no esophageal varices. On colonoscopy, the patient had medium-sized non-bleeding external and internal hemorrhoids but the entire colonic mucosa was unremarkable. The terminal ileum was intubated and showed old coffee-ground appearing contents. His video capsule endoscopic examination was unremarkable. The patient was conservatively managed but the exact source of bleeding was not identified, and his bleeding stopped. Two weeks later, the patient underwent endoscopic ultrasound (EUS) to evaluate for chronic pancreatitis, and also follow up of pancreatic tail cyst found on imaging four months prior. EUS demonstrated an overall atrophic appearance of pancreas with diffuse hypoechogenicity with lobularity and honeycombing, and shadowing calcifications, with areas of hyperechogenic stranding, most prominent in the distal body and tail of the pancreas. Additionally was seen a round peripancreatic complex collection measuring about 27.3 x 23.7 mm, with some anechoic areas along with hyperechogenic contents, which was suspected to be old blood (Figure ). The pancreatic duct (PD) appeared normal, without any disruption or stricture, and no obvious communication with the cyst was identified, and the common bile duct (CBD) was unremarkable. Given complex nature of the collection, fine needle aspiration (FNA) was deferred.\nFor further evaluation of the complex pancreatic collection containing hyperchogenic blood-like material, with the background presentation of recurrent melena and persistent anemia, a duodenoscope was used to evaluate the ampulla, and small amount of blood was seen extruding, raising differentials for HP or hemobilia (Figure ). The upper enteroscopic examination to proximal jejunum was otherwise unremarkable. CT angiography (CTA) of the abdomen did not show any mass in the liver or biliary system, but was notable for pancreatic pseudocyst with no evidence of active arterial extravasation, findings suggestive of chronic pancreatitis, thrombosis of distal splenic vein (associated perisplenic and upper abdominal collaterals).\nAfter multi-disciplinary discussion, IR approach was preferred over endoscopic approach, and through ultrasound-guide access of the left radial artery, selective catheterization of the celiac trunk, with subselective catheterization of the left gastric artery/splenic artery/gastroepiploic artery branch of the gastroduodenal artery with angiogram was performed. Superselective gastroepiploic angiogram demonstrated abnormal area of contrast pooling at the level of the midportion of the gastroepiploic artery corresponding with site of pancreatic complex collection abutting the stomach (Figure , ), and finally coil embolization of the gastroepiploic artery (bleeding source), and splenic artery (to decrease flow to the area of bleeding) was achieved with "back and front door" technique (Figure , ). The patient did well post-procedure, and did not have further bleeding, and hemoglobin remained stable on two-month follow-up.
We discuss the case of a 25-year old woman suffering from progressive limb weakness and clinical presentation of a proximal tetraparesis with slightly reduced muscular tone and mild Trendelenburg’s sign. She has had difficulties in walking longer distances since the age of 14 when she underwent corrective osteotomy for congenital genu valgum. However during the last years she noticed a newly developed decrease in tone and strength of the proximal muscles, predominantly affecting the lower limbs, especially when climbing stairs. Muscular weakness had slowly progressed, being most prominent in the evening hours. For the past 6 months she had been dependent on walking aids (canes or crutches). Moreover she reported of exertion induced myalgia. Family history at this time was unremarkable.\nClinical examination showed a symmetric proximal tetraparesis particular of the limb girdle muscles (MRC grade 4+ / 5 on arm elevation, rotation and abduction and 4 / 5 on hip and knee flexion and extension on both sides). Strength of the distal muscles was unremarkable. Muscle tone was slightly decreased. There were no relevant muscular atrophies. Tendon reflexes were normal. Examination of gait revealed slight bilateral Trendelenburg’s sign. History and clinical features were consistent with signs of a neuromuscular disorder.\nOn diagnostic work-up, both cranial and spinal MRI scans were unremarkable, as well as the lumbar punction. ECG and echocardiogram showed no abnormalities. Electrophysiological examination showed neither signs of peripheral sensory or motoric neuropathy nor of central nervous disorders, however EMG revealed myopathic changes in the proximal muscles of the upper and lower limbs with few myotonic discharges. MRI of the thighs revealed fatty degeneration of the gluteal and quadriceps muscles ( and ). X-ray of pelvis, hip and knee joints showed no osseous destruction but moderate signs of osteoporosis. Computed tomography of the spine and pelvis measuring bone mineral density showed a Z-score of -4.0 compatible with generalized osteoporosis.\nBlood tests showed serum creatininkinase and myoglobine within normal range, however Alkaline phosphatase (ALP) was elevated (254 U/l, ref. < 104). Thyroid function tests were unremarkable including thyroid antibodies. Screening of autoantibodies showed elevation of antinuclear antibody (ANA) titer (1:320, ref. < 1:80) and positive antibodies against Sjoegren’s syndrom A (SSA-AB), whereas all other antibodies including p- and c-ANCA, antibodies against the acetylcholine receptor, the MuSK protein and Titinantibodies were unremarkable. We also found regular increase of lactate and ammoniac following physical exercise. Particularly remarkable results were found when testing for the parathyroid function, revealing excessively increased parathyroid hormone (PTH) levels (959 pg/ml, ref. < 72) along with decreased serum phosphate (1.8 mg/dl, ref. 2.7-4.5) and serum calcium levels (2.00 mmol/l, ref. 2.08-2.65). Further workup revealed markedly decreased vitamin D levels (< 4.0 ng/ml, ref. 31-100).\nBased on these results, we diagnosed secondary hyperparathyroidism with osteomalacia and a neuromuscular disorder as a result of presumably long standing vitamin D deficiency. Further rheumatological workup confirmed the diagnosis of Sjögren’s syndrome, but causal relationship with the neuromuscular symptoms was found to be unlikely. Endocrinologists recommended a therapy with high dose vitamin D substitution (single i.m. injection of cholecalciferol 200,000 IE, followed by weekly oral intake of cholecalciferol 20,000 IE) and oral substitution of calcium 2000 mg daily. Three months after the recommended therapy was started, serum calcium levels had normalized and serum PTH levels had decreased to 90 pg/ml. Moreover both neurological symptoms and clinical appearance markedly improved: the patient did not suffer from myalgia anymore and was able to walk without aid again.
An eighteen-year-old male patient was brought to our emergency department five hours after an alleged history of suicidal attempt with gunshot under the chin. He was presented with a burst open face and no recognizable structures on the face, except for the eyes. He was uncooperative and drowsy. His arterial blood pressure was 80/60 mm Hg and pulse rate was 124/min. His respiratory rate was 26/min and was slightly distressed. He was unable to lie supine as the shredded structures tended to fall back; causing airway obstruction and blood trickled into the oropharynx causing him considerable distress.\nAfter initial resuscitation in emergency department with intravenous fluids (colloid and crystalloids) and blood transfusion patient was stabilized and suctioning of the blood from the upper airway, a closer examination of the face was done. There was comminuted fracture of mandible, maxilla, and nasal bones. Tongue, hard palate, and nasal structures were not recognizable []. Three loose teeth were seen embedded in the lower half of the face. The eyes were spared and his vision was unimpaired. Cerebrospinal fluid leak could not be made out because of the presence of blood. The expired gases had an exit near the root of nasal structure, which could be made out by the movement of cotton strands and no foreign body was present. The neck was not injured. Nervous system examination, as far as could be elicited, was normal with no cranial nerve damage or sensory and motor weakness. On auscultation, the breath sounds were normal with no added sounds, suggesting no aspiration of blood into trachea. Rest of the systemic examination was also normal. Chest radiograph was normal and no foreign body was present. He was scheduled to undergo emergency tracheostomy for airway management, debridement, and closure of facial laceration under general anaesthesia.[]\nThe patient was shifted to the operating room in the sitting position. We planned for tracheal intubation under sedation using light wand, in anticipation of a difficult airway.[] Pulse oximeter, ECG leads, non-invasive blood pressure monitor etc. were connected. Difficult airway cart was kept ready as patient was resuscitated and stabilized vitals (BP 110\84 mm Hg, PR 96\min, SPO2 96%). Injection Propofol 60 mg in sedation doses was administered intravenously slowly to enable the patient to lie supine. Airway was cleared by suctioning and holding forward of the fractured bones. The loose teeth were easily pulled out lest they should get dislodged. After adequate preoxygenation with a mask held close to the face we tried to perform direct laryngoscopy, but to our dismay, the blade could not be maneuvered once it was introduced into the oral cavity. Thereafter, we went for intubation using light wand.[] The operating room lights were dimmed and a well-lubricated, 8.0 mm endotracheal tube mounted light wand, which had been pre-shaped in the hockey stick manner was inserted in the oral cavity The usual technique of light wand intubation was used and the patient was easily intubated in the first attempt. The correct placement of the endotracheal tube was confirmed by chest auscultation. The entire process took thirty seconds. The tube was fixed securely with the help of a bandage. The anaesthesia was maintained with 40:60 O2: N2O, inj. Vecuronium bromide and halothane. The surgeons proceeded with the tracheostomy. Despite meticulous dissection, they had difficulty in locating the trachea. The light wand was introduced into the endotracheal tube and the transillumination directed the surgeons towards the exact location.[] As the surgeon was about to incise the trachea, the endotracheal tube cuff was deflated and the light wand was removed. When the trachea was adequately incised, the orally placed endotracheal tube was slowly pulled out and a 7.5 mm ID cuffed tracheostomy tube was inserted. The breathing circuit was connected to the tracheotomy tube. Facial laceration was debrided and closed. The entire surgical procedure took 1.5 hours. Giving reversal reversed the residual effect of neuromuscular blockade. After ensuring an adequate tidal volume and when the patient was following commands patient was shifted in the postoperative room. Oxygen inhalation was continued in the postoperative period. The patient was monitored till 24 hours for any complications. Then patient was referred for a CT scan of head, face, and neck. Three-dimensional CT scan of face showed complex comminuted fracture of face involving bilateral maxilla (anterior, lateral, medial wall, and alveolar process), hard palate, orbital wall, nasal bones, septum, and roof of nose and left sided base of pterygoid plate with opacification of bilateral maxillary, ethmoidal, and sphenoid sinuses. Zygomatic bones, eyeball, and optic nerve were normal. CT scan of head and neck were normal.\nHe was posted for definitive repair of face three days later when the edema of the bilateral ramus and body of mandible, left coronoid process of mandible face had subsided. The surgical procedure took 3.5 hours. The patient continues to do well waiting for bone grafting and prosthesis implant [].
A 12-year-old girl visited our hospital with “a right abdominal mass and spinal deformity 1 month” on July 22, 2014. When she was 3, the girl was received a mass resection operation in the local hospital, but without postoperative pathologic examination. She recovered well after the surgery. At the age of 5, her parents found that their daughter had mild claudication, but they did not take it seriously until 1 month ago, when she complained of a right abdominal mass with mild pain. In the meantime, the parents noticed the girl had scoliosis. She was then hospitalized in our orthopedics department. The child did not have a family history of tumors. Upon physical examination, we found an old vertical operation scar sized 5 cm in the right abdominal region. In the right lumbar region, we identified a lump measuring 10 cm × 7 cm that was solid, without clear boundaries, immobilized, and having no tenderness. A lumbar right curvature could be observed, and there was no direct or indirect percussion pain or neurological deficits. From a radiographic examination, the lower segment of the thoracic and lumbar spine showed a right tumefied thoracolumbar curve and left thoracolumbar curve around the 1st lumbar body at a Cobb angle of 33.7° and Ferguson angle of 69.4° (Fig. ), respectively. A CT and an MRI showed a paravertebral soft tissue mass from the T12 to L2 vertebrae (Fig. –). The preoperative value of NSE was 17.51 μg/L↑. Other laboratory examinations revealed no other abnormalities.\nThe large tumor was adjacent to important organs such as the right kidney and inferior vena cava. Moreover, the patient presented with scoliosis. Therefore, a staging operation was performed. The first stage operation by posterior approach aimed to resect the giant tumor, which was derived from the nerve, and to correct the scoliosis. In surgery, we saw that the mass originated from the L1 nerve root and extended into the intervertebral space between L1 and L2. The posterior and partial paravertebral elements of the lump were removed following the reclamation of a vertebral column using a screw-rod system as an internal fixation by a posterior approach. The mass was 11 cm × 7 cm × 4 cm and well encapsulated, luidity section, and was solid with a tough texture (Fig. ). Postoperative pathologic examination confirmed it was a ganglioneuroma (Fig. –). The second stage operation by the thoracoabdominal anterior-lateral approach was performed 2 months later to eradicate the thoracolumbar paravertebral giant ganglioneuroma. An intraoperative exploration found that it was a retroperitoneal mass, derived from the intervertebral foramen from L1 to L2, extending T10 to L4, measuring 13 cm × 8 cm × 6 cm, identical in character to the first mass, the giant tumor (Fig. ). The postoperative pathologic examination also presented identically.\nIn postoperative plain X-radiographs, the instrument, used for internal fixation, formed an image in the vertebral body and paravertebral region. The lower thoracic and lumbar vertebra surrounding the L1 vertebral body maintained a right direction thoracolumbar scoliosis. We measured a Cobb angle of 15.3° and a Ferguson angle of 44.3° (Fig. , ). A further CT revealed that, compared to preoperation, the right direction paravertebral soft tissue clump had disappeared, the right kidney had descended, and the right renal vessels imaging was clearer (Fig. , ). After the staging operation, the patient’s claudication has been disappeared, the patient was 5 cm taller, and her general condition recuperated well.\nTo review the cases of paravertebral ganglioneuroma and scoliosis, we used “scoliosis” and “ganglioneuroma” as keywords to search Medline for publications from the preceding 40 years. Using the strategy, we found 16 reported cases in 13 papers. For details regarding age, position, clinical characteristics, and follow-up, see Table .\nNeurogenic tumors may be broadly classified as arising from nerve cells or nerve sheaths. The former group includes ganglioneuroma, ganglioglioma, ganglioneuroblastoma, and neuroblastoma, and the latter includes neurilemmoma, neurofibroma, and malignant schwannoma []. In 1941, Eden [] classified dumbbell-shaped tumors into four categories according to the anatomical relationship, that is, the spinal cord and vertebrae: intradural and extradural; intradural, extradural, and paravertebral; extradural and paravertebral; and foraminal and paravertebral. In this case, the tumor, which was located in the intraspinal region, passes through the intervertebral foramen to form a paravertebral mass resembling a dumbbell. It is extradural and paravertebral according to the Eden categorization. The most common dumbbell-shaped tumor is the Schwann cell tumor, whereas ganglioneuroma is relatively rare [].\nOsteoidosteoma is one of the most common types of scoliosis deformities caused by tumors []. The scoliosis caused by osteoidosteoma is mainly connected with pain and paravertebral myositis [, ]. But patients with paravertebral ganglioneuroma and scoliosis feel no pain and experience no paravertebral myositis. There are three types of paravertebral ganglioneuroma and scoliosis: (1) the tumor grows expansively, leading to damages in the side and front vertebrae and eventually to scoliosis; (2) scoliosis is mechanically stimulated, induced the tumor; (3) paravertebral ganglioneuroma and scoliosis occur simultaneously. It was reported 60~80 % of dumbbell tumors can cause nerve root compression symptoms, while 20~40 % of the patients had no nerve compression symptoms []. Combined with the patient’s medical history and related literatures, in this case, we consider that scoliosis was caused by ganglioneuroma. One possible reason is that the tumor stimulated the affected side vertebral epiphyseal plate, leading to its overgrowth []. Another possible reason is that the tumor involved paravertebral muscle of convex side, causing the convex side muscle atrophy [].\nThe typical manifestation of ganglioneuroma is low density and punctate calcification on plain CT, showing a high T2 signal on MRI and a gradual increase in enhancement on dynamic images; it presents as non-enhancement or mild enhancement in the arterial phase of CTs or MRIs and progressive mild enhancement in the delayed phase. If ganglioneuroma shows an atypical manifestation on a CT and an MRI, we consider the tumor to contain a malignant component []. In recent years, 3D printing technology has proven helpful not only to make an operation plan and simulate the operation in the preoperative stage but also for patients to learn more about their conditions and facilitate communication with doctors [, ].\nThe ganglioneuroma shows complete capsular and basal growth by expansive patterns. The most effective therapy is a surgical removal operation as soon as possible, which can reduce the risk of malignant transformation, paraplegia, and other abnormalities. Moreover, surgery has a good prognosis. If the tumor does not affect the vital organs, it should be removed completely; however, if complete resection may cause serious complications, then a partial resection should be performed []. In this case, the preoperative Cobb angle was 33.7°, but due to the combined thoracolumbar paravertebral giant ganglioneuroma, a complete resection required a laminectomy. To maintain the spinal stability, in this case, the patient also required a scoliosis deformity correction and an internal fixation. The postoperative Cobb angle is 15.3°, and it was corrected well. Thoracolumbar paravertebral giant ganglioneuroma and scoliosis make a single surgical approach difficult to correct a spine deformity and resect the tumor completely. Therefore, the operation is divided into two stages. The interval time between two operations should be 1 to 7 weeks [, , , –] according to literature reports. However, as for us, the interval time should be considered from patients’ recovery condition after the first operation. If the patient recovers well and shows no signs of surgical contraindication, then the second operation can be performed. A piecemeal resection of the tumor can reduce the injury of paravertebral nerve and muscle. Adopting different surgical approaches can avoid injury of important blood vessels, nerves, and organs, so that the tumor is fully exposed. On the other hand, adopting different surgical approaches is good for operation and shortening the operation time; in addition, patients will better be able to tolerate the operation, while doctors can maintain their physical stamina. And at the same time, it increases the safety during the operation. Paravertebral giant ganglioneuroma and scoliosis usually affect the lung, kidney, intestine, large blood vessels, and other important organs. The postoperative complications include cerebrospinal fluid leakage, paraparesis, intestinal obstruction, perioperative bleeding, and pneumothorax, among other complications []. Consequently, multidisciplinary consultation preoperation and intraoperative multidisciplinary joint surgery can lower the risks of developing complications.\nGanglioneuroma has low recurrence rate, a good prognosis, and does not require chemotherapy, radiation therapy, or other treatments following complete resection [, ]. The patients should accept ultrasonography, DR, and CT checks regularly for a more thorough evaluation of partial recurrence after the surgery. The patient has been followed up for 18 months and shows a good scoliosis correction, a good internal fixation, and no tumor recurrence. At this point, the patient is still under follow-up.
A 20-year-old woman with a history of root canal treatment of the upper maxillary (left) central incisor was referred to our department from a private clinic. The root canal treatment had been initiated 4 months prior but could not be finished. A clinical examination revealed a temporary filling on the palatal surface. The coronal part of the tooth was discolored, and multiple visible cracks were present on the buccal surface (). The tooth was asymptomatic; the mobility was normal, and the probing depth at all sites was ≤ 3 mm. A periapical radiograph revealed a lesion around the apex and an enlarged and clearly irregular root canal (). When specifically asked, the patient denied knowledge of any previous dental trauma. Upon checking the electronic documentation of the faculty of the institution, we found that the patient had visited the department 2 years prior for other reasons, and X-rays were taken at that appointment. From a comparison of the periapical X-rays, it was clear that something must have happened since her last visit (). After gaining her confidence, the patient and her mother admitted that the patient had suffered from domestic abuse at the hands of her boyfriend, but that had been taken care of by the police. At this point, a small-field-of-view high-resolution cone-beam computed tomography (CBCT) scan was taken to investigate the extent and irregularity of the lesion. The scan confirmed the presence of a periapical lesion, which had destroyed most of the buccal bony wall around the root. The scan also showed massive internal resorption inside the root canal (). The patient was informed of the findings and the poor prognosis of the tooth, but since she desperately wanted to keep her tooth, she provided consent for endodontic treatment, which was initialized at a later appointment. The patient was also informed that due to the irregular nature and more pronounced buccolingual extent of the resorptive lesion and the massive destruction of the buccal bony plate, which cannot be seen or monitored on periapical X-rays, it would be beneficial to control the steps of the treatment and perform the monitoring via CBCT scans. The patient was informed of the extra radiation exposure resulting from the CBCT scans, but she insisted on undergoing scans instead of periapical X-rays when deemed beneficial by the clinicians.\nThe working length was established with an electronic apex locator (Root ZX, J. Morita Corp., Tokyo, Japan) and confirmed via radiography. The canal was subjected to instrumentation only with NiTi files (K-File Nitiflex, Dentsply-Maillefer, Ballaigues, Switzerland), stainless steel hand files (K-File, Dentsply-Maillefer), and Hedstrom files (ReadySteel, Dentsply-Maillefer). The last instrument used for apical preparation was a size 120 file, and at that point a definite apical stop could be established. The site was irrigated with copious amounts of 5% sodium hypochlorite. At the time of drying, the electronic apex locator, the hand files, and the paper points indicated no sign of perforation. At the most apical part of the resorption defect, the root canal appeared extremely thin; thus, the use of warm gutta-percha for obturation was deemed risky and difficult to control. An approximately 4- to 5-mm-thick mineral trioxide aggregate (MTA) plug (MTA+, CERKAMED Medical Company, Stalowa Wola, Poland) was made to establish a proper apical barrier without error in the affected apical part (). The MTA was covered with a moist cotton pellet, and the tooth was temporized with Cavit W filling material (3M ESPE, Seefeld, Germany) for 48 hours. At the next appointment, the root canal was adhesively treated with a dual-cure self-etch adhesive system (Gradia Core Self-Etching Bond, GC Europe, Leuven, Belgium) and was filled with SFRC (EverX Posterior, GC Europe) according to the Bioblock technique [] to the point of the root canal orifice. During this procedure, an approximately 4-mm-thick increment of SFRC material was placed in the root canal and applied to the most apical part to make contact with the MTA plug. This can be accomplished by pressing alternately with any small-headed microbrush and a periodontal probe or plugger. After the first layer appeared to be in position, a light-transmitting FRC post (1.4 mm GC Fiber Post, GC Europe) was inserted into the canal to facilitate the transmission of the light to the apically-positioned layers. The light-transmitting post was withdrawn to 0.5–1 mm from the surface of the uncured SFRC layer so as not to directly contact it. The first layer of SFRC was light-cured through the post for 80 seconds using a light source with an average power density of 900 mW/cm2. This was continued to the level of the cemento-enamel junction. After the root canal was filled to that level, the coronal portion of the tooth was internally bleached several times with sodium perborate (made by the pharmacy of the University of Szeged) mixed with distilled water at a ratio of 2:1 (g/mL) and covered with glass ionomer filling (Equia Fore, GC Europe). After the desired color was reached, the coronal cavity and the SFRC material in the orifice were refreshed with a diamond bur, adhesively treated (G-premio Bond, GC Europe), and restored with SFRC and a 2-mm-thick composite covering on the palatal aspect (G-aenial Anterior, GC Europe) ().\nA follow-up CBCT scan was taken 1 year after the final root canal treatment and revealed continued healing of the periapical radiolucency and reformation of the buccal bony wall around the apex (). The tooth has remained asymptomatic ever since, and the patient was satisfied with the result.
A 9-year-old boy presented with complaints of excessive duration of sleep, increased appetite, weight gain, excessive daytime sleepiness, loss of interest in sports activities, irritability and snoring since 1.5 years after he was shifted to a residential school.\nBefore attending residential school, he used to follow a regular sleep schedule, with bedtime at 10 p.m. He did not have any issue with sleep onset and used to sleep alone in the bed. According to his father, he used to sleep in supine position and did not show any sign of sleep-related breathing disorder. His mother used to wake him up at 5 a.m. and he usually left the bed within 10 minutes, feeling fresh. He was good in studies till the age of 7 years and had many friends.\nAfter 6 months of shifting to residential school, his father received complaints from the school regarding deterioration in studies and sleeping in the class. His teachers had also noticed that he was gaining weight and losing interest in sports activities. Then, his father took him back home before 1 year.\nAfter bringing him home, since the past 1 year, his parents noticed a gross change in his appetite with an increase in frequency and amount of food. A delay in meeting his demand of food used to result in irritability shown by the child. He continued to put on weight at home. His parents also noticed an increase in the time spent in sleep. For the past 1 year, he started feeling sleepy by 9 p.m. During the night, he would snore and spent most of his sleep in prone position. On some occasions, his father had found saliva on the pillow in the morning. His parents were not able to wake him up till 7 a.m., that too, with a lot of difficulty. After waking up, he took nearly an hour to become active. In addition, he started taking 3–4 hour nap after lunch each day. If he was not allowed to take nap any day, he would fall asleep by 5 p.m to wake up at 7 a.m. in the morning. Any force to avoid nap resulted in irritability. He lost interest in sports activities since then.\nThe child's father also noticed frequent memory lapses resulting in misplacing his belongings. There was no history to suggest childhood depression, frequent rhinitis, tonsillitis, attention deficit hyperactivity disorder, restless leg syndrome, cataplexy, hypnogogic or hypnopompic hallucinations, sleep paralysis or any other parasomnia. There was no evidence of any neurological disorder, epilepsy, head trauma or substance abuse. His birth history and developmental history were noncontributory. Family history was also nonremarkable.\nHis craniofacial examination showed presence of central obesity, dental overjet, Mallampatti grade IV upper airway, submental fat and high arched palate. Epsworth Sleepiness Scale score was 24. His weight was 56 kg and height was 132 cm, leading to body mass index (BMI) of 32.18. His neck circumference was 34 cm.\nMental status examination showed normal psychomotor activity. Child was irritable on occasions following trivial issues and was reluctant to comply with examination procedure. He failed to comply when higher mental functions were being tested.\nHis lateral neck skiagram showed adenoid hypertrophy [] and magnetic resonance imaging (MRI) brain was noncontributory. IQ assessment was also ordered. Since the child did not comply on the first day, the tests were performed after 2 days. At the time of administering tests, child was in better mood and performed all the tests. On developmental screening test, he attained a score of 90; on Vineland Social Maturity scale, his score was 70; on Malin's Intelligence Scale (Indian Adaptation) – Wechsler Intelligence Scale for Children (WICS) (performance test and verbal test), he attained a score of 70 each. Thus, the comprehensive score was 75.\nConsidering the clinical picture, diagnosis of obstructive sleep apnea was made and narcolepsy without cataplexy and KLS were kept as differential diagnoses. Consequently, a level-I video polysomnography followed by multiple sleep latency test (MSLT) was ordered.\nLevel I polysomnography was done with a total recording time of 452 minutes. Total sleep time was 362 minutes. Objectively, the boy had sleep efficiency of 90% (100 × Total Sleep Time (TST)/ Sleep Period Time (SPT)); sleep onset latency of 44 minutes; Rapid Eye Movement (REM) latency of 187minutes and Wake after Sleep Onset (WASO) of 47 minutes. During the study night, N1 was 12%; N2 was 48%; N3 was 18% and REM was 22%. Hypnogram suggested frequent arousals []. He spent most of time in right lateral position [] and Respiratory Disturbance Index (RDI) was 2 (REM=4; Non Rapid Eye Movement (NREM)=1). Respiratory events were position dependent. Average saturation during REM was 97% and during NREM was 98%. Oxygen saturation dropped to 87% during REM and 94% during NREM. Snoring was also observed with an index of 8.9 without any effect of sleep stage.\nMSLT was done the following day. Four naps were recorded as per the standard protocol []. Results of this test are depicted in .
A 24-year-old man was admitted to our facility for IR. The patient had a history of percutaneous kidney biopsy under real-time ultrasound guidance using a cutting needle at 15 years of age. Macroscopic hematuria started shortly after biopsy, but ceased within a couple of days without development of significant anemia. The size of a subcapsular hematoma (up to 38 mm long by 7 mm wide), which occurs in most renal biopsies, also decreased over time. Thus, common complications such as gross hematuria and subcapsular hematoma occurred, but were settled within days of the biopsy. Based on our institutional protocol, the patient was discharged 7 days after biopsy. However, immediate rehospitalization was required on the day of discharge due to abrupt onset of severe left flank pain. Gross hematuria reemerged and was intermittently observed for several days, resulting in hypotension (94/58 mmHg), a significant decrease of blood hemoglobin (14.2 to 10.7 g/dl), and acute dysuria. Embolization via IR was considered as an emergency therapy to stop bleeding, but gross hematuria eventually disappeared within days with intravenous injection of hemostatic agents such as carbazochrome sodium sulfonate and tranexamic acid, in addition to bed rest. It was noteworthy that color-coded Doppler US detected AVF as a mosaic signal in the lower pole of the left kidney (Fig. ), which was the puncture site of the biopsy. However, in terms of size, this lesion was morphologically undetectable, even by dynamic contrast-enhanced computed tomography (CT) (Fig. ). After disappearance of gross hematuria, the patient was free from renal AVF-related manifestations, including abdominal pain, hypertension, and renal impairment. Thus, it was determined that IR was not required at this time.\nIgA nephropathy was diagnosed based on the findings of renal biopsy. The patient was hospitalized again six months later to receive steroid pulse therapy followed by tonsillectomy [] for this glomerulopathy. A striking improvement in urinary abnormalities was gradually achieved, with urinary protein reduced from 4.2 to 0.2 g/day and red blood cells decreased to a level of 5 cells per high-power field in sediment. Ultrasound examination was performed again, but growth of renal AVF was not evident in this period. Following the combination therapy, the patient regularly visited our hospital and took 300 mg dipyridamole and 6 mg candesartan orally each day. The angiotensin II receptor antagonist was prescribed as a renoprotective agent, rather than for a depressor effect. Blood pressure (125/75 mmHg), blood hemoglobin, and serum creatinine remained normal in this period and for over eight years.\nDespite the uneventful course, at the age of 24 the patient underwent abdominal ultrasound because of a slight elevation of serum alanine aminotransferase (up to 36 U/l), which had been noted approximately one year earlier. Unexpectedly, a mass lesion was found in the left kidney, whereas there were few morphological abnormalities in the liver. Dynamic contrast-enhanced CT subsequently delineated a gourd-shaped mass (26 × 22 and 12 × 11 mm) in the left kidney (Fig. ) and marked dilatation of the left renal vein (Fig. ). Three-dimensional CT (Fig. ) and maximum intensity projection CT (Fig. ) clearly revealed that this lesion directly led to the renal artery and vein, suggesting that the renal AVF had grown subclinically to a giant size over many years and had resulted in marked dilatation of the venous system. Therefore, IR was required to prevent further growth of the fistula and manifestation of symptoms.\nTranscatheter embolization of high-flow left renal AVF was performed using detachable coils by radiologists. The fistula was solidly packed with 7 Coil 400 Standard Complex® coils (Penumbra, Inc., Alameda, CA, USA) and 22 Presidio® coils (Codman, Inc., Raynham, MA, USA) under digital subtraction angiography. As shown in Fig. , the high-flow fistula was successfully blocked out, with conservation of renal blood flow. The patient was discharged on postoperative day 3 without adverse events. A follow-up test by contrast-enhanced MRI at about 3 months after IR showed maintained discontinuation of blood flow to the AVF (Fig. ), indicating the success of the treatment.
Diana (a pseudonym) is a young woman of 25 years old that reached the Eating Disorders Centre, Division of Endocrine and Metabolic Diseases, San Luca Hospital in Milan, following a dramatic weight loss. Diana reached the Centre with a BMI of 16.06 kg/m2 reporting several disruptions in her eating patterns and several distressful alterations in her body image perception. As reported in the clinical history, Diana’s first eating related crisis was dated back 2 years before her current admission, with a subtle episode when she started a diet to lose some weight after health issues related to her thyroid. During that period, Diana was located abroad for work and – under moderate stress – she began a restrictive diet with a low caloric intake that brought her to lose 10 kg in 6 months. Diana’s weight remained constant in the following months but she developed an obsessive attention to the caloric intake along with intrusive thoughts regarding her weight and regarding specific types of foods. Diana also reported body-related image distortions such as overvaluation of her weight, mirror and body checking, and avoidance of body exposure. Moreover, during the crises, she reported frequent crying spells observing her body in front of a mirror.\nIn the last year, Diana reported a stressful situation at the University that heightened her psychological symptoms. Following these new difficulties, Diana re-enacted the restrictive conducts, reducing the caloric intake with a consequent weight loss of 4 kg in a month. In the period before the admission, the restrictive conduits were accompanied by self-induced vomit and daily binge episodes.\nDiana matched all the DSM-5 criteria for a diagnosis of AN, binge-purge subtype. Compatible with the diagnosis, Diana presented distortions in her body perception and obsessive thoughts regarding her weight and her body image; for these reasons, she was considered as an optimal candidate for the interoceptive assessment.\nAt the begin of the rehabilitative protocol, Diana’s blood panels showed no signs of metabolic distress, with values in normal ranges. At her admission, Diana’s thyroid levels were within normal range and they remained within the normal range during the curse of the treatment. Endocrinologist suggested a chronic autoimmune normal-functioning condition. The psychiatric assessment indicated mood alterations toward a depressive condition accompanied by severe sleep difficulties and insomnia.\nThe rehabilitative program was composed of a multidisciplinary approach that included several experts in different fields: endocrinology, psychiatry, psychology, and nutrition. The specialists collaborated in an outpatients service tailored to the specific users’ needs. The rehabilitative program could extend from two to four cycles of treatment. Diana followed a two-cycle rehabilitative protocol with a frequency of 3 sessions a week for a total of 37 sessions. The protocol was composed of psychological intervention with group psychotherapy and individual sessions focused upon a psyco-corporal therapy approach (body-oriented psychotherapy). Psychological intervention was accompanied by psychiatric and pharmacological support (citalopram and mirtazapine), to moderate Diana’s mood alterations. The rehabilitative protocol was integrated with alimentary education sessions provided by the nutritionist. Additionally, the protocol was also accompanied by a nutritional program with fixed meals (both in quantity and composition) that Diana consumed under supervision. Scheduled assessment sessions ensured an adequate monitoring of the progress.\nFrom the beginning, Diana showed a deep insight regarding her condition. Nonetheless, from her first session, Diana showed severe difficulties in following the assigned rehabilitative diet due to obsessive thoughts connected to her body weight and to certain types of food. In the following weeks, Diana improved her adherence to the recovery protocol with a better ability to follow the changes in the diet both on quantitative both on the qualitative level (e.g., types of foods consumed). Diana reached a BMI of 19.00 kg/m2 at the end of her second cycle of rehabilitative treatment. Considering the noticeable improvements regarding her eating behaviors and her general clinical condition, Diana was dismissed from the Centre and continued her program following only monthly assessments accompanied by individual psychotherapy.
A 61-year-old man went through a pelvic fracture due to wall collapse and was diagnosed with posterior urethral rupture. Five months after the accident, he came to our department to seek for urethral reconstruction.\nWe retrieved his medical history after injury from the medical record system in the orthopedic department. After the injury, the patient went through acute urinary retention (). Computed tomography (CT) scan showed edematous lower limbs and perineum (), purpura, and obvious local tenderness. The condition of the scrotum and testicles was not recorded. CT scan showed a comminuted fracture of the pelvis and sacrum (). The catheter balloon was not located in the bladder (). At that time, the orthopedists asked a urologist for a consultation on the acute urinary retention of the patient. The patient was then diagnosed as having a pelvic fracture complicated with acute urinary retention caused by posterior urethral rupture. Cystostomy and external pelvic fixation were performed.\nThe patient then came to the urology department to seek for urethral reconstruction 5 months after injury. Physical examination revealed a tough, round palpable mass in his right inguinal region, with a size of about 42 cm. The right scrotum was shrunken whereas the other side was normal. After further questioning the medical history, the patient confirmed that his bilateral testicles were palpable in scrotum and denied history of retractile testes before the accident, and the right scrotum was found empty after he was discharged from the hospital last time. He supposed the testicle had been completely destroyed during the accident. The patient was old, had no fertility requirements, and still had erectile function, so he did not take the initiative to mention it to his doctor.\nPelvic floor ultrasonography () was done during transurethral injection of normal saline, and presented that the penile part of the urethra was naturally dilated and the mucosa was smooth. However, the membranous urethra was like the shape of the mouth of a bird. There was no dilatation of membranous, bulbar, and prostatic urethra (indicating posterior urethral rupture). Color Doppler ultrasound image of right inguinal mass () presented a moderate echo with a size of 4.11.2 cm in the subcutaneous fat layer, with clear boundary, intact shape, and blood flow signals, which was consistent with a normal testicular echo. Reexamination of the pelvic CT showed that the right scrotum was empty and asymmetrical to the left (). The patient was diagnosed with membranous urethral rupture and right traumatic testicular dislocation.\nAfter adequate antiinfection treatment, fiber cystourethroscopy, urethral reconstruction, and orchiopexy for the dislocated testicle were performed under general anesthesia. The posterior urethral atresia was confirmed by fiber endoscopy through the external urethral orifice and cystostomy catheter. An invert “Y” incision was made in the perineum. Then the urethra was exposed and separated. Then we excised the scar tissue around the urethra, and the defect length was measured at about 1 cm. After eight stitches of tension-free anastomosis of the proximal and distal urethra, the incision was sutured. Negative pressure drainage was retained. Then, an oblique incision of about 3 cm was made in the right inguinal region, and a well-formed dislocated testicle was exposed outside the subcutaneous inguinal ring with no rupture of the tunica albuginea and any hematoma. We separated the spermatic cord and vas deferens, pulled the testicles out of the scrotum, and fixed them with three stitches to prevent from torsion.
A 9-year-old girl was presented to our hospital on referral from a peripheral hospital with a one-week history of darkish discoloration of both feet and her left hand and a six-day history of a purulent feculent discharge from an exploratory laparotomy wound. She had presented to the referring hospital eight days earlier, with a two-week history of high-grade fever and a one-week history of a generalized abdominal pain and diarrhea. She had an exploratory laparotomy at the referring hospital. Intraoperative findings include a feculent intraperitoneal collection and a single perforation on the antimesenteric border of the terminal ileum. An intraoperative diagnosis of perforated typhoid enteritis was made, and the perforation was closed in two layers. One unit of blood was transfused postoperatively. On the first day of postsurgery, the patient developed darkish discoloration of the left hand and both feet which were associated with pain. She was not a known sickle cell disease patient and had no past histories suggestive of intermittent claudication. On the second day of postoperation, a purulent discharge from the lower aspect of the laparotomy wound was noted, and there was darkening of the feet and duskiness of the distal one-third of both legs. Due to the progressive worsening of the patient's condition, she was referred to our hospital on the seventh day of postoperation.\nAt presentation to our unit, she was acutely ill looking, febrile with a temperature of 38.1°C, dehydrated, not pale, and anicteric. Her respiratory rate was 30/minute with reduced air entry and coarse crepitations in both lower lung fields. She had a pulse rate of 130/minute, which was regular and of moderate volume. Only first and second heart sounds were heard. Her abdomen was full, and did not move with respiration, and had a midline dressing soaked with a feculent fluid. There was a complete wound dehiscence of the abdominal wound, with both edges being necrotic. There was no bowel evisceration. Both feet were dark and cold, with duskiness of the skin of the distal one-third of both legs (). There was loss of sensation in the feet and absent dorsalis pedis, anterior and posterior tibial pulses bilaterally. Popliteal pulses were palpable bilaterally. The left hand was dark, dry, and shriveled up to the wrist. The radial pulse was however present. The right upper limb was normal. A diagnosis of complete wound dehiscence, fecal fistula with peripheral gangrene of both feet and left hand in a patient who had surgery for typhoid intestinal perforation, was made. The patient was commenced on intravenous (IV) fluid resuscitation and intravenous antibiotics (ceftriaxone and metronidazole). She was placed on nil per os (NPO) and nasogastric decompression of the stomach. A consult was sent to the orthopedic surgery team who advised that the gangrene be allowed to demarcate before any decision could be taken on the limbs.\nHematological investigations after fluid resuscitation showed a hematocrit of 23.9% and a white blood cell count of 18.9 × 109/l and a hemoglobin genotype of AS. She was transfused with 200 ml of packed cells, in preparation for a wound exploration. She was however noted to be making scanty urine, and due to her unstable clinical condition, the surgery was postponed while IV fluid resuscitation and local wound care continued. By the fourth day of admission in our unit, the left upper limb gangrene had extended to the distal forearm, while on the lower limbs, the gangrene had extended to the middle aspect of the legs. The patient's condition continued to deteriorate in spite of efforts at resuscitation. She died on the eighth day of admission in our unit.
A 43-year-old man with a history of cocaine and amphetamine use and no medical contact since childhood presented to an outside hospital with severe, acute-onset, tearing back and flank pain. On initial evaluation his blood pressure was 240/130 mmHg with a heart rate of 100 beats per minute and he was noted to have an altered mental status. Computerized tomography (CT) angiography at the outside hospital demonstrated a descending aortic dissection. He received an intravenous labetalol bolus followed by continuous infusions of esmolol and nitroprusside. He was transferred to our institution for further management.\nOn arrival, the heart rate was 91 beats per minute and noninvasively measured blood pressure was 139/66 mmHg in both arms. The radial, dorsalis pedis, and posterior tibialis pulses were full and equal bilaterally. Laboratory evaluation was notable for potassium of 5.9 mmol/L, creatinine of 1.57 mg/dL, lactic acid of 2.9 mg/dL, white blood cell count of 20.17 × 103/µL, and a mild troponin-T elevation at 0.06 ng/mL (Table ). The electrocardiogram showed sinus rhythm at a rate of 63 beats per minute with left axis deviation as well as an R wave in aVL measuring 14 mm, consistent with left ventricular hypertrophy. Urine toxicology screen was positive for amphetamines, tetrahydrocannabinol, and opiates (the latter had been administered prior to transfer).\nReview of the CT obtained at the hospital of first presentation revealed a descending aortic dissection that began distal to the origin of the left subclavian artery and extended to both iliac arteries (Fig. ). The left renal artery received blood supply from the false lumen, which compressed the true lumen at the site of the right renal artery takeoff. There was lack of contrast enhancement of the left kidney consistent with malperfusion. Enhancement of the right kidney was normal.\nFollowing multidisciplinary evaluation, consensus was reached that the time for surgical intervention to preserve the left kidney had passed. It was noted that the right kidney appeared to be perfused and the left kidney had a warm ischemic time of approximately 12 h. Given the duration of warm ischemic time and lack of evidence of other limb or visceral malperfusion, surgical intervention was deemed unlikely to be beneficial and intensive medical management was recommended. Medical therapy was selected with careful monitoring of renal function and visceral and extremity perfusion. The infusions of esmolol and nitroprusside were uptitrated and the patient was given hydromorphone and fentanyl for pain. He was intubated for airway protection in the setting of altered mental status and was monitored in the cardiac intensive care unit with goal heart rate <60 beats per minute and goal systolic blood pressure 100–120 mmHg.\nDespite control of heart rate and blood pressure, the urine output declined to <20 cc/h and creatinine rose to 2.9 mg/dL within the first 24 h. It was felt that expansion of the false lumen with compression of the true lumen was leading to malperfusion of the right kidney. In addition, he was noted to have diminished pulses in the right foot. After multidisciplinary consultation, the decision was made to proceed with aortic fenestration with the goal of decompressing the false lumen to restore flow to the right kidney (Fig. , Movie 1). The right renal artery pressure was lower than the proximal aorta pressure with a peak gradient of 50 mmHg and mean gradient of 22 mmHg. Intravascular ultrasound (IVUS) of the aortic true lumen showed it to be severely compressed in the suprarenal segment with a dynamic component. Fenestration was performed using a Pioneer® Plus catheter (Medtronic CardioVascular, Santa Rosa, CA) and dilations across the dissection flap were performed with 10 and 14 mm balloons. Intravascular ultrasound showed an expanded true lumen and a patent right renal artery. The urine output increased to 60 cc/h over the next 4 hours.\nDespite the initial improvement in renal function, the urine output again declined to <30 cc/h with progression to anuria over the subsequent 8 h. The patient was taken back to the catheterization laboratory 12 h after the first procedure. IVUS of the aorta showed that in the thoracic aorta the true lumen was widely patent but in the sub-diaphragmatic segment at the level of the visceral vessels the true lumen became quite small before enlarging again in the infrarenal aorta. An endovascular endarterectomy from T11 to the aortic bifurcation was undertaken (Fig. ) with restoration of flow to the right renal artery. Urine output post-procedure was 400 cc/h for the next hour and then returned to baseline.\nThe remainder of the hospital course was complicated by ventilator-associated pneumonia, right internal jugular vein thrombosis, and new-onset left arm weakness with magnetic resonance imaging of the brain demonstrating likely embolic infarcts in the right middle cerebral artery territory. The patient was successfully extubated and transitioned to oral medications to control the blood pressure and heart rate. No embolic source was found and the left arm weakness had markedly improved by discharge on hospital day 20. The creatinine, which had peaked at 3.36 mg/dL on hospital day three, had declined to 1.3 mg/dL by discharge. At follow-up, the patient was compliant with a stable regimen of metoprolol, hydrochlorothiazide, lisinopril, and amlodipine to maintain a normal blood pressure and had not used cocaine or amphetamines since discharge. The creatinine stabilized between 1.3 and 1.5 mg/dL. Contrast enhanced CT scan performed three and a half months after discharge (Fig. ) demonstrated a stable dissection beginning immediately distal to the left subclavian artery and extending to the distal thoracic aorta without involvement of the great vessels. The upper pole of the left kidney was infarcted and atrophied. The proximal abdominal aortic dimensions remained stable at 3.3 × 3.3 cm.
A 47-year-old male presented to the emergency department at a primary care hospital complaining of sudden onset, severe chest pain radiating to his back. Four years prior, the patient suffered a type A aortic dissection and underwent emergency surgery that included Dacron graft replacement of the ascending aorta and a mechanical aortic valve. The patient was anticoagulated with warfarin for his mechanical valve. INR 2 days before presentation was 4.3; the patient was advised to reduce his warfarin dose by one half. Vital signs at the outside hospital were reported as temperature 37.1°C, heart rate 86 bpm, blood pressure 141/89 mmHg and oxygen saturation 98% breathing ambient air. The patient appeared comfortable. Cardiopulmonary examination was benign and consistent with a normal mechanical aortic valve. The remainder of the physical exam was unremarkable. Electrocardiogram showed normal sinus rhythm. Complete blood count, electrolytes and renal function were within normal limits; however, hemoglobin was 40 points lower than 2 days before. Troponin was negative. INR was 3.0.\nWorkup at the outside hospital included computed tomography (CT) of the chest with administration of contrast material. This revealed a heterogeneous collection in the right prevascular space immediately anterior to the ascending aorta measuring 6.0 × 3.2 × 12.8 cm3, concerning for aortic rupture with contained hematoma (Fig. , top left and right). Vitamin K 10 mg IV was administered, and the patient was urgently transferred to our tertiary care hospital.\nUpon arrival, the patient was hemodynamically stable with no signs of acute hemorrhage or cardiovascular compromise. The patient was admitted for monitoring and further evaluation. Contrast-enhanced CT scan of the chest was repeated at our hospital. The aortic graft appeared intact with no evidence of contrast extravasation. The configuration of the mediastinal process was stable compared to the outside study 6 h earlier. Furthermore, the appearance of the ascending graft was unchanged from a postoperative study 4 years prior. The anterior mediastinal process was consistent with a lobulated soft tissue mass such as lymphoma, thymoma or teratoma. No additional lymphadenopathy was identified. Review of remote CT imaging performed at the time of the aortic dissection did not demonstrate a mediastinal mass.\nThe patient underwent evaluation for an undifferentiated anterior mediastinal mass, which included CT abdomen and pelvis, testicular ultrasound and tumor markers. These were negative. Two CT-guided biopsies of the soft tissue mass were non-diagnostic. Cardiac MRI was unable to conclusively characterize the lesion (Fig. , bottom left). Given that this mass in the anterior mediastinum defied diagnosis by imaging and biopsy, a decision was made to operate.\nOperative planning involved concerns that this may indeed have been a malignant lesion with hemorrhagic transformation or a complication related to the aortic graft, such as infected hematoma or chronic inflammation (Fig. ). The patient underwent complete resection of the anterior mediastinal mass, which included intubation with a double-lumen endotracheal tube and re-do sternotomy with cardiopulmonary bypass on standby. Intraoperatively, the mass was found to overly and densely adhere to the aorta (Fig. , left and middle). There appeared to be an intact capsule with no obvious invasion of adjacent structures. Under lung isolation, the mass was resected in its entirely. All thymic tissue was removed from the upper thymic poles superiorly to the right and left phrenic nerves medially and the diaphragm inferiorly. The mass was adherent to the proximal suture line of the Dacron graft to aorta and required careful dissection to ensure the aorta and tumor capsule were not entered. Frozen section tissue analysis from this anastomotic area was consistent with inflammatory adhesions only. Other analyses of frozen sections at the time of operation were pathologically inconclusive. Given the suspicion of malignancy, the mass was resected with adequate margins for an effective cancer operation if indeed the final histopathological analysis was malignant. Final pathology revealed a large multilocular thymic cyst with evidence of cyst rupture, hemorrhage and organizing hematoma but no evidence of malignancy (Fig. , right).\nThe patient’s postoperative course was uncomplicated. He was bridged with therapeutic dalteparin to warfarin therapy for his mechanical aortic valve. Follow-up in 6 months will include a chest CT scan to monitor for recurrence of the mediastinal mass.
A 74-year-old male with past medical history of atrial fibrillation, hypertension, dyslipidemia, and benign prostatic hypertrophy presented to the emergency department with testicular swelling and pain for four days. Physical exam was notable for an abscess on the inner gluteal fold of the perineal region that was spontaneously draining serosanguinous output. His left hemi-scrotum appeared erythematous and indurated without overt signs of cellulitis. There was mild tenderness to palpation of the scrotal area although no crepitus was felt in the thighs or scrotum. He also endorsed blood-tinged drainage from the wound for several weeks. He was admitted for the management of his scrotal wound. He was evaluated by urology and general surgery who had low suspicion for Fournier’s gangrene. Medical records demonstrated several urological procedures for ureterolithiasis and nephrolithiasis in the few months prior to this admission. The procedures included a bilateral ureteroscopy with laser lithotripsy followed by stone removal, stricturotomy, and placement of ureteral stents to prevent the progression of hydronephrosis. He had developed iatrogenic urinary incontinence after the procedures and had an indwelling Foley catheter inserted in view of incontinence.\nHe also reported a fall at home three weeks ago, following which he developed persistent back pain but did nothing about it. On our evaluation the computed tomography (CT) scan of the spine (Figure ) revealed a recent L1 vertebral compression fracture with sclerosis and slightly ill-defined margins with additional lumbar spine compression fractures of T12 , L3 and L4 with several other multilevel degenerative changes in the lumbar spine. The patient also reported a gradual drop in height from 6 feet 3 inches to 5 feet 9 inches over the course of many years.\nUpon admission to our inpatient department, review of his CT findings was out of proportion to age-related bone changes which led us to go back and do an extensive chart review which revealed that the patient had a long-concealed history of hyperparathyroidism, which was diagnosed incidentally from a routine blood draw which demonstrated hypercalcemia 12 years ago. He was initially referred by his primary care physician to endocrinology who strongly recommended surgical removal of the adenoma. However, the patient declined surgical intervention because he felt he was asymptomatic.The patient instead opted for medical management of his hyperparathyroidism and was started on long-term alendronate therapy by his primary care physician for approximately 10 years. Home care records demonstrate that he was non-adherent to routine follow-up and was predominantly home bound with minimal activity and minimal sunlight exposure. His laboratory records persistently demonstrated hypercalcemia and increased parathyroid hormone levels. At current admission his calcium was 8.9 mg/dl, parathyroid hormone was at 102 ng/L, vitamin D was 24 ng/ml and alkaline phosphatase was 75 IU/L.\nUpon directed evaluation in the inpatient setting we found that along with nephrolithiasis and bone changes he had also developed other classical signs such as neurocognitive changes such as hallucinations of men standing in the air with food carts. He continued to decline parathyroidectomy despite worsening of his disease. His 10-year-long alendronate course was recently replaced by denosumab in view of treatment failure. Alternatively at the current admission denosumab was discontinued in view of increased risk of infections and he was started on cinacalcet therapy and intravenous antibiotic and was referred to the neurosurgery unit for the management of his chronic unstable L1 vertebral fracture.
A 30-year-old man was admitted to the intensive care unit (ICU) of our hospital with an approximately 12-hour history of oliguria. He was 180 cm tall and weighed 70 kg. The patient and his sister provided the history.\nTwo days before admission, the patient had orally ingested about 160 mL of enriched diquat (20 g per 100 mL) because of debt-related problems. Three hours later, the patient presented to another hospital for treatment and underwent gastric lavage. At that time, his blood pressure and heart rate were normal, and he had no shortness of breath. His blood creatinine level, routine blood tests, transaminase levels, bilirubin level, coagulation function, troponin level, and electrocardiogram findings were all normal. Chest computed tomography (CT) showed no abnormalities (). The patient refused further treatment and went home on his own. Moreover, a history of systematic disease including hypertension, diabetes, respiratory illness, hepatic or renal dysfunction, and mental disorder was denied by the patient and his family member, as was drug or alcohol abuse. His previous health condition was claimed to be quite good.\nOne day before admission, the patient presented to the emergency department of our hospital because of pharyngeal pain and mild pain in the upper abdomen. At that time, blood tests showed a high creatinine level (267 μmol/L). The patient was given antioxidation and infusion therapy. However, his urine output decreased and his blood creatinine level increased to 541 μmol/L after 24 hours. The doctor recommended a blood purification treatment. However, the patient and his family refused for economic reasons. Seven hours later, the patient and his family agreed to the blood purification treatment. At that time, the patient’s transaminase and bilirubin levels were elevated. He was transferred to the ICU 57 hours after the oral diquat ingestion and developed kidney failure, liver dysfunction, and respiratory failure. He died of respiratory failure on day 13 of hospitalization.\nDiquat is a small molecular substance with molecular weight of 344.05, and it can easily pass through the cell membrane. Hemoperfusion is an important treatment method in the early stage of diquat poisoning. In the present case, hemoperfusion was performed immediately after admission to the ICU; however, only one 2-hour session of hemoperfusion was performed. Oliguria persisted during hospitalization; therefore, continuous renal replacement therapy (CRRT) was initiated following the hemoperfusion treatment. Postdilution continuous venovenous hemodiafiltration with an effluent flow of 30 mL/kg of body weight per hour was implemented until the patient died.\nUpon presentation, the patient was conscious, had no shortness of breath, had a percutaneous oxygen saturation (SpO2) of 98% (fraction of inspired oxygen (FiO2), 21%), and had a normal blood pressure and heart rate. His arterial partial pressure of oxygen was acceptable (). Chest CT findings at that time appeared normal (). He was not treated with oxygen therapy because the mechanism of diquat poisoning is mainly the generation of oxygen free radicals leading to cell apoptosis.\n Although the leukocyte count was high, the procalcitonin (PCT) level was within the low range (). No antimicrobial agent was administered at that time. The patient’s clinical condition rapidly became exacerbated thereafter. On day 3 of hospitalization, the patient developed progressive shortness of breath and hypoxemia, and his SpO2 was maintained at only about 88% with conservative oxygen therapy. The patient was intubated and mechanically ventilated when the severe hypoxemia became unacceptable. After endotracheal intubation, piperacillin/tazobactam was empirically given for anti-infective therapy. Fiberbronchoscopy was performed after intubation, and a large amount of yellow plasma exudate was observed. Sputum was collected for culture (). Chest CT examination revealed exudation, consolidation, and ground-glass opacity in the bilateral lower lungs, confirming a diagnosis of acute respiratory distress syndrome (ARDS) (). The patient’s oxygenation significantly improved after he was moved into the prone position; his FiO2 increased to 30% and his SpO2 was maintained at about 90%. On day 6 of hospitalization, another chest CT examination showed that the lung exudation was significantly reduced ().\nOn day 8, however, the SpO2 began to gradually decline, especially after position changes. The FiO2 had to be increased, and higher inspiratory pressure was required to achieve a satisfactory tidal volume because of deterioration in the respiratory compliance. Deep sedation was required to relieve the sensation of asphyxia. At that time, the previous sputum culture revealed ampicillin-sensitive Acinetobacter baumannii. Thus, the treatment with piperacillin/tazobactam was continued. On day 10, a bedside chest radiograph showed interstitial lung lesions (). Necrotic mucosal tissue could be seen in the bronchi branches under fiberbronchoscopy. However, the mucosal injury of the oropharynx had healed without mucosal abscission. No plasma exudate was present in the airway, but scattered bleeding was noted after mucosal exfoliation.\nOn day 12, the patient’s dyspnea was extremely severe, and his autonomic inspiratory drive was so strong that muscle relaxants were required to maintain deep sedation. However, the respiratory compliance was attenuated and the arterial pressure of carbon dioxide increased rapidly as a result. The patient eventually developed severe carbon dioxide retention, respiratory acidosis, and distributed shock (), which led to his death on day 13.\nWhen the patient was transferred to the ICU, his serum levels of alanine aminotransferase and aspartate aminotransferase were 184 and 275 IU/L, respectively. His total bilirubin level was 60.7 μmol/L, and his direct bilirubin level was 41.0 μmol/L; both decreased slightly after hemoperfusion treatment. Vitamin C, vitamin B6, N-acetyl-L-cysteine, and glutathione were intravenously infused for antioxidation. Additionally, magnesium isoglycyrrhizinate injection, ademetionine 1,4-butanedisulfonate for injection, and ursodeoxycholic acid capsules were administered for liver protection from the beginning of hospitalization.\nThe patient’s total bilirubin and direct bilirubin levels increased again during CRRT maintenance (). An abdominal CT scan showed no reduction in liver volume on days 3 and 6. However, the prealbumin level was independent of the abnormal liver function. Both the prothrombin time and activated partial thromboplastin time were also normal.\nThe troponin level was closely monitored after admission, and the troponin I level slightly increased to a maximum of 0.36 ng/mL. In addition, bedside ultrasound monitoring showed no obvious abnormalities in cardiac function.
An 80-year-old Caucasian man who initially presented with back pain was found to have a 9.8 cm infrarenal abdominal aortic aneurysm on computed tomography (CT). This was suitable for endovascular aneurysm repair (EVAR) but the left common iliac artery (CIA) was short, necessitating the extension of the left iliac limb into the external iliac artery (EIA). The decision was made to proceed with an on table coiling of the left internal iliac artery (IIA) and EVAR. However, a tortuous right CIA made coiling of the left IIA extremely difficult and following a prolonged attempt, the decision was made to proceed with EVAR without coiling the IIA (Fig. ).\nOn a 1-month follow up CT the left limb extension had disengaged from the main body, resulting in a large Type 3 endoleak. The disengagement was presumed to be due to the tortuosity of a heavily calcified external iliac artery returning to its anatomical position on removal of the Meir wire (Boston Scientific). The left IIA remained patent and acted as an outflow for the type 3 endoleak. The left limb was then realigned successfully with termination of the type 3 endoleak.\nOn a follow up CT 1 month post repair, the left IIA remained patent with a large type 2 endoleak demonstrated but the sac size remained static (Fig. ). The failure of the IIA to occlude was presumed due to the cavity created within the aneurysm sac from the type 3 endoleak. Due to the large size of the aneurysm and the relatively large size of the type 2 endoleak the decision was made at multi-disciplinary team meeting to embolise the type 2 endoleak.\nAs antegrade access to the left IIA was not possible due to the presence of the stent graft, an attempt was made to access the internal iliac artery via the buttock. Direct sac puncture was not possible as the endoleak was located within the pelvis and surrounded by bony structures and pelvic viscera. The intention was to puncture the posterior division of the IIA but visualisation with ultrasound was limited and a vessel was punctured, which on subsequent angiography was shown to be the anterior division of the left IIA (Fig. ). The left IIA was embolised using Spirali (Pyramed) coils in a retrograde manner. Due to the absence of a solid structure which to compress the access vessel against, especially as the anterior division of the IIA had been punctured, the arteriotomy needed to be closed using an Angio-Seal (Terumo). Correct intraluminal placement of the Angio-Seal (Terumo) is dependent on backflow of blood into the Angio-Seal (Terumo) sheath from the artery. However, as the left IIA had been embolised, backflow from the Angio-Seal (Terumo) was non-existent. The angioseal was not visualised on ultrasound and therefore a 4 French dilator was inserted within the Angio-Seal (Terumo) sheath through which contrast was injected to directly visualise the tip to the sheath within the artery (Fig. ). The sheath was then withdrawn until just inside the vessel lumen and the plug deployed with immediate haemostasis (Fig. ). This was an off-label use of the Angio-Seal (Terumo).\nA follow up CT 1 month post procedure revealed resolution of the Type 2 endoleak.
This is a case of a 59-year-old morbidly obese female with situs inversus totalis who presented for a laparoscopic sleeve gastrectomy. Her BMI was 38 (height 4 ft 11.5 inches, weight 188.2 pounds), and she had a lifelong history of morbid obesity and obesity-related comorbidities, including obstructive sleep apnea requiring a continuous positive airway pressure machine, an elevated hemoglobin A1c (5.8) and a fasting blood glucose increasing her risk of developing diabetes mellitus, and degenerative joint disease which significantly impacted her ability to exercise. The main challenges she identified in losing weight involved eating carbohydrate rich foods, overeating during meals and limited activity due to musculoskeletal pain. The patient had made multiple attempts to lose weight through commercial dieting programs but had been unsuccessful. The patient also completed a six-month medically supervised diet through her primary care provider, which also included working closely with a bariatric registered dietician, following a strict diet of about 1800 calories/day and performing modified exercise, about 120 minutes/week. Despite these intense medical weight loss efforts, she was unable to maintain a healthy weight. The patient was motivated to try bariatric surgery after she witnessed the significant weight loss success her daughters had from this intervention. Her greatest hope from the bariatric surgery was to be healthier and to alleviate her obesity-related comorbidities.\nThe patient’s surgical history included cesarean section and evacuation of an ectopic pregnancy. She was a former smoker, quitting over 25 years ago, and has no other history of substance or alcohol use. Family history is positive for obesity, diabetes, hypertension, coronary artery disease and hypercholesterolemia. She did not take any medications, including supplements, except for Ibuprofen 800 mg 1–3/daily for musculoskeletal pain.\nThe patient underwent a comprehensive evaluation and treatment plan prior to the surgery including: psychiatric evaluation and clearance, nutritional consultation with a registered dietician, education about bariatric surgery and pre/post op expectations, routine preoperative labs, UGI, esophagogastroduodenoscopy (EGD), pulmonary function analysis, and a cardiovascular exam. Situs inversus totalis was confirmed with abdominal X-ray and CT, and echocardiogram. Pre-procedural evaluation with UGI revealed mild gastroesophageal reflux observed to the level of the distal one-third esophagus and small sliding-type hiatal hernia. EGD revealed normal esophagus and duodenum. Stomach biopsy was obtained for antral gastritis, and no helicobacter organism was identified. Abdominal ultrasound confirmed fatty liver disease. Pulmonary function analysis including spirometry, lung volumes and diffusion was normal. A pre-procedural cardiovascular exam was performed due to dextrocardia to exclude other structural cardiac abnormalities. 2-D echocardiography with M-mode demonstrated a left ventricular ejection fraction of 60%, no structural abnormalities in the aortic, pulmonic, tricuspid and mitral valve, or right and left atrium and ventricles. Color Doppler and continuous and pulse wave Doppler demonstrated mild pulmonic regurgitation and mild tricuspid regurgitation.\nThe patient discontinued the Ibuprofen more than one month before her surgery and began a low-calorie liquid diet two weeks prior to surgery. On the day of surgery, preoperative antibiotics, Cefazolin, were given within 60 minutes before the first incision. Prior to induction, the patient received 5000 units of heparin subcutaneously and sequential compression device (SCD) boots were placed for deep vein thrombosis (DVT) prophylaxis. No beta-blockers were administered. The patient was placed in the supine position and general anesthesia was induced. A Foley catheter was placed and the patient was supported with positioning devices including: arms on padded arm boards, gel pad under left axilla, footboard with gel pads, hover mat and a bariatric safety belt over patient’s thighs. The abdomen was then prepped and draped in the standard surgical fashion.\nA Veress needle was used in the right upper quadrant to access the abdomen and insufflation was created to 15 mmHg. Veress was removed and replaced with a 5 mm trocar and the scope was placed. Additional trocars were placed in the following position: right and left 5 mm lateral trocars, right and left 12 and 15 mm supra-umbilical trocars. The placement of all retractors and graspers was adjusted accordingly to the mirror image anatomy of the intraabdominal organs. The primary surgeon was positioned on the left side of the patient and the assisting surgeon on right side of the patient. Situs inversus totalis was confirmed with the majority of the liver oriented to the patient’s right, spleen on the right, greater curvature of the stomach on the right and gallbladder on the left.\nThe operating table was placed in the reverse Trendelenburg position. The patient’s right-sided half of the liver was retracted cephalically using a Nathanson retractor to expose the vicinity of the esophageal hiatus. The peritoneum over the cardia was incised using the Ethicon Harmonic scalpel, and the plane between the cardia and the left crus of the diaphragm was opened to expose the right diaphragmatic crus. No hiatal hernia was present. A point 5 cm proximal to the pylorus along the greater curvature of the stomach was marked corresponding to the incisura angularis just proximal to the crow’s foot of Latarjet’s nerve. The vessels along the greater curvature and all the short gastric vessels were sealed and divided using the Ethicon Harmonic scalpel, freeing the greater curvature and the fundus of the stomach. A 42-French bougie was placed and oriented towards the antrum along the lesser curvature (Figure ). The stomach was stapled and divided alongside the tube in a vertical fashion towards the angle of His (Figures -).\nAn Ethicon Echelon Flex triple staple line power stapler with a total of one black, three green, and one gold staple loads was used. All staple loads were 60 millimeters in length with staple line bio-absorbable reinforcement. Hemostasis at the external staple line was achieved (Figure ). The stomach was removed from the abdomen via the left-sided 15 mm trocar (Figures , ). Intraoperative endoscopy was performed using a 5 mm Olympus Ultrathin gastroscope revealing no areas of stenosis, internal staple line bleeding, nor staple malformation or leak seen. Total operation time was 108 minutes. There were no post-operative complications.\nPost-operative care included pain management and DVT prophylaxis with SCD, heparin and early ambulation four hours post-surgery. A UGI was performed on post-op day 2 with normal findings for a post-bariatric surgery evaluation and clear fluids were started. The patient was discharged on post-op day 3 without any complications. Upon discharge, the patient had a treatment plan that was previously discussed that included instructions for diet and medications, including vitamin supplementation and antacids. At her three-month follow-up, the patient was still without complications and very pleased with her weight loss of 46.2 lbs, weighing 142 lbs (BMI 29).
The patient was a 57-year old female, five years post-menopausal, without hormone replacement therapy. Past medical history was positive for hypertension. The patient was diagnosed with adenocarcinoma of the parotid gland, for which she underwent total parotidectomy and ipsilateral neck dissection. The pathology report indicated an adenocarcinoma, 1.4 cm in size, grade 3, with perineural and lymphovascular invasion, and involvement of one lymph node adjacent to the parotid gland. Systemic evaluation included chest computerized tomography (CT) and bone scan, which demonstrated no evidence of metastatic disease. The patient received adjuvant radiotherapy to a total dose of 70 Gy to the tumor bed and involved lymph node and 50 Gy to the ipsilateral neck.\nTen years later, she presented with respiratory distress after a trans-Atlantic flight. She underwent CT angiography (CTA) to rule out pulmonary emboli. The CTA was negative for emboli, but showed numerous small, bilateral, lung nodules (largest diameter 1 cm). The respiratory distress resolved with conservative and supportive treatment. Further evaluation with fluorodeoxyglucose positron emission computed tomography (PET-CT) scan showed an uptake in her left breast and internal mammary chain, lung and liver. A biopsy from a lung nodule was positive for adenocarcinoma similar to the primary parotid gland tumor. A biopsy from her breast lesion indicated ductal carcinoma in situ (DCIS). The patient underwent left lumpectomy and sentinel lymph node dissection. The pathology report showed DCIS with a focus of invasion. Adjuvant radiotherapy was applied to her left breast and then she was started on anti-hormonal treatment using letrozole as adjuvant treatment. As the patient was asymptomatic and the metastatic disease was slow growing (according to repeated chest CT scan), it was decided to continue with letrozole for the breast DCIS, with follow-up by imaging of the lung lesions.\nThree years later, there was evidence of progressive disease. PET-CT showed new uptakes in the mediastinal lymph nodes, right lung hilus and left mandible. After a discussion with the patient and a multidisciplinary team, it was decided to send the histology for a molecular profiling (MP) analysis. MP was conducted on paraffin-embedded tissue taken from the 2008 left lumpectomy of a metastatic lesion. The MP analysis was performed with the Caris Molecular Intelligence (CMI™) tumor profiling service (Caris Life Sciences, Irving TX) at the Caris Life Sciences laboratories (Phoenix AZ) (Von Hoff et al. ). The analysis included immunohistochemistry (IHC) staining of 10 molecular targets, FISH to identify amplification in cMET and HER2. RT-PCR was used to determine gene expression of 12 genes and 6 genes were sequenced by Sanger sequencing ((EGFR, KRAS, NRAS, C-KIT, PIK3CA and BRAF). Of the molecular targets analyzed, agents associated with potential benefit were cisplatin due to low expression of excision repair cross-complementation 1 (ERCC1) by RT-PCR, taxanes due to high expression of transducin-like enhancer of split 3 (TLE3) by RT-PCR, and anti-androgens due to high expression by IHC. The estrogen receptor (ER) (Figure ), and progesterone receptor (PR) (Figure ) status evaluated by IHC were negative and weakly focally positive respectively; however, androgen receptor (AR) (Figure ) was strongly positive in 90% of cells with +2 level of staining. Therefore, we decided to start treatment with bicalutamide 50 mg daily and to continue the adjuvant treatment of the DCIS with letrozole. PET-CT after three months indicated a complete response in the pulmonary nodules (Figures and ). The patient is currently 18 months after starting bicalutamide, with no evidence of progression in her pulmonary nodules originating from the SGC, while continuing adjuvant treatment with letrozole for her DCIS for 5 years as acceptable in breast cancer. She will continue treatment with bicalutamide until proof of disease progression.
The case of this report is a 36-year-old married, nonsmoking, non-alcoholic female with primary infertility since 9 years of married life undergoing continuous interventions and repeated ART. Previous specialized evaluations include hormonal assays such as follicle-stimulating hormone, luteinizing hormone, estradiol, thyroid function test and AMH; ultrasonography (USG); and karyotyping. Investigations into the male factor revealed OAT. She underwent three courses of intrauterine insemination (IUI) and three courses of ICSI which were unsuccessful. AMH value before fourth ICSI was very low after which she was advised for an ICSI with a donor ovum.\nThe patient got married in the year 2005 at the age of 27 years. Investigations carried out on both partners after 2 years of married life revealed OAT. After correcting the sperm count with oral medications, she underwent three cycles of IUI in 2010 which was unsuccessful. Due to OAT and failed IUIs, she was subjected for ICSI after necessary hematological evaluation and hormonal assays which came out to be normal. AMH value was 2.5 ng/mL before first ICSI on November 3, 2011. Six eggs were retrieved and one embryo was transferred, but there was no rise in human chorionic gonadotropin (hCG) value. Second ICSI was on May 10, 2012, in which three eggs were retrieved and three matured embryos were transferred. hCG value raised to >1500 mIU/ml on May 31, 2012, but had tubal abortion on June 3, 2012. Third ICSI was on July 21, 2013 and three embryos were transferred from five retrieved eggs. She had threatened abortion on August 19, 2013, and USG on August 20, 2013, showed single intrauterine gestational sac with subchorionic hematoma and a repeated scan on September 3, 2013, revealed an empty endometrial cavity. Routine investigations carried out on January 2, 2014, before fourth ICSI revealed very low AMH value (0.07 ng/mL). She was advised for undergoing next ICSI with a donor ovum due to the low AMH value. As the couple was not willing to accept a donor egg, they visited our outpatient department (OPD) on February 1, 2014 in the hope of undergoing ICSI with her own ova by improving the AMH value with Ayurvedic treatment. Her medication includes Eltroxin 50 mcg for hypothyroidism since 2010. Her history was relevant only for chickenpox in 2005 and jaundice in 2012. Family history was negative for any premature ovarian failure or low AMH. Her personal history revealed a regular bowel habit and sound sleep. Her appetite was apparently normal and the tongue was uncoated. She attained menarche at the age of 12 years with 2–3 days’ duration in 26–28 days’ interval. The amount was scanty with noticeable vaginal dryness since 3 months. Her obstetric history was gravida 2 abortion 2 ectopic pregnancy 1 (G2A2E1). On examination, she was calm and pain free. Her blood pressure was 120/80 mmHg, pulse rate 70/min and body mass index 23 kg/m2. She is of Vata Kapha Prakṛiti with Madhyama Satva (moderate mental strength) and Madhyama Koshtha (moderate bowel). Her physical examination and examination of external genitalia did not revealed any abnormal findings. Per speculum examination showed a healthy nulliparous cervix without any significant abnormality. Bimanual examination revealed an anteverted mobile uterus with a negative cervical motion tenderness. Medical history of the patient is detailed in .\nIn the view of symptoms of Artava Kshaya (hypomenorrhea), the present case was diagnosed as Dhatukshayajanya Vandhyata (infertility due to depletion of body tissues). The assessment was done by comparing baseline AMH value with repeated evaluation after 3 months of Shamana (oral medication) and Shodhana treatment (purification therapy). Baseline AMH value was 0.07 ng/ml (reference range: 2–6.80).\nThe therapeutic plan was to administer Shamana Chikitsa (oral medication) followed by Shodhana Chikitsa (purification therapy). Shamana Chikitsa was done for 3 months with Vaishavanara Churna and Mahanarayana Taila. As her clinical symptoms and AMH value after Samana Chikitsa showed satisfactory improvement, she was subjected to Shodhana Chikitsa. Initially, her Agni was corrected by Dipana-Pachana (improving digestion) with Vaishavanara Churna as a Purvakarma (preparation) of Shodhana. She attained Nirama Lakshana (signs of digestion of Ama) by 2 days after which Accha Snehapana (intake of oil) was started with Mahanarayana Taila. Samyak Snigdha Laksana (signs of proper oleation) was observed after 6 days of Snehapāna. Mridu Virechana (mild purgation) was performed on the administration of Trivritlehya. After Virechana, she was subjected to Yoga Basti and Uttara Basti (medicated enema). After completion of Shodhana therapy, she was discharged from the hospital [].\nAfter 3 months, the patient was advised to have a follow-up in the OPD with a reassessment of AMH value. It was observed that there was a marked improvement in the AMH value as shown in .
We present the case of a 48-year-old male, who was evaluated by the medical genetics service because he had noticed weakening of his voice with a high pitch since age 35, associated with premature graying since his 30s and skin lesions since about the age of 40. At the age of 32, bilateral cataracts were diagnosed and at 44 he was diagnosed with diabetes mellitus, currently on oral hypoglycemic agents. Additionally, he has hypothyroidism and hypertriglyceridemia in management and calcification of the Achilles tendon. Patient endorses lack of an early adolescent growth spurt; however, final stature is similar to his other 3 siblings (164 cm). Patient reports he had no child by choice.\nPatient is product of the union of consanguineous parents (second cousins) and has a 49-year-old brother with similar clinical characteristics, including voice changes since the age of 28, bilateral cataracts at age 29 (subsequently presents complications from corneal ulceration and is currently legally blind), and premature graying since age 33, moreover, scleroderma-like skin changes since his 30s and diagnosis of type 2 diabetes mellitus at age 35. His brother also endorses no child by choice. No other complications such as atherosclerosis, dyslipidemia, hypertension, osteoporosis, or tumors were reported.\nUnfortunately, patient's brother and parents declined genetic testing. There are no other relatives with clinical suspicion of WS.\nPatient states maternal aunt has unspecified type leukemia and father with a history of acute myocardial infarction at age 65 and a diagnosis of melanoma at age 85. Maternal uncle diagnosed with lung cancer at age 72 and maternal grandfather with prostate cancer diagnosed at age 73.\nOn initial physical examination, he appeared much older than his age with “bird-like” facial appearance, beak-shaped nose, and bilateral cataracts, his voice was high-pitched and his hair and eyebrows were scarce and markedly gray. He had thin upper limbs with decreased subcutaneous fat and truncal obesity (). Moreover, we found short stature, hypogenitalism, lower limbs with markedly atrophied skin and subcutaneous fat, abnormal pigmentation of the skin and hyperkeratosis, and flat feet (Figures and ).\nWRN gene sequencing identified the homozygous variant NM_00553.4: c.2581C>T (NP_000544.2: pGln861Ter). WRN gene sequencing report can be found in Supplementary . This variant generates a stop codon at position 861 and has been classified as pathogenic and previously described in homozygous status in a Caucasian patient from the United States in 2006 [].\nLaboratory findings included normal renal function, high blood glucose (164 mg/dl), elevated glycosylated hemoglobin (9.4%), and elevated triglycerides (324.6 mg/dl) with normal cholesterol (162.4 mg/dl). EKG showed an elevation of the J point by early repolarization. Abdominopelvic CT-scan showed bilateral renal cysts, small umbilical hernia, and no fatty liver. Testicular ultrasound showed decreased bilateral testicular volume mainly left side.\nRegular screening for malignancies is recommended for patients with WS, due to the high risk of early-onset neoplasms. Also, it is very important to rule out cardiovascular and metabolic diseases during the follow-up of these patients. Our patient is still under periodic clinical observation and follow-up. Currently, he is on treatment with oral hypoglycemic agents for DM2 with adequate glucose control and in treatment of hypertriglyceridemia. Until now no signs of atherosclerosis or cardiovascular disease have been detected. However, he was recently diagnosed with refractory cytopenia with multilineage dysplasia, a form of myelodysplastic syndrome, which has required multiple transfusions.\nAccording to a clinical history, the patient's brother is being monitored for inadequate control of diabetes mellitus and severe skin lesions that have been difficult to treat, but no cancer has been documented.
The patient is a 20-year-old single woman who was referred to the ER by her group home for altered mental status. The staff declared her missing when she did not return from work. When she met with the treatment team on the psychiatric unit, she offered a scattered story related to the events prior to her admission. The team asked for clarification and further explanation, but the following story was pieced together.\nShe aged out of her group home with her most recent birthday and was forced to leave. She was left homeless and living on the streets for months. She narrowly escaped being raped on one occasion and desperately needed a place to live. She was walking the streets one day hoping to meet someone who would take care of her. She eventually met a nice man who brought her to his home and stayed with him overnight. However, the next day he started using cocaine and she needed to leave immediately. She was not threatened or abused by the man but perceived the environment as unsafe given his substance use. She asked for the custody of his two-year-old daughter in fear of the environment. She starts referring to the toddler as if she is her own child. The man agreed to her taking the child without any questions. The patient and the toddler left to begin walking down the turnpike. Her mother later joined the journey despite the patient’s warning of the long walk ahead of them. Eventually, a police car stopped, and an officer started walking toward them. He asked them to get into the car and they drove to a strange house. The patient went into the house with the officer, leaving her mother and toddler in the car. A woman in the house offered her some pills which she and the officer insisted the patient to take. The officer eventually left and drove off with her mother and the two-year-old in the car. She developed severe abdominal discomfort after taking the pills and an emergency medical services (EMS) vehicle brought her to the hospital.\nThe patient had no memory of the information obtained from the group home while she was in the ER. She did not remember her group home, staff, current medications, deteriorating mental status, hospitalization five weeks ago, intensive outpatient program, place of employment, or anything else that occurred while living in the home. The more questions the team asked the more puzzled the patient became.\nMental status examination demonstrated a well-groomed woman who appeared her stated age. Her hair was somewhat unkempt, but she was generally well-groomed. She made continual facial expressions of disbelief and confusion, maintained good impulse control, and displayed normal psychomotor activity. Her speech was over productive with normal volume, rhythm, and rate. Her mood was “okay” and affect was in full range. Her thought process was circumstantial. Her thought content was illogical only when discussing the history of present illness, otherwise linear. She denied hallucinations and no delusions were elicited.\nThe patient was admitted to the hospital approximately a month prior and discharged on olanzapine. Subsequently, olanzapine was restarted in the ER and she reported no side effects. When learning ziprasidone was given to her in the home, she did not want to restart the medication due to the previous abdominal discomfort.\nThe hospital internist did not find any physical abnormalities during exam. Laboratory data, including urine toxicology and blood alcohol level, were of no clinical significance. Computed axial tomography (CAT) scan of the head without contrast was benign. The consulting neurologist denied any acute findings and the electroencephalogram did not demonstrate any seizure activity. The patient received a diagnosis of dissociative amnesia with dissociative fugue.\nThe hospital was flooded with calls from the patient’s case managers that offered further needed information. She had been residing in a group home for the past seven months. She had a history of schizoaffective disorder, borderline personality disorder, and PTSD. She had an extensive history of trauma and an extremely strained relationship with her mother as a result. She was compliant with her medications, an active participant in her intensive outpatient program, and a responsible employee. She served as a role model for the other residents and was thriving in all aspects of her life until she started to decompensate approximately six weeks prior.\nShe was becoming extremely worried about her upcoming birthday and associated departure from the group home. She was constantly reassured of an appropriate transition, yet she was certain of homelessness. She started becoming paranoid and acting bizarre. She was often engaged in self-dialog, cursing at herself and speaking with a Spanish accent. She believed her belongings were being stolen and frequently screamed at the other residents. On the day the patient went missing she accused her roommate of theft which escalated to a physical altercation. She angrily left the home and went to work. She completed her shift and told her co-workers she was waiting for a ride, although she usually went home on her own. She remained outside until all the employees left. The patient never returned home. Early the next morning she was reported missing. A police officer found her wandering along the highway that morning approximately 10 miles from her place of employment. He brought her to the group home where she had no memory of the staff or facility. She hesitantly agreed to take her ziprasidone, complained of abdominal distress, and accused the staff of poisoning her. EMS was called, and she was brought to the hospital. \nDuring her next encounter with the psychiatrist she described her memory loss to be like a nightmare. She remained completely puzzled with the reports of the last seven months. She often displayed a look of complete disbelief each time the psychiatrist asked about or revealed more information related to that time. When asked about the ongoing conflict with her mother, she quickly shook her head in disagreement and reported her to be supportive. She was skeptical about going back to the facility as it was an unknown place to her. However, she agreed to return if the treatment team thought it to be appropriate.\nWhile on the unit she was calm, cooperative, and pleasant. She was compliant with her medications and participated in the group therapy and milieu of the unit. She was interactive with her peers and often spoke to her mother on the phone. There were no signs of mood instability or psychosis. The hospital staff reported her to be helpful, appropriate, and with good behavior control. The patient’s case workers and support staff were hesitant for her return given continued amnesia, the other residents’ fears, and previous psychosis and violence. They did not feel it was appropriate for her return with such memory loss. The case workers thought the amnesia itself is enough to have the patient transferred to a higher level of care or discharge to a more supervised facility. Their apprehensions and requests were understandable; but the patient was mentally stable and did not meet the criteria for commitment. They were educated, had all their concerns addressed, and agreed to take her home with a discharge meeting prior to her return. She was discharged to the group home and would return to her intensive outpatient program and monthly sessions with the consulting psychiatrist.\nOne week later, the psychiatrist called to learn that the patient had not recovered any parts of her memory but had a smooth transition back into the facility. She was doing well and getting along with the other residents. She even started working at her previous place of employment. Her employer was willing to retain her given her work history. The psychiatrist called again in six months and learned she had transitioned into her new apartment. Faint pieces of memory had returned but she remained generally amnestic to those seven months. She continued to remain compliant with her psychiatric treatment, works, and does well.
A 74-year-old, smoker, male patient came urgently on the 30 December 2018 to the Accident and Emergency Department of our clinic, reporting heavy rectal bleeding and intense pain during defecation for 2 days; however, there were no clinical signs of anemia. Past medical history included coronary artery disease (CAD) and angioplasty with the placement of three stents 6 years ago. As a result, he was on anticoagulation protocol with 75 mg clopidogrel and 75 mg aspirin. There were no other medications and no known drug allergies. Furthermore, the patient has never had any similar symptoms and neither has ever suffered from hemorrhoids in the past. Initial physical examination showed HD Grade III, with three nodules protruding outside the anal canal and required manual reduction; the two nodules at 3 and 7 o’clock, respectively, were thrombosed and bleeding, while there was also a smaller one at 11 o’clock. Once the pre-operative testing protocol was ready, a colonoscopy was performed to exclude any other additional anorectal diseases or possible differential diagnoses for bleeding, and the findings were normal. Due to the urgent character of the case and taking into consideration the blood thinners the patient was on, the surgeon decided, after having the informed consent of the patient, to proceed with the expert laser intrahemorrhoidal therapy (ELITE) minimal invasive technique for the hemorrhoids with the use of a 1470 nm diode laser in order to limit the potential complications including any post-operative (post-op) bleeding.\nAn urgent bowel preparation took place with a cleansing enema, and surgery took place under local anesthesia in the peri-anal region along with sedation. For prevention of an infection, a dose of IV antibiotics (metronidazole 500 mg) was administered to the patient during the procedure and it was repeated every 8 h until the patient’s discharge. The ELITE was initiated with the use of a 1470 nm laser unit. A designated optic fiber with a specific conical glass tip (hemorrhoid probe) helped with the treatment of each hemorrhoidal nodule. The laser unit was set at 7.0 W/3 s and single pulse, and each nodule received the dedicated energy homogenously in pulses for better control during the thermal seal-off of the hemorrhoidal nodules. Total operating time was 31 min. and the energy delivered to the area was 1270 J, without the need for any sutures.\nRight after the end of the procedure, the patient got admitted for a precautionary overnight stay due to the possible, yet minor, risk of bleeding due to his CAD and medications.\nEighteen hours after the surgery, the pain experienced by the patient was a Level 4 on Visual Analog Pain Scale (VAPS), compared to a pre-operatively 10/10. Clinical post-op examination showed normal findings with extended tissue ecchymosis due to the large amount of energy given. The patient was discharged the day after the surgery and returned to daily activities after 5 days; he was prescribed only with paracetamol 500 mg as needed for analgesia in the possibility of pain during the next 48 h. First bowel movement occurred 30 h after the surgery without bleeding, with a slight discomfort, yet without any difficulty. During the follow-up visit that took place a week later, the reported pain dropped to 2/10 (VAPS), without any discomfort. On the second follow-up, 15-days post-op, physical examination confirmed that the hemorrhoidal piles have been substantially shrunk and the healing process was normal. Next follow-up visits took place on the 6th, 12th and 18th months after surgery, with the latter being in June 2020; there have never been any complications, any bleeding from the points of entry of the optic fiber, and of course, no signs of recurrence of the hemorrhoidal nodules.
A 38-year-old man with schizophrenia presented after his second suicide attempt through an overdose with 48 tablets of burotizolam, 42 tablets of haloxazolam and 14 tablets of levomepromazine. The patient's childhood and adolescent development was normal. He was a good student and an active soccer player in high school. His social skills were standard, and he had no family history of mental illness. When he was 23 years old and a fourth year university student, he became convinced that he was being observed and he withdrew from social activities. His parents brought him to a psychiatric hospital, and he was diagnosed with schizophrenia according to DSM-IV-TR (). The prescribed medication worked well and he was able to graduate from university at 27 years old. After graduating, he worked part time in a convenience store or at a nursery for several years. He then started to work at a distribution business under a handicapped employment program. His father committed suicide 3 years before he first presented at our hospital and a friend also died from a sickness. Because his auditory hallucinations repeatedly told him that he was responsible for their deaths, he could not stop blaming himself for their passing, in spite of his mother and brother telling him that he was not responsible. He was pessimistic about his future partly because he was able to earn only a meager income. In order to increase his income, he started a second part-time job at a supermarket in addition to his distribution job. He slept less and felt the accumulation of fatigue. He started to stockpile sleeping medications and he eventually took 76 tablets of brotizolam and 30 tablets of eszopiclone. The next morning his mother found him unconscious and called an ambulance. His mother brought his empty medicine containers to the hospital. At his first presentation, his physical examinations and vital signs were normal. He appeared to be very sleepy, but he managed to speak. The emergency department doctor ordered a blood test, a chest x-ray, an electrocardiogram test, a urine toxicology test, and a computed tomography brain scan. All results were within normal range, except a positive result for benzodiazepine in his urine and a slightly elevated white blood cell count (10.92 × 103/μL). The emergency doctor enlisted a psychiatric doctor to evaluate his mental state. The patient claimed that his auditory hallucinations sounded like someone was booing in addition to radio sounds from a distance. He also claimed he was being tracked by the police. He admitted suicidal ideation and reported that he was sad because he could not die. Because his depressive symptoms occurred 4 weeks prior to his first admission, the authors carefully excluded the possibility of schizoaffective disorder and depressive disorders or bipolar disorder. However, the patient did not show manic symptom or markedly diminished interest, and his depressive thoughts seemed to ease shortly after his admission. Obviously, his mood episodes have been present for a minority of the total duration of the active and residual phases of illness; however, his memory changing delusion and auditory hallucination remains continuously. Furthermore, he showed negative symptom that he had withdrew from social activity except working. The authors diagnosed schizophrenia according to DSM 5 (). His decreased ability to discriminate between his thought and true memories as mentioned previously suggests the presence of disturbance of the self which also supports this diagnosis (). The authors prescribed risperidone 6 mg, brotizolam 0.25 mg, and eszopiclone 2 mg. Soon after the treatment started, he became calm and claimed his suicidal ideation disappeared. However, during the patient's second hospitalization, 6 months later, he admitted that he had lied. He wanted to go home quickly so he pretended to be healthy. He subsequently obtained a distribution job contract for the coming season by himself and he was supposed to be followed by a nearby clinic as a condition of his hospital discharge. He started his distribution job but he could not work regularly. Again, he wanted to earn more money so he started attending lectures to get a healthcare worker license. Consequently, his sleep time was reduced and he started to feel life was troublesome once again. He subsequently overdosed as mentioned previously. The next morning, his mother brought him to the emergency department again. She had no idea when he attempted to commit suicide but she last saw him the previous night at 10 p.m. His mother brought his empty medicine containers. His vital signs were normal, and he managed to speak. The emergency doctor conducted a blood test, a chest x-ray and a computed tomography brain scan. All the results were normal, except an elevated white blood cell count (12.16 × 103/μL), creatine kinase (429 U/L), and chloride (109 mmol/L). His mother brought with her more than 100 risperidone tablets. It became obvious that he had not taken his pills regularly. The authors thought his adherence worsened during his psychotic period and started a long acting injectable antipsychotic (LAI). Because the patient worked regularly, the authors choose an injection given once in a 4-week period. Furthermore, because several studies showed it made significant improvements in the quality of life (), the authors chose aripiprazole LAI at 400 mg. The authors also prescribed 20 mg of suvorexant per day and gradually discontinued brotizolam 0.25 mg and flunitrazepam 2 mg because the authors were concerned about a possible third suicide attempt while using benzodiazepine. Because both of the patient's admissions were associated with poor sleep, the authors examined the patient by polysomnography (PSG) and a multiple sleep latency test (MSLT) to exclude comorbid diseases such as sleep apnea syndrome or restless legs syndrome. As shown in Figure , he woke frequently during his sleep (25.6 times per hour on average as shown in Figure ) and he lived with excessive daytime sleepiness (he fell asleep within 2 min; on four out of five trials during the MSLT, as shown in Figure ). His Apnea-Hypopnea Index (AHI) was slightly elevated (5.1 times/hour), and respiratory events were not associated with significant desaturations (the minimum SpO2 was 95%). His BMI was 19.8. Malocclusion or tonsil swelling was not observed. Figure shows the patient's sleep log. The patient did not show sleep phase advance or delay. The patient's Pittsburgh Sleep Quality Index (PSQI) score was 13, while over 5 points on the PSQI represents insomnia (). Two months after his second admission, he was discharged while being prescribed suvorexant 20 mg, and chlorpromazine 25 mg per day in addition to aripiprazole LAI 400 mg per month. His Brief Psychiatric Rating Scale (BPRS) () dropped form 48 at admission to 42 at discharge. Six months after his second admission, the authors and the patient started CBT-i according to the CBT-i therapeutic manual (). The authors also referred to the four causes cited by Chiu et al. (): (a) beliefs that sleep problems cannot be changed; (b) trauma and adversity; (c) lifestyle choices and lack of motivation; (d) medication side effects and the 12 problems cited by Waite et al. (): (a) Poor sleep environment; (b) Lack of daytime activity; (c) Lack of evening activity; (d) Disrupted circadian rhythm; (e) Sleep as an escape from distressing experiences; (f) Fear of bed; (g) Nightmares; (h) Night-time awakenings; (i) Sleep disrupted by voices/paranoia; (j) Worry; (k) Neuroleptic medication side effects; and (l) Reducing hypnotics. Our CBT-i consisted of eight sessions with each session ranging from 30 to 45 min. The first two sessions were educational sessions that attempted to find disturbances such as a misunderstanding of sleep hygiene or an inadequate sleep environment. In the other six sessions the authors and the patient tried to find other targets to tackle. For instance, the patient tried eating a carbohydrate (banana) before sleep, stopped checking his watch, warmed his body before going to bed, turned off small lights in his room, changed his routine of taking a bath before eating dinner to prevent him from taking a nap after dinner, bought a blackout curtain and an air conditioner. He also tried to wake up early in order to exercise in the morning instead of doing in the middle of the night because he believed he can fall asleep soon after the exercise. The whole course of sleep and psychological tendencies are shown in Figure . The patient's BPRS dropped to 24 and his PSQI dropped to 8. His sleep time increased steadily however, at his sixth session, he claimed that he could not sleep at night and he felt a strong sense of sleepiness during the day. His mental health care team consisted of two physician groups; with one group treating his psychiatric symptoms and the other group (the authors) treating his sleep abnormality. The first physician group increased the patient's chlorpromazine from 25 to 37.5 mg. The authors, as the second physician group treating the patient's sleep abnormality, discussed reducing the patient's chlorpromazine with the first physician group because the authors believed that his sleep troubles were not caused by a difficulty in falling asleep but by the dosage of chlorpromazine being too high for the patient's current ability to fall asleep which was gradually being strengthened by CBT-i. At the seventh session, the authors encountered another misunderstanding of the patient in which the authors believed the patient's headaches were being caused by a lack of sleep, while the patient used chlorpromazine as a painkiller. The authors prescribed acetaminophen 400 mg as a painkiller, and stopped the administration of chlorpromazine. At the eighth session, the patient claimed that he had almost no trouble sleeping except when he forgets to take suvorexant.
A 43-year-old, premenopausal, lady, was evaluated at an outside hospital for complaints of a lump in her right breast of three months duration. She also had severe bone pains and numbness in her chin, since the last one month. She was diagnosed to have metastatic breast cancer. Biopsy was suggestive of high grade infiltrating duct carcinoma which was further characterised as being estrogen, progesterone receptor positive and Her 2 neu 3+ by immunohistochemistry. Contrast Enhanced Computerised Tomography scans (CECT scans) of the chest and abdomen and Magnetic Resonance Imaging (MRI) of the brain, head and neck region revealed multiple lung, liver and bone metastases including the right acromion as well as parameningeal and right mandibular bony infiltration which was consistent with mental nerve neuropathy.\nShe was given palliative radiation to the painful right acromial lesion. She was then started on chemotherapy with albumen bound paclitaxel 100 mg/m2 weekly and transtuzumab 4 mg/kg as loading dose followed by 2 mg/kg weekly. Two to four hours following each dose of transtuzumab, she would have episodes of high grade fever of up to 39 °C associated with mild chills, resolving spontaneously without intervention over the next 1–3 days. After the fourth dose, she developed bilateral pneumonitis (non-neutropenic) for which she had to be admitted to hospital for treatment with intravenous antibiotics. The CECT scan of the chest revealed extensive bilateral patchy areas of air space disease with interstitial involvement. Two weeks later, she improved and was given another dose of transtuzumab and albumen bound paclitaxel and then discharged. A week after receipt of her last dose, she presented to our hospital emergency with complaints of respiratory distress and hypoxia. CECT Scan of the chest and abdomen revealed dense extensive right upper and left lower lobe para-mediastinal consolidation along with smaller, bilateral patches and air bronchograms consistent with a diagnosis of organizing pneumonia (Fig. a, b). Septal lines and interstitial opacities in the mid lower zones suggested lymphangitis. There was evidence of progressive disease with multiple liver and bone metastases. The patient was started on oxygen, antibiotics, intravenous dexamethasone and supportive care. In view of progressive disease, albumen bound paclitaxel was discontinued. Hormonal therapy comprising goserelin depot preparation 3.6 mg administered subcutaneously every 28 days and anastrazole orally 1 mg daily was commenced.\nFour days later, single agent transtuzumab was administered, to which she developed an anaphylactoid reaction with symptoms of acute breathlessness and hypotension. This was managed successfully with steroids, antihistaminics and intravenous fluids. The reaction resolved but her breathlessness did not and she in fact developed progressively worsening dyspnoea, with an oxygen saturation of 84% on room air, requiring supplemental oxygen, antibiotics and parenteral steroids. Her condition improved after 3 days of supportive care. Parenteral steroids were then stopped and oral dexamethasone 8 mg thrice daily was commenced. A bronchoscopy was conducted.\nThe biopsy revealed patchy intra-alveolar haemorrhage and fibrin deposition with collections of intra-alveolar foamy macrophages, patchy reactive type II pneumocyte hyperplasia and focal organizing pneumonia (Fig. a). There was also a localised interstitial infiltrate of polygonal cells forming tubular structures and a small ill-defined sheet (Fig. b). Changes suggestive of lymphatic invasion were evident. The polygonal cells were positive for estrogen receptor and GATA 3 (Fig. c) and negative for TTF1, consistent with metastatic breast carcinoma. There was no history of exposure to fumes and no microbiological or serological evidence of any pathogens or collagen-vascular disease.\nHer symptoms resolved over the next two weeks and she was discharged home. The CECT scan of the chest done at discharge (Fig. c, d) revealed marked reduction in the organizing pneumonia and interstitial infiltrates. Lapatinib 1250 mg daily was added to her treatment regimen. Dexamethasone was slowly tapered off over the next 4 weeks. At last follow-up, 8 weeks post-discharge, she was doing well on therapy.\nOrganizing pneumonia or bronchiolitis obliterans organizing pneumonia (BOOP) is one of the major Idiopathic Interstitial Pneumonias (IIP) (Travis et al. ). Typical radiological features include multiple alveolar opacities, usually bilateral and peripheral, often migratory, varying in size from a few centimeters to a whole lobe, with air bronchograms often present in consolidated opacities: as in our case. The disease is pathologically defined by the presence of buds of granulation tissue formed by fibrin exudates, fibroblasts, myofibroblasts and loose connective tissue in distal air spaces (alveolar spaces and bronchiolar lumen), although lung structure is preserved. Treatment tends to be steroid-responsive, though scarring may persist (Travis et al. ; King and Mortenson ). In our case, organizing pneumonia was held to be secondary and differentials included infections, malignancy, transtuzumab, paclitaxel or prior radiation. However the absence of any demonstrable pathogen and the occurrence of bilateral lesions outside the known extrathoracic radiation field precluded infections or radiation as a cause. The strong temporal association of hypersensitivity reactions with single agent transtuzumab and marked resolution of the disease process on stopping the drug, strongly suggests a role of transtuzumab in its aetiology. A probable causative or contributory role of malignancy in its aetiology, cannot be fully excluded.\nDrug-induced infiltrative lung disease (DI-ILD) is the most common form of anti-neoplastic agent-induced respiratory disease. Its spectrum includes non-specific interstitial pneumonia, eosinophilic pneumonia, hypersensitivity pneumonitis, pulmonary fibrosis, or organizing pneumonia (Travis et al. ; King and Mortenson ).\nThe association of malignancy with BOOP is an infrequently described clinical entity and mimics various respiratory conditions. In the largest retrospective study describing this phenomenon, 43 patients with an underlying diagnosis of cancer were found on lung biopsy to have BOOP as an isolated entity. All patients had received therapy including chemotherapy (71%) or radiotherapy 9(21%), only 7 had surgery alone. Two patients who had received extra-thoracic radiation developed BOOP 2–3 years after treatment, while others who received thoracic radiation developed BOOP ipsilateral to the radiation port. One case of breast cancer was also reported. Patients with solid organ tumours were more likely to have nodular or mass like radiographic abnormalities (81%) rather than diffuse infiltrates (19%). The opposite pattern was observed in patients with hematologic malignancies (22 vs. 67%). Most patients recovered on steroids or macrolides but the disease was fatal in 3 patients with haematological malignancies (Mokhtari et al. ).\nTranstuzumab is reasonably safe to use and only about 0.3% patients have serious reactions with features of anaphylaxis or bronchospasm, usually occurring within 2.5 h of drug administration (Cook-Burns ; Vahid and Mehrotra ).\nTrastuzumab-associated pneumonitis is a rare, potentially fatal side effect, presenting as hypoxemia, dyspnoea and respiratory failure. Common radiological findings are interstitial infiltrates with a ground-glass appearance and patchy foci of airspace consolidation. In the trial National Surgical Adjuvant Breast and Bowel Program (NSABP) B-31, of 2102 women enrolled, four patients in the trastuzumab group had interstitial pneumonitis, one of whom who died (Vahid and Mehrotra ; Perez et al. ). In the North Central Cancer Treatment Group (NCCTG) N9831 trial, of 1944 women, five patients in the trastuzumab group had grade 3+ pneumonitis or pulmonary infiltrates, one of whom who died (Perez et al. ; Pepels et al. ; Romond et al. ).\nOnly three cases of transtuzumab induced organizing pneumonitis have been described so far. One patient, 49 years old, developed it 6 weeks after starting transtuzumab and this resolved three months after its cessation. Another patient, 60 years old, developed it after 11 cycles and this too resolved three months after drug cessation (Radzikowska et al. ; Taus-García et al. ; Grudny et al. ).\nPneumonitis rates of 4% have been described with albumen bound paclitaxel in combination with gemcitabine, especially in patients with pancreatic cancer. However none of these episodes were Grade 3 or higher and were not fatal (Von Hoff et al. ).\nPaclitaxel is also known to cause pneumonitis with an estimated frequency of 0.7%, more so in combination with gemcitabine or irinotecan. Dyspnea, cough, hypoxemia, and pulmonary infiltrates usually develop 1 week to 3 months into therapy. Severe pneumonitis and pulmonary fibrosis resulting in death have been described but mild cases tend to resolve spontaneously (Khan et al. ).
A previously healthy 14-year-old female presented to the office with sharp, generalized abdominal pain and dark brown urine since the previous night. Additionally, the patient noticed that her whole body ached after running for two hours that same day. She was afebrile and had a cough and runny nose for the past three days. All of these symptoms were not preceded by trauma or Streptococcus infection within the past month. Her last menstrual period was 20 days earlier.\nOn observation, the patient looked well. All vital signs were normal. Physical examination findings were unremarkable (non-tender abdomen, no edema noted on extremities). Urine sampling demonstrated light yellow clear urine. A urine dipstick was not performed because it was unavailable in the office. Consequently, the patient was advised to call the office or go to the emergency room should she have the reemergence of abdominal pain or brown urine. Shockingly, the office was notified that the patient had been admitted to a hospital with acute renal failure the following day. She was in the hospital for three days before finally being discharged home to be followed up by the renal clinic.\nFour months later, the patient presented to the office with diffuse back pain of one day's duration. The pain did not radiate anywhere. She also presented with chest pain that had been going on for five months. The pain occurred mainly in the left lower breast area, and it typically increased with inspiration. It occurred roughly one to three times per week and, along with a cough, was associated with recreational sports. The patient stated that the last time she had any chest pain was the previous week. An electrocardiogram (EKG) taken four months earlier was unremarkable. She denied any palpitations, nausea, vomiting, and any dizziness during exercise or rest. Upon further questioning, it was discovered that her throat had felt ‘tight’ for the past year when running, and she often tried to cough because it hurt to swallow. This did not negatively affect her ability to participate until the end of the run, however. In general, she didn’t experience any fever, changes in weight, muscle weakness, or swelling of extremities. She denied having any episodes of dark brown urine ever since being discharged from the hospital. Consequently, the patient was diagnosed with exercise-induced asthma and back muscle strain.\nAt a follow-up visit four days later, the patient felt well, but her creatine phosphokinase (CPK) was 2,439 U/L (reference range: 26.0 - 192.0 U/L). This was observed even eight days after she played kickball for one hour. At that time, she was advised to avoid sports and to do a weekly CPK test for three weeks. Table shows the results.\nOn further questioning, the patient disclosed that eight months earlier, she had presented to a clinic with dark brown blood in the urine of three days duration (four days after the completion of her menses). The patient reported starting a strenuous exercise regimen on the day she started seeing the brown urine and had generalized exercise-related soreness. She mentioned having been in school sports (mainly basketball), which she had been doing for three years now. The basketball practice and games lasted up to a maximum of two hours, with the first 15 to 30 minutes generally being running. She did not participate in any weightlifting. On average, she drank two bottles of water and half a bottle of Gatorade a day. Urine dipsticks showed the presence of protein and blood in the urine. She was sent home with a diagnosis of dehydration due to strenuous exercise and not drinking enough fluids.\nThe patient’s past medical history was unrevealing. Occasionally, she took acetaminophen for pain as needed. The patient had been performing really well in school, honoring in all of her classes. She denied any smoking, alcohol consumption, or recreational drug usage. Her mother has attention deficit hyperactivity disorder (ADHD), hypertension, and arrhythmias. Her maternal grandfather suffered from a heart attack, and her maternal grandmother had melanoma three times. One of her second cousins suffered a cardiac arrest and was found to have a prolonged QTc (it was unclear whether this developed before or after the cardiac arrest). Her half-brother has an autism spectrum disorder and ADHD. Her full biological brother is very healthy.\nAt the time of the follow-up visit in the office, the patient weighed 132 lbs (59.9 kg, 74th percentile) and was 67 inches tall (1.7 meters, 92nd percentile). Her percentiles for weight and height have been the same since the age of three. Her body mass index (BMI) was 20.7 (62nd percentile). Muscle tone, strength, and mass were normal. There were no signs of calf muscle hypertrophy. Gower’s sign was negative (which was performed because the CPK levels were greater than 100 times the upper normal limit when she was previously hospitalized). In the hospital, the following were also unremarkable: CT scan, antineutrophil cytoplasmic antibodies (ANCA), C3/C4 complement, antistreptolysin O titers, Streptococcus A direct probe, and a pregnancy test. Erythrocyte sedimentation rate and liver function tests were also normal. Table compares various measurements taken at the time of hospitalization to the time of follow-up.\nThe patient’s history of recurrent rhabdomyolysis related to sports, acute renal failure, absence of weakness, and persistently elevated CPK levels with low exercise threshold raised suspicion for metabolic myopathy. She was consequently referred to a geneticist for evaluation. The geneticists performed lactic acid and acylcarnitine panels, both of which were normal. Whole exome sequencing (WES) revealed a pathogenic partial gene deletion in the DMD gene. Pathogenic variants in this gene are associated with Duchenne/Becker muscular dystrophy (DMD/BMD). This was surprising because DMD is X-linked. It was traditionally thought that only males developed this condition.\nOn recent subsequent evaluations, she tended to present with calf muscle pain and cramping during exercise, muscle twitching all over (although mostly on extremities), and weakness with climbing and trying to get up from a chair. She was still able to participate in sports after school with frequent drinking and resting as needed. She was advised to drink enough fluids and monitor her urine color so that it remained clear.
A 75-year-old male was admitted to the Thoracic Surgery Department in order to undergo an elective left lung upper lobe resection for a moderately differentiated lung adenocarcinoma. Patient medical history included diabetes mellitus, hypertension and dyslipidemia under medication. Moreover, the patient was a heavy smoker (140 Pack/Years). One year ago, he presented to his general practitioner (GP) suffering from cough, anemia, fatigue and weight loss of 12 kg during 3 months. The patient eventually underwent computed tomography (CT) scan, bronchoscopy and tumor biopsy, and he was diagnosed with a moderately differentiated lung cancer, stage IIIa, T3N2M0 according to seventh edition of the TNM staging system. T3 was attributed to tumor size (maximum diameter 10.8 cm) and N2 to metastasis to inferior mediastinal (subcarinal) lymph node according to the initial CT scan. The patient subsequently underwent six cycles of chemotherapy with pemetrexed and carboplatin. After that, a new CT scan, a fluorodeoxyglucose (FDG) uptake positron emission tomography (PET)-CT scan and a transthoracic echocardiogram were performed in order to evaluate tumor stage following chemotherapy. The tumor at that time was staged T3N1M0. Size was decreased (maximum diameter 9 cm) but remained T3, while only ipsilateral hilar lymph nodal metastases but no mediastinal nodes were found (downgrade to N1). Additionally, there was close proximity of the tumor to the aortic arch, but vessel wall invasion was not evident according to the radiology report (). Therefore, the patient was scheduled for left upper lung lobe resection.\nUnder general and thoracic epidural anesthesia, a left anterolateral thoracotomy was performed. In the left lung apex, the tumor was strongly adhered to the thoracic wall and the aortic arch and it seemed to invade the superior pulmonary vein, although this was not evident on the CT scan. Ligation of pulmonary vein and complete resection of tumor due to aortic arch invasion were not possible, so the tumor was considered nonresectable and thus resection was not attempted. A specimen of tumor adjacent to aortic arch and lymph nodes were resected for histopathological examination.\nWhile still in recovery, the patient complained of sudden onset of lower back pain and paralysis of the lower limbs. Initially, symptoms were attributed to epidural anesthesia, but subsequently the lower limbs became pale and cold and vascular consultation was requested. On clinical examination, arterial pulses of bilateral femoral, popliteal, dorsalis pedis and posterior tibial arteries were absent and there was no blood flow on handheld Doppler examination at the ankle level. The patient immediately underwent a CT angiography that revealed thrombosis of the infrarenal aorta in the presence of atherosclerotic lesions of the iliac arteries bilaterally ().\nThe patient was emergently taken back to the operating theater where bilateral femoral artery thromboembolectomies were performed. Although thrombus was removed after multiple embolectomies, blood flow was not adequately restored. Since arterial inflow was inadequate while the patient had just undergone a thoracotomy, it was decided to proceed to revascularization of the lower limbs with a subclavian-bifemoral bypass, in order to avoid a second major abdominal procedure. The bypass was performed with a Dacron 8-mm ringed graft. Femoral pulses were restored and postoperative ankle-brachial index was 0.6 on the right side and 0.85 on the left side which were similar to preoperative recordings.\nPostoperatively, the patient was transferred to intensive care unit (ICU), hemodynamically unstable with metabolic acidosis due to prolonged ischemia. The postoperative course was unfavorable and patient died on the fifth postoperative day due to multi-organ failure caused by reperfusion syndrome.\nHistopathological characteristics of thrombus obtained during embolectomy (–) were consistent with the histopathology of the biopsy of the primary lung tumor (–). The histopathology of the fraction of tumor adjacent to aortic arch revealed invasion of aortic adventitia (–).
A 77-year-old man with a past medical history of type 2 diabetes, hypertension, and ESRD underwent deceased donor renal transplantation. Two months following his renal transplant, the patient was admitted for an acute kidney injury discovered on routine follow-up laboratory testing. From a baseline creatinine of 1.3 mg/dl after the transplant, he was noted to have a creatinine of 2.7 mg/dl and a renal biopsy was obtained. Histopathology was suggestive of mildly active cellular rejection and acute tubular injury but with no concerns for antibody-mediated rejection. The patient received three days of methyl-prednisolone 250 mg daily intravenously and was subsequently transitioned to high-dose oral prednisone with a taper.\nThe patient was readmitted within three weeks of his renal biopsy with worsening renal functions, now with a creatinine level of 3.8 mg/dl. At this time, he was on prednisone five mg daily, tacrolimus four mg twice daily, and mycophenolate 500 mg twice daily. His family reported poor oral intake and that he had been taking furosemide at home. He received intravenous (IV) normal saline and diuretics were held. However, as his renal functions did not improve beyond a creatinine level of 2.1 mg/dl, the decision was made to perform a repeat renal biopsy. During ultrasonography for the renal biopsy, concerns were raised for a possible renal artery aneurysm. An ultrasound of the right lower quadrant and transplant kidney subsequently showed a 3 cm x 3.4 cm x 4 cm aneurysm proximal to the renal artery anastomosis to the right external iliac artery (Figure ). The right external iliac artery to renal artery anastomosis was patent and the renal vein was noted to be patent as well. No peri-transplant kidney fluid collections were noted and no hydronephrosis was seen.\nSubsequently, a pelvic arteriogram was performed, which showed patent pelvic and iliac arterial flow. However, a large pseudoaneurysm arising directly off the right external iliac artery was re-noted and the renal transplant artery was noted to be filling from the distal side of the aneurysm and remained patent, although with sluggish flow (Figure ). A second known renal transplant artery was not seen and was presumed to be thrombosed.\nWhile the interventional radiologist considered closing off the pseudoaneurysm by stent placement, the origin of the aneurysm was noted to be too close to the anastomosis to allow for stent placement. Hence, transplant surgery was consulted. The patient was taken to the operation room for a re-exploration of his transplanted kidney and revision of the arterial anastomosis. The patient underwent exploratory laparotomy, and intraoperatively, significant inflammation and scar tissue surrounding the hilum of the transplanted kidney was seen. Once the pseudoaneurysm was entered, necrotic tissue and purulence within the pseudoaneurysm were noted. It was felt that the arterial flow to the transplanted kidney could not be restored and, therefore, a decision was made to proceed with the resection of the infected pseudoaneurysm and transplant nephrectomy. The transplanted kidney was explanted. A portion of the pseudoaneurysm was sent to pathology for further characterization, and a portion was sent to microbiology for culture. The explanted kidney was also sent to pathology for further analysis. The patient was transferred to the intensive care unit (ICU) postoperatively for further management and close monitoring. During the surgery, the patient received crystalloids and multiple blood products.\nOn arrival to the ICU, the patient had temporary dialysis access placed and started on continuous renal replacement therapy (CRRT). Given concerns for an infected pseudoaneurysm, he was empirically started on renally dosed vancomycin, piperacillin-tazobactam, and fluconazole. All immunosuppressive agents were discontinued at this time. Due to persistent vasopressor requirements while in the ICU, the patient was switched to vancomycin, meropenem, and micafungin. The infectious disease team was consulted for recommendations. Blood bacterial and fungal cultures remained negative. However, fungal cultures from the pseudoaneurysm grew Aspergillus flavus on three separate samples. The patient was then transitioned to IV isavuconazonium 372 mg every eight hours for six doses, followed by 372 mg daily with a plan to continue for at least six weeks. Vancomycin and meropenem were discontinued. The pathology of the explanted kidney showed transmural necrosis of the renal artery, no evidence of rejection, and scattered micro-abscesses within the parenchyma. As the patient improved clinically, he was transitioned from CRRT to intermittent hemodialysis. He was subsequently transferred out of the ICU in stable condition. His hospitalization was complicated by colitis secondary to Clostridium difficile for which oral vancomycin was initiated. Preparation was made to discharge him to an acute rehabilitation facility; however, a day prior to discharge, he was found to be unresponsive by his nurse and despite prolonged resuscitation attempts for cardiac arrest, could not be revived. The exact etiology for this sudden demise could not be determined.
A 67-year-old, right leg dominant male (1.63 m; 61.4 kg) underwent physical assessment pertaining to reports of unilateral left knee pain and muscle weakness. He first experienced moderate left knee pain five months previously when attempting to carry a heavy object up a flight of stairs. There were no reports of previous injuries to the feet, knees, hips, or back that required medical attention. The pain was self-managed using anti-inflammatory medication and glucosamine with chondroitin, however he still reported a moderate (4/10) amount of knee pain on most days. He was able to ambulate unaided, but occasionally used a cane when walking longer distances. Radiographs confirmed the presence of mild OA in the left knee with definite medial joint space narrowing and one small medial tibial osteophyte (Kellgren and Lawrence grade 2 []). Radiographs of the right knee were unremarkable. Clinical examination did not reveal any ligamentous laxity or patellofemoral involvement, or any observable difference in knee circumference or thigh muscle girth. In accordance with established and institutional safety guidelines for the use of TMS, the participant was screened for history of seizure, medication use, metal implants in the brain or head, and history of neurological diagnosis prior to inclusion in this study [, ].\nAssessment of neuromuscular function included measurement of CS excitability (as quantified using MEPs) and isometric muscle strength. CS excitability was assessed using TMS with a 70 mm double cone coil (Magstim Super Rapid, Magstim Company, Ltd.). Surface bipolar Ag-AgCl electrodes (10 mm diameter) were placed 25 mm apart on the belly of the left and right rectus femoris (RF), and a ground electrode was placed on the patella. The skin was shaved, cleaned, and abraded prior to application of the electrodes to reduce electrical impedance. During stimulation, the coil trajectory that elicited the best MEP for the RF for each leg was marked using BrainSight software (Rogue, Montreal) and stored for future reference, and the marker for the RF trajectory was placed on a template MRI brain image. To reduce both intra- and intersession variability in the application of TMS, the same reference brain and RF trajectory marker were used in each TMS mapping time point. Active motor threshold (AMT) was determined while the participant maintained a low level contraction and defined as the lowest stimulator intensity that generated 5 MEPs across 10 trials, each with a peak-to-peak amplitude () of at least 200 μV. MEPs were then elicited from the motor cortex at stimulus intensities of 105%, 115%, 120%, 125%, 130%, 135%, and 145% AMT. A stimulus response (motor recruitment) curve [] was generated for each limb by calculating the average peak MEP magnitude from ten consecutive stimuli at each intensity, with approximately 1 sec to 4 sec between each stimulus. To account for the possibility of baseline variability, data from each stimulus intensity were normalized to 105% AMT for each stimulation session. Linear regression was then used to compute the slope parameter for the curve, as suggested previously [, ]. This procedure was repeated immediately following a 30-minute exercise session, using the same electrode placement and coil trajectory. This exercise session was intended to familiarize the participant with a home-based exercise program and included unilateral (affected limb only) open-kinetic chain knee extension in sitting, knee flexion in standing, and hip abduction in standing and side lying. Closed kinetic chain exercises include half-squats against a wall, forward lunges, and seated leg presses (not part of the home program due to equipment requirements), all involving both limbs. Resistance, when applicable, was provided from cuff weights attached around the ankle and chosen based on the ability to perform 10 repetitions with moderate difficulty.\nOn a separate visit to the laboratory within the same week as the assessment of CS excitability, maximal isometric muscle strength was measured while the participant was seated in an isokinetic dynamometer (Biodex System 4; Biodex Medical Systems Inc., Shirley, NY). Maximal isometric knee extension torque from each limb was measured with the hip in 90° of flexion and the knee in 30° of flexion. After a warm-up trial for the purposes of equipment familiarization, three isometric trials of five-second duration were completed for each limb. The maximum torque exerted onto the dynamometer from the three trials was identified and normalized to body mass (Nm/kg).\nFollowing baseline muscle strength testing, the participant was prescribed the series of six muscle-strengthening exercises described above and instructed to complete each exercise for 3 sets of 10 repetitions at home on at least four days per week over 8 weeks. Ankle cuff weights were provided for resistance to the seated knee extension, side lying hip abduction, as well as the standing knee flexion, and hip abduction. The participant met with a physiotherapist four times over this period to ensure proper exercise performance and safe progression of resistance, and to perform additional exercises (e.g., seated leg press and standing hip adduction with cable resistance) not possible as part of the home program. CS excitability (using the same electrode placement and coil trajectory from baseline, AMT was determined again) and muscle strength were assessed again at the end of the 8-week muscle strengthening intervention, using the same techniques with the isokinetic dynamometer and outcomes previously described. Finally, overall average knee pain was assessed before and after the strengthening intervention, using an 11-point numerical rating scale with 0 representing “no pain” and 10 representing “worst pain imaginable”.\nBetween-limb differences in MEP amplitude were evident across all stimulus intensities at baseline (). In general, RF MEPs were higher in the unaffected limb compared to the affected limb across all stimulus intensities prior to 30 minutes of exercise. Following the 30-minute exercise familiarization session, MEP amplitudes in both limbs increased at all stimulus intensities, with the exception of 120% and 145% AMT in the unaffected limb. Differences between limbs in the postexercise session were small at all stimulus intensities. Following 8 weeks of lower limb muscle strengthening, MEP amplitudes in the affected limb increased, compared to baseline at all stimulus intensities except 115% of AMT (). Finally, overall knee pain was reduced from 4/10 to 0/10, and maximal isometric quadriceps torque increased from 1.25 Nm/kg to 1.60 Nm/kg following the 8-week strengthening intervention.
34 years old patient, Gravida 6 Parity 3, previous 2 miscarriages (18 weeks & 12 weeks), was seen first at 23 weeks 4 days of pregnancy. She had undergone previous 3 cesarean sections and an evacuation of retained products of conception by curettage in 2013 for partial hydatidiform mole. At 27 weeks 5 days, she was admitted for vaginal bleeding. On further evaluation by ultrasound (), the diagnosis of placenta percreta was made (later confirmed by MRI). At 29 weeks, she had constipation with 2 episodes of urinary retention and she was put on continuous bladder drainage. She developed urinary tract infection and treated with appropriate antibiotics based on culture sensitivity. She continued to have repeated bouts of vaginal bleeding of varying amounts and severe constipation from 31 weeks of gestation.\nAt 32 weeks 4 days, patient underwent cystoscopy, which had shown signs of cystitis with no definite infiltration. She underwent classical cesarean section under combined anesthesia (Epidural + General). The umbilical cord was tied near insertion and the placenta was left in situ because there was no spontaneous separation. Then, the uterus was closed. Prophylactic temporary bilateral internal iliac artery balloons were inserted and inflated earlier. Uterine artery embolization was performed post cesarean section and selective angiograms confirmed adequate positioning. The patient required large volume of particles and still had incomplete embolization with the lower part of the uterus still showing some unblocked branches on both sides.\nPost-operatively, she was transferred to labor ward and within 4 hours, she developed clinical features of pulmonary embolism (PE). Some of her symptoms included drop in O2 saturation to 81%, tachycardia, chest pain, peripheral cyanosis, and signs of respiratory distress. Then, she was transferred to ICU and was initiated on heparin infusion. On chest X-ray, she had no atelectasis, pneumothorax, or pleural effusion. An immediate CT scan did not show any PE. There was no Doppler evidence of venous thrombosis in the femoral and popliteal venous systems. Later on day 1 post-operative, she had focal patchy consolidation left base and was started on parenteral meropenem, linezolid and fluconazole for the next 5 days. She had two consecutive CT scans on post-operative on days 2 and 3, which were negative. On ECG, there was right heart strain. She was now on enoxaparin. On the post-operative day 5, she was prescribed parenteral piperacillin-tazobactam for 5 days and she was shifted out of ICU next day. She had 500ml vaginal bleeding on the 9th post-operative day. 2 units PRBC were transfused. She was switched to oral cefuroxime and metronidazole and planned to continue on long-term low dose antibiotic. On post-operative day 11, she received methotrexate. On day 12, the MRA had shown the placenta was still enhancing with some areas of infarct and separation, fluid collection in the uterine cavity (present from day 1 post op, not increasing), with large ovarian veins, hugely distended and extensive pelvic varices, R>L, extensive collaterals. Her CRP was 12.7 mg/L.\nOn post-operative day 13, she underwent total abdominal hysterectomy. Intraoperatively, the bladder was densely adherent, drawn up, with large vessels in the broad ligament. The lower segment was bulging due to the presence of the placenta. The uterus was about 24 weeks’ size with adherent omentum. There was 100 mL of old blood in the cavity and the placenta was partially infarcted. The total blood loss was 2000 mL.\nPost-operatively, she was in ICU for 2 days receiving anticoagulation treatment (bridging treatment with enoxaparin + warfarin) and patient controlled analgesia. She had a bout of severe cough on day 4 and loose motions on day 5. She was diagnosed with vault hematoma, which was retro-vesical, about 120 ml in volume, treated conservatively. On day 10 she had been discharged from the hospital.\nShe presented to the ER on the post-operative day 16 and was diagnosed with chronic pulmonary embolism. Patient had a pulmonary embolus within the right middle lobe pulmonary artery; areas of sub-segmental embolus within the right lower lobe pulmonary arteries. She had no pleural effusions or consolidation and no mediastinal lymphadenopathy. She was readmitted for 4 days. She was started on therapeutic enoxaparin + warfarin. She was continued on 6 mg warfarin for 4 weeks after discharge.
A 29-year-old woman who had been bit by an insect on the left calf was admitted to our hospital with a chief complaint of continuous painful swelling of the bit area for 3 days. After scratched, the bite area became red and inflamed. The injury was not considered severe by the patient initially and the swelling of the calf was treated by self-medication with heat-clearing and detoxifying effects. The aggravating swelling and pain of the left calf impelled her to seek medical advice. After admission to our hospital, the patient developed septic shock symptoms characterized with diminished consciousness, pale skin, hypothermia, lack of urine output, and undetectable blood pressure. Laboratory studies revealed a white blood count of 13.8 × 109 cells/L, neutrophil count of 12.24 × 109 cells/L, and 88.7% polymorphonuclear neutrophils. She was admitted to the intensive care unit, receiving intravenous fluids and broad spectrum antibiotics treatments. Besides, she denied any history of diabetes mellitus, alcoholism, liver diseases, or trauma.\nIn the intensive care unit, the swelling increased and extended proximally to left knee and foot, complicated with blisters (Fig. A). Additionally, the patient developed cutaneous necrosis in the left ankle and popliteal space. Clinical examination showed her entire left calf was tensely swollen both medially and laterally, and the most obvious pain was localized to the bit area of the left calf. She had a loss of superficial touch sensation and 2-point discrimination over the entire sole of the left calf. She was unable to move her left leg actively, and any passive movements of the left calf, knee, and ankle joints caused severe pain. Palpation of the whole left leg revealed a mildly increased skin temperature and exquisite pain compared with her contralateral leg. The main differential diagnose was from deep vein thrombosis (DVT). Subsequent venous Doppler ultrasonography found no evidence of DVT, and only subcutaneous edema at the lower leg. On the basis of the postmedical history and clinical findings, in particular, increasing pain, loss of sensation, tense swelling, and severe pain to any stretch of the tissues, we diagnosed acute compartment syndrome affecting her left leg.\nAccording to the diagnosis, the patient was then transferred to the operating room for surgical inspection of the tissues and decompression of the compartments by fasciotomy. A medial incision was made 2 cm posterior to the posteromedial border of the tibia. Extensive purulence was encountered in the subcutaneous fat and superficial fascia, which confirmed the diagnosis of infectious cellulitis. The purulence was collected for aerobic and anaerobic bacterial culture. After fascia was incised, the deep layer of gastrocnemius and partial soleus were found dark red, with widespread necrosis as well as drainage of thick and amber pus, which confirmed the diagnosis of infectious myositis. Next, the dissection proceeded to transverse intermuscular septum over flexor hallucis longus muscle, flexor digitorum longus muscle, and tibialis posterior muscle to release the deep posterior compartment. The necrotic muscle and the grossly infected soft tissue were thoroughly debrided without the injury of posterior tibial artery, veins, and tibial nerve. Then, the lateral incision was made 3 cm lateral to the crest of the tibia. Layer dissection through this incision to the periosteum revealed a little purulence but no necrotic muscle. The fascia over the anterior and lateral compartments was completely released. After inspection, decompression, and debridement, the areas were irrigated sequentially with 3% perhydrol, diluent iodophor, and normal saline. Eventually, the wounds were closed by vacuum sealing drainage to improve the wound circumstance and further reduce intracompartment pressures. She was treated with elevation of the left leg, empirical antibiotic therapy, and daily monitoring of peripheral blood circulation. Considering the severity of the left calf, we closely monitored the function of renal and prepared to amputate the infected limb in case of possible renal failure and death due to acute rhabdomyolysis.\nOn day 3 postoperation, the patient reported a remarkable pain relief in the area of the incisions. A reduction of the edema was also found. Cefoperazone–Sulbactam was treated intravenously twice a day on the basis of bacteria culture revealing heavy infection of Staphylococcus aureus and Escherichia coli. On day 10 postoperation, vacuum sealing drainage was removed, and the surgical site was still riddled with necrotic and purulent tissue (Fig. B). Additionally, the skin of former swelling popliteal fossa and foot were ulcerated and it was thought to arise from the spread of infection. To reduce the severity of deep wound infection, thorough debridement was performed on day 12. We explored and found purulence and necrosis bestrewed with segmental posterior muscular group of calf, especially the fascial spaces. Different from calf, wounds in foot and popliteal fossa just deepened to subcutaneous tissue. Then the necrotic, injected tissue, and inflammatory granulation were completely excised until healthy, and bleeding tissue was exposed. Eventually, the wounds, especially the deep muscle tissues, were irrigated repeatedly with 3% perhydrol, diluent iodophor, and normal saline, and were closed by vacuum sealing drainage. Scheduled redebridements were performed every 7 to 8 days until all necrotic and injection tissues were removed. When the wounds were well granulated, we applied an anterolateral thigh flap transplantation to close the medial and lateral calf wounds and a split-thickness skin gift to close the medial and lateral foot. After 6 weeks, medial and lateral calf wounds healed well (Fig. C).\nWhen the wound healed completely, the patient underwent systematic rehabilitation for approximately 3 weeks, including rehabilitation assessment and treatment. The rehabilitation assessment was performed firstly. As shown in Table , the main problems were the ability barrier of daily life activities (standing, walking, transfer, lavatory, stairs, etc) and the movement dysfunction of the left knee and ankle (strength, mobility, etc). Besides, the sensory of the left lower extremity below the knee, the motor and sensory nerve conduction function of the left tibial nerve, and peroneal nerves were partly impaired. So the following rehabilitation programs were drew up: the low-power helium-neon laser to promote wound healing[; the ultrasound and audio pulse therapy to soften the scar and loosen the adhesion; the wax, arthrosis, and cold therapy to improve the motion of the joints. Also, the functional transcranial magnetic stimulation (10 Hz trains for 2 seconds; repeated 70 times with an inter-train interval of 4 seconds, a total of 1400 pulses and 7 minutes) was used to promote nerve function recovery. All the above treatments were performed daily. After 3 weeks, the patient underwent a second rehabilitation evaluation (Table ). All the scores of the evaluation index increased, albeit limited. Considering the high costs for the continued in-hospital rehabilitation, the patient chose to wear an orthopedic insole to achieve standing and then returned home. During the following 3 months after discharge, we conducted a telephone follow-up for the patient. After 1 month, she was able to walk independently, but slowly and instability. Moving up and down the stairs was limited. Three months later, the activities of daily living are markedly improved. She is able to walk, and moves up and down the stairs, independently.
An 80-year-old female was referred to the Department of Oral and Maxillofacial Surgery, Narakasuga Hospital because of pus discharge from an oroantral fistula at the right posterior maxilla. The right upper second molar had been extracted 43 days before because of chronic periodontitis. She noticed swelling of the right cheek, purulent rhinorrhea, and discharge through the oroantral fistula 20 days before. She was prescribed antibiotics for seven days by her family doctor. The cheek swelling improved, but the discharge through the oroantral fistula persisted. She had a medical history of hypertension and chronic hepatitis C, both of which were well controlled with an angiotensin II receptor blocker, calcium channel blocker, and ursodeoxycholic acid. The right chronic maxillary sinusitis was identified in X-ray findings by her family doctor several years ago. However, it was asymptomatic and was not treated. A physical extra-oral examination was unremarkable. Oral examination revealed an oroantral fistula with a diameter of 3 mm in the posterior alveolar ridge of the right maxilla (Fig. ). By irrigation of the right maxillary sinus through the oroantral fistula, purulent discharge was observed through the fistula.\nA panoramic radiograph showed a bone defect at the posterior alveolar ridge of the right maxilla (Fig. ). Computed tomography (CT) showed partial thickening of the mucosal lining and the presence of a hyperdense foreign body with a density of 240 Hounsfield units in the posterior lower part of the right maxillary sinus (Fig. ). The patient was unaware of the presence of the foreign body. Because the upper complete denture was repaired with acrylic resin and relined with silicone soft lining material 36 days before (seven days after the tooth extraction), the foreign body was suspected to be the denture lining material.\nAntibiotic therapy with cefditoren pivoxil was conducted for seven days. The oroantral fistula became almost closed. The patient refused to undergo immediate surgical treatment. Low dose macrolide was administered to improve the maxillary sinusitis for a month. However, CT examination showed complete opacification in the maxillary, ethmoid and frontal sinuses on the right side (Fig. ).\nThe foreign body was removed by endoscopic surgery under general anesthesia 11 weeks after the first visit, and maxillary antrostomy and ethmoidectomy were also performed. First, the maxillary antrostomy was performed. The maxillary sinus was filled with inflamed hypertrophic mucosa and granulation tissue. The foreign body was found and removed endoscopically (Fig. ). After removal of the granulation tissue, markedly inflamed mucous membrane was removed and the residual mucous membrane was irrigated and preserved. Then, anterior ethmoidectomy and revision of the frontal recess were performed. The foreign body was 12 × 6 mm in size, oval in shape, light pink in color, and compatible with silicone soft lining material (Fig. ).\nThe oroantral fistula closed spontaneously four weeks after the surgery (Fig. ). Macrolide antibiotics were administered for six months. The postoperative course was uneventful. After seven months of follow-up, CT scanning showed complete aeration of the right maxillary sinus (Fig. ).\nWe report a case of maxillary sinusitis caused by denture lining material entering through an oroantral fistula, which was successfully treated with endoscopic surgery. This case calls attention to an unusual cause of maxillary sinusitis related to dental manipulation.
A 66-year-old right-handed male presented to the emergency room with orbitocranial penetrating injury after falling down the stairs. During the fall, he impacted his face on the coat rack at the bottom of the stairs. One of the coat hooks on the coat rack penetrated into his left orbit and lodged into the posterior orbital wall. The coat hook was cut in the field by the paramedics two to three centimeters from the point of entry. On presentation, he was alert and fully oriented with a Glasgow Coma Scale (GCS) of 15 and his neurological exam was nonfocal. His ophthalmologic exam revealed an edematous left lower eyelid, with a visible large metallic foreign body breached through the lower eyelid bordering the inferior orbital rim (). The visual acuity in his left eye (OS) was 20/200 with no improvement on the pinhole, and intraocular pressure (IOP) assessment was deferred in the left eye. There was a left relative afferent pupillary defect (RAPD). Ocular movements were intact in both eyes. The subsequent ophthalmic exam did not suggest an occult globe rupture.\nComputed tomography (CT) head was obtained and did not appear to show any significant intracranial injury, although the cast iron hook created a significant metal artifact that limited the quality of the study. CT angiography (CTA) did not show any vascular injury but was also severely limited by the metal streak artifact. He was started on meningitis prophylaxis, and a tetanus booster was administered. He was taken for an urgent diagnostic cerebral angiogram to better investigate the intracranial vascular structures prior to any attempts to remove the hook. This was critical as the metal streak artifact may have obscured an underlying vascular injury that could complicate hook removal. Six-vessel cerebral angiography did not show injury to any intracranial vessels (). The majority of the intraorbital vessels appeared uninjured, although the left superior ophthalmic vein was not visualized; this was concluded to be secondary to compression from the foreign object or possibly from thrombosis. A reconstructed 3-dimensional (3D) image confirmed that there was no major violation of vascular structures ().\nThe patient underwent an orbital foreign body removal under fluoroscopic guidance in a multidisciplinary setting with neurosurgery, ophthalmology, and neurointerventional radiology. The neurointerventional radiologist was in the room for immediate support for potential vascular injuries. The groin and the orbit were prepped and draped in the usual sterile fashion. The hook was rotated with ease, but initial resistance to withdrawal of the hook suggested that there may have been a ball-socket interaction with the orbital wall and the ball of the hook, which had to be overcome. The hook then was released successfully from the orbital wall with slightly increased force. The hook was then removed from the orbit under live fluoroscopy by twisting the Kocher in order to allow the hook to exit along the entrance trajectory to prevent iatrogenic injury (Figures and ). No significant hemorrhage was appreciated from the puncture site after removal of the IOFB, and the wound was copiously irrigated with bacitracin-infused saline. Cerebrospinal fluid (CSF) leak was not appreciated.\nPostoperatively, he was taken for a conventional CT which showed a displaced comminuted fracture of the greater sphenoid wing, a small amount of retrobulbar hematoma, and a trace left anterior temporal contusion with adjacent subarachnoid hemorrhage (). The optic nerve was not suspected to be injured by the fracture fragments as the fractures were lateral to the optic canal. The sphenoid wing fractures were assessed by the neurosurgery service, and no operative repair was indicated given the lack of CSF leak. He was admitted to intensive care unit for blood pressure control and frequent neurologic checks as a result of cerebral contusion and subarachnoid hemorrhage. A repeat CT scan six hours after his surgery was performed to rule out further hemorrhage. The patient underwent ophthalmologic examination daily, without further deterioration of the visual acuity or signs of retrobulbar hematoma or other neurological deficits. Three weeks after the initial injury, the patient had a persistent RAPD and no further improvement in vision. Follow-up vascular imaging two weeks after the incident in the form of CT angiography of the head ruled out delayed vascular injury, pseudoaneurysm, or carotid-cavernous fistula. A formal Humphrey visual field test was performed two weeks after the initial injury which showed a full visual field in the right eye and a central and cecocentral defect with superior and inferior nasal steps in the left eye (). At 3 months, the patient's best-corrected Snellen vision improved to 20/30, with persistent RAPD in the left eye.

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