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An 82-year-old woman presented to our hospital with shortness of breath and left chest pain. On primary investigation with chest X-ray a moderate left-sided pleural effusion was revealed. On history the patient complained of weight loss, fatigue, and mild afternoon fever for the last 3 months. From her past medical history, hypertension and breast cancer treated with surgery and adjuvant chemotherapy and radiation therapy 16 years ago, were significant. More specifically the patient had received six cycles of cyclophosphamide, methotrexate, and 5-fluorouracil. Physical examination revealed a palpable mass in her left upper abdominal quadrant. Laboratory findings at initial presentation revealed marked leukocytosis 44.41 × 109/L, with 55% neutrophils, 42% lymphocytes, 2% monocytes, and 1% eosinophils and elevated platelet count, 551 × 109/L. After admission, a thoracentesis was performed, for shortness of breath relief, and pleural fluid had findings consistent with a reactive effusion. A computed tomography (CT) scan was done, revealing splenic enlargement and two solid mass lesions measuring 10 cm and 12.5 cm in greatest diameter with ill-defined margins within the spleen. These two mass lesions were isodense to the splenic parenchyma, while they did not present contrast blush on the arterial phase and no other specific characteristic was revealed. The fundus and body of the stomach seemed compressed and dislocated anteriorly due to splenomegaly. A CT scan of the brain and thorax followed, but no other enlarged lymph nodes were found, nor indications of metastatic disease. Despite elevated white cell count and platelet count, splenomegaly was due to distinct masses identified on CT scan and no other signs indicated hematological disease.\nSurgical approach in an open manner was decided for accurate diagnosis and treatment. The patient was placed on a supine position and a midline incision was preferred. Open splenectomy was performed with care not to violate the splenic capsule and not to disrupt the pancreatic tail. During the procedure, the patient received 2 units of concentrated red blood cells and 2 units of fresh frozen plasma. Her postoperative hospital course was uneventful.\nThe spleen was 1.200 g in weight and 19 × 15 × 11 cm in size, with nodular appearance and areas of necrosis (). The final histopathological diagnosis was angiosarcoma originating from the spleen (Figures and ). Immunohistochemical staining was positive for vimentin, CD31, CD34 (), and factor VIII () and negative for CD68. The Ki67 index was less than 10%. No signs of neoplastic disease were found in an additional hilar splenule, which was found. The patient remains disease free six months later on follow-up visit.
An 82-year-old male patient presented at the emergency department complaining of pain in his left leg. He reported painful swelling in both popliteal fossae that had emerged 2 years previously and increased in size thereafter. Around two days previously, the swelling on the left had enlarged significantly and become more painful. Physical examination revealed a pulsatile tumor in the left popliteal region ().\nThe patient underwent computed tomography (CT) with contrast, showing an enlarged left popliteal artery (LPA), with thrombus and contrast leakage delimited by a sac (), findings characteristic of a ruptured left PAA measuring 14.4 centimeters at its greatest diameter. As soon as laboratory test results had been checked and the patient had been informed of the situation, he was transferred to the operating room for immediate repair of the ruptured aneurysm.\nThe approach chosen was via medial access in the thigh and leg, with the patient in dorsal decubitus under general anesthesia. The aneurysm sac was identified and then dissected until the proximal and distal portions of the LPA were identified. Both were clamped to control proximal and distal arterial flow (). The aneurysm sac was incised and its thrombotic contents removed. A ringed polytetrafluoroethylene (PTFE) graft was interposed between the proximal and distal segments of the LPA (). The patient had previously undergone saphenectomy, precluding use of his great saphenous vein (GSV) for grafting.\nThe patient was transferred to the intensive care unit (ICU). He had persistent leukocytosis during his stay in the ICU and we considered the possibility of a graft infection. On the 7th day in the ICU, he underwent another CT of the lower extremities with contrast. This examination showed that the graft, the distal LPA, and its terminal branches, the anterior tibial artery, the posterior tibial artery, and the fibular artery, were all patent (). These images ruled out the hypothesis of graft infection. The patient’s leukocytosis improved after antibiotic therapy to treat pneumonia and he was discharged from hospital 14 days after surgery.\nDuring the initial physical examination, we identified physical characteristics of MFS - arachnodactyly and enophthalmos. One week after surgery, another physical examination was conducted, following the revised Ghent Nosology diagnostic criteria for MFS. The patient had dilation of the aortic root, with a diameter of 48 millimeters and an aortic root z score of 4.19. The following findings were also present: wrist and thumb sign, valgus hindfoot deformity, thoracic asymmetry, thoracolumbar scoliosis, and protrusio acetabuli. These findings, in conjunction with a z score > 2 fulfill the criteria for a clinical diagnosis of MFS.
A 55-year-old Caucasian woman with a 5-month history of abdominal pain and vomiting was diagnosed as having a probable high-grade ovarian malignancy with a large volume of peritoneal disease. She had a prior ultrasound of her pelvis which demonstrated a 17 cm large irregular solid vascularized mass in her right ovary. Her comorbidities included obesity (body mass index of 33), asthma, and she had previously undergone a laparoscopic cholecystectomy. There was no significant family history. She did not smoke tobacco and she drank alcohol occasionally. She was scheduled for debulking surgery; however, she presented to our emergency department with worsening abdominal pain, vomiting, and diarrhea. A physical examination showed dry mucous membranes, capillary refill < 3 seconds, and jugular venous pressure of 4 cm. Her chest was clear on auscultation with dual heart sounds. Her abdomen was distended with generalized tenderness but no guarding or signs of peritonism. Bowel sounds were present. A computed tomography (CT) scan of her abdomen and pelvis was performed which demonstrated the large right ovarian tumor, peritoneal tumor deposits, and ascites. There was extrinsic compression of her sigmoid colon due to the tumor without radiological signs of large bowel obstruction. There were no other abnormalities of her bowel. She received intravenously administered fluids for rehydration, anti-emetics for nausea, and intravenously administered morphine for abdominal pain. She remained overnight in our emergency department for treatment. She was reassessed the following morning after resolution of her symptoms and was found to be hemodynamically stable and subsequently discharged home.\nShe re-presented 1 week later with similar symptoms and was admitted to hospital for further management. Following multidisciplinary discussion, she was recommended for neoadjuvant chemotherapy prior to surgical debulking. An urgent core biopsy of the mass confirmed the likely diagnosis of ovarian malignancy. This biopsy indicated a high-grade serous adenocarcinoma. The histology and CT findings were consistent with an International Federation of Gynaecology and Obstetrics (FIGO) stage III ovarian cancer. She was appropriately counselled as to the benefits and risks of chemotherapy prior to commencing treatment.\nShe was commenced on a first cycle of the commonly used platinum-based two-drug chemotherapy regime of paclitaxel and carboplatin []. The dose prescribed was a three weekly cycle of paclitaxel 100 mg/m2 and carboplatin 385 mg/body to achieve area under the curve (AUC) of 5 using the Calvert formula. The results of her pre-chemotherapy blood tests were within acceptable ranges. Specifically, her white cell count (WCC) was 10.1 × 109/L (normal 4–11 × 109/L) and neutrophils were 8.5 × 109/L (normal 1.5–8 × 109/L). She reported feeling better 1 day after chemotherapy. However, 3 days following the commencement of chemotherapy, persistent diarrhea developed. Stool cultures were negative for stool pathogens including Clostridium difficile. On the sixth day post-chemotherapy, she became febrile and acutely unwell with severe abdominal pain. Blood tests at the time revealed a WCC of 0.6 × 109/L and neutropenia of 0.1 × 109/L. A repeat CT of her abdomen and pelvis showed a thickened descending colon and rectal pneumatosis with perforation into the mesorectum (Figs. and ). Blood cultures were positive for Escherichia coli and meropenem was commenced. She was transferred to our intensive care unit for hemodynamic support due to septic shock. A surgical assessment was conducted but immediate surgical intervention was deemed inappropriate due to high predicted mortality. She was administered granulocyte colony-stimulating factor but her white blood cell counts did not improve and her respiratory function deteriorated. She died 9 days after the administration of the first dose of chemotherapy. An autopsy was not performed due to the wishes of our patient’s family.
A 67-year-old male patient visited the endocrinology department with increased blood sugar for 5 years. During a routine abdominal computed tomography (CT) examination, an abnormal shadow was incidentally found in the right liver. Further magnetic resonance imaging (MRI) of the mass revealed signs of a malignant liver tumor. Therefore, he was transferred to our hepatobiliary surgery department for further treatment. Since the onset of disease, the patient had no obvious discomfort, except for weight loss of approximately 8 kg within 4 months. He had a history of chronic viral hepatitis B for 5 years and is regularly treated with tenofovir at 300 mg/day. Blood test results are summarized in . The levels of alpha-fetoprotein, serum carbohydrate antigen (CA) 19-9, total bilirubin, and direct bilirubin were abnormally elevated. Abdominal CT scanning of the tumors was then performed. Plain scanning revealed a giant hypointense mass (10 × 7 × 6 cm) involving the right liver that was oval-like, irregular, and with septations inside (). On dynamic CT scanning, the tumor edge showed irregular ring-like enhancement with strengthened septations, moderate enhancement in arterial phase (), continued enhancement in portal vein phase (), but attenuation in the equilibrium phase (). On MRI, the lesion appeared as an inhomogeneous high and low intensity on T1-weighted images () and T2-weighted images () with septations inside. On enhanced MRI scanning, the arterial phase of the enhancement scan was slightly inhomogeneously intensified, there was no signal enhancement in the inner part, and slight edge and separation enhancement (), with the enhancement range gradually increasing in the portal vein phase and attenuating in the equilibrium phase (). A sub-focus with a diameter of approximately 8 mm was found in the lateral segment of the left liver, which presented as slightly enhanced (). The tumor was accompanied by an embolus in the right posterior portal vein ().\nThe patient was diagnosed with atypical HCC and radical resection of the tumors was recommended to prolong survival. During laparotomy, the liver showed pink sclerosis, and the tumor was located in the right posterior hepatic segments. No extrahepatic lesions were found. Thus, a right posterior hepatectomy with removal of the right posterior portal venous thrombus, and ultrasound-guided radiofrequency ablation of the sub-focus were performed. On pathology, the resected lesion was macroscopically of the multi-nodular type and had a size of 10 × 8 × 6.5 cm in the largest cross-section. The cut surface of the tumor was grayish-white and grayish-red with a central necrotic area ().\nMicroscopically, the tumor was composed of both carcinomatous and sarcomatous elements, with histological features of a collision tumor containing HCC, CCC, and undifferentiated spindle cell sarcoma. Unequivocal glandular structures comprising atypical gland cells with pleomorphic hyperchromatic nuclei and prominent nucleoli were interpreted as moderately differentiated CCC (). Additionally, carcinomatous cells with nestlike distribution, obvious atypia, high nucleus:cytoplasm ratios, and hyperchromatic nuclei were found, suggesting poorly differentiated HCC (). There was a clear boundary between the two elements. The sarcomatous component, which occupied approximately 20% of the whole tumor, consisted of long spindle-shaped cells with elongated nuclei arranged in bundle patterns (). There was a clear boundary between the carcinomatous and sarcomatous components (). The tumor was 2 cm from the closest liver resection margin, and the surrounding liver showed nodular cirrhotic changes. Cutting edges of the tumor were negative. On immunostaining, part of the epithelial component with a nested growth pattern was positive for HepPar-1 (), CKpan, arginase-1, and focally positive for glypican 3, which was suggestive of HCC, while the other part of the epithelial component with glandular architecture was positive for CK7 () and CK19 (), and negative for arginase-1 and glypican 3, suggesting CCC. Both epithelial components were negative for vimentin. Sarcomatous cells were positive for vimentin () and negative for cytokeratins 7 and 19, epithelial membrane antigen, and CD34.\nThus, the tumor was finally diagnosed as HCS (T4N0M0 stage IIIB) with a mix of HCC, CCC, and sarcoma. The patient’s tumor pathological classification and immunohistochemical indexes are shown in . The patient recovered well and was discharged 11 days later. Transhepatic arterial chemotherapy and embolization was given after surgery. Unfortunately, during the follow-up visit at one and a half months post-surgery, a chest CT scan revealed a single small nodule in the right lung. After comparing the with preoperative CT scans, this nodule was suspected of being metastatic lesions, and the CA19-9 level was elevated to 53.97 U/mL.
A 12-year-old Moroccan female who presented a nephrotic syndrome was treated with corticosteroids, without any effect. A renal biopsy performed 8 years later showed typical focal glomerulosclerosis. Renal insufficiency developed rapidly, with the concomitant development of cardiac failure. Dialysis was required at the age of 22 years. Two years later the patient moved to France and was dialysed in our unit. Dialysis was carried out via an arteriovenous fistula and comprised 4-h sessions three times a week, with a dialysate Ca level consistently maintained at 1.5 mmol/L. When the patient first presented in our unit, our main concern was severe cardiac failure due to uraemic cardiomyopathy, with a left ventricular ejection fraction (LVEF) of 28%. After a few months, following treatment adjustment using diuretics, β-blockers and angiotensin-converting enzyme (ACE) inhibitors, as well as a decrease in body weight, the LVEF improved to 54%. The next important problem was SHPT. The main biological parameters and medications prescribed to the patient are shown in . Despite an increase in the dosage of C-HCl, the level of PTH did not decrease but paradoxically increased. To ensure that the prescribed doses were effectively taken by the patient, the medication was given at the dialysis unit on the days of dialysis at the end of the dialysis session. However, the level of PTH consistently increased.\nIn May 2007, an ultrasound scan of the parathyroid glands revealed normal upper right and upper left parathyroids of ~3 mm in diameter and two enlarged parathyroids, with the right lower gland measuring 8 × 6 × 5.2 mm and the left lower gland measuring 7 × 6 × 4.5 mm. Consequently, a parathyroidectomy was performed in July 2007, at a preoperative PTH level of 2162 pg/mL. The two upper parathyroid glands were slightly hyperplastic but were left intact and controlled with a clip to ensure that they could be easily identified, if necessary. The two lower glands, each ~10 mm in diameter, were resected, with each gland weighing 5.3 and 5.2 g, respectively. The histopathological examination revealed the presence of a benign parathyroid adenoma consisting of chief cells in each resected gland; however, 30–40% of each gland contained oxyphil cells, which was classified as an oxyphil adenoma (), and a parathyroid carcinoma was excluded.\nTwo hours post-surgery, the serum Ca level dropped to 1.30 mmol/L and the PTH level to 8 pg/mL. The patient was prescribed 12 g/day of intravenous Ca and 6 µg/day of alfacalcidol. Four months later, with a treatment regimen of 4 g/day of Ca and 4 µg/day of alfacalcidol, the Ca level was 2.00 mmol/L, P level 1.20 mmol/L and PTH level 22 pg/mL. In the following year, the patient received a kidney transplant, was pregnant 3 years later and remained well 9 years later, with a creatinine level of 118 µmol/L, Ca level of 2.36 mmol/L and PTH level of 36 pg/ml on a treatment regimen of 2 g/day of Ca and 2 µg/day of alfacalcidol.
A 38-year-old man presented with multiple nodular lesions over the pubic region with ulceration and excoriation over the penile skin for the previous 2 months []. The lesions had been increasing in size and number over a period of time. The patient first consulted a dermatologist who referred him to a surgeon, given the history of malignancy in this patient with suspicion of cutaneous metastases. He had complaints of severe and intractable pain over the lesions. He had a 2-year history of carcinoma of the rectum with liver metastases. According to the American Joint Committee on Cancer Tumor-Node-Metastasis Staging System, the disease was Stage 4, so surgery was not feasible. A colonoscopy performed 9 months previously had revealed an ulceroproliferative lesion in the rectum 8 cm from the anal verge. Since a biopsy proved it to be an adenocarcinoma, a repeat biopsy was not performed. He underwent a colostomy 9 months previously because of obstructive symptoms. He received chemotherapy with three series of combinations of irinotecan, oxaliplatin, 5-fluorouracil (5 FU), and leucovorin (LV) (FOLFOX). A positron emission tomography-computed tomography scan revealed liver metastases, and he was encouraged to receive further chemotherapy, which he refused. Two months later, the patient returned to the surgery department and was found to have extensive new skin lesions involving the pubic region and external genital area. The cutaneous lesions were not close to the colostomy wound. His vital signs and laboratory investigations were normal. A hard, ulcerated lesion was felt on rectal examination. Histopathological examination of a biopsy from the skin nodules and the penile region revealed metastatic adenocarcinoma []. The tumor was assessed to identify histological features suggestive of high-frequency microsatellite instability which was negative in the index case. Palliative radiation therapy was advised, but the patient refused any further treatment. He also did not receive any traditional treatment such as Indian herbal medications. The patient was followed up for 2 months for symptomatic treatment and died.
An 86-year-old woman who had been living with her elderly husband was admitted to our hospital with the complaints of nausea, anorexia, and epigastric discomfort. When she was 73 years old, the patient had undergone laparotomy and right hemicolectomy for resection of colon cancer. Five years after hemicolectomy, she had received hernia repair surgery using mesh for an abdominal incisional hernia. There was no history of abdominal or thoracic trauma. Laboratory tests were all within the normal range. Her electrocardiogram showed no ischemic changes. Although chest X-ray revealed an abnormal gas-filled mass in the left thoracic cavity, the patient had no symptom of dyspnea. Contrast-enhanced computed tomography (CT) scan confirmed herniation of the gastric corpus through the left posterior part of the diaphragm (). We diagnosed adult Bochdalek hernia and planned its surgical treatment. As mesh had been placed under the previous upper abdominal midline incision more than 10 years earlier, dense adhesions between the mesh and abdominal tissues were expected. Therefore, we decided to perform diaphragmatic hernia repair by HALS, considering the patient’s safety. After induction of general anesthesia, the patient was placed in the supine position with her legs apart. The previous midline incision was opened carefully to insert a LAP DISC® (Hakko, Nagano, Japan) for a hand port. Dense adhesions, which had to be divided, were found between the mesh used to repair her incisional hernia and loops of the small intestine. The disk for HALS was placed after complete removal of the mesh. Subsequently, a 12 mm trocar was inserted into the inferior umbilical region for the laparoscope. A 5 mm trocar was also inserted into the left upper abdominal region. Herniation of the gastric corpus into the left thoracic cavity through a hernial orifice in the left posterior diaphragm was confirmed. The gastric corpus could not be pulled back into the abdominal cavity because of adhesions around the hernial orifice. After these adhesions were carefully removed by HALS using Harmonic ACE shears (Ethicon, NJ, USA) (a), the stomach was completely freed and could be returned to the abdominal cavity. A 5 × 3 cm hernial defect with sac was observed (b). We decided to perform simple closure of the defect without mesh reinforcement because the rim of the hernial orifice was relatively strong. Accordingly, the defect was repaired with interrupted nonabsorbable sutures (2-0 Nesporen; Alfresa Pharma Corporation, Osaka, Japan) using a 5 mm port on the left upper abdomen and the surgeon’s left hand via the hand port (c). The midline incision for the hand port was closed without mesh reinforcement. A drain tube was placed under the left hemidiaphragm. The operating time was 244 min and there was no significant bleeding. Her postoperative course was uneventful. The patient was discharged on postoperative day 20. There was no evidence of recurrence at 1-year follow-up.
A 62 year-old gentleman presented to thyroid clinic with a neck lump. It had been growing slowly over a six-month period and became more noticeable. He did not report any hoarse voice, dyspnoea or dysphagia. There was no loss of appetite or weight. On examination there was a palpable well circumscribed lump arising from the left thyroid lobe measuring 5 × 3 cm. There was no tracheal deviation and the lump moved on swallowing. There were no other palpable lymph nodes and the patient was clinically euthyroid.\nHis previous medical history of note includes a sub-arachnoid haemorrhage ten years previously leaving the patient with residual mild left sided weakness. He was a smoker of ten cigarettes a day and was previously a heavy drinker.\nThyroid function tests were within normal limits and thyroid ultrasound demonstrated a lump that appeared benign arising from the superior pole of the left lobe of thyroid gland so the initial diagnosis was an adenoma in the left lobe of thyroid.\nDuring the operation it was noted that there was a cystic lump that did not originate from the thyroid gland but from the lower pharynx extending to the upper oesophagus. The lump was found to be full of necrotic tissue. The lump and left lobe of the thyroid were excised.\nThe histopathology demonstrated a normal left thyroid gland. The 5 cm tumour was well circumscribed with focally invasive spindle cells (Figure ) with some areas that were highly cellular with a fascicular fibrosarcomatous like growth pattern. Elsewhere there was rather loose fibrous and partly myxoid stroma with abundant mast cells. In addition immunochemistry including EMA, CD99 and BCL2 were all positive supporting the diagnosis of monophasic synovial sarcoma originating from the lower pharynx.\nStaging CT-scan showed local disease recurrence with infiltration of the strap muscles and trachea. He was started on a course of radical radiotherapy. Follow-up CT-scan revealed multiple pulmonary metastases although the patient remains clinically stable.
Here, we report a case of an 83-year-old Hispanic male with end-stage renal disease (ESRD) on dialysis three times a week who presented to the emergency department with the chief complaint of bilateral lower extremity weakness. The patient noted the weakness in his leg after having his early dinner around 3PM. He first noticed weakness in his knee joint and later he had trouble getting out of his chair and walking to the restroom. Initially he managed to walk with help. Over the next several hours the weakness in his legs persisted and became so severe that he decided to come to the hospital.\nThe patient stated that he had similar problems 3 days prior to presentation and on the day of his dialysis and the symptoms resolved after a 3-hour dialysis. Prior to these events and in between the two episodes the patient had normal, age appropriate activities of daily living. He walked without help and without any difficulty.\nHis past medical history included was significant for end-stage renal disease on hemodialysis three times a week, hypertension, and coronary artery disease with stent placement, myocardial infarction, and seizure disorder.\nThe patient had undergone a colectomy for perforated bowel with colostomy several years ago.\nThe patient denied cough, fever, diarrhea, and change in bowel or urinary habits. There was no prior history of trauma.\nHis home medication list included Aspirin 81 mg per day, Clopidogrel 75 mg per day, Simvastatin 40 mg per day, Metoprolol 25 mg per day, Multivitamin one tablet a day, folic acid one tablet twice a day, Calcium acetate 667 mg two tablet with each meal daily, Phenytoin 100 mg twice a day, and Omeprazol 20 mg per day.\nThe review of systems was significant for a recent history of new-onset seizure that required intubation for air way protection. Patient was admitted to Medical Intensive Care Unit (MICU). Patient was later discharged home on Phenytoin. Shortly before this admission, patient has presented to the hospital multiple times with serum potassium levels as high as 8.3 milliEquivalents/liter (mEq/L).\nPhysical examination was remarkable for bilateral lower extremity weakness pronounced at the hips (motor strength 2/5 in both legs). Furthermore, cogwheel rigidity was appreciated bilaterally, more pronounced in the knee joints. Sensory system examination and the reflexes were within normal limit. No saddle anesthesia was appreciated on neurological examination. Motor strength and cranial nerve examination were essentially unremarkable.\nECG revealed peaked T-wave and the serum chemistry study was remarkable for potassium of 7.8 mEq/dL ().\nA portable chest radiograph showed no acute infiltration or fluid overload.\nThe patient's diet includes rice, beans, pork chops, beef, chicken, and salad.\nIn the emergency department, the patient was given 10 cc of Calcium gluconate, 5 units of regular insulin, 25 cc of Dextrose 50%, and 60 mg of oral Sodium polystyrene sulfonate immediately based on the ECG and laboratory abnormalities.\nPatient was attached to the monitor and observed with serial neurological exam, ECG and serial labs.\nAfter an hour, patient's weakness and cogwheel rigidity partially improved and the patient was able to stand with help. The patient was transferred to the MICU service for close monitored observation. Patient was subsequently dialyzed. Repeat laboratory values and the ECG returned to previous values (Figures and ), and the patient was able to stand up and walk without difficulty maintaining his preevent status.
A male patient aged 11 years reported with the complaint of swelling of the gums since 6 months. A detailed history was taken and revealed no positive features.\nOn clinical examination, the patient had gingival enlargement in the upper arch. This enlargement involved attached gingiva and covered almost one-third to one-half of the crown length. Periodontal probing showed presence of false pocket with no signs of clinical attachment loss. As the patient was in mixed dentition stage, few primary teeth were mobile. There was presence of grade II local factors.\nLooking at the history and clinical picture, the enlargement was considered to be due to the local factors and the mixed dentition stage that made the maintenance difficult by the patient.\nHe was treated with scaling followed by gingivectomy operation. The patient was perfectly fine. Regular follow-ups could not be carried out due to patient's poor compliance.\nAfter 4 years of treatment, the patient came with milder form of gingival enlargement. This time, the enlargement was accompanied by swelling of upper lip.\nOn elaborating the history, the swelling of gingiva began before 2 months with no change in the oral hygiene or food habits. His medical history was unremarkable and he had no history of any intestinal disease, TB, or any signs of chronic fatigue.\nThe gingival examination revealed rubbery consistency involving the attached gingiva, with the presence of false pockets, slight bleeding on probing, and very less local deposits []. This enlargement was resembling the previous enlargement in all aspects except the severity which was less this time. On palpation, the upper lip felt nontender and soft in consistency. There were no appreciable changes on the dorsal surface of the tongue. The rest of the intraoral examination was unremarkable.\nUpper lip enlargement with the given background and good oral hygiene led us to think about the granulomatous disease. The clinical differential diagnosis included OFG, angioedema (idiopathic or hereditary), sarcoidosis, Crohn's disease, and an allergic reaction.\nMelkersson-Rosenthal syndrome (MRS) was ruled out because of the absence of facial paralysis and clinically normal tongue.\nTo rule out Crohn's disease, chest radiography and a series of blood tests were requested. They turned out to be normal. An in-depth gastrointestinal investigation did not appear justified in this case, since there were no signs of anemia or symptoms suggestive of Crohn's disease.\nA biopsy sample of the upper lip was obtained for histopathologic evaluation. The histopathologic analysis showed nodular tuberculoid granulomatous inflammation in patchy pattern throughout the submucosa. The granuloma consisted of lymphocytes, histiocytes, epitheloid cells, and occasional plasma cells with scattering of neutrophils. Overlying epidermis showed mild spongiosis and slight hyperplasia, giving the histological impression of granulomatous cheilitis []. The results of the other investigations were negative. Therefore, a final diagnosis of idiopathic OFG was made.\nOnce the diagnosis was made, we started with the local treatment. To our surprise, as it occurs in very rare cases, the lip swelling reduced in size without any intervention during this investigation period. So, we decided to wait for further reduction and treat the gingival enlargement by gingivectomy meanwhile. The patient was explained regarding the recurrence and was kept on regular follow-up regimen. After 2 months of treatment, the lip swelling subsided completely. He is responding well with the recent recall showing no signs of recurrence even after 2 years.
A 46-year-old female had a tracheotomy under diagnosis of pneumonia during her course of conservative treatment () after being hospitalized in a neurosurgery center for intracerebral bleeding at the right basal ganglia region. After two months of the intracerebral hemorrhage, she was transferred to a rehabilitation center. In the third month after the tracheotomy, massive hemorrhage occurred through the tracheotomy tube when tracheal suction was carried out. The patient demonstrated cyanosis, respiratory failure, and shocks so that the cuff air in tracheotomy tube was maximally expanded. However, the bleeding did not stop. The tube was immediately removed while the suction was consistently carried out. A cuffed endotracheal intubation was tried through the oral cavity but the attempt was a failure due to visual obstruction. The cuffed endotracheal tube was inserted deep into the tracheal opening, and the cuff was expanded, followed by moving up the tube. The bleeding stopped at last. She was immediately transferred to an intensive care center, and blood test was carried out. The hemoglobin level decreased from 10.9 g/dl to 8.9 g/dl (about a drop of 2 g/dl), and cervical CT was carried out to identify the cause of bleeding. No special abnormality was identified, but the bronchoscopy did not show endotracheal area due to blood clot. Complete examination could not be done due to the risk of re-bleeding, hence conservative treatment was continued. One week after the bleeding, transfemoral angiography findings revealed that innominate artery made small luminal outpouching to trachea () at the carotid artery and subclavian artery bifurcation level, and thus diagnosis of TIAF was made. She was transferred to the thoracic surgery unit, and had an operation for TIAF, tracheoplasty with bypass graft. A rupture area of 1×0.5 cm was observed by a median sternotomy, and the innominate artery was rebuilt through replacement of the detached innominate artery by an artificial blood vessel that connected the right subclavian artery and the right aorta while the tracheoplasty was done using the fifth costal cartilage. A month later, tracheostenosis and inflammation were observed during a bronchoscopy that was carried out due to wheezing and tachypnea and subsequently, endotracheal balloon dilatation was conducted. However, no improvement in the symptoms was seen, hence replacement of Montgomery T-tube was carried out followed by continuation of rehabilitation after transferring her to the rehabilitation center. It has been eleven months since the surgery has been carried out, and the patient survives without further complications.
Parents brought their three-year-old son to the ED complaining that he had put a watch battery in his nose. The provider did radiographs of the chest and abdomen and did not see a foreign body. On nasal examination they were unable to visualize the battery. An otolaryngologist (ENT) was called; the emergency provider was instructed to have the parents call the ENT office at eight in the morning for their son to be seen, and to have the child not take any oral liquids or solids after midnight and wait until he was seen by the ENT physician. The parents asked if their child could be taken to a nearby children’s hospital, but they were reassured that it was not necessary and were also instructed to return if there was any respiratory distress.\nAbout six hours later the child began having discharge from his nose. His parents took him to another hospital where a specialist removed the watch battery from his nose. By that time there was extensive tissue necrosis resulting in a perforated nasal septum requiring surgery. A lawsuit for malpractice was filed with the court for delay in care. The lawsuit was filed against the emergency provider and the ENT specialist. The ENT physician asked for the case to be dismissed with respect to his care, claiming he did not have a physician-patient relationship. He also claimed that the plan was a general one in the event he did see the patient the next day and that it was not specific care.\nA physician-patient relationship is required as the first element of a malpractice action. The court stated: “there is no physician patient relationship if the physician does not see, treat, or in any way participate in the care and diagnosis.” The court also said “the physician must perform some affirmative act.” The court examined the facts and declared that the ENT physician had placed an order for “nothing per mouth” status and directed when follow-up care was required, demonstrating a participation in care that constituted an affirmative act.
A 51 year old man presented to the ED for the third time in 2 weeks with complaints of flank pain and hematuria. He had complained of left sided flank pain during the initial 2 visits. During the first visit, the patient was diagnosed to have renal colic. X-ray KUB did not show any renal stone. He was treated symptomatically with analgesia and discharged. During the second visit with complaints of persistent left flank pain despite taking analgesia given at discharge, he was admitted to the emergency observation ward for pain management. Bedside ultrasound done then was noted to have mild left sided hydronephrosis. He was pain free at the end of the observation and was then discharged with analgesia and an outpatient follow-up with the urology department. He was also scheduled to have an outpatient computed tomography scan of the kidneys, ureters, and bladder (CT KUB). However, 2 days before the scheduled CT, he represented to the ED with right sided flank pain since morning on the day of his visit. The pain radiated to the right groin and was associated with hematuria. He was not passing blood clots. He denied any other complaints of fever, weight loss, vomiting, diarrhea, or constipation. He had a past medical history of hypertension, diabetes mellitus, and hyperlipidemia. He had previous surgeries for appendicectomy and cholecystectomy.\nHis vital signs were stable. There was tenderness over the right flank on physical examination. There was also a palpable tender mass measuring about 5x5cm over the right lumbar region. There was no renal angel tenderness. Bedside ultrasound in the ED showed an appearance of a 6 cm cystic lesion around the inferior pole of the right kidney with internal echogenicity within the cyst. The provisional diagnosis was a bleeding renal cyst versus a tumor. Blood tests done in the ED showed a mildly elevated white cell count 12.64 × 10(9), hemoglobin of 14 gm/dL, and creatinine of 109 Umol/L.\nPatient was seen by the urology on-call in the ED and his bedside ultrasound done by the urologist revealed a 10 cms cystic lesion anterior to the lower pole of the right kidney with irregular internal echogenicity. Patient was admitted to urology department for further evaluation and management.\nInpatient CT Intravenous Pyelography (IVP) was done and the left kidney showed relative hypoenhancement and reduced excretion of contrast, associated with diffuse ureteric thickening and periureteric fat stranding which could be related to a passed left renal calculus or a differential diagnosis of ureteropyelonephritis which was less likely due to diffuse involvement of left kidney. There was a tubular cystic structure in the right flank with inferior tip at the same site of previously inflamed appendix stump base which could represent mucocele of the appendix stump ().\nPatient was then transferred under the care of the general surgery department, where he underwent a colonoscopy which demonstrated an extrinsic compression at the caecum and appendiceal orifice and a smooth pedunculated polyp measuring 10 mm in the sigmoid colon (). Exploratory laparotomy was done, the mucocele was resected, and patient recovered well. Histopathology revealed a benign mucinous cystadenoma. The patient was discharged well with an outpatient surgical follow-up.
A 63-year-old male with a past medical history of hypertension, hyperlipidemia, and obstructive sleep apnea initially presented with altered mental status and was admitted for septic shock secondary to methicillin-sensitive Staphylococcus aureus bacteremia and NSTEMI with troponin level up to 40 ng/mL. His cardiopulmonary exam was unremarkable on the initial exam. Due to poor images on a transthoracic echocardiography (TTE), a transesophageal echocardiography (TEE) and soft tissue chest CT scan was also performed which revealed a severely stenotic bicuspid aortic valve with fusion of the left and noncoronary cusps as well as an ascending aorta dilatation of 4.8 cm and aortic root dilatation of 4.5 cm with concern for an aortic root abscess (Figures , , and ). The patient's hospital course was complicated by an embolic stroke and embolic phenomenon of his right thumb, requiring stabilization prior to surgical intervention. The patient underwent an uncomplicated bioprosthetic aortic valve replacement with a 23 mm Magna Ease Tissue valve, a patch exclusion of the left coronary sinus abscess, and aortoplasty. A coronary artery bypass grafting with a saphenous vein graft to the left anterior descending (LAD) artery was also performed, as the abscess had involved the LAD artery as well. The final pathological report demonstrated thrombotic vegetations on the valve leaflets consistent with acute endocarditis. Once stabilized, the patient was discharged to acute rehabilitation on a 6-week course of IV nafcillin based on culture sensitivities.\nAbout four months after discharge, the patient presented to an outpatient cardiology clinic for a routine postoperative follow-up. At that time, he was asymptomatic and reported having completed the antibiotic course. Physical exam, however, was notable for a left lower sternal harsh holosystolic murmur. He underwent stat TTE, which revealed an abnormal aortic valve with a possible leak near the patch. Upon arrival back to the hospital, TEE revealed a normally seated bioprosthetic aortic valve with no abnormal valvular motion or apparent dysfunction but had a large ventral septal defect (VSD) involving the lower border of the valve which entered the right ventricle adjacent to the septal leaflet of the tricuspid valve (). It also showed an aortopulmonary artery fistula connecting the sinus of Valsalva to the pulmonary artery and moderate dilatation of the aortic root with an aneurysmal appearance (). Given the level of complexity of the case, he was transferred to a tertiary care facility, at which he successfully underwent freestyle aortic root (23 mm) and ascending aortic replacement (24 mm), reimplantation of SVG to LAD bypass graft, and VSD closure.
A 58-year-old male with a history of well-controlled hypertension, chronic smoking, and recently diagnosed biopsy-confirmed small cell lung cancer (SCLC) of the left upper lobe presented to the hospital with a left field cut, right gaze palsy, and left hand weakness (). He had previously been admitted to an outside hospital after developing sudden-onset tonic-clonic seizures and expressive aphasia. A magnetic resonance imaging (MRI) scan of the brain at that time revealed scattered subcentimeter ischemic infarcts in the right and left frontal lobes, right parietal lobe, and the left cerebellar hemisphere. He was transferred to our institution for further management.\nUpon admission, the patient was admitted to the neurosciences intensive care unit for further monitoring. He was placed on continuous electroencephalographic monitoring. Computed tomography (CT) of the brain showed new mild hypodensities in the right subcortical frontal lobe with stable hypodensities in the periventricular white matter, left subcortical frontal and left occipital lobes (). A transthoracic echocardiogram (TTE) revealed a moderately dilated left atrium with a 40 × 39 mm spherical echogenic, heterogeneous mass originating from a left pulmonary vein (, ). Given that the patient had no significant cardiovascular history, it was concluded that the embolic infarcts were likely secondary to tumor shower from the left atrial metastasis from his primary SCLC. The patient was scheduled to undergo cardiac MRI imaging to further characterize the left atrial lesion and extent of invasion, but given the risk of intubation and sedation, this was deferred. A CT-angiogram and chest X-ray of the chest was conducted which demonstrated encasement of the left main pulmonary artery with direct extension of the left pulmonary vein and left atrium (, ).\nIn the setting of metastatic left atrial invasion with metastatic thromboembolic spread to the brain, the patient was started on anticoagulation with a heparin drip in the hospital and later transitioned to enoxaparin. His neurologic status improved and he was transferred to the oncology service for initiation of chemotherapy. After initiation, he developed atrial fibrillation with rapid ventricular response that was subsequently controlled with metoprolol. He was discharged in stable condition from the hospital and has been following up with his oncologist and cardio-oncologist for continued management of SCLC.
This is a 50-year-old right-handed male, with 33-year history of T6 AIS A SCI from a gunshot wound complicated by chronic pain, left hip and knee heterotophic ossification, and a chronic dislocation of his right hip, who initially presented to the emergency room with a right shoulder mass in September 2014. While he initially noticed the mass about 2 months earlier, he presented for evaluation now because of acute onset of pain, weakness and paresthesias in the right arm. He was admitted to the general medicine service for pain management and underwent an initial work up for his right shoulder mass, including advanced imaging and a core biopsy. Physiatry was consulted due to his functional deterioration that precluded him from returning to his previous independent living arrangement. He demonstrated diffuse, mild weakness throughout the right arm that was variable and seemed to be correlated with his reported pain level, but his most consistent and weakest movement patterns were his grade 4/5 weakness in finger abduction and distal interphalangeal joint flexion. He had reduced pin prick sensation over the volar surface of digits 3–5, palm and forearm of the right arm and hand. He was not able to perform transfers to or from his manual wheelchair due to his level of pain. The magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass with a maximum diameter of 6.9 cm (Fig. ) that involved the right deltoid and pectoralis major muscles. His core biopsy demonstrated a STS that was classified as a high grade (III) spindle cell sarcoma.\nOncology recommended treatment for his STS with a course of outpatient neo-adjuvant radiation therapy followed by gross total resection with wide margins. Physiatry pre-operative consult focused heavily on functional prognostication. The patient expressed multiple times that he placed the highest priority on return to his previous modified independent living arrangement and not only survival after his STS treatment course. The patient’s personal values combined with the physiatric assessment informed the pre-surgical planning. In particular a decision was made to take a narrower surgical margin around key muscle group (pectoralis major and deltoids) in order to help maintain the man’s manual wheelchair mobility and his ability to independently transfer himself.\nHe completed neo-adjuvant radiation therapy in November of 2014 and underwent radical excision of his right shoulder mass with flap closure that December. He began intensive inpatient rehabilitation after he was given clearance to weight bear through his arm about 8 weeks later. Initially he required total assistance for most ADL’s, including transfers, manual wheelchair propulsion, dressing and toileting. Despite the extensive surgery and radiation treatments, the gentleman was able to return to a functional level, approaching his pre-morbid status (modified independence). He was successfully discharged home to live alone in his accessible apartment complex.
A 30-year-old woman presented for an elective nephrectomy and consented to epidural analgesia for postoperative pain relief. She had a past medical history of duplex kidney and previous partial nephrectomy following recurrent urinary tract injections from pelviureteric junction obstruction. Epidural insertion at the mid-thoracic level prior to surgery resulted in inadvertent dural puncture with a Tuohy needle.\nOn the second postoperative day, she developed bilateral frontal headaches that worsened when sitting forward, tinnitus, nausea and vomiting. A diagnosis of PDPH was made and treatment initiated with caffeine, oral analgesia and increased fluid intake. Although the patient subsequently failed to respond to conservative management, she developed wound sepsis, which the microbiology team felt would preclude an epidural blood patch due to the risk of neuraxial infectious complications. Computer tomography imaging revealed no space-occupying lesion in the brain, and a diagnosis of meningitis was excluded on the basis of a normal lumbar puncture. An autoimmune screen was negative.\nThe pain management team carried out a therapeutic epidural blood patch on the eighth postoperative day, after complete resolution of the sepsis. The patient was placed in the left lateral position and the epidural space located using a 16G Tuohy needle and loss of resistance to air technique. Correct placement of the needle in the epidural space was visually confirmed using C-arm fluoroscopy (Figure ), prior to injection of twenty millilitres of sterile autologous blood.\nThe following morning the patient reported no further headaches, but stated that her right pupil was smaller than the left, and that her right eyelid was starting to droop. A neurologist reviewed her and made a diagnosis of Horner's syndrome (miosis, ptosis and enophthalmos). Magnetic resonance imaging of her neck and brain revealed no brainstem lesion or carotid artery dissection.\nOver the next two days her right pupil was starting to resume normal size and the patient was discharged home. During a telephone follow-up six weeks later, she reported that she had returned to work full-time and the headaches had not recurred.
This is the case of a 65-year-old male, who originally presented on August 18, 2008 with obstructive jaundice. His past medical history includes diabetes and hypertension. He underwent ERCP with stenting of the bile duct. The patient also had a spiral CT and an endoscopic ultrasound of the pancreas. These tests helped to determine resectability. He underwent a pancreaticoduodenectomy (Whipple procedure) on Sept. 8, 2008 for a pancreatic head adenocarcinoma. He had a standard whipple performed, with removal of the gallbladder and distal stomach, as well as the head of the pancreas and duodenum. There were no intraoperative events and no gallstones were spilled. He received adjuvant chemotherapy for 7 months. The chemotherapy was gemzar, oxaliplatinum and tarceva. The patient had routine follow-up and surveillance for recurrence every 4 months the first year and every 6 months for the second year. This included a CT scan and CA 19-9 level. Over the course of one year he was admitted to the hospital on several occasions for low-grade fever, for which the diagnosis was not established, and at times treated as an outpatient for multiple urinary tract infections. During these episodes the patient always had a normal white blood cell count and normal neutrophils/lymphocytes on differential. At no time did he complain of any abdominal pain or any difficulty tolerating food. All CT scans during this first year were negative for recurrent malignant disease.\nAt approximately 18 months in follow-up, the repeated computed tomography (CT) scan showed a non-specific abnormality in the right lobe of the liver, suspicious for a mass. A magnetic resonance imaging (MRI) and positron emission tomography (PET) scan were then performed, which was positive for a mass, suspicious for an isolated tumor recurrence between segments 5 and 6 in the liver. Both the CT and MRI showed a large liver lesion present in the inferior aspect of segment 6 measuring 7 cm AP, 5 cm transverse, and 4.5 cm craniocaudal (Figure ). This mass demonstrated heterogeneous thick peripheral rim enhancement with central hypo-enhancement and likely necrosis. The PET scan showed an abnormal robust focus of metabolic activity in inferior lateral right hepatic lobe, concerning for metastatic disease, SUV 11.6 (Figure ).\nLaboratory examination of the patient's blood demonstrated: white blood cell count 6.1 (reference range 4.5-10.8 K/uL), alkaline phosphatase level 141 (reference range 38-126 U/L), and AST 36 (reference range 15-46 U/L), ALT 50 (reference range 13-69 U/L). The total and direct bilirubin levels were normal. The CA 19-9 was 84, which was increased from 39 on the previous sample from 3 months prior. Physical exam of the abdomen was unremarkable. No masses were palpated, the liver was not enlarged, and the abdomen was non-tender. There was no cervical, umbilical, or inguinal nodes appreciated.\nFNA biopsy was discussed at the GI tumor board, but in the setting of a rising CA19-9 and suspicious CT, MRI, and PET scan, the decision was made to proceed to surgery without a biopsy. The tumor board felt that this was recurrent pancreatic cancer and because of this decided the small risk of seeding the needle tract by doing an FNA was not warranted in this case.\nPatient underwent a resection of segment 5 and 6 of the liver without any complications. Final pathology of the specimen returned as inflammatory granulation tissue and granules consistent with hepatic actinomycosis. This was confirmed on tissue cultures. Special staining of this specimen showed gram positive filamentous bacteria.\nInfectious disease consult was called to review the case. They selected doxcycline iv, based on culture and sensitivity report, which would be changed to po doxcycline on discharge. The patient had an uneventful recovery and was discharged home on post-operative day 5. He was discharged home on oral doxycycline for a 6 month treatment course. 30-day follow-up shows no recurrence of this lesion and he continues to remain afebrile.
A 26-year-old Saudi patient was first referred to our outpatient department (OPD) at King Fahad Hospital of the University in January 1994 as a case of recurrent molar pregnancy for the third time with persistent trophoblastic disease. She gave a history of three previous histologically proven molar pregnancies since her marriage two years ago, treated in a peripheral hospital. The patient continued to have vaginal bleeding for a month after the procedure. The serum β-human chorionic gonadotropin (β-hCG) was found to be 15,444 IU and a chest X-ray, renal and liver function tests were normal. She underwent a repeat uterine curettage in her base hospital which showed necrotic endometrial tissue. Unfortunately, she continued to have intermittent vaginal bleeding and hence referred to our hospital after 10 days. Her β-hCG on presentation was 4 458 IU and the ultrasound showed endometrial thickening, so she underwent dilatation and curettage. Histopathology reported necrotic decidua only. The patient was given the oral contraceptive pill and followed up regularly in the OPD with serial quantitative β-hCG estimations until it was undetectable after 4 weeks.\nHer subsequent clinic attendance at our hospital was irregular. The patient returned to the OPD in June, 2004 and reported to have had three more molar pregnancies treated at her initial hospital with suction and curettage in May, 1995; June, 1996; and three weeks prior to presentation. Histopathology report revealed avillous sheets of trophoblastic cells with hemorrhage and necrotic background. She was followed up in her base hospital in the last two pregnancies with serial quantitative β-hCG, until it became negative. By the time she came to us after 3 weeks of her last molar pregnancy, she complained of abdominal pain and shortness of breath. A chest X-ray showed bilateral cannon ball opacities in both lung fields which were thought to be due to metastatic choriocarcinoma; her β-hCG was 987 576.5 IU. The ultrasound of the pelvis showed an enlarged uterus (12.5 × 6.6 cm) with some cystic changes in the endometrial cavity and hypervascularity of the hypoechoic areas and intramyometrium on color Doppler. The left ovary showed three cysts, the largest was 1.7 cm. The CBC, RFT, LFT, PT, and PTT were normal.\nThe patient was referred to our Medical Oncology department for chemotherapy with a diagnosis of metastatic choriocarcinoma. Multiple drug regimen consisting of etoposide, methotrexate, actinomycin, cyclophosphamide, and vincristine was started. The patient required a total of 10 courses. Her last course ended in July, 2007 when she was asymptomatic, the lung shadows had disappeared, and the β-hCG was negative.\nHer last visit to the follow-up clinic was in February, 2010, at 42 years of age, when she complained of six-months of amenorrhea and mood changes. β-hCG was negative, but the FSH was 36 IU/ml. She was considered to be in the phase of menopause and was given a further follow-up appointment after 3 months.
This patient is a 29-year-old white woman from the USA with a medical history significant for severe IBS-D (diagnosed at age 12) and anxiety disorder. In July 2015, she presented with severe bleeding hemorrhoids secondary to IBS, which required hemorrhoidectomy and anal sphincterotomy in August 2015. The week before the surgery she developed pharyngitis and was treated with azithromycin, which resulted in mucousy diarrhea and abdominal discomfort. She tested negative for C. difficile antigen and toxins at that time.\nA week after surgery, she developed a perirectal abscess that had formed at the site of the sphincterotomy and was prescribed orally administered ciprofloxacin. Despite moderate symptom improvement, in September 2015 she required an abscess incision and drainage procedure and Penrose drain insertion. Prior to the surgery she was given a single dose of clindamycin. An additional 2-week course of ciprofloxacin and metronidazole was then prescribed. In late September 2015 she was admitted to the hospital for two nights due to further complications related to the abscess and was then diagnosed as having a perianal fistula.\nIn November 2015, she was prescribed clindamycin for an episode of group C streptococcal-positive pharyngitis. In late November 2015, she was also diagnosed as having Ehlers–Danlos syndrome, which according to her medical record may partially explain the poor wound healing from the perirectal abscess. In December 2015, her fistula required an anus seton placement. She was treated with multiple courses of ciprofloxacin and metronidazole off and on from December 2015 to January 2016.\nIn January 2016, following up on her recurrent pharyngitis, she was diagnosed as having chronic tonsillitis which led to tonsillectomy. In February 2016, 2 weeks after the surgery she was prescribed clindamycin. At the beginning of March 2016, she was diagnosed as having bacterial vaginosis and was prescribed orally administered metronidazole. A week later she was diagnosed as having vaginal candidiasis and was prescribed orally administered fluconazole. In April 2016, she complained of dysuria and was prescribed ciprofloxacin. After 2 days, when urine analysis results came back negative, she was asked by her physician to stop the treatment.\nIn June 2016, she presented for follow-up with ongoing diarrhea and abdominal pain. She was diagnosed as having C. difficile diarrhea, her antigen and toxins laboratory results were indeterminate, and a toxigenic strain was confirmed by polymerase chain reaction (PCR). She was prescribed a 6-week course of orally administered vancomycin. After a week of treatment her symptoms worsened, and following discussion with her gastroenterologist her treatment was switched to a 2-week course of metronidazole. Hours later, she was admitted to the hospital for a 4-day period for colitis. Her C. difficile antigen and toxin test returned negative during her admission. She received intravenously administered metronidazole treatment during her hospitalization. Her symptoms improved during her hospital stay, with 1–2 soft bowel movements a day. At discharge her metronidazole course was stopped and she was again prescribed vancomycin, which she took for over a month. She continued to experience GI irregularity (3–5 bowel movements a day) beyond what she had experienced secondary to her IBS prior to her surgeries. In March 2017, she was prescribed rifaximin for 2 weeks to treat chronic diarrhea.\nIn November 2017, she was prescribed a series of clinical intestinal tests (SmartGut™, uBiome Inc., San Francisco, USA) with the instructions to administer the test at home whenever she was experiencing a noticeable change of GI symptoms, then follow-up with her health care provider to discuss the results. This sequencing-based test requires that patients use a sterile swab to transfer a small amount of fecal material from toilet paper into a vial containing a lysis and stabilization buffer that preserves the microbial DNA for transport by mail back to the laboratory for processing, which involves DNA extraction, 16S ribosomal RNA (rRNA) gene amplification, and sequencing []. She first used this test in November 2017, about a month after completing a 2-week course of rifaximin. The results revealed a number of microbial organisms that were outside the healthy reference ranges, but she was negative for all pathogenic organisms included in the test, including C. difficile (Fig. ).\nBetween November and December 2017, her GI symptoms worsened considerably; her daily bowel movements increased from 3–4 to 6–10, stool consistency became more mucous-like and gelatinous, and she was experiencing more pain with defecation. She re-tested with SmartGut™ test again in January 2018. Her results continued to reveal a number of microbial organisms outside the healthy range and, this time, her sample also indicated the presence of C. difficile (Fig. ). She immediately contacted her primary care provider, who re-tested her for C. difficile and confirmed indeterminate CDI by antigen and toxins A and B. Additional PCR testing at a regional laboratory confirmed the sample was positive for a toxigenic C. difficile strain. As a result of testing, her clinician started her on fidaxomicin; her symptoms improved rapidly. By April 2018, she had returned to her baseline in regard to her IBS-related GI symptoms with no blood in her stools.\nIn addition, the second SmartGut™ sample was tested for toxins A and B by sequencing at uBiome Inc. laboratory in San Francisco, USA, which resulted positive for both and confirmed the toxigenic nature of the C. difficile strain.
A 72 year-old male was diagnosed with subungual melanoma removed from the nail bed of his right thumb in 2007 with a depth of 1.0 mm at the site of prior melanoma in situ dating back to 1981. In 2009, he had a recurrence at the same site extending down his digit and necessitating amputation of the distal phalanx. Resected sentinel nodes showed a melan-A positive focus of hyperchromatic atypical melanocytes in one node and S-100 positive dendritic cell staining in the other two nodes. Due to potential surgical complications and patient preference for observation, complete dissection was not performed.\nIn 2010, the patient presented with multiple bullae on the back. The diagnosis of BP was made by a skin biopsy and clinical and pathological correlation [Fig. ]. Disease activity was managed with high dose oral prednisone with eventual taper and disease remission after one year. In 2011, the patient noticed a new lump in his right axilla. Pathology confirmed malignant melanoma with a cKIT mutation (L576P in exon 11) completely replacing a lymph node. Positron-emission tomography (PET) revealed lung nodules, suspicious for metastatic disease. The patient declined a recommended lung biopsy and, after discussion of the risk of BP flare, therapy was initiated with ipilimumab at 3 mg/kg. Several days after the second dose, he experienced severe exacerbation of his BP with mucous membrane involvement for which he was hospitalized and treated with a 9-week course of 60 mg of prednisone. He subsequently maintained control of BP with 15 mg of prednisone daily.\nAfter discontinuing ipilimumab, computed tomographic (CT) scan showed numerous enlarging bilateral pulmonary lesions and mediastinal lymphadenopathy. Magnetic resonance imaging (MRI) of the brain showed small volume brain metastases to the left internal auditory canal and the inferior aspect of right cerebellum, which were clinically asymptomatic. He declined radiation therapy for the brain lesion and began therapy with nilotinib, a small molecule tyrosine kinase inhibitor for cKIT mutant melanoma, at a dose of 400 mg twice daily. After three weeks of treatment, CT scan showed interval improvement in previously noted mediastinal lymphadenopathy. Over the following year, his nilotinib dose was reduced to 200 mg BID or held due to elevated liver function tests and prolonged QT interval. During this time, imaging showed interval progression of his brain and lung metastases. He received stereotactic radiosurgery to the right cerebellar lesion with subsequent stabilization of the brain lesions on imaging.\nIn 2014, he developed new osseous metastases to the thoracic vertebrae. Nilotinib was discontinued and he received palliative radiation to the spine for local disease control. At this time the patient completed prednisone taper without flare of his BP. The patient then received pembrolizumab, at a dose of 2 mg/kg given every three weeks. The first 3 doses of pembrolizumab were well tolerated with minimal bullous eruption, which was controlled with clobetasol 0.05 % topical cream. Imaging after 10 weeks of treatment showed essentially stable disease, and a cutaneous metastasis on his wrist flattened and regressed [Fig. ]. However, following the fourth cycle, the patient developed a severe BP flare resulting in discontinuation of pembrolizumab and the start of oral prednisone at 60 mg a day. Over the next three months, the patient’s BP lesions had resolved and prednisone was tapered off, allowing for a fifth cycle of pembrolizumab [Fig. ]. The patient again developed a flare of BP requiring patient to stop anti-PD1 and again start prednisone at the same dose. He had improvement of skin lesions but was unable to taper below 15 mg without worsening symptoms. One year following initial treatment with pembrolizumab, scans show stable disease with unchanged brain, pulmonary, and osseous metastases. Clinically, the patient feels well and denies symptoms related to his metastases. He is undergoing continued monitoring of both his metastatic melanoma and his BP.
A 44-year-old Japanese male, an office worker, was transported to our emergency department with a complaint of temporary loss of consciousness. He had experienced fatigue for 3 years and had fallen easily in his daily life starting 2 months before admission. He was feverish and had a cough and phlegm for several days before admission. On the way to work, he felt drowsy and fell down the stairs of the train station, necessitating transport to our hospital by ambulance. The peripheral blood analysis noted mild elevation of white blood cells. The blood chemistry test disclosed mild elevation of C-reactive protein and mild liver dysfunction (). He was thin, i.e., his height was 174 cm and he weighed 52 kg (). Chest computed tomography (CT) showed severe infiltration in the upper posterior fields of both lungs as well as food debris in the esophagus. He was diagnosed as having aspiration pneumonia. His past medical history included diabetes mellitus that had been treated by a local doctor; glycated hemoglobin was approximately 7%. He had experienced ileus six times since age 30 years. He was not married. His father had died of dilated cardiomyopathy at the age of 70 and had also been thin. On the other hand, his mother was healthy but his maternal uncle was diabetic. His younger sister (Case 2) also had mild muscle weakness of the four extremities. Aspiration pneumonia was treated by intravenous administration of antibiotics under conditions of food-take restriction combined with temporary insulin infusion, and the respiratory symptoms subsided. On the neurological examinations after improvement of pneumonia, he presented with typical clinical manifestations of DM1 (), such as forehead balding, hatchet face with bilateral ptosis, nasal speech, mild muscle weakness of the four extremities, handgrip myotonia, and diffuse muscle atrophy. Electromyography performed to test the biceps brachii muscle and femoral quadriceps muscle on the left side revealed frequent myotonic discharges. Because DM1 was strongly suspected, we recommended that, after providing informed consent during genetic counseling, he undergo genetic testing for DM1 together with his younger sister. The number of CTG repeats in the DMPK gene was abnormally expanded to about 600 repeats in the elder brother and about 900 repeats in the younger sister, in contrast to those of healthy individuals who have 5 to 37 repeats (), thereby confirming the diagnosis of DM1 in these siblings ().
The second patient is a 47-year-old male with a nonischemic cardiomyopathy who was admitted to the hospital in October 2010 with signs and symptoms of acute sepsis. The patient had a single-chamber ICD implanted in July 2008, followed by implantation of the LAP monitor in September 2008. Both the ICD and LAP monitor were implanted via the left subclavian vein from a superior approach with two separate subcutaneous device pockets created in the left pectoral region. The patient had recurrent fevers with core body temperature measurements recorded by the LAP monitor up to 39.8°C for 3 days prior to hospitalization, and in-hospital blood cultures growing methicillin-sensitive S. aureus. The ICD pocket had some erythema without fluctuance. A computed tomography scan of the chest showed multiple pulmonary lesions that were suspicious for septic emboli. A TEE study showed a density located on the ISL within the right atrium that was suspicious for possible vegetation. There was no evidence of any thrombus or vegetation within the left atrium. Because of the worsening clinical status and progressive sepsis despite antibiotic therapy, a decision was made to remove the ICD and LAP monitoring systems.\nUnder intravenous sedation with local anesthesia, the device pockets were opened and all hardware was dissected free of the fibrous tissues and externalized. Under fluoroscopic guidance, the right ventricular defibrillation lead (Durata™, Model 7120, St. Jude Medical) was extracted using direct traction and rotation from the left subclavian superior approach without difficulty. The ISL was then extracted from the interatrial septum with the locking stylet (Liberator™, Cook Medical) using traction alone also from the left subclavian superior approach. The ISL was liberated easily from the septum, and there did not appear to be any other intracardiac or vascular adhesions associated with the ISL. However, upon withdrawing the ISL toward the left subclavian venous entry site, the sensor module could not be removed, and it was necessary to apply counter traction with an 11.5-French polypropylene sheath (Bryd, Cook Medical) to allow the sensor module to slide out of the vasculature. The extracted ISL had a faint amount of a tissue capsule partially over the sensor module and no tissue covering the remainder of the ISL, including the fixation anchors and the ring electrode. There was no damage caused to the fixation anchors. The patient tolerated the procedure well without complications.
A 40 year old woman presented at our clinic with severe mitral valve rheumatic stenosis. Intraoperative findings showed a typical rheumatic disease with commissural fusion and thickened valve leaflets, but a relatively preserved mitral valve apparatus.\nWe performed a mitral valve repair consisting of bilateral commissurotomy papillary muscle splitting and placement of a rigid anuloplasty ring (SEGUIN, St. Jude Medical) N. 30. Intraoperative echocardiography showed a good performance of the valve. No stenosis and regurgitation were detected. The postoperative period was uneventful and the patient was discharged on the 7th postoperative day in good conditions. No signs of pericardial effusion were noted on echocardiography.\nThe patient was readmitted at our institution a week after on an emergency basis with clinical signs of cardiac tamponade. After confirmation by echocardiography of the presence of massive pericardial effusion, a central venous 18 F catheter was placed in the pericardial space. An amount of 1300 milky fluid was drained gradually and the patient get better. The pericardial fluid laboratory analysis showed chylomicrons and elevated triglycerides. Lymphocytes were seen on microscopy. The diagnosis of chy lopericardium was obvious. Microbiology studies were negative. Parenteral nutrition was initiated and oral nutrition was stopped. Somatostatin 0,6 mg three times a day and antibiotic prophylaxis was constituted. The patient was treated conservatively for 30 days.\nThe average daily drainage was 75 ml but it did not decrease and stop during this period. Under these circumstances and taking in consideration the prevention of other complications we decided to reexplore the patient in sternotomy in an attempt to find the place of the leakage and close it. The patient was given to eat fat 12 hours before surgery and on the operating room by gastric tube. No place leakage was observed. We ligated and sutured thymus gland remnants and the pericardial edges with double suture prolen 4/0. Additionally Bioglue (Bio Glue Surgical Adhesive, Cryolife) was place over the sutured tissues to increase the possibility of closing the point of leakage. The right pleural space was opened for drainage and the patient was closed in the standard manner.\nThe postoperative period was without any complications. The drain tubes were taken off three days after with no signs of chyle leakage. Oral nutrition was normally commenced. On echocardiography there was no pericardial or pleural fluid 10 days after the intervention on discharge of the patient and 25 days later on control.
A 31-year-old Sri Lankan woman, who was a known patient with systemic lupus erythematosus (SLE), mild persistent bronchial asthma and a history of recurrent urinary tract infections, presented with left wrist pain.\nShe was diagnosed with SLE 5 years previously, when she presented with photosensitivity, malar rash, inflammatory polyarthritis and supportive immunological criteria. She was treated with 1 mg/kg dose of oral prednisolone initially which was subsequently tapered to a lower dose. She was on mycophenolate mofetil with good disease control and without any organ threatening complications, when she developed wrist pain.\nOne year prior to the admission she developed gradually worsening pain in her left wrist, which was more towards the end of the day and worse with activity. She did not notice morning or inactivity stiffness. There was no proceeding history of trauma and other joints were not involved. She did not notice fever, oral ulcers or rashes during this period. However, she complained of fatigue. She did not notice numbness or weakness over the hand. She denied productive cough with hemoptysis or a contact history of TB.\nThe symptoms worsened over time with worsening disability and limitation in wrist movements. She developed intermittent short lasting bouts of fever during the course of the illness, which was treated by her general practitioner. The diagnosis was urinary tract infections in these instances based on urine full reports showing pyuria and organisms. Urine cultures were not done at these instances.\nThe patient was assessed by the physicians and rheumatologists from the onset of symptoms. She underwent radiography and hematological investigations, which did not reveal significant abnormalities. The pain was presumed to be due to SLE related synovitis.\nShe then developed persistent low-grade pyrexia for about 1 month, predominantly in the evenings with worsening joint pain. She noted joint swelling over 1 week, and the joint became excruciatingly painful with restriction of movements in all directions.\nApart from mild pallor and left wrist swelling, the general examination was unremarkable. There was clinical evidence of synovial thickening and effusion of the wrist joint, with marked tenderness and impairment of function. The power of the hand muscles was slightly diminished with wasting, without sensory impairment. System examination was unremarkable.\nOn admission the patient was clinically diagnosed with septic arthritis and urgent arthrotomy and wash out was done. She was started on intravenous cloxacillin and ceftriaxone after obtaining cultures. Investigations to assess the presentation and SLE disease activity were done (Table —investigation findings).\nShe had normal wrist and chest radiographs, serum creatinine and liver function tests. The positivity of acid fast bacilli and granulomatous inflammation in synovial biopsy was suggestive of tuberculous arthritis. Mantoux test was negative; however this was interpreted with caution in the background of immunosuppression. Her chest radiograph did not show evidence of past or present TB. Although the presence of acid fast bacilli and granuloma on synovial biopsy were suggestive of TB, non-tuberculous mycobacteria causing infection had to be excluded.\nTB PCR and cultures were subsequently available confirming Mycobacterium tuberculosis infection.\nIntravenous cloxacillin and ceftriaxone were continued for 3 weeks until the diagnosis of TB was confirmed. The patient underwent repeated surgical drainage of the wrist 2 weeks later, as there was increasing joint swelling with expanding abscess formation. She was seen by the rheumatologist in order to optimize therapy, and was also referred for physiotherapy and occupational therapy.\nThe patient was educated on the diagnosis and the need of long term treatment. She was referred to the chest clinic where she was started on isoniazid, rifampicin, ethambutol and pyrazinamide along with pyridoxine. The baseline liver and renal functions were assessed prior to commencement of therapy. The treatment was continued for 2 months and a continuation phase of 10 months with isoniazid and rifampicin was planned.
A 56-year-old female presented with an acute onset of severe headache after coughing described as "a disaster for the first time in my life", associated with mild nausea. There was no focal neurologic deficit. The patient's blood pressure was 150/90 mm Hg. There was no previous history of migraine or other type of headaches, and she denied any regular medication. Initially, head CT scan revealed isolated cortical SAH within the sulcus of right high frontal lobe (). No cerebral vasoconstriction, venous sinus thrombosis, cerebral aneurysms or arteriovenous malformations were found on digital subtraction angiography (DSA) and CT angiography performed immediately after admission (). After four days, CT angiography revealed multiple segments of irregularity consisting of narrowed areas of both middle cerebral arteries (M2 tracts) and right posterior cerebral artery (P2 tract) with no evidence of arterial vasoconstriction at proximal cisternal segment indicating SAH-related vasospasm (). However, no aneurysms or arteriovenous malformation were identified.\nPhysical and neurological examination findings were normal, and ophthalmologic examination excluded the possibility of retinal vasculitis or inflammation. Blood chemistry workup including coagulation function test, hormonal workup, hepatic and renal function test, and serum immunological screening for rheumatologic disease or vasculitis were all negative or within normal limits. Cerebrospinal fluid analysis was near normal except for the presence of red blood cells indicating SAH. There was neither leukocytosis nor increased protein levels typical of primary angiitis of the central nervous system (PACNS). Transthoracic echocardiography and electrocardiography showed no definitive wall motion abnormality or arrhythmia. Intravenous nimodipine for vasodilatation with blood pressure control was applied under suspicion of RCVS, and oral analgesics were prescribed for her severe headache. Her symptoms improved over the next three days.\nFive days later, she suffered deterioration of neurological status, stuporous mental change and motor weakness of the right side extremities after coughing. Her blood pressure was checked up to 200/120 mm Hg. CT scan showed huge intracerebral hematoma on left cerebral hemisphere, opposite to the first SAH presentation (). There was no newly developed lesion in CT angiography. Emergent decompressive craniectomy and hematoma removal was performed, and she was transferred to the intensive care unit for close observation. Intravenous nimodipine was used for blood pressure control and treatment of RCVS, combined with mannitolization for intracranial pressure control.\nAfter 12 day, CT scan showed reduction of cerebral edema and hematoma (). Her mental status gradually improved to drowsy, and she was transferred to general ward and then received oral amlodipine mesylate. But, her hemiplegia did not change and motor aphasia was found according to improvement of mental status. She complained intermittent mild headache, improved with analgesics. On day 30, she was transferred to department of rehabilitation for efficient recovery of motor and speech function. One week thereafter, she experienced sudden-onset headache of moderate degree and nausea not severe than her first symptoms, and brain MRI showed hyperintense signals in the pons on diffusion-weighted imaging, which are suggestive of acute to subacute cerebral infarction (). Without additional treatment, this event was settled uneventfully. After 6 weeks and 8 weeks on admission, CT angiography and DSA exhibited marked resolution of vasoconstriction (). By the end of three months, there was no evidence of recurrent symptoms and signs.
The patient is a 61-year-old Korean male with a significant past medical history of rheumatoid arthritis, hypertension, and diabetes mellitus who presented to the hospital with sudden onset of blurry vision in his left eye three days prior. A head CT had been performed at an outside facility, which was negative for acute ischemia, hemorrhage, midline shift, or extra-axial fluid collection. The patient denied visual complaints in his right eye and had no history of similar events.\nOphthalmologic and Neurologic consultations initially revealed visual acuity was 20/40 on right eye; however, on the left eye, there was an apparent left-sided visual loss. Patient's intraocular pressure was normal in both eyes, pupils were equally round and reactive to light, and no afferent pupillary defect was observed. Moreover, the extraocular muscles were intact and with full range of motion. In the right eye, visual fields were full to finger count. In the left eye, the acuity in the nasal visual field was greater than that in the temporal visual field. In the nasal visual field, the patient was able to count fingers; however, in the temporal visual field, the patient could barely detect gross hand movement at one foot out. External examination was within normal limits, and pen light examination was only remarkable for nuclear sclerotic cataracts bilaterally. Dilated fundus examination demonstrated no evidence of pathology to the vitreous, optic nerve, or retina that might explain the vision loss. Four days later, repeat examination by Ophthalmology demonstrated a stable right eye; nonetheless, the left eye temporal visual field was 20/25 and patient was able to count fingers without mistake on left visual field. Pupil and retina examination were unchanged from previous examination. However, visual field mapping could not be done as this patient was seen in the inpatient setting.\nConsequently, an initial head MRI with and without contrast was performed, which showed focal areas of restricted diffusion in the right medial temporal lobe, inferior right basal ganglia with possible involvement of the right lateral geniculate nucleus. The head MRI did not show any other pathology such as masses or hemorrhages (Figure ). The subsequent head MR angiogram done demonstrated no focal occlusion or stenosis, and the MRI of the orbits showed no focal defects bilaterally. At this point, it was evident that the patient was suffering from a left temporal monocular hemianopia and that there must be a correlation with the subtle but definitely present right LGN ischemic lesion. An infarct affecting the right lateral geniculate nucleus would most likely cause bilateral left homonymous hemianopia. Nevertheless, a lesion at the vicinity of right LGN that had taken out the inputs going to layers 1, 4, and 6 became generally accepted among all physicians involved at this point in the care of this patient as the only possible explanation. Patient's left visual symptoms slowly started to improve, and after four days of hospitalization and monitoring, patient's left monocular temporal hemianopia had almost resolved and his vision had almost returned to his normal baseline.
A 28-year old native female patient complained about recurrent pain in the right hip joint without previous trauma progressing by physical strain starting about 1.5 years before. A MRI of the pelvis performed 3 months before admittance showed a swelling of the capsule and reduction of the cartilage in the right hip joint which was considered as an early arthritis or coxitis. After treatment with physiotherapy and oral non-steroidal antirheumatic medication the symptoms were regressive and no further diagnostic procedures were performed.\nCaused by recently aggravation of pain in the right hip joint within the days before, the patient was not able to stand and walk and the range of motion (ROM) was painfully reduced. Blood samples were normal except slightly increased CRP of 4.2mg/l (reference: <0.5mg/l) without any local signs of inflammation in the pelvic region. X-ray of the right pelvis showed a slight joint space narrowing with affection of the acetabulum and the femoral head (Fig. ). An effusion was detected by ultrasound, aspiration of the hip joint led to no further diagnosis as aerobic and anaerobic cultures were sterile. The MRI showed a joint space narrowing in the cranial part of the acetabulum with increased subchondral sclerosis as well as a synovial enhancement with bone marrow edema in the femoral head and the corresponding acetabulum.\nThe further clinical examination with detailed reconstruction of patient´s history revealed a surgical procedure with extirpation of a lymph node from the left supraclavicular region two years before. The histological result was suspicious for tuberculosis with marked granulomatous lymphadenitis and caseous degenerated confluent necrosis, but tuberculin test and sputum sample examinations by microscopy and culture medium were negative. A CT without any proof of lymphadenopathy and pulmonary infiltration could not confirm this suspicion. In addition there were no other risk factors except her profession as a veterinarian, especially as she was neither an immigrant nor had long-termed stays in foreign countries. So no further treatment had been started at that time.\nAlthough the initial microbiological examination of the first aspirate being inconspicuous, a current Quantiferon-Test [] showed a positive reaction as an indication of a latent or active infection with Mycobacterium tuberculosis (M. tbc), Mycobacterium Kansasii, Mycobacterium Szulgai or Mycobacterium Marinum. X-ray of the chest showed an enhanced parenchymal change in both apical segments. A consequently performed CT of the chest and abdomen revealed the clinical sign of reactivated tuberculosis in the apical and posterior bronchopulmonary segments with nodular peribronchiovascular focus as a sign of open lung tuberculosis. Furthermore a second aspiration of the right hip joint confirmed M. tbc by microscopical analysis and bacterial culture. Gastric secretion examined by polymerase chain reaction (PCR) as well as bacterial culture and microscopy of the sputum showed an amount of M. tbc.\nThereafter by confirming TBC and open lung tuberculosis, the patient was treated with combination of oral Isoniazid, Rifampicin, Ethambutol and Pyrazinamid. She received physiotherapy with partial weight bearing of 10 kg.\nThree months later X-ray showed an increasing patchy transparency in the femoral head and neck as well as in the greater trochanter and acetabulum with clear progressive joint space narrowing in the corresponding parts of the hip joint (Fig. ). A complementary MRI with contrast medium five months after starting therapy showed active arthritis with progressive destruction of the right hip joint and a new appearance of liquid retention in the right acetabulum as a sign of tuberculous osteomyelitis (Fig. ).\nAfter seven months the microbiological examination of gastric secretion and sputum analysis did not detect open lung tuberculosis, so the oral therapy was reduced to double combination with Isoniazid and Rifampicin. In contrast to X-ray and MRI results with progressive destruction, the ROM of the right hip joint improved. The patient underwent reconstructive surgery by extirpation of the granuloma from the acetabulum and augmentation with autologous spongiosaplasty from the ipsilateral iliac crest. Microbiological and microscopic examination of intraoperative swabs and surface biopsies did not detect M. tbc or other pathological results.\nSix weeks after surgery, continuing physiotherapy and oral double combination, the patient was almost free of pain and ROM was improved. X-ray of the right pelvis revealed an unchanged result compared to the examination after operation without any progression of destruction and regular positioned autologous spongiosaplasty without dislocation. The physical load for the right leg was gradually increased and ROM was continuously improved.\nTen months after starting oral therapy, a CT of the thorax detected scars without nodular pathologic infiltrates or pleural effusion in both apical parts of the lungs.\nThe ROM of the hip joint in spite of the radiological findings with progressive sclerosis in the acetabulum and joint space narrowing (Fig. ) was satisfactory measured with 125° for flexion, 35° for abduction, 20° for adduction, 5° for internal rotation and 30° for external rotation. The patient was able to walk without crutches suffering only minor pain.
A 67-year-old man with a medical history of kidney transplant, chronic renal dysfunction, prior cytomegalovirus infection causing retinal damage and vision loss and prescribed valacyclovir presented to the emergency department (ED) with a complaint of hallucinations and weakness. This was the patient’s fifth healthcare encounter in three weeks. The first visit was to the ED for heel pain, and he was discharged home after an unremarkable right foot radiograph. The patient then returned to the ED for his second visit with a painful vesicular rash along the second sacral dermatome of his right leg and was prescribed valacyclovir 1 gram orally three times a day for seven days for shingles. Vaccination status was unknown at the time of diagnosis.\nOn the third ED visit two days later, the patient presented with vomiting after being seen by his primary care doctor that morning. The patient was able to tolerate two doses of valacyclovir; and while being seen by his primary care doctor, his valacyclovir dosing was adjusted to account for his renal disease. The patient also was experiencing hallucinations but was discharged home with the explanation that his symptoms could have been due to dehydration after a “negative workup.” On his fourth visit to the ED seven days later, the patient stated that he would “close his eyes and see bands playing and rolling plains of green grass.” He stated that these images were very vivid but would go away when he opened his eyes. The patient also had difficulty ambulating and generalized weakness. A family member reported that he also had difficulty with finding words.\nVital signs during this fourth ED visit included the following: temperature 99.4° Fahrenheit; pulse 92 beats per minute; respiratory rate 20 respirations per minute; room air pulse oximetry 98%, and a blood pressure of 196/91 millimeters of mercury. Physical examination revealed crusted lesions following the second sacral dermatome on the posterior right leg extending from the sacral region to the lower calf. A neurological exam revealed generalized weakness and difficulty with ambulation without any focal deficits.\nLaboratory testing, including complete blood count, metabolic panel and urinalysis were unremarkable except for serum blood urea nitrogen, creatinine and glomerular filtration rate, which were 23.1 milligrams per deciliter (mg/dL) (normal range 6.0–20.0 mg/dL), 3.03 mg/dL (normal range 0.67–1.17 mg/dL) and 22 milliliters per minute (mL/min) (normal is >60 mL/min), respectively. Chest radiograph was unremarkable and brain computed tomography (CT)demonstrated only chronic mild to moderate degenerative changes. Based on the recent diagnosis of shingles, history of immunocompromise and hallucinations with weakness, lumbar puncture was performed. Results included elevated protein with lymphocyte predominance consistent with viral infection. Cerebral spinal fluid (CSF) culture was ordered, and the patient was administered one gram of acyclovir intravenously and admitted to the hospital.\nOn hospital day one CSF culture demonstrated VZV via polymerase chain reaction (PCR). The patient also underwent brain magnetic resonance imaging (MRI) on hospital day two, which showed moderate chronic microvascular ischemia and abnormal appearance of the distal left vertebral artery. Infectious disease, neurology and hospital medicine teams all evaluated the patient and agreed with the diagnosis of VZV encephalitis in the setting of recent shingles, CSF findings, and patient presentation. The patient was administered a two-week course of acyclovir with improvement of his hallucinations and presenting symptoms prior to discharge on hospital day four.
A 69-year-old woman was referred to our institute for further evaluation of a tongue mass. She had a 5-year history of pain involving the right lateral tongue edge, and the lesion had been diagnosed as lichen planus by incisional biopsy performed by her dentist. The disease was stable for a long time; however, the tongue pain suddenly worsened. Physical examination revealed an elastic, hard, 1.2 × 0.7 cm mass of the right tongue (Fig. ). There was no palpable lymphadenopathy in the head and neck area. The patient was being medically treated for hypertension and diabetes mellitus, both of which were well controlled. She denied using tobacco or alcohol but had been exposed to smoke from her family. She had no history of exposure to ionizing radiation or having undergone cancer chemotherapy. Regarding her family history, her younger brother had been treated for rectal cancer and liver metastasis.\nContrast-enhanced CT scans of the head and neck, chest, and liver were performed as a routine procedure in patients with oral cavity cancer. CT showed no lesions in the tongue, cervical lymph nodes, lungs, bone, or liver; however, three masses were detected in both breasts and the right kidney. Whole-body FDG-PET was performed to identify any other lesions; no other lesions were found. An upper gastrointestinal examination, indirect laryngoscopic examination, and bone scintigraphy revealed no abnormalities. The patient had no breast- or kidney-related signs or symptoms before we identified the masses. Fine-needle aspiration cytology was performed to confirm the diagnosis of the bilateral breast masses and revealed malignancy on both sides. Renal cancer was suspected based on the CT findings (Fig. , arrow). All four lesions were diagnosed as early-stage cancers at the initial presentation.\nWe removed the four tumors over three surgical procedures because the patient had diabetes mellitus. The patient underwent wide local excision of the tongue tumor. Histopathological examination revealed a moderately differentiated SCC. The bilateral breast lesions were then resected, and histopathological examination revealed an invasive ductal carcinoma (positive for estrogen receptor and human epidermal growth factor receptor 2, negative for progesterone receptor and MIB-1; 7–8 %) of the right breast representing a scirrhous growth with one metastatic axillary lymph node. An intraductal carcinoma of the left breast (MIB-1, 7–8 %) was also found. After resection of these lesions, laparoscopically assisted partial resection of the right kidney tumor was performed. Histopathological examination revealed a chromophobe RCC with positive cytoplasmic Hale’s colloidal iron staining. Immunohistochemistry, cytokeratin 7, CD10, and E-cadherin were positive.\nIn summary, we resected four synchronous MPCs including a moderately differentiated SCC of the mobile tongue, invasive ductal carcinoma of the right breast, intraductal carcinoma of the left breast, and chromophobe RCC of the right kidney. The patient received adjuvant therapy (radiation therapy and anastrozole) for both breast cancers. After a 5-year follow-up period, the patient was alive and well with no clinical or radiologic signs of recurrent or metastatic disease.\nThis case illustrates two important clinical issues. This combination of MPCs, namely that involving the tongue, bilateral breasts, and kidney, has not been reported to date. To avoid overlooking kidney MPC, we suggest that contrast-enhanced CT should cover an extended region ranging from the liver to the inferior margin of the kidney in patients with oral cavity cancer.\nFirst, regarding the fact that this combination of MPCs has not been reported to date, we searched PubMed and the Japan Medical Abstracts Society databases but found no case reports of the same combination of cancers. The current case was defined as MPCs according to the Warren and Gates criteria []; namely, each cancer must be distinct, and the probability of one being a metastasis of the other must be excluded. Using this definition, all four neoplasms were determined to be primary cancers. Furthermore, we defined these cancers as synchronous cancers based on Moertel’s definition; that is, a primary tumor recognized within or after 6 months of diagnosis of another primary tumor is defined as synchronous or metachronous, respectively []. We thus diagnosed the present case as synchronous quadruple MPCs. We believe that the patient had three risk factors that contributed to this combination of MPCs. First, the patient had a previous diagnosis of diabetes mellitus, which may increase the risk of cancer [, ]. Second, the patient’s family members were chronic, heavy cigarette smokers []. Third, the patient’s younger brother had colon cancer []. Synchronous quadruple MPCs are extremely rare, and all such cancers can be found in the early stage. MPCs in patients with oral cavity cancer mainly occur in the HNLE region []. In contrast, the incidence of non-HNLE cancer, including cancer of the kidney or breast, is relatively low in patients with oral cavity cancer []. The 1973–2000 SEER Cancer Registries indicates that of 26,984 patients with oral cavity cancer, the rate of second primary kidney cancer and breast cancer occurring within 1 year after oral cavity cancer diagnosis was only about 0.03 and 0.11 %, respectively []. There are few reports of oral-cavity–kidney combinations of MPCs [, , , ]. We found no other English-language literature describing synchronous MPCs as oral-cavity–kidney combinations [, –].\nThe second important clinical issue illustrated by this case report is that contrast-enhanced CT should cover an extended region ranging from the liver to the inferior margin of the kidney in patients with oral cavity cancer to avoid overlooking kidney MPC. We usually perform neck, chest, and liver CT scans to detect MPCs or distant metastases in patients with oral cavity cancer. In these cases, we must evaluate the MPCs or distant metastases with nothing but contrast-enhanced CT. We performed a liver CT scan containing the kidneys and incidentally noticed an early-stage mass of the right kidney. In patients with oral cavity cancer, the probability of detecting kidney cancer is low according to the 1973–2000 SEER Cancer Registries []. However, recent reports have indicated that the lifetime risk for developing kidney cancer is about 1.6 % [] and that the incidence of RCC has continued to increase []. Furthermore, early-stage RCC can remain clinically silent for a long time, and the clinical symptomatology is generally expressed in patients with advanced disease []. Advanced or metastatic RCC is associated with a much lower 5-year disease-specific survival rate than is early-stage RCC []. According to the lack of symptoms and the invisibility with FDG-PET, hidden synchronous renal cancer in patients with oral cavity cancer may be much more frequently present than previously thought. Therefore, clinicians should detect the kidney cancer in the early stage using an extended CT scan with consideration of both the benefits and risks, the latter of which include high cost and radiation exposure. The risk of development of secondary kidney or breast cancer after oral cavity cancer is not particularly higher than the risk of after other HNLE or skin (non-melanoma) cancers [, –]. Therefore, in patients with not only oral cavity cancers but also other HNLE or skin cancers, hidden kidney cancer may be detected by CT covering an extended region ranging from each routine region to the inferior margin of the kidney.\nIn general, the performance of several types of examinations can help to prevent overlooking synchronous MPCs. FDG-PET is usually used to identify synchronous MPCs; however, FDG-PET sometimes identifies false-positive lesions. In the current case, we could not detect the kidney mass by FDG-PET. The application of FDG-PET to the urinary tract is relatively limited because this tract is the major excretion route for FDG, and background activity may thus obscure the presence of lesions []. Additionally, FDG-PET is generally expensive; therefore, some patients with oral cavity cancer are denied this examination before cancer therapy.
A 25-year-old woman presented to an emergency department complaining of a year long history of worsening back pain, 6 months of worsening right leg weakness and 3 months of progressive right foot drop. She denied any bowel or bladder incontinence but did admit to decreased perianal sensation over the previous 3 weeks. A lumbar spine MRI revealed a large sacral mass. She was then transferred to our tertiary care facility where she was evaluated by the Spine service. Physical examination revealed profound right lower extremity weakness in the L5-S1 distribution with absence of deep tendon reflexes. Sensation was decreased in the anterior tibial, plantar and dorsal foot distributions. She had decreased perianal sensation but intact rectal tone.\nImaging\nPlain film imaging revealed intact vertebral alignment and no suspicious vertebral deformities. A lytic lesion of the right sacrum however was evident. A lumbar spine MRI with and without contrast demonstrated a large 6 by 8 centimeter multilobulated heterogeneously enhancing mass centered over the right iliac wing that extended into the posterior elements of S1 and the right paraspinal muscles. There was extension into the right S1 and L5 nerve roots and sciatic notch.\nOperation\nThe patient was taken for an urgent decompression without further workup. A midline incision was used for a bilateral L4 to S2 laminectomies, facetectomies and foraminotomies with microscopic assisted epidural tumor resection from the right epidural space. The specimens were sent to pathology and were consistent with a chondrosarcoma. Due to these findings, the musculoskeletal oncologist was consulted for further recommendations.\nPostoperative Course\nThe patient had partial resolution of her numbness but continued to have a dense foot drop. She completed the appropriate malignancy workup which revealed no evidence of metastatic disease. As the imaging and patient’s age were not consistent with a chondrosarcoma she underwent an open biopsy of the right ilium. Her final pathology was consistent with a chondroblastic osteosarcoma. She was discharged and underwent neoadjuvant chemotherapy with plans for a definitive resection after completing chemotherapy.\nThe patient then underwent repeat staging studies which were negative for metastatic disease. Her pelvic and lumbar spine imaging demonstrated tumor growth in the areas of contamination from the initial surgery. The subsequent procedure required a more extensive resection due to seeding from her index surgery. She underwent a staged procedure with an anterior approach followed by resection from a posterior approach. Her previous posterior midline incision was excised and her paraspinal muscles and spinal cord had to be resected bilaterally at the level of the previous surgery. She underwent a right internal hemipelvectomy, complete sacrectomy, and complete L5 resection, ligation of the thecal sac at the L4-5 level with ligation of the sciatic nerve at the sciatic notch, and L1 to ilium fixation and arthrodesis with morselized allograft and a fibular strut allograft. Coverage of the posterior defect was necessary with a pedicled vertical rectus myocutaneous flap. The initial decompression forced her left sacrum, L5 vertebrae and spinal cord to be resected. Her final pathology demonstrated negative margins with 75% tumor necrosis.\nFollowing the definitive resection, she required three irrigation and debridements for superficial wound breakdown. She also underwent revision of a failed iliac screw with harvest of autograft from the right femur using a Synthes Reamer Irrigator Aspirator as there was no evidence of fusion three months from her initial fusion. She then completed a full course of physical therapy and returned to her home four months following her definitive resection. The patient was able to transfer to a wheelchair at the time of discharge and had evidence of early consolidation of the fusion. She was found to have a deep vein thrombosis in her right iliac vein at nine months after surgery. Twelve months postoperatively, she is without evidence of recurrent or metastatic disease.
A 35-year-old male patient reported to the outpatient department of our institute, Maratha Mandal's Nathajirao G. Halgekar Institute of Dental Sciences and Research Centre, Belagavi, on July 7, 2019, with a chief complaint of pain in the left and right lower jaw area for 5 months while having food. On detailed history, the patient informed that he experiences acute pain (10/10 on Numerical Rating Scale) on having the first bite of meal and gradually subsides on subsequent intake of food and subsides completely in few minutes after completion of meal. The pain was described as sharp and stabbing type. He experiences maximum pain while having the first bite of his food in the morning, and subsequently, with every first bite of the meal in a day, he experienced pain, but the intensity of pain reduced from morning to evening. During dinner, the intensity of pain experienced on the first bite of the meal was least. Because of pain during eating, the patient developed aversion for food which led to his weight loss.\nPertaining to medical history, the patient had met with an accident in 2014 and was hospitalized for 2 months and recovery was uneventful. Later in October 2018, he had a head injury following a fight where he was hit with a rod on the head, which was sutured, followed by which he was treated as a day-care patient. During this time, the patient was diagnosed with diabetes. Since then, he is on medication for diabetes (Glycomet 250 mg). The patient's blood glucose level was in normal limits with controlled level of glycosylated hemoglobin. Later in January 2019, he started experiencing pain in the parotid region while having food.\nHe initially visited an ENT specialist for the same, where he was diagnosed to have parotitis and was prescribed with medication which did not give any permanent relief. The patient is unaware of the medicine prescribed. Later, he visited another two ENT specialists. He was advised for scanning. Ultrasonographies of both right and left parotid glands were taken []. A computed tomography scan was taken from the base of the skull to the lung apex. No relevant findings or abnormalities were noted except for a slight enlargement of the glands []. He was asked to continue with the same medication, which did not give him any permanent relief. Hence, he was referred to dental institute.\nOn examination, bilateral swelling was seen on the angle of mandible with raised earlobe []. On palpation, tenderness was elicited on the left side. On milking of parotid glands, clear but scanty fluid was noted. On intraoral and extraoral examination, no other abnormalities were detected.\nBased on the history, symptoms observed and records of imaging, a final diagnosis of FBS was arrived at.\nFrom July, the patient was prescribed with drugs for neuropathic pain that is Pregeb M 75, one tablet once daily, and Teedal, one tablet twice daily. The patient was called for weekly follow-up. After 5 months patient showed 80% reduction in pain and rated 2/10 on pain scale for pain. The patient is still under follow-up.\nOn literature search, we found six published reports (one case series and five case reports) of IFBS, out of which two case reports were in Japanese language and abstract was available in English; hence, it was also included in review [].\nThe first case was reported in the year 2010 by Kurokawa et al. of a patient with a 6-month history of pain in the parotid region that occurred after the first bite of every meal. Pain decreased after 6 repeats of stellate ganglion blocks (SGBs) and completely disappeared after 16 repeats of SGB.[] The patient had no history of trauma or surgery.\nWemyss et al. reported a case of the patient who had severe, sharp unilateral pain on the first bite of eating. No abnormalities were detected with respect to TMJ, PPS, trigeminal nerve or parotid gland. FBS was suspected, and the patient was immediately started with carbamazepine 200 mg, three times daily. It was proved to be successful in treating, and in 4 months, symptoms resolved completely.[]\nA similar case with symptoms consistent with FBS was reported by Stoopler et al. Medical therapy of the patient included oxcarbazepine and gabapentin without benefit. Later, patient was given botulinum toxin A injections to the parotid gland and patient reported improvement in pain with the treatment.[]\nChiba et al. reported case series of 14 patients with idiopathic parotid pain (IPP), and they opined that clinical picture and pathogenesis of IFBS remains unknown and also only a few cases have been reported in the literature, where the difference in clinical presentation of IPP and IFBS has not been clearly described. Hence, they referred their cases as IPP instead of IFBS. Out of 14 cases analyzed retrospectively by checking the medical records, they considered only 9 cases that were diagnosed with diabetes as IPP and the rest 5 cases were not considered as they had no history of diabetes. Their main aim was to find if there was any association of diabetic neuropathy with FBS. In this study, the authors ruled out various pathogeneses reported earlier such as the loss of sympathetic innervations to the parotid gland and gustatory stimuli which evoked facial pain. Thus, the pathogenesis of IPP in the study remained to be elucidated. They believed that there might be an association between diabetic neuropathy and pathogenesis of IPP, but it is unlikely that the diabetic neuropathy alone contributed to the development of IPP. Thus, the relationship between IPP and diabetes was speculated and required further studies.[]\nHayashi et al. reported a case of IFBS treated with rikkosan. Here, the treatment with carbamazepine was ineffective and was, in turn, treated with the Kampo medicine rikkosan gargles along with dietary strategy. In this case, in 1 year, IFBS improved with no recurrence.[]\nThe literature review provided with total of five individual case reports of IFBS and one case series of five cases without diabetes diagnosed as IFBS and nine cases with diabetes. We report an additional case of IFBS with diabetes.
A 69-year-old female from eastern Nepal presented to our outpatient clinic with a history suggestive of right sided sciatica for the last 2 years. She had been evaluated before for the same, but without any positive diagnosis. The patient denied any history of trauma or any alteration in her bladder and bowel habits, or of any symptoms which is suggestive of intermittent claudication. Upon neurological examination, the power in all the muscle groups in her lower limbs was normal - 5/5 as per the MRC Muscle scale (used with the permission of the Medical Research Council). Her ankle and the knee reflexes were normal and she had no sensory indifference in any of the dermatomes in the affected limb, as compared to the normal limb. There was no wasting of the extensor digitorum brevis muscle. Straight leg raise test and a crossed straight leg raise test were both negative. Her stance was also normal. While sitting in a squatting position, the patient complained of an exaggeration of her symptoms. We thereafter made a differential diagnosis of either a sciatic nerve tumor or a Pyriformis syndrome. Radio imaging with help of an MRI scan revealed the presence of a sciatic tumor alongside the sciatic nerve, near the ischial tuberosity on the right side (\n). The unusual location of the lesion was in favor of a schwannoma rather than a neurofibroma (\n).\nThe patient was counseled for the operative intervention that would remedy her persistent symptoms. A subgluteal approach was taken for the surgical corridor. Intra-operatively, a 3×3 cm\n2 well circumscribed lesion was seen lying within the sciatic nerve. It was carefully dissected off the nerve fascicles and fully removed (\n). The sciatic nerve was confirmed to be intact intra-operatively with the aid of an intra-operative nerve monitor.\nPostoperatively, the patient was completely free of her previous symptoms. She made a full recovery with no adverse events and was discharged on the fifth day. The histopathological report confirmed the diagnosis of a sciatic nerve schwannoma, owing to the presence of Antoni A and B areas and Verocay bodies (\n). The patient returned to her follow up visit at 1 month completely asymptomatic.
A 62-year-old woman was referred to the Plastic and Reconstructive Surgery Department for treatment of a bleeding exophytic tumor located on the right inner canthus. She had had the lesion for approximately 11 years. Initially, the patient was treated with excision and primary closure ten years ago. At this time the tumor had a diameter of 5 cm. The tumor was diagnosed as adenoid BCC microscopically and surgical margins were tumor-positive. The patient was operated on two years later when the diameter of the recurrent tumor was 15 mm. Histological examination of this second specimen revealed an "adenoid BCC" with clear surgical margins.\nAlthough the tumor recurred again after the second excision, the patient neglected medical advice and did not undertake any treatment (Figure ). More recently, however, the tumor began growing rapidly and became hemorrhagic. On examination the lesion was located on the right inner canthus and involved 1/3 of the eyelid. The size of tumor was approximately 55 mm × 45 mm. Visual functions of the patient were normal. However, a fixed mass developed in the patient's periauricular area six months ago (Figure ), although there were no palpable cervical nodes. We therefore investigated this region with computed tomography (CT), which revealed a tumor involving the right orbital structures extending to the ethmoidal cells. The tumor also involved the right parotid gland and multiple cervical lymph nodes.\nWe also investigated this patient with a bone scan (normal), abdominal and cranial CT scans (also normal) and a thoracic CT. Multiple metastatic lesions were seen in the chest CT (Figure ).\nExaminations of the cardiovascular, gastrointestinal, neurological, urogenital and hematological systems and other parts of the skin were performed by physical and routine laboratory and radiological techniques. There were no abnormal findings. Biopsy was performed from the tumor located on the inner canthus and revealed "adenoid basal cell carcinoma" (Figure ). Also, Fine needle biopsies were performed on the parotid and pulmonary lesions which confirmed the presence of adenoid BCC in these regions.\nThe patient did not accept the offer of surgical treatment for the tumor on the inner canthus. She was referred to the Oncology Department and treated with radiotherapy and chemotherapy. The patient received approximately 6000 cGy of external beam radiation over 3 weeks totally. Also, chemotherapy was initiated with cisplatin and 5-fluoruracil. She was followed up with physical examination and CT scans for six months and there were no metastases to other organs. She is still being followed.
A 57-year-old male was admitted to an emergency unit with abdominal trauma due to 2 motorbike crashes. His right flank hit on one side of the motorbike. The blood pressure on admission was 95/60 mm Hg, heart rate was 90 beats per minute, he was not afebrile, and had a normal respiratory system. He had minor abrasions and pain on the right flank. Examination showed that this was a blunt injury with no related medical history. Laboratory tests included red blood cells count 4 100 000 µL, hemoglobin concentration 10 g/dL, platelet count 160 000/µL, blood urea nitrogen 19 mg/dL, and serum creatinine 1.2 mg/dL. The blood in the urine was positive with >150 red blood cells and 1 to 2 white blood cells per high-power field. Other tests were within normal limits.\nUltrasound revealed the disruption of polar structures under the right kidney with surrounding hematoma, but abnormal shunt flow was not detected. Computed tomography (CT) proved kidney injury IV degree according to the American Association for Surgery of Trauma (AAST) renal injury grading scale. CT images showed stamping and deep laceration reaching up to the hilum. Besides, there was the presence of hematoma around the polar under the kidney spreading down pelvic lumbar muscles (). There was a mass structure in the renal navel region with enhancement after contrast injection. There was enhanced material in the arterial phase, and there was washout in the venous and delayed phase. These images were initially misdiagnosed as a kidney tumor because it had the form of a mass. However, when we analyzed carefully, this lesion was related to artery and vein in the renal navel region and defined as AVF. The areas of enhancement in this AVF matched the attenuation of the appropriate vessels (blood pool) at all phases (). In the arterial phase, the enhancement of AVF had the same attenuation value as the enhancement of aorta, while in the venous phase, it matched the enhancement of the vein.\nThe patient underwent subsequent vascular intervention to resolve the AVF. Digital subtraction angiography (DSA) images showed that the right renal artery consists of 2 main branches that come from the aorta. The upper branch supplied blood to a small portion of the upper pole and adrenal glands. The lower branch supplied blood to the upper, middle, and lower areas of the right kidney. The fistula artery came from the middle branch (). Besides, the patient was actively treated to avoid shock and used antibiotics to fight infections. He got operated to collect hematoma and stitched to repair renal parenchymal tear through the incision at the diagonal line of the outer abdominal wall. The patient was treated stably and was discharged from the hospital after 10 days. The patient was followed up after 2 months, and then he was fully recovered.
A 60-year-old African American male presented with progressively increasing erythema and pain and swelling of the right wrist associated with restricted wrist movement. His symptoms began three months prior to presentation when he injured the same wrist while fixing a used tire. He abraded his wrist against the dirty tire and 2 weeks thereafter his wrist started to progressively swell. A week prior to presentation yellowish pus started emanating from the wrist wound. He tried soaking his wrist in warm water and applied topical antibiotic cream without relief. He had a long-standing history of chronic right wrist pain following a closed fracture of his right wrist from a motor vehicle accident sustained 9 years prior to presentation, with preexisting radiographic evidence of wrist joint erosion with cystic changes in the distal radius, ulna, and carpal bones together with joint space narrowing suggesting secondary posttraumatic osteoarthritis. The patient also had a history of polysubstance abuse which included intravenous (IV) heroin use and alcohol abuse. This may have rendered him immunosuppressed and therefore susceptible to this invasive and uncommon infectious agent.\nPhysical examination findings included an elevated blood pressure of 156/83 and a heart rate of 115/min likely reactive to pain. He was afebrile. Examination of the right hand and wrist revealed an erythematous, fluctuant mass, 3 × 3 cm in size with an open wound on the radial and volar aspect of the wrist draining a purulent foul-smelling discharge.\nWrist radiography at admission showed extensive cystic changes involving the distal radius and ulna. There was almost complete loss of the distal ulna and loss of several carpal bones. Cystic erosions extended into the bases of metacarpals. Proximally the radius and ulna appeared normal. These changes were compatible with extensive destructive arthritic disease, the foremost consideration for which being pyogenic arthritis. There was no evidence of soft tissue gas ().\nComputed tomography (CT) scan of the wrist showed destructive changes involving the base of the second, third, fourth, and fifth metacarpals. More proximally destructive changes extended to involve all carpal bones. There was complete loss of normal anatomic bony relationships. Intercarpal, carpometacarpal, radiocarpal, and ulnocarpal joints were all lost. Similar extensive permeative and destructive changes were seen in the distal radius and ulna. Even more proximally were subtle permeative changes involving the cortex. Associated with this extensive destructive change in the bone were also seen irregularly shaped, thick-walled, septated fluid collections with enhancing borders. These collections were largest on the volar aspect of the wrist and proximal forearm along the radial border. There was no clear compartmentalization. Edema was seen in the subcutaneous tissue. These changes reflected an extensive pyogenic joint with osteomyelitis (Figures , , and ).\nLaboratory values showed a leukocytosis with mild left shift and 7% bandemia, C-reactive protein elevation of 34.80 mg/L, and creatinine of 1.5 mg/dL.\nThe patient was started on empiric IV Vancomycin 1 g Q12H and IV Ceftriaxone 1gm Q24H at the time of presentation. Blood cultures were sent prior to the administration of antibiotics. CT guided aspiration was performed thereafter and the aspirate was sent for microbiologic analysis. The patient also underwent an open arthrotomy with incision and drainage of frank pus from the right wrist after his wrist swelling persisted status after CT guided aspiration. The patient was on empiric antibiotic therapy prior to collecting intraoperative microbiological samples for analysis. Anaerobic blood agar plates (AnBAP) Becton Dickinson, Kanamycin-vancomycin laked blood agar (KV-BAP) Becton Dickinson, Colistin-nalidixic acid blood agar (CNA-BAP) Becton Dickinson, and prereduced chopped meat broth (CMG) Becton Dickinson showed growth of bacterial colonies. F. magna was identified by gram stain and biochemical reactions on the RapID Ana II (Innovative Diagnostic Systems, Inc., Atlanta, GA) identification strip.\nCT abdomen and pelvis as well as an echocardiogram were performed to look for a potential source for hematogenous spread of infection to the wrist. These tests were normal suggesting that the infection was acquired through direct inoculation of wound at the time of injury and/or contamination of the wound with oral saliva applied on the wound by the patient.\nAntibiotic therapy was deescalated to IV Clindamycin 900 mg Q8H once fluid cultures from the CT guided aspiration of the wrist joint grew F. magna. The patient was treated with a total of nine weeks of Clindamycin which is comprised of Clindamycin 900 mg intravenously Q8H for the first two weeks followed by oral Clindamycin 300 mg Q6H for the next seven weeks. This high dose and extended duration of therapy for 9 weeks, as opposed to the conventional therapy of 4 weeks for native joint septic arthritis, was undertaken because of the patients persistently elevated inflammatory marker (C-reactive protein of 23.87) and residual clinical examination findings of a low grade wrist swelling and erythema at the time of discharge and during subsequent outpatient followup visits. All of these resolved completely by extending the antibiotic therapy beyond the stipulated conventional time frame based upon ongoing clinical symptomatology, physical examination findings, and laboratory markers such as CRP.\nAfter completion of antibiotic therapy the subsequent outpatient orthopedic and primary care visits indicated that the right wrist swelling, tenderness, warmth, and erythema resolved completely. The incisions healed completely. The surgical scar was healthy and well approximated. A functional wrist brace was prescribed. No recurrent infections occurred. No osteosynthetic wrist implants were required. There was residual pain of low intensity upon active movements. He did have a mild flexion contracture. He had mildly restricted movements in regard to his dorsiflexion, volar flexion, and rotation of the wrist. His power was about 50% less than the opposite hand when attempting to make a fist. He was able to write with some effort and able to oppose his thumb. To improvise on these residual deficits Naprosyn and physical therapy were prescribed and he was subsequently able to return to work with minor restrictions on lifting weights and performing strenuous activity with his wrist. He became occupationally functional after approximately 2.5 months from the time of his initial presentation.
A 25-year-old active young man presented to emergency medicine and trauma care in June 2014 for progressive bilateral lower limbs weakness and spasticity for the past 6 months. His lower limbs weakness and spasticity were initially episodic with full recovery without treatment. Later it became more frequent and affected his daily activity. He also complained of urinary hesitancy and recurrent multiple painful genitalia and oral ulcers. He was treated with oral carbamazepine 200 mg daily for lower limbs spasticity. Subsequently, he developed cerebellar signs, dysarthria, nystagmus, and intentional tremor. MRI of brain and spine was performed to rule out compressive myelopathy and the result was normal. Blood investigations for sexual communicable diseases and autoimmune diseases were negative. Based on clinical finding and inconclusive investigations, he was diagnosed as primary spinocerebellar degeneration with a differential diagnosis of vasculitic Behçet’s. Despite being on treatment, his limb weakness progressed. He was paraplegic and wheel chair dependent.\nOne year after his initial presentation, he developed painful right eye with reduction of vision for 3 weeks. He was then referred to ophthalmology team for further eye assessment. Retrospectively, he had history of right eye painless progressive blurring of vision for the past 1 year. His vision was perception to light on the right eye while left eye vision was 6/12. Ocular examination showed the presence of bilateral uveitis. Anterior segment of the right eye revealed severe anterior chamber inflammation with the presence of hypopyon, 360 degree rubeosis iridis with ectropion uvea (), and dense diffuse vitritis that impaired the visualization of the fundus. There was mild anterior segment inflammation in the left eye () with the presence of mild vitritis and vitreous opacities. There was no vasculitis, retinitis, or retinal hemorrhage and exudate seen in the left eye. Right eye was complicated with neovascular glaucoma with IOP of 44 mmHg. IOP of left eye was 14 mmHg.\nFurther assessment was performed to support the diagnosis of BD; pathergy test positive (). Screening for infective and connective tissue diseases was negative. Another MRI of the brain revealed atrophy of the cerebellar and frontal lobe (). He refused lumbar puncture. He was diagnosed as neuro-Behçet’s based on progressive neurological deficits of the lower limbs, recurrent multiple painful genitalia and oral ulcers, bilateral uveitis, and positive pathergy test. He was then treated with intravenous methylprednisolone 1 g daily for 5 days followed by oral prednisolone 50 mg daily. His IOP was not controlled with antiglaucoma drugs, and right eye augmented trabeculectomy was performed (). However, the bleb site was complicated with suture abscess () and followed with blebitis (), then subsequently developed endophthalmitis. In view of that, the oral prednisolone was tapered down slowly. Despite being on intravenous, intravitreal, and topical antibiotic, the right eye progressed to painless blind eye. There was no progression of neurological deficit throughout follow-up. However, he continued to develop recurrent attacks of posterior uveitis of the left eye. Ocular inflammation was well controlled with higher doses of oral prednisolone and addition of oral cyclosporine. The summary of clinical presentation and treatment is shown in .
A 22-year-old woman with a known history of giant cell tumor of the left first metatarsal was evaluated for worsening left medial midfoot pain. She had previously undergone curettage and polymethylmethacrylate (PMMA) cementation of a biopsy-proven giant cell tumor of the first metatarsal two and a half years prior at a different institution. Six months following the index surgery, she had a recurrence treated with repeat curettage, liquid nitrogen local adjuvant, and placement of bone substitute graft. After the second procedure, she was ambulatory, but occasionally used a boot for comfort and avoided high-impact activities. Physical examination demonstrated a prominent first metatarsal base and a well-healed dorsal incision. She exhibited tenderness to palpation throughout the area of the scar.\nRadiographs of the left foot showed an expansile, radiolucent lesion of the first metatarsal base surrounding the known cement implantation of the first metatarsal (). Magnetic resonance imaging showed a large soft-tissue mass surrounding the first metatarsal circumferentially and abutting the second metatarsal (). There was high T2 signal with no T1 signal changes at the base of the second metatarsal. Computed tomography (CT) scan demonstrated an expansile lucency at the proximal aspect of the first metatarsal base toward the dorsal lateral side (). There were some areas of cortical breach, and the bone was expanded and abutting the base of the second metatarsal. There was no evidence of direct extension into the second metatarsal or areas of osteolysis within the second metatarsal base. She was diagnosed with a second recurrence of giant cell tumor of bone of the first metatarsal base. As the tumor was recurrent, with destruction of the articular surface and little residual bone of the first metatarsal base, a wide resection of the tumor was recommended.\nThe procedure began by harvesting iliac crest bone graft (5 cm in length, 2 cm in depth). The prior dorsal incision was extended along the entire first metatarsal and proximally across the tarsometatarsal (TMT) joint. A wide resection was performed by disarticulating the TMT joint, maintaining periosteal coverage surrounding the metatarsal, and osteotomizing the metatarsal about 4.5 cm from the TMT joint. The damaged cuneiform articular surface (from articulation with cement) was resected with a wafer osteotomy. The resection bed was treated with adjuvant thermal necrosis. Margins were extended further with 10% hydrogen peroxide. The first toe was held in appropriate length and rotation and pinned to the second metatarsal. The iliac crest graft was fashioned to fit appropriately, contouring the angles for an appropriate junction. The graft was secured with an anatomic plate and screw construct (Figures and ).\nNonweight bearing was maintained until the three month postoperative visit, at which time she began weight bearing as tolerated through the heel and 50% weight bearing through the forefoot with a removable boot. Four months following her operation, she was advanced to full weight bearing as tolerated in the boot. Five months after surgery, she began weight bearing as tolerated in a regular shoe and nonimpact exercises. Nine months after surgery, plain radiographs and CT demonstrated osseous bridging at both the proximal and distal graft sites with no signs of tumor recurrence (). At one year following surgery, she denied pain in the left foot and was able to complete all activities of daily living without difficulty. Eighteen months following surgery, the patient had no left foot pain, was exercising without difficulty, and there was no radiographic evidence of recurrence. The patient was informed that data concerning the case would be submitted for publication, and she agreed.
A 41-year-old woman presented with a non-tender swelling in the right posterior neck, increasing in size over a period of 2 months. A history of previous significant trauma incurred during adolescence was noted, in which the patient had fallen from a horse and had been kicked in the right side of the neck. Further questioning revealed a 10-year history of severe ongoing pain over the right scapular region, shoulder and neck with no other sensory deficit. Neurological examination demonstrated weakness of palmar grip of the right hand, and limitation of right lateral rotation and flexion of the neck. She had previously been clinically diagnosed with unilateral cervical spondylosis but no radiological evidence was sought at the time. Examination of the neck revealed the presence of a 3 × 2 cm2 firm non-mobile mass located deep to the posterior border of the right sternocleidomastoid muscle at the level of C3/C4 (Fig. ).\nUltrasound scanning showed a hyperechoic mass within the pre-vertebral muscles of the posterior triangle of the neck and fine needle aspiration failed to yield sufficient aspirate for cytopathological analysis. A computerised tomography scan revealed a soft tissue mass arising laterally to the origin of the deltoid muscle, suggestive of a soft tissue sarcoma. Magnetic resonance imaging (MRI) of the neck showed a 3 × 2 × 1 cm3 lesion of intermediate T1 and low T2 signal intensity within the right posterior cervical space, that could not be separated from the adjacent scalene and levator scapulae muscles. There was no evidence of cervical spondylosis. A peripheral area of enhancement surrounded the lesion, suggesting a neoplastic or inflammatory process (Fig. ). An incisional biopsy was recommended by the multidisciplinary head and neck oncology team and the histological analysis confirmed the diagnosis of collagenous fibroma.\nFollowing a discussion by the multidisciplinary head and neck oncology team, a surgical excision of the mass was recommended. This was performed through a transverse cervical incision. The tumour was identified intramuscularly, deep to the levator scapulae, scalenus anterior and posterior muscles, involving the upper cervical nerve roots (Figs. and ). Macroscopically, the tumour was a well-circumscribed firm oval mass, tan-grey in colour with a homogeneous consistency. The resected specimen consisted of mature fat, lymphoid tissue and nerve trunks containing a paucicellular fibrous lesion with spindle to stellate-shaped fibroblasts and a diffuse, permeative architecture mimicking infiltration and extending between and around nerves and muscle bundles. No mitotic figures or nuclear hyperchromasia was seen (Fig. ). Fibroblasts exhibited a myofibroblastic phenotype on immunocytochemistry, expressing calponin but not smooth muscle actin, S100 protein, CD34 or HMB45. Nerve sheath tumours and solitary fibrous tumour were thus excluded. There were no cytological atypia to suggest a malignant neoplasm. The patient’s neurological symptoms improved following surgery and no recurrence was reported at 18-month follow-up.
A 68-year-old man who had undergone subtotal esophagectomy for squamous cell carcinoma of the lower thoracic esophagus eight years previously was admitted with high fever and right hemiplegia. On examination, body temperature was elevated at 40.1°C, heart rate was 126 beats/min, and blood pressure was 170/110 mmHg. The abdomen was soft and flat, and no tenderness was observed. Antibiotic treatment was started. Immediately after drinking a glass of water at 3 h after admission, the patient suddenly developed respiratory distress and lost consciousness. He remained unresponsive and developed a flaccid paralysis. Cranial computed tomography (CT) demonstrated small collections of gas within the right hemisphere and frontal lobe, while cranial magnetic resonance imaging (MRI) revealed small collections of gas within the right hemisphere and frontal lobe (Figure ) and internal carotid artery siphon (Figure ). Contrast-enhanced thoraco-abdominal CT showed wall thickening and emphysema of the gastric tube, but no metastatic lesions were found in the mediastinum. The patient was diagnosed with cerebral air embolism as a complication of a gastric tube disorder. Despite supportive therapy, his status continued to deteriorate, and he was declared dead 13 days later. Autopsy showed an ulcer of 1.5 cm diameter in the middle of the gastric tube which had caused complete rupture down to the muscularis propria (Figure ). Immunostaining with CD34 showed complete loss of the endothelial cell structure of the branch of the pulmonary vein beneath the ulcer base of the gastric tube, indicating perforation of the vessel (Figure ). No evidence of Helicobacter pylori infection was seen.\nSeveral etiological mechanisms for the formation of gastric tube ulcers have been postulated, including hypersecretion of gastric juice, inadequate drainage from the pylorus, and breakdown of the mucous and mucosal barrier as a result of disturbed vascular circulation of the gastric tube []. In our case, gastric pedicle stasis in the eight years after surgery due to denervation or the gastric tube itself may have caused delayed gastric emptying, with a resulting increase in gastrin secretion and high acidity. Peptic ulcer of the gastric tube may penetrate into any adjacent organ [], including the right pleural cavity [], bronchi [], pericardial cavity [], thoracic aorta [], pulmonary artery [], left brachiocephalic vein [], and sternum []. Cerebral air emboli are usually caused by trauma or an invasive procedure, including upper gastrointestinal endoscopy [], affecting one of the blood vessels. In the present case, however, the patient had no trauma, invasive procedures, or central line access. To our knowledge, this is the first reported case of cerebral air embolism resulting from spontaneous perforation of a gastric tube ulcer into the pulmonary vein.
A 29-year-old military male basic trainee (71 inches, 200 pounds, BMI 27.89) presented with a one-month history of atraumatic bilateral leg pain. He reported pain within the first week of initiating running at basic training. He was diagnosed with bilateral proximal tibia stress fractures at three weeks with instructions to stop impact activities. After failure of his symptoms to improve, he was referred to our facility. On presentation, he had tenderness about the medial aspect of each proximal tibia. Radiographs obtained at that time demonstrated bilateral proximal tibia stress fractures with varus alignment about each knee and articular collapse of the left tibial plateau. Metabolic labs were obtained and significant for a low vitamin D (15), but the remainders of labs including calcium, thyroid-stimulating hormone (TSH), and testosterone were unremarkable. MRI of the left knee demonstrated a medial tibial plateau fracture with two millimeters of articular depression and extension of the fracture to the tibial spine (). MRI of the right knee demonstrated a hypointense linear T2 signal surrounded by diffuse hyperintense signal suggesting a medial tibial plateau stress fracture with surrounding bone edema, without articular collapse or extension (). These fractures were at the level of the physis, and there was surrounding sclerosis on plain radiographs. Given the collapse of the articular surface in his left knee, the patient was indicated for open reduction internal fixation with allograft bone. The patient underwent the aforementioned procedure without complication, and the patient remained nonweight bearing to his bilateral lower extremities (). At the time of surgery, the bone at the fracture distal to the articular surface was softer and more friable in composition than expected for the stress fracture. It was easily debrided back to stable, healthy bone edges, and we were interested in analyzing its composition, so a biopsy was obtained and sent to the lab for analysis. Histology demonstrated nonossifying hyaline cartilage with admixed fibroconnective tissue consistent with a persistent physis ().\nAt the six-week follow-up visit after the procedure on his left knee, the patient was still having significant pain in his right lower extremity and, after extensive counseling, elected to undergo the same procedure on the contralateral limb in an effort to return to running and continue his military career (). We suspected that a similar pathology of a persistent physis was present in the right proximal tibia and that this was contributing to his slow healing progression. Similar intraoperative pathology specimens were again obtained at the fracture site distal to the articular surface (), and these demonstrated fragments of purple to purple-gray staining matrix with chondroid metaplasia. This presence of cartilage distal to the articular surface was consistent with a persistent physis.\nAt four-and-a-half months from the index procedure (three months from the staged procedure), the patient was ambulating without assisted devices and had painless range of motion from 0 to 120° in each knee with well-healed incisions, maintained hardware and alignment, but had not returned to impact activities. Due to his status as a basic trainee, he was released from active duty at six months' time postoperatively and was lost to follow-up.
In April 2012 a 44-year-old man pedestrian was referred to our hospital (Khatam-al-anbia, Zahedan, Iran) 18 hours after a motor-vehicle accident. First, He was transferred to the nearest hospital, but his dislocation was missed on initial X-ray images (). Few hours later, a CT scan was performed owing to his increasing pain that revealed an anterior dislocation of his right hip joint (). Consequently, he was referred to our center. In Emergency room, his right lower extremity was in external rotation position. He explained an anesthesia in medial side of right leg and ankle. Active extension of the right knee was impossible and the muscle force could not be assessed due to extreme pain. Motor and sensory functions of the sciatic nerve were normal. The right hip was reduced under general anesthesia in the theater and was followed by skin traction. Post-reduction radiographic images demonstrated an appropriate anatomical position of the right reduced hip without any fracture.\nAfter reduction, marked wasting of knee extensor muscles and decreased sensation in the femoral nerve distribution were noted on examination. Electrophysiological studies shown a neurapraxia and the patient was informed that the muscles force would be restored and the numbness would be resolved. On discharge, skin traction for two weeks, gentle range of motion and knee extensors strengthening exercise, and analgesic were prescribed. Three weeks later, there has been a steady improvement in the patient's symptoms. He could extend the right knee against gravity and the sensation was regained in the medial of the right leg. Therefore, gait training was initiated and physical therapy was continued. At two months follow-up, complete clinical recovery of right femoral nerve was achieved and he was able to resume his job. Findings of an electromyography and nerve conduction study were normal at three months after injury. Recent eight months review has not shown any neurological deficit and he had full range of motion in both right knee and hip joints.
An 83-year-old man presented with a 1-year history of symptomatic osteoarthritis of the right hip. He became to our attention because in the last months he has exhibited symptoms of anterior thigh pain and paresthesia without trauma. The symptoms have gradually worsened over time. A physical examination showed the following multidirectional limits of motion; flexion, 85°; extension, -10°; abduction, 25°; adduction, 30°; internal rotation, 25°; and external rotation, 50°. Laboratory tests of the blood and urine did not suggest any other underlying disease states. The initial radiographs showed joint space narrowing and joint erosion in both hip joints compatible with hip osteoarthritis. Magnetic resonance imaging (MRI) showed a cystic mass with abnormal intensities in correspondence with the right hip and revealed a communication between the cystic mass and the hip joint (). The mass was close to the proximal insertion of the rectus femoral muscle, strictly associated with the femoral nerve and femoral vessels ().\nSurgical excision was performed through a longitudinal incision over the swelling (), with the patient in the supine position. The subcutaneous fat and fascia were incised, revealing the vascular bundle dislocated superficially (). The femoral nerve also appeared to be compressed by the mass and has been adequately exposed and protected (). A cystic mass has been detected deeper to femoral nerve and femoral vessels () and has been isolated up to the joint capsule (). The hip joint was not opened and the entire mass was excised (). The cyst was opened and it was filled with synovial fluid. Microscopically, the cyst wall was composed of vascular synovial tissue which contained loose fibrous tissue and aggregates of lymphocytes and plasma cells. All histopathologic findings were consistent with the diagnosis of a synovial cyst. At the time of this report, the clinical course has been uneventful and there was no evidence of recurrence of the hip pain and paresthesia of the right thigh at 1 year of follow-up.
A 75-year-old woman (nonsmoker with history of hypertension and depression) had repeatedly been administered an intra-articular injection with diazepam for left-sided chronic wrist pain due to osteoarthritis by a primary physician and chiropractor. After the last injection, she immediately developed progressing, cyanosis and hypaesthesia pain in her hand (). The symptoms became unbearable after 3 days and the patient presented to our institution. After immediate admission digital angiography showed a complete blockage of perfusion to her 4th and 5th left fingers (). Intra-arterial lysis therapy with urokinase was initiated but only slightly improved arterial flow in the ulnar digital artery of the 5th digit in the control angiography and was discontinued after 2 days due to an increasing hematoma on the upper arm. Intravenous heparinisation was administered from the day of hospitalization, and vasodilatation with prostaglandin was started on the second day. Both medications were continued until day 9 when an inhibition of platelet aggregation was started with aspirin 100 mg and clopidogrel 75 mg daily. CK was elevated from 481 U/l on day 3 to 642 U/l on day 5. The patient was discharged on the following day after a total of 10 days of hospitalization. During this time, the cyanotic 4th and 5th digits showed some improvement and the pain and hypaesthesia resolved. The patient was followed up as an outpatient for a total of 5 weeks during which time the acral areas of the digits progressed into dry necrosis (). With no further treatment option left, the 4th digit was amputated at the distal middle phalanx and the 5th digit was exarticulated in the distal interphalangeal joint. shows the intraoperative view of a thrombosed digital artery in the 5th digit. Wound healing was uneventful. Histological workup of the amputated tissue showed necrotic soft tissue and bone, a mildly active osteomyelitis, and small vessel thrombosis. shows the result 2 weeks postoperatively.\nThe patient denied to name the primary physician because she “has always been treated so well.”
A 49-year-old female suffered from a fever and rectal ulcer after undergoing internal hemorrhoid treatment with a submucosal injection of ALTA at a previous clinic. Detailed information concerning the ALTA therapy was not available. One week after ALTA therapy, she noted the obvious passage of flatus and stool through the vagina. She was diagnosed with RVF by anoscope in another clinic and referred to our hospital 3 weeks after ALTA therapy. A sigmoid colostomy was performed for fecal diversion as a preliminary step for fistula repair. The fistula did not improve at all (Fig. ). She had no symptoms such as pain and fever caused by severe damage of the surrounding tissue. However, she had been suffering from poor QOL as an ostomate. Ten months after ALTA therapy, we performed RVF repair by gluteal-fold flap. Under general anesthesia, the patient was placed in the lithotomy position. The 2 cm fistula at the vaginal entrance was easy to manipulate under direct vision. A fistulectomy was performed to dissect the fistula tract and the circumferential scar tissue (Fig. ). The rectal defect was closed primarily with interrupted absorbable sutures through the vaginal side. The vaginal wall was not sutured, and the space above the sutured rectal wall was opened (Fig. ).\nA Doppler probe was used to identify and mark the points of the perforator vessels of the internal pudendal artery on the medial point of the right ischial tubercle. A gluteal-fold flap was designed around the right gluteal fold including these marked points (Fig. ), the entire circumference of the skin was incised, and the distal side of the flap was elevated from the subcutaneous muscular layer. The proximal subcutaneous flap fat under the Doppler marking was left attached to the muscle to avoid injury to the pedicle vessel (Fig. b). After confirming the pedicle vessel of the flap, the flap was carefully elevated with blunt dissection. A subcutaneous tunnel to the vaginal introitus was prepared to minimize tension on the gluteal flap, and the flap was rotated 180 degrees and transferred through the tunnel to cover the posterior vaginal mucosal defect. The proximal skin portion of the flap was denuded and conformed to the shape of the posterior vaginal mucosal defect, and the subcutaneous fat tissue on the distal area of the flap was removed to thin the flap, while confirming bleeding from the flap margin (Fig. c). The flap was sutured to the surrounding vaginal wall with absorbable interrupted horizontal mattress sutures (Fig. d). Before donor site skin closure, a small suction drain was placed in the gracilis muscle harvested space. We show the illustrations of the operative procedure in Fig. . The patient had open drainage for subcutaneous abscess and was discharged on the 21st postoperative day. Seven months after the radical operation, we confirmed the fistula repair by pelvic examination of the vaginal wall and colonoscopic exam of the anorectal wall (Fig. ). Sigmoid-colostomy reversal was performed. The patient has experienced no RVF and complete anal function without soiling and has not been trying to do sexual intercourse in the three years since the sigmoid-colostomy reversal.
A 5-year-old girl presented to the emergency with history of painful mass in the left inguinolabial region noticed a week before presentation. The child has a positive history of trivial trauma in school while playing with other children. The inguinal swelling and pain got gradually worse over the week. There was no history of recent illness in near future. Examination revealed temperature of 38°C, heart rate of 140/m, and mild dehydration. Abdominal and chest examinations were unremarkable. Local examination revealed a 6 × 3 cm irreducible tense swelling in the left inguinolabial region, with overlying skin redness and edematous. Blood investigations revealed white blood cell count of 14000/mm3, C-reactive protein 50, normal electrolytes, and coagulation profile. Her urinalysis was normal. Blood culture and sensitivity was sent and was reported normal. An ultrasound of inguinal region performed in the emergency room revealed a cystic mass in left inguinal region containing fluid in the upper part and fluid with internal echoes in the lower part of mass, and there was no free fluid noted in the abdomen. After resuscitation with fluids and administration of IV antibiotics, she was taken to the operating room for exploration. Operative findings included a cystic mass in the left inguinal canal extending down to the ipsilateral labia majora (), cyst containing dark brown fluid in the proximal part, and clotted blood with dark fluid in the distal part (). The cystic swelling was confirmed to be an encysted hydrocele of canal of the Nuck with no evidence of associated inguinal hernia. The cyst was easily isolated from the surrounding edematous tissue planes and after complete separation high ligation of the canal of Nuck was performed and cyst was excised (). Postoperative course was uneventful. Microbiology of fluid collected from cyst was negative and cytology showed foamy macrophages and scattered lymphocytes and neutrophils. Histopathology of excised cyst revealed inflammatory cells infiltrated in the hydrocele sac with some degenerate blood and cholesterol clefts. No epithelial lining was seen and calretinin staining showed a few mesothelial cells. The histology findings were consistent with infected hydrocele of the canal of Nuck. In followup at 6 weeks, 6 months and 18 months after surgery, patient is well and there is no swelling or recurrence on the operated side.
A 60-year-old, right-handed male with involuntary shaking movements of the right-sided extremities visited our service. These episodes resolved within 5 min and were restricted to the extremities of the right side. He complained that the attacks were elicited by using the right hand or foot when for instance writing, tightening a bolt with a driver, and fitting shoes, but not by postural changes. A personal note he wrote with his right hand during an attack proved an intermittent slight trembling movement (Fig ). He was always alert, and no other neurological symptoms arose during the course. Neurological examination at the first visit did not show any abnormal findings such as cerebellar ataxia and extrapyramidal signs. He had a history of hypertension but no other diseases leading to hypercoagulability such as infection, head trauma, malignancy, dehydration, chronic inflammatory disease, drugs, or other. Systemic workup revealed a normal serum analysis, including coagulation factors. Brain magnetic resonance imaging (MRI) and MR angiography neither showed a parenchymal lesion nor artery stenosis/occlusion. We performed a standard electroencephalogram (EEG) to differentiate epilepsy as a probable diagnosis, but no epileptic activity was detected. He received a diagnosis of kinetic tremor based on the characteristics of his attacks. Arotinolol was administrated initially and subsequently switched to clonazepam. However, the paroxysmal tremor continuously persisted with exacerbations.\nOne year later, the patient suffered a sudden weakness of the right extremities, verbal disturbance, and sensory deficit on the right side of his body. Initial MRI and MR angiography revealed no responsible lesion. An EEG showed no evidence of epilepsy. He was admitted to our service with a suspicion of cerebral ischemia and antiplatelet therapy was started. The tremor did not occur after hospitalization. On day 5, the right paralysis deteriorated. MRI showed dilatation of vessels, from deep medullary veins toward superficial medullary veins on the left frontal/parietal lobe and on the right frontal/parietal lobe (Fig ). White matter changes surrounding these dilated veins were observed as well. In contrast, there were no changes in bilateral basal ganglia, thalamus, brainstem and cerebellum. MR venography proved occluded superior sagittal sinus in the anterior part (Fig ). The diagnosis of cerebral venous thrombosis (CVT) was established and anticoagulation by intravenous low-molecular-weight heparin was immediately initiated. He refused to undergo any contrast studies, including cerebral angiography. Ten days later, he had a partial seizure starting in his right upper limb and passing over to the left upper limb and face. Brain computed tomography detected cortical hemorrhage in the left frontal lobe where gradual dilatation of cortical veins had been observed. Multiple cortical hemorrhages recurred with subsequent complication of obstructive hydrocephalus. Despite decompressive surgery and ventriculoperitoneal shunt placement, the patient stayed in coma and died of disseminated intravascular coagulation and multiple-organ failure induced by shunt infection on the 90th hospital day.
A 38-year old female felt pain in the left ankle after sprain. Two months after the injury, she visited a local hospital because the pain had gotten worse. She was suspected of having a bone tumor at the distal end of the fibula by X-ray, and referred to our hospital. At the initial visit, the pain decreased while resting and walking. Physical examinations revealed a tenderness on the distal end of the fibula, but there was no swelling and restriction in range of motion of the ankle joint. She had no family history of this type of injury. Plain radiographs showed multilocular osteolytic lesions in the distal end of the fibula with thinning of the cortex (). On magnetic resonance imaging (MRI), the lesion showed intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The septum was enhanced by an injection of gadolinium (). Although the tumor extended surrounding soft tissue, distal tibiofibular joint was not involved (). Osteofibrous dysplasia, fibrous dysplasia, giant cell tumor, metastasis, abscess or adamantinoma were suspected from the image analyses.\nAn open biopsy was performed under local anesthesia. Histology showed biphasic pattern of epithelial cells and fibrous components with a tubular epithelial pattern (). Strong immunoreactivity of CK5 and vimentin were observed in the epithelial cells (). From these findings, she was diagnosed as the classical adamantinoma of the fibula.\nOne month after the open biopsy, we planned en bloc wide resection of the tumor. An oval shaped skin incision was made around the liner scar of the open biopsy. The distance between the oval skin incision and liner scar was kept 3 cm. At the proximal side, the fibula was cut at the same level of the oval shaped skin incision. At the distal side, lateral ligaments of the ankle and the tendon of the peroneal muscle were resected together with the distal end of the fibula. In the anterior and posterior sides, posterior part of the extensor digitorum longus muscle (EDL) and anterior part of the flexor hallucis longus muscle (FHL) were resected with fibula to cover the tumor, respectively. In the medial side, the interosseous membrane was resected with the fibula at the margin of the tibia. Finally, distal half of the fibula, lateral ligaments of the ankle, EDL, peroneal muscle, FHL and the interosseous membrane were resected. Because instability of the ankle joint was expected after the resection, arthrodesis of the ankle with intramedullary Nail with Fins (Smith and Nephew, London, UK) were performed. Reconstruction of soft tissues was performed to cover the defect by using free muscle flap of the Latissimus dorsi muscle (). The surgical margin of the resected specimen was evaluated according to the evaluation system of the Japanese Orthopaedic Association and a 1cm wide margin was achieved. From the surgical specimen, histology showed the same findings to the biopsy specimens. Her left ankle had been immobilized for 4 weeks using splints. She was allowed partial weight bearing (one-third of her body weight) gait at 4 weeks after the surgery. Weight bearing was increased gradually after 4 weeks, and she back to full weight bearing gait at 8 weeks. During the follow-up period of 7 years after the surgery, she has lived without any metastasis and local recurrence, and joined social activities without any prosthesis.
The patient was a 33-year-old male with a previously diagnosed Spetzler-Martin Grade 5 AVM in the left parietal lobe that had been treated conservatively. On the night prior to presentation he developed a sudden-onset, diffuse, severe headache that radiated to the suboccipital region. There was no nausea, vomiting, or loss of consciousness.\nOn the initial assessment the patient was awake, alert, and fully oriented. There were no cranial nerve, motor, or sensory deficits. A fundoscopic examination revealed no papilledema or retinal hemorrhage. A CT scan at admission revealed IVH within the left frontal horn, both occipital horns, both temporal horns, and the third ventricle. There was no ventriculomegaly (). Comparison of this scan with previous CT scans did not suggest the early hydrocephalus. We were concerned about the possibility of recurrent hemorrhage or the development of hydrocephalus, and hence the patient was admitted to the neurosurgical intensive care unit (ICU) for close observation. A catheter angiogram and MRI scan obtained on the following morning revealed no change in the AVM (). A repeat head CT scan on the second day of hospitalization demonstrated stable IVH without ventriculomegaly. The patient was then transferred from the ICU to the neurosurgical ward for observation.\nApproximately 40 hours after initial presentation, the patient experienced severe headache with associated nausea. An urgent CT scan revealed the interval development of hydrocephalus, with the suggestion that a portion of the intraventricular thrombus had migrated from the left lateral ventricle to the junction of the third ventricle and the cerebral aqueduct (). There was no recurrence of IVH or subarachnoid hemorrhage. The patient was immediately transferred back to the ICU with anticipation of a need for ventriculostomy placement.\nShortly thereafter his headache began to improve. On examination the patient appeared more comfortable and was not lethargic. He remained without focal neurological deficits. The headache resolved within 4 hours of onset. A ventricular drain was not placed, and the patient was monitored. Head CT performed 6 hours after the onset of headache and the first CT revealing hydrocephalus, showed an interval decrease in the ventricular size and further clot migration through the cerebral aqueduct (). There were no further clinical events during the hospital stay and the patient was discharged without further sequelae from his transient hydrocephalus.
A 55-year-old gentleman, ex-smoker, presented to our hospital complaining of mild epigastric pain, regurgitation, and heartburn. On top of that, he has a long-standing history of gastroesophageal reflux disease (GERD), which was managed by proton pump inhibitors. His past medical history was significant for hypertension. He was previously diagnosed with a liver hemangioma based on abdominal ultrasound two years before the presentation. He had no relevant family history. Physical examination revealed mild epigastric tenderness with no palpable abdominal mass. Laboratory data showed no anemia but positive stool occult blood test. Tumor markers including AFP, CEA, and CA 19-9 were all within normal range. Upper GI endoscopy revealed mild esophagitis, Los Angles grade A along with Barrett's esophagus without dysplasia and a 1 cm polyp at the GEJ. A sample was sent for histopathology; the rest of the stomach and duodenum were normal. The patient did not have a previous endoscopy prior to this one.\nInfused computed tomography (CT) of the abdomen and chest showed mild GEJ thickness with no evidence of mediastinal or celiac lymphadenopathy and no signs of metastasis. It also demonstrated a large heterogeneously enhancing mass about 6 × 9.5 cm with central necrosis in the upper abdomen that appears to be originating from the gastric antrum (greater curve). The mass was highly suggestive of GIST based on CT; it was the same mass that was previously misdiagnosed as a liver hemangioma (). Endoscopic ultrasound confirmed the previous findings. However, no biopsy was attempted due to the risk of bleeding.\nHistopathological examination of the GEJ polyp revealed tubulovillous adenoma with elements of adenocarcinoma in situ. The patient was admitted with a provisional diagnosis of early-stage adenocarcinoma of GEJ along with the incidental finding of enlarging gastric GIST. A trial of endoscopic mucosal resection of GEJ polyp was attempted but failed because of the polyp location that created a technical difficulty. Therefore, the patient was taken to the operating room with a plan to perform a wedge resection of the gastric mass and a submucosal resection of GEJ polyp through the same gastric opening. We planned to use frozen section (FS) to document negative margin resection and determine the need for a formal esophagectomy. Intraoperatively; a large (10 × 7 × 6 cm), extraluminal pedunculated mass was found at the posterior wall of the greater curvature of the stomach (). Wedge resection of the gastric mass with negative margins was achieved along with a transgastric submucosal resection of the GEJ polyp. Fortunately, the FS examination of the polyp showed negative margins as well with no evidence of deep invasion. Postoperatively, the patient had a smooth course and was discharged home in a stable condition. The final pathological examination revealed a GEJ polyp around 1.7 × 1.4 × 0.6 cm. Microscopically, there was a focus of invasive adenocarcinoma involving the superficial submucosa of the polypoid lesion, negative margins, and no lymphovascular invasion (T1a NxM0). Furthermore, the gastric wall mass measured around 10 × 7 × 6 cm with a 2 × 1.5 cm stalk. Histopathology revealed encapsulated high-grade epithelioid GIST tumor with negative margins (pT3). The mitotic rate of 6/50 HPF and immunohistochemical stains were positive for DOG1 and CD34 but negative for CD117 (c-Kit) ().\nThe final diagnosis was synchronous early-stage GEJ adenocarcinoma and a high-grade gastric GIST. Therefore, the patient was started on adjuvant imatinib treatment, along with endoscopic surveillance every six months and proton pump inhibitors.
We report a case of a 62-year-old male who developed DRESS syndrome after seven weeks of antibiotic treatment with vancomycin. He initially underwent instrumented thoracic spinal fusion (T1–7) due to cord compression from a metastatic T4 lesion from renal cell carcinoma and developed a postoperative deep spinal infection. He underwent multiple washouts and vacuum-assisted closure over a period of twelve weeks, with various antimicrobial regimes, initially receiving seven weeks of vancomycin as well as a shorter duration of ciprofloxacin. He developed a maculopapular morbilliform rash, () initially on the right arm and scalp, before spreading to cover the entire head, trunk, and upper legs () which progressed to become exfoliative and was intensely pruritic and painful (). This was accompanied by a fever and eosinophil count of 9.77 × 10−9/L at the highest, occurring simultaneously with the development of the rash, and which remained elevated over the course of a month of regular blood tests. Other haematological abnormalities were also present, with a rise in both lymphocytes and neutrophils. Vancomycin was discontinued immediately, and other causes for these results were excluded, with negative blood cultures, CMV, EBV, ANA, and hepatitis B, hepatitis C, and HIV titres. There was no clinically apparent lymphadenopathy; however, a CT scan performed after the onset of symptoms showed new prominent right hilar lymph nodes, although this may have been due to metastatic cancer and not DRESS syndrome. Skin biopsy showed superficial perivascular lymphocytic infiltrate and rare eosinophils, consistent with a morbilliform drug rash. Ciprofloxacin was felt to be unlikely to be the cause of his DRESS, as he had been prescribed the drug several times in the past, as well as having a shorter duration of treatment which would not fit with the typical timeframe for DRESS, so this was continued to treat his infection.\nThe patient initially received a single dose of intravenous high-dose hydrocortisone, but due to the severity of infection and the risk of immunosuppression, he was subsequently treated exclusively with topical steroids, emollients, and antihistamines (). No liver or renal function abnormalities were noted during this time; however his eosinophils remained raised as described. He developed acute chest pain and shortness of breath four weeks after the initial rash, with new onset fast atrial fibrillation and negative troponin and creatinine kinase. A CT scan demonstrated bilateral pleural effusions, as well as progression of lung and rib metastases. An echocardiogram showed mild left ventricular and right ventricular impairment and a rim of pericardial fluid. Unfortunately, within three months of initial surgery, the metastatic spinal load increased causing further cord injury and paraplegia. Further surgical intervention was deemed inappropriate at this point, and the patient was discharged to the community palliative care team.
A 65-year-old man presented with a 14-d history of numbness and pain in the left lower limb, which became aggravated after physical activity.\nThe patient had a 2-wk history of numbness and pain in his left lower limb when carrying heavy loads, which became aggravated during walking and showed intermittent claudication. The numbness and pain were mainly located on the medial aspect of the thigh and extended to the left knee. The patient could not sleep supinely due to the pain. He had previously visited a local hospital and received symptomatic treatment with painkillers for 2 wk. The pain, however, was still severe, and the patient’s quality of life was severely affected. For further diagnosis and treatment, this patient came to our hospital. The initial diagnosis was "left lower extremity pain of unknown origin with dyskinesia". The patient had a good general condition since onset.\nThe patient could not sleep supinely due to the pain. He had previously visited a local hospital and received symptomatic treatment with painkillers for 2 wk. The pain, however, was still severe, and the patient’s quality of life was severely affected.\nThe patient had no family history of genetic disease.\nThe physical examination revealed numbness and pain in his left groin and inner thigh, as well as tenderness on the medial side of the left knee joint. The visual analog scale (VAS) pain score was 7 (severe pain). The patient walked with an abnormal pattern and posture: He bent his back and put his left hand on his left thigh. The passive knee flexion test result was positive. This patient had a normal muscle tone, and the level of muscle strength was grade V in the right lower limb and IV in the left lower limb. The knee and Achilles tendon reflexes were normal, and the Babinski sign was negative on both sides.\nNo obvious abnormalities were found in laboratory examinations.\nThe plain X-ray scans of the two knees and right hip showed no obvious abnormalities except mild hyperosteogeny. The plain and dynamic enhanced magnetic resonance imaging scans showed slight disc bulging at the L4-L5 level (Figure -). The pelvic computed tomography (CT) scan plus 3D reconstruction revealed bilateral sacroiliac joint osteophyte formation (Figure -), and the left osteophyte was larger (arrow). The electromyography and nerve conduction velocity studies showed partial peripheral nerve damage in both lower extremities (Figure -).\nA CT-guided diagnostic obturator nerve local block was performed at the site of the left sacroiliac joint osteophyte[,] (Figure and ). Immediately after surgery, the patient felt that the pain had significantly reduced, and the passive knee flexion test result became negative. In addition, intermittent claudication disappeared. However, the pain recurred the next day after the local block and could not be relieved after conservative therapy for a week.
A 69-year-old male patient, with a three months history of abdominal pain asthenia and macroscopic hematuria, was admitted to the outpatient clinic. Abdominal CT revealed an 8 cm left renal growth suggestive of neoplasia, with the involvement of the tail of the pancreas, tumor thrombus in the left renal vein and multiple left para-aortic adenopathies (). A biopsy was performed and showed to be inconclusive regarding the possibility of renal cell carcinoma. Radical left nephrectomy with distal pancreatectomy and splenectomy was proposed.\nThe procedure (Video 1 in Supplementary data) started with an extensive Cattel-Braasch Maneuver, allowing exposure of the inferior vena cava and the aorta, and thus isolation of the left renal vessels. The tail of the pancreas and spleen were freed, and a no-touch approach [] was adopted to minimize the spread of tumor cells with proximal splenic vein ligation. Caudal splenopancreatectomy was performed with section of the pancreas with a GIA 80 (vascular load), followed by proximal ligation and section of the splenic artery and what was thought to be the left renal artery (). The junction of the left renal vein with the vena cava was opened, and the tumor thrombus was resected, followed by caval suture. The renal vessels were, at this point, presumably controlled. The nephrectomy was continued with the release of the kidney and para-aortic lymphadenectomy, during which only the stump of the left renal vein could be identified, lacking the previously ligated renal artery stump. The renal artery was located inside the mass of lymph node tissue in the left para-aortic space, and the stump belonged to the superior mesenteric artery, ligated flush with the aorta. There was no arterial pulse in the mesentery confirming the injury. After removing the specimen, the distal stump of the superior mesenteric artery was exposed, and a repair with a terminoterminal anastomosis was performed from the proximal stump of the splenic artery (). The viability of the gut was assessed by palpation of an arterial pulse in the superior mesenteric artery.\nThe postoperative period went uneventfully. Histological examination showed a renal sarcomatoid carcinoma pT4N1M0G3. Control imaging at three months showed permeability of the celiac trunk and the superior mesenteric artery ().
The patient is a 61-year-old Korean male with a significant past medical history of rheumatoid arthritis, hypertension, and diabetes mellitus who presented to the hospital with sudden onset of blurry vision in his left eye three days prior. A head CT had been performed at an outside facility, which was negative for acute ischemia, hemorrhage, midline shift, or extra-axial fluid collection. The patient denied visual complaints in his right eye and had no history of similar events.\nOphthalmologic and Neurologic consultations initially revealed visual acuity was 20/40 on right eye; however, on the left eye, there was an apparent left-sided visual loss. Patient's intraocular pressure was normal in both eyes, pupils were equally round and reactive to light, and no afferent pupillary defect was observed. Moreover, the extraocular muscles were intact and with full range of motion. In the right eye, visual fields were full to finger count. In the left eye, the acuity in the nasal visual field was greater than that in the temporal visual field. In the nasal visual field, the patient was able to count fingers; however, in the temporal visual field, the patient could barely detect gross hand movement at one foot out. External examination was within normal limits, and pen light examination was only remarkable for nuclear sclerotic cataracts bilaterally. Dilated fundus examination demonstrated no evidence of pathology to the vitreous, optic nerve, or retina that might explain the vision loss. Four days later, repeat examination by Ophthalmology demonstrated a stable right eye; nonetheless, the left eye temporal visual field was 20/25 and patient was able to count fingers without mistake on left visual field. Pupil and retina examination were unchanged from previous examination. However, visual field mapping could not be done as this patient was seen in the inpatient setting.\nConsequently, an initial head MRI with and without contrast was performed, which showed focal areas of restricted diffusion in the right medial temporal lobe, inferior right basal ganglia with possible involvement of the right lateral geniculate nucleus. The head MRI did not show any other pathology such as masses or hemorrhages (Figure ). The subsequent head MR angiogram done demonstrated no focal occlusion or stenosis, and the MRI of the orbits showed no focal defects bilaterally. At this point, it was evident that the patient was suffering from a left temporal monocular hemianopia and that there must be a correlation with the subtle but definitely present right LGN ischemic lesion. An infarct affecting the right lateral geniculate nucleus would most likely cause bilateral left homonymous hemianopia. Nevertheless, a lesion at the vicinity of right LGN that had taken out the inputs going to layers 1, 4, and 6 became generally accepted among all physicians involved at this point in the care of this patient as the only possible explanation. Patient's left visual symptoms slowly started to improve, and after four days of hospitalization and monitoring, patient's left monocular temporal hemianopia had almost resolved and his vision had almost returned to his normal baseline.
A 21 month-old girl was brought to Asan Medical Center suffering fever, cough and tachypnea for 4 months. Abnormal lung findings were first noted by a chest radiography taken due to fever when she was 8 months old. No further evaluation was performed at that time. The symptoms of the patient at 21 months of age were the same as those that her sister had shown at age 4 yr.\nPhysical examination of the patient revealed tachypnea at rest (beyond 60/min) and chest retraction without rales or wheezing, but no digital clubbing. On arrival at the hospital, the Denver symptom score () of ILD gave a value of 2 points meaning the patient showed respiratory symptoms but oxygen saturation was normal in room air under all conditions. Blood, urine, sputum and stool studies revealed no evidence of acute viral or bacterial infection. Neither virus (adenovirus, influenza virus, parainfluenza virus, respiratory syncytial virus, cytomegalovirus) nor bacteria was found in bronchoalveolar lavage fluid. The chest radiography showed a dense hazy area at the central region of both lungs and blunting on the left costophrenic angle. Chest CT () demonstrated diffuse fibrosis on the medial portion of both lung fields and subpleural consolidation along both lateral pleura. Based on the similar findings from chest CT (), the sister of the patient had been diagnosed with uncharacterized ILD, and died 5 months after diagnosis. Surgical open lung biopsy () was performed at 22 months of age. A diagnosis of NSIP was made based on the findings of diffuse, uniform thickening of the interstitium with lymphoplasmacytic infiltration and collagen fibrosis. Some alveoli contain accumulation of intra-alveolar macrophages, while some alveoli had hyperplastic type II alveolar pneumocytes. Genetic studies were performed with the lung biopsy specimens and peripheral blood mononuclear cells. These revealed no identifiable mutation in the genes encoding surfactant proteins. Examination of peripheral blood cells from the patient's parents also showed no evidence of mutations in these genes. The patient was treated with high-dose intravenous methylprednisolone (30 mg/kg/day, 3 doses every other day, monthly) and oral hydroxychloroquine (daily).\nDuring treatment, tachypnea and dyspnea on exertion were still evident and the Denver symptom score was 2 points at follow-up. Follow-up high resolution CT (HRCT) showed that there was no further disease progression after 21 months.
Mr. AA is a 50-year-old man who emigrated from South Korea to the U. S. 10 years ago. Mr. AA holds an MBA from a high-ranking university in Korea, and his wife has a BA in fine arts. Mr. AA and his wife run a small laundry and dry cleaning business in a Midwestern city. They do not have health insurance and speak limited English. At one time, Mr. AA had health insurance, but he decided to discontinue his health insurance because he was not sick and the copayment for his clinical visits was too expensive. Over the last year, Mr. AA developed mild indigestion, so he purchased digestive medicine at a drug store to treat this condition. He also had general fatigue, a low appetite, and weight loss which his family thought was due to his workload or immigration-related stress. His wife encouraged him to visit family in Korea and have a medical exam. Health care in Korea would be less expensive than in the U. S. with no language barrier. When he arrived in Korea, he experienced more severe indigestion, itching in his legs, and mild jaundice. He scheduled a comprehensive physical examination that showed a chronic HBV infection with large primary liver cancer (>10 cm). Clinical history examination revealed that his mother was also infected with HBV and developed liver cirrhosis and died of liver cancer 10 years ago.\nGiven their high rate of HBV infection, and without appropriate intervention, mortality rates among Asian Americans with liver cancer will increase substantially in the near future. Chronic HBV infection usually does not produce symptoms, and people who have this infection feel healthy, even in the early stages of liver cancer,[] and the disease can progress without patients even knowing they are infected. This is why HBV infection is called a silent killer and many cases of liver cancer are detected in the late stages, leading to a low survival rate after diagnosis (5%).[] The CDC[] recommends screening tests for all people born in regions of the world with high or moderate rates of HBV or who were born to parents from a country with high HBV rates. This includes all countries in Asia. It appears that Mr. AA failed to undergo screening for HBV because he did not realize that he was at risk, did not recognize his symptoms, nor did his HCP screen for HBV.\nMr. AA did have health insurance, which he canceled. Mr. AA and his family did not use the insurance; he never went to the hospital. He believed that unless he had serious health problems, it would not be necessary for him to see a health-care provider. His language barriers and unease regarding using the health care system in the U. S., including making an appointment with his HCP, were sociocultural factors that impacted his cancer screening decision making. He also worked long hours and perceived that getting tests at a hospital would take too much time. In short, getting a screening test was not a priority.
A 51 year old man presented to the ED for the third time in 2 weeks with complaints of flank pain and hematuria. He had complained of left sided flank pain during the initial 2 visits. During the first visit, the patient was diagnosed to have renal colic. X-ray KUB did not show any renal stone. He was treated symptomatically with analgesia and discharged. During the second visit with complaints of persistent left flank pain despite taking analgesia given at discharge, he was admitted to the emergency observation ward for pain management. Bedside ultrasound done then was noted to have mild left sided hydronephrosis. He was pain free at the end of the observation and was then discharged with analgesia and an outpatient follow-up with the urology department. He was also scheduled to have an outpatient computed tomography scan of the kidneys, ureters, and bladder (CT KUB). However, 2 days before the scheduled CT, he represented to the ED with right sided flank pain since morning on the day of his visit. The pain radiated to the right groin and was associated with hematuria. He was not passing blood clots. He denied any other complaints of fever, weight loss, vomiting, diarrhea, or constipation. He had a past medical history of hypertension, diabetes mellitus, and hyperlipidemia. He had previous surgeries for appendicectomy and cholecystectomy.\nHis vital signs were stable. There was tenderness over the right flank on physical examination. There was also a palpable tender mass measuring about 5x5cm over the right lumbar region. There was no renal angel tenderness. Bedside ultrasound in the ED showed an appearance of a 6 cm cystic lesion around the inferior pole of the right kidney with internal echogenicity within the cyst. The provisional diagnosis was a bleeding renal cyst versus a tumor. Blood tests done in the ED showed a mildly elevated white cell count 12.64 × 10(9), hemoglobin of 14 gm/dL, and creatinine of 109 Umol/L.\nPatient was seen by the urology on-call in the ED and his bedside ultrasound done by the urologist revealed a 10 cms cystic lesion anterior to the lower pole of the right kidney with irregular internal echogenicity. Patient was admitted to urology department for further evaluation and management.\nInpatient CT Intravenous Pyelography (IVP) was done and the left kidney showed relative hypoenhancement and reduced excretion of contrast, associated with diffuse ureteric thickening and periureteric fat stranding which could be related to a passed left renal calculus or a differential diagnosis of ureteropyelonephritis which was less likely due to diffuse involvement of left kidney. There was a tubular cystic structure in the right flank with inferior tip at the same site of previously inflamed appendix stump base which could represent mucocele of the appendix stump ().\nPatient was then transferred under the care of the general surgery department, where he underwent a colonoscopy which demonstrated an extrinsic compression at the caecum and appendiceal orifice and a smooth pedunculated polyp measuring 10 mm in the sigmoid colon (). Exploratory laparotomy was done, the mucocele was resected, and patient recovered well. Histopathology revealed a benign mucinous cystadenoma. The patient was discharged well with an outpatient surgical follow-up.
A 32-year-old male presented to our service with complaints of headache, nausea, vomiting, and a “funny feeling” is his left abdomen, which had been progressing for the past 3 weeks. Patient worked as a commercial truck driver and had been experiencing increasing symptoms when driving at higher altitudes, with some resolution of symptoms as he descended. Patient's past medical history was significant for premature birth with a VP shunt placed when he was approximately 2-month-old for unknown reasons. Patient reported that his first shunt revision occurred in 2009 where his shunt was revised to a right frontal VP shunt. Patient later required a revision at an outside hospital in 2015 because the shunt was complicated distally with a pseudocyst. At that time, the distal portion of the catheter was replaced. Pseudocyst cultures did not grow any organic species.\nOn previous admission to an outside hospital, patient presented with a 3-week history of abdominal pain, gradually worsening. No headaches, nausea, or vomiting were reported at that time. Computed tomography (CT) scan of the abdomen revealed a 13 cm pseudocyst along the liver margin [], with the distal tip of the catheter within the cyst. At that time, patient's shunt was externalized, and the cyst was drained by interventional radiology. Cultures from the pseudocyst were negative. Patient was placed on cefepime empirically while in the hospital. On day 22, shunt was inserted with the assistance of general surgery, using laparoscopic guidance. A choice was made to internalize the shunt in the left upper quadrant. Of note, upon entrance into the abdominal cavity, the descending colon was found to be adherent to the left anterior abdominal wall. Lysis of adhesions was performed with blunt dissection. There was no evidence of bowel injury or significant bleeding. Catheter was passed into the peritoneum and the incision was closed in the usual manner.\nOn admission to our hospital 1 year later, patient's CT of the abdomen demonstrated a 10-cm fluid-filled cyst around the distal end of the catheter. On admission, erythrocyte sedimentation rate (ESR) was 39 and C-reactive protein (CRP) was 2.13. CT of the head demonstrated no evidence of hydrocephalus, however, there were no other images for comparison. A lumbar puncture was performed, which demonstrated an opening pressure greater than 40 cm H2O. CSF studies were negative for infection. Next, the patient's shunt was tapped. Proximal (intracranial) catheter demonstrated significant resistance and was unable to be drained. Distal catheter flushed with ease and demonstrated a pressure of 13 cm H2O, with a manometer held at EAM. CSF studies from this tap demonstrated gram positive rods on gram stain, however, cultures were negative. A decision was made to externalize the shunt (proximal catheter, valve, and distal catheter) and place an external ventricular drain. Patient was placed on vancomycin at the recommendation of infectious disease. Three negative sets of CSF were drawn, each 2 days apart, before returning to the operating room for shunt revision. He was taken to the operating room with the assistance of general surgery for removal of the orphan catheter in his abdomen with the placement of a new catheter.
Our patient was a 25-year-old Caucasian Iranian man who was referred to our center in 2010 for further evaluation of paraparesis and urinary incontinence. There was no significant point in his past medical or familial medical history. He was well until 2008, when he developed blurred vision in his right eye and was admitted to a local center with the clinical impression of optic neuritis. He received intravenous methylprednisolone 1000mg for 5 days without a good response. Two months later he experienced optic neuritis in his left eye, but he had a good recovery after receiving intravenous methylprednisolone. According to his medical records, his physical examination was normal at that time, with the exception of mild optic atrophy of the right eye. Brain MRI was performed, which revealed some hyperintense T2-weighted lesions around the ventricles, with linear callosal involvement. The lesion was nicely enhanced in a linear pattern in a T1-weighted image with contrast agent (Figs. and ). Linear enhancement of ventricular surfaces (pencil-thin appearance) in both the frontal and occipital horns was another important finding in this patient.\nThe patient was diagnosed with MS and received interferon (IFN) beta 1a three times per week. Six months later he developed another episode of optic neuritis in his left eye, and he had a less than 50 % recovery with intravenous steroids and plasmapheresis. After 1 year, he developed paraparesis and urinary incontinence while receiving IFN beta 1a three times per week. He was admitted to a local hospital and received intravenous methylprednisolone 1000mg/day for 5 days. He responded to this therapy and was able to walk in 4 weeks. At that stage, the patient was referred to our center to escalate the disease-modifying therapy.\nA neurologic examination of the patient revealed bilateral optic atrophy, weakness of both lower limbs (grade 4/5) and bilateral Babinski sign. Although we did not perform detailed neuropsychological tests, the patient did not show any apparent decrease in cognition or any other signs or symptoms of callosal involvement. New MRI showed a longitudinal cord lesion extending more than four vertebral segments without any enhancement. Regarding episodes of optic neuritis, longitudinal extensive spinal cord lesion and atypical callosal involvement, anti-AQP4 was checked, and the result was positive. Other laboratory tests, such as anti-nuclear antibody and anti-phospholipid antibody, for collagen vascular diseases were negative. The patient was diagnosed with NMO, and azathioprine was started for him at a dose of 50mg/day with a gradual increase to 150mg/day.
A 69-year-old male was admitted to a hospital in June 2016 because of right arm asthenia and dysarthria and was diagnosed as having cerebral infarction in the left middle cerebral artery area along with deep vein thrombosis. ECG demonstrated normal sinus rhythm and echocardiography revealed no intra-cardiac thrombus or vegetation. The patient was discharged from the hospital following administration of apixaban.\nIn August 2016, the patient was readmitted to the hospital because of recurrent right arm asthenia and dysarthria. MRI revealed multiple cerebral infarctions in not only the bilateral cerebral hemispheres but also the cerebellum. Trousseau’s syndrome was suspected at this time. Apixaban administration was stopped and an intravenous drip of heparin was started. Echocardiography revealed mild mitral regurgitation with vegetation on the mitral valve. Although the laboratory data suggested no evidence of infection, ceftriaxone and gentamicin were added as a precaution against infective endocarditis. The patient was then referred to our hospital for surgery.\nA CT scan revealed a left renal infarction and multiple swollen lymph nodes around both the abdominal aorta and stomach with antral hypertrophy, suggesting an advanced gastric cancer or lymphoma. As the vegetation showed no change despite the heparin and antibiotics therapy, cardiac surgery was performed on day 5 after referral. Extracorporeal circulation was instituted employing aortic and bicaval cannulation. After aortic cross-clamping, the mitral valve was exposed via a left atriotomy. Both mitral leaflets had vegetation on the surface, and major vegetation 15 mm in width was evident on the anterior leaflet (Fig. ). These were resected in their entirety and replaced with a 25-mm Epic bioprosthesis (Abbott). Continuous intravenous heparin administration was resumed on the following day, aiming for an activated partial thromboplastin time of between 40 and 50 s. Histologic analysis revealed that the vegetations were thrombi covered with vascular endothelium and that the mitral leaflet tissue was not damaged (Fig. ). On the basis of these findings, the patient was diagnosed as having NBTE.\nAn endoscopic stomach biopsy was performed on the seventh postoperative day, and histologic analysis revealed non-solid poorly differentiated adenocarcinoma with components of signet-ring cell carcinoma and moderately differentiated tubular adenocarcinoma. The patient was definitively diagnosed as having Trousseau’s syndrome and, subsequently, transferred to the department of surgery. A Billroth I distal gastrectomy was performed, and a continuous intravenous heparin drip was employed during the operation. Histologic analysis revealed poorly differentiated adenocarcinoma with a component of moderately differentiated tubular adenocarcinoma and metastatic tumor cells in the dissected lymph nodes (T4aN3bM0; stage IIIb). Further histologic analysis using alcian blue staining confirmed the presence of mucin in the tumor.\nSubcutaneous heparin injection was introduced on day 8 after the gastric surgery, and the patient was discharged from our hospital in October after acquisition of the self-injection technique. During this long hospitalization, no thromboembolic events were observed. Chemotherapy was started in November. The patient has survived for 18 months after the diagnosis of Trousseau’s syndrome without any recurrence of thromboembolism.
A 67-year-old man presented to an outside institution's emergency department with right groin and abdominal pain, nausea, and emesis. Three years prior he had undergone hybrid open and endovascular repair of common iliac and infrarenal aortic aneurysms consisting of open bilateral femoral artery access for placement of an aorto-left iliac device, as well as a left to right femorofemoral bypass with an 8 mm ringed polytetrafluoroethylene (PTFE) graft. Physical examination now revealed a large right inguinal hernia extending into the right hemiscrotum, as well as abdominal distension and tenderness. A contrast-enhanced computed tomography scan of the abdomen revealed a bowel-containing inguinal hernia with upstream small bowel dilation. The constriction of the bowel appeared to be occurring at the level of the femorofemoral bypass graft where the hernia sac abutted the anastomosis (Figs and ).\nDue to the large scrotal component of the hernia and potential adherence of the sac to the vascular graft, an open approach was undertaken. A vascular surgery consultant was apprised of the case, reviewed the imaging prior to surgery and was available during the surgery. Bilateral groins, thighs, abdomen were included in the operative field in case revision of his vascular reconstruction should become necessary. The graft's course was traced utilizing a handheld Doppler and a transverse, curvilinear incision was made extending laterally from the pubic tubercle, superior to his vertical femoral cutdown scar. A large indirect hernia sac was found to be protruding from the external inguinal ring and to be adherent to the vascular graft. In addition, the graft with its associated fibrosis had contributed to the constriction at the internal ring and to the resulting incarceration. Meticulous, technique was employed to carefully dissect the hernia sac from the cord structures and from the graft, as well to avoid entry into the sac or injury to the bowel within the sac. After opening the external oblique aponeurosis, the vascular graft had to be mobilized superiorly in order to allow for exposure of the internal inguinal ring. The sac, which was large, was then reduced intact into the preperitoneal space through the internal inguinal ring. A weakness of the transversalis fascia was also noted. A tension-free Lichtenstein mesh repair was performed using a lightweight polypropylene mesh (Fig. ). The external oblique aponeurosis was carefully re-approximated to separate the mesh and graft, and the incision closed in layers. Graft and distal arterial Doppler signals were unchanged at the termination of the procedure.\nThe patient had an unremarkable recovery. He was ambulating 4 h after surgery and returned to his assisted living facility on the third postoperative day.
A 32-year-old woman with a diagnosis of a hydatidiform mole at a gestational age of 16 weeks underwent uterine curettage in another hospital. On the 6th postoperative day, the patient presented with fever and aggravated abdominal pain. Then, she was transferred to our department for severe peritonitis and underwent an emergency exploratory laparotomy. The intraoperative findings included massive intra-abdominal intestinal contents and pus, severe edematous and adhesive intestines, a 1-cm defect on the intestinal wall located approximately 30 cm from the caecum and a perforation on the uterus. Simple repair of the intestinal defect and uterus and peritoneal irrigation were performed during the operation. Unfortunately, the patient suffered lower abdominal pain and fever again on the 7th day after the operation. Blood examination showed increased leukocytes and neutrophils. Abdominal CT showed a lower intra-abdominal abscess of approximately 10 cm in diameter and surrounding disordered bowel structures. Under the guidance of color Doppler ultrasound, diagnostic abdominal puncture was carried out, which yielded puncture fluid containing intestinal contents. Intestinal suture line leakage was diagnosed. Because of severe edematous and adhesive intestines, conservative treatment was selected. Under ultrasound guidance, an appropriate path that would allow subsequent dilatation was selected. Under local anesthesia, access to the abscess cavity was maintained achieved using a guidewire, over which the catheter tract was dilated to 30F using graduated dilators of a percutaneous nephroscope. Then, the triple-cavity tube was placed through the tract and advanced to the bottom of the abscess cavity. Leakage along the suture line with an approximately 2-cm defect was identified by fistulography. Continuous irrigation and suction with negative pressure at approximately −100 mm Hg was initiated, and the net output exceeded 600 ml/day for the first few days. A broader spectrum antibiotic was administered for sepsis control, and octreotide was given to decrease the output. TPN was given because of poor intestinal function. Fistulography was performed again 7 days later, which revealed fistula tract formation. Because of anus exhaust, oral feeding with starchy foods, complete protein foods, and fruit juice was encouraged, and recombinant human growth hormone was administered at a dosage of 8 U/day instead of octreotide. Although the output increased for a few days, the drainage was unobstructed, and anal defecation always occurred. Due to the antagonism between somatostatin and somatotropin, oral loperamide hydrochloride capsules but not octreotide were given to reduce intestinal secretions. Sixteen days after recombinant human growth hormone administration, the fistula tract had healed completely. Follow-up at 1 week, 1 month, and 3 months were performed without fistula recurrence and other relevant complications.
A 27-year-old man with a duodenal injury from a traffic accident was treated with simple closure for duodenal rupture, retrograde placement of a drainage tube through the jejunum for duodenal decompression, and preventive jejunostomy for enteral nutrition (EN) in another hospital. Seven days after the operation, he was found with bile leaking from the drainage tube, which gradually developed into an intra-abdominal abscess; septic episodes occurred within the next 3 days. Then, he was transferred to the intensive care unit of our hospital due to persistent sepsis. On admission, bile leakage from the drainage tube exceeded 800 ml in the first 24 hours. For sepsis control, a broader spectrum antibiotic was administered. An acid inhibitor drug, omeprazole, and somatostatin analog, octreotide, were administered to reduce the output. Total parenteral nutrition (TPN) was given due to poor intestinal function. An abdominal computed tomography (CT) exam and fistulography revealed a 2-cm defect on the junction of the descending part and horizontal part of the duodenum and a large surrounding fluid collection. Because of the high risk of a second operation, a damage control strategy should be considered in these cases. Thus, conservative treatment was selected. To facilitate effective drainage, active suction drainage was needed instead of the previous passive drainage method. Thus, a homemade triple-cavity tube (Fig. ) with functions of continuous irrigation and suction, which was designed with an insertion length of approximately 14 cm as determined by measuring the previous drainage tube of the intra-abdominal part on fistulography, was immediately inserted in the direction of the duodenal defect along the initial drainage channel when the previous drainage tube was removed. Then, the location of the triple-cavity tube was identified and slightly adjusted near the duodenal defect under fistulography. Negative pressure was applied at approximately −100 mm Hg. With the patient's stabilization 7 days later, the fistula tract formed gradually and was identified by repeated fistulography. Due to intestinal function recovery, octreotide was reduced and eventually stopped, and EN was administered through the jejunostomy tube. After EN was well tolerated at 40 kcal/kg/day of energy and completely replaced TPN, somatotropin (recombinant human growth hormone) was administered at a dosage of 8 U/day. An interesting phenomenon was that the triple-cavity tube was frequently plugged by exudative fibrous tissue approximately 14 days after recombinant human growth hormone administration and thus needed to be rinsed at least twice a day. To promote healing, the tube was pulled out approximately 2 cm to facilitate fistula tract closure. Eighteen days after recombinant human growth hormone administration, the duodenal fistula had healed completely, and the patient resumed oral intake. The patient was followed without fistula recurrence and other relevant complications at 1 week, 1 month, and 3 months.
The patient was a 34-year-old woman, G4 L2 Ab1 who had married her cousin 7 years ago. She had a history of two normal vaginal deliveries and one abortion in the 1st trimester. The first pregnancy in the age of 28 had terminated with a normal vaginal delivery (NVD) resulting in a term baby girl who weighed 3150 gr. The second pregnancy had occurred two years later; curettage was done at week 6 due to spontaneous abortion. Her 3rd pregnancy was in the age 32 resulting in a healthy term baby girl weighing 3400gr with NVD. Due to her unwillingness for becoming pregnant she had withdrawal contraception, whereas because of the non-occurrence of menstruation during breastfeeding and 6 months after her last pregnancy, a pregnancy test was requested. Due to the positive pregnancy result, ultrasound study was done which revealed a 10-week spontaneous gestation with 4 gestational sacs and 4 fetuses. There was no case of multiple pregnancies in her or her husband's family.\nShe received prenatal care during her pregnancy but there was no need for prophylactic cerclage. At week 24 of gestation she was hospitalized due to premature contractions. The contractions were controlled with the prescription of pethidine and hydration and she was discharged 3 days later. She was once again admitted at 28 weeks of gestation due to similar contractions; this time she was treated with indomethacin and pethidine and discharged 3 days after the contractions suppression. She also received two doses of betamethasone during hospitalization.\nShe was admitted a week later due to labour contractions. In vaginal examination 2 finger dilatation with no effacement was detected. Serum test results were reported all in the normal range and the vital signs during hospitalization were normal. At this stage she was treated with tocolytics (adalat). The fetuses' health was monitored by Doppler ultrasound imaging, biophysical profile and fetal non stress test (NST). After the labour contractions' suppression and due to the presence of sporadic contractions she was monitored while being hospitalized up to the time of delivery.\nAt 32 weeks and 4 days of gestation, due to the resumption of labour contractions and dilatation progression, after receiving the rescue dose of betamethasone, cesarean section and tubectomy (upon the request of the patient and her husband) was performed. The outcome of cesarean section was 4 fetuses, 3 girls and a boy, quadriamniotic and quadrichorionic. Quadruplet A weighed 1820 gram with an Apgar score of 9 to 10; quadruplet B weighed 1810 gram with an Apgar score of 6-7. Quadruplets C and D weighed 2100 and 1980 gram with an Apgar score of 7-8 and 9-10, respectively. Among the 4 neonates, only quadruplet B was transferred to the NICU; she was discharged after 2 days in good health. and show the quadruplets after birth.\nBecause of atonic uterus during the cesarean section, after the administration of the appropriate dosage of oxytocin and methylergonovine and 800µgr of rectal misoprostol, the uterine arteries were blocked and the B-Lynch suture was done. No blood transfusion was required for the mother and her hemoglobin (Hb) level 6 hours after the operation was 9 g/dl; her pre-operational Hb level was 10g/dl. The mother was discharged 3 days after delivery with no complications.\nFor close follow up, the mother and her newborns were visited two weeks after delivery; they were all healthy and had no problem. The infants were visited once again 6 months later revealing normal physical and mental development in all four. shows the babies at 6 months of age.\nThis project has been approved by Ethical Committee and Vice Chancellor for Research of Mashhad University of Medical Sciences (97/429008).
A 56-year-old Caucasian woman with a history of hypertension and migraine headaches was found by her husband walking in circles and acting strangely. She was last acting normally the night before and there were no signs of trauma. She was evaluated in an emergency department and found to have an intracerebral hemorrhage (ICH) in the left frontal cortex on head CT (Fig. ). She had a history of mild hypertension but had not been on antihypertensive therapy.\nShe had not taken any anti-thrombotic medications prior to the ICH except for occasional aspirin-containing migraine therapy. Her coagulation profile was normal except for elevated activated partial thrombin time (aPTT). CT angiogram and MR angiogram did not detect any abnormal blood vessels. MRI of the brain did not show an underlying tumor or infarction. She was discharged on a low-dose antihypertensive agent. A repeat MRI of the brain was done a month later (Fig. ) and showed 9 microhemorrhages and superficial siderosis meeting the Boston criteria for probable CAA []. Five months after the ICH, a repeat brain MRI showed a new area of encephalomalacia in the left occipital cortex that was not present on the prior MRI (Fig. ). The radiological features indicated a chronic infarction []. The MRI also showed an area of encephalomalacia of the right frontal cortex/subcortex that had been seen on the initial CT and MRI but had unclear significance. With the development of the left occipital encephalomalacia, it became apparent that the right frontal lesion was also a chronic infarction. A transthoracic echocardiogram was done as part of the ischemic stroke investigations and showed an echogenic structure on the atrial aspect of the anterior mitral leaflet. Transesophageal echocardiogram was done on the same day and showed a 6-mm, sessile, thickened mass on the mitral valve (Fig. ). She was admitted to the hospital for expedited workup. She was asymptomatic, did not show any systemic manifestations of infective endocarditis, and had normal vital signs. Her laboratory tests were significant for mild leukopenia, thrombocytopenia, and mild renal insufficiency. She also was noted to have aPTT of 54 s (normal: 22.5–36.5 s). Further hypercoagulable workup revealed positive lupus anticoagulant (LA), high-titer anticardiolipin (aCL) IgG antibody, and high-titer beta-2-glycoprotein 1 IgG antibody (aB2GP1) suggestive of APS. Additional autoimmune evaluation revealed low-titer ANA 1:40 and hypocomplementemia. The patient was diagnosed with nonbacterial thrombotic (Libman-Sacks) endocarditis due to primary APS. She did not meet criteria for another connective tissue disease. After multidisciplinary discussion regarding her underlying CAA and recent ICH, anticoagulation with warfarin was recommended for at least 3 months with INR goal of 2–3.\nAfter 3 months on warfarin with INR documented between 1.6–2.6, a follow-up transthoracic echocardiogram showed a slightly smaller mass on the mitral valve. Anticoagulation was continued due to the persistent mass, as well as lack of adverse events. Repeat testing at that time for antiphospholipid antibodies (aPL) showed they were present in high titer confirming the diagnosis of APS. Indefinite anticoagulation was recommended.\nIn the one and half years since her initiation on warfarin, our patient has not had any major bleeding or thrombotic events, including intracranial bleeding. She has mild cognitive impairment without focal findings. Her most recent brain MRI, completed 18 months after the ICH, showed no new areas of ischemia or lobar hemorrhages, although there were 4 new microhemorrhages compared to the prior study (Fig. ). No symptoms of another collagen vascular disease have developed.
In November 2005, a 55-year-old Japanese woman underwent upper endoscopy at another hospital and was found to have a pedunculated polyp, measuring 10 mm in diameter, on the greater curvature of the lower gastric body (Fig. ). On biopsy, it was diagnosed as a GFHP. At the moment of the first diagnosis, there was only an endoscopic report and the endoscopic appearance could be compatible with an atrophic mucosa. In January 2013, upper endoscopy was repeated, which revealed that the polyp had grown to 12 mm in diameter with a slightly rounded head (Fig. ). Biopsy was not performed at the time.\nIn June 2014, the patient underwent positron emission tomography-computed tomography to evaluate the treatment effect on her right breast cancer, for which she had undergone surgery and received chemotherapy and radiation therapy at the age of 53 years. There was an abnormal accumulation in the lower gastric body (Fig. ). Subsequent upper endoscopy showed that the polyp had grown to 20 mm in diameter, and the surface of the head was slightly reddish and tense (Fig. ). The biopsy specimen obtained from the head of the polyp was histologically diagnosed as tubular adenocarcinoma and was determined to be unrelated to the breast cancer. It was suggested that the polyp became malignant during the nine years of follow-up, and the patient was referred to our institution for further evaluation and treatment.\nOn physical examination, scars from the right breast mastectomy with bilateral axillary lymph node dissection were noted. No other remarkable findings were observed.\nNo abnormalities were found in the blood counts or liver and renal function tests. The serum H. pylori immunoglobulin G antibody and stool H. pylori antigen were both negative. The anti-gastric parietal cell antibody was positive (80-fold increase), as was the anti-intrinsic factor antibody. The fasting serum gastrin level was markedly increased at > 3000 pg/ml (normal range, < 200 pg/ml). The serum pepsinogenI level and pepsinogen I/II ratio were both low (Table ).\nThe patient was not taking any medication at the time.\nA well-circumscribed mass with a maximum standardized uptake value of 2.7 was observed in the lower gastric body. There were no findings suggesting the presence of gastrinoma.\nIn August 2014, en bloc resection of the pedunculated polyp, measuring 20 mm in diameter, on the greater curvature of the lower gastric body was performed by endoscopic submucosal dissection (Fig. ).\nHistopathological examination showed tumor lesions, which had well-defined borders with hyperplastic glands (Fig. , ). The tumor lesions were strongly positive for p53 and Ki-67, while the hyperplastic lesions were not (Fig. ). Submucosal invasion was observed in the stalk, with an invasion depth of 300 μm (Fig. ). In D2-40-positive and cluster of differentiation 34-negative lymph ducts, cells with acidophilic cytoplasm and large nuclei were observed. The cells were positive for keratin, indicating lymphovascular invasion (Fig. ). As a result the final histological diagnosis was adenocarcinoma of the stomach, tubular adenocarcinoma > papillary adenocarcinoma, size of resected lesion 20 mm in size, submucosal invasion of 0.3 mm, venous invasion (-), lymphatic invasion (+).\nConsequently, additional radical distal gastrectomy was performed. Examination of the surgical specimens showed a scar resulting from the endoscopic submucosal dissection on the anterior wall of the gastric body. There were no residual tumor cells or lymph node metastasis. Examination of the background mucosa showed that the proper gastric glands in the pyloric region were preserved, while those in the mucosa of the gastric body were atrophic and showed intestinal metaplasia and pseudo-pyloric metaplasia (Fig. ). In the OLGA staging system, the corpus and the antrum were scored as 2 and 0, respectively, indicating stageII gastritis at the time of surgery [, ]. Chromogranin A staining revealed enterochromaffin-like cell hyperplasia (linear hyperplasia and micronodular hyperplasia) (Fig. , arrows indicate micronodular hyperplasia). H. pylori infection was negative (Fig. ).
Patient is a 34-year-old female without past medical history. Two years before she sought medical attention for the first time, she noticed a mass in her upper abdomen that has grown to gigantic proportions, however, due to a lack of adequate access to healthcare facilities in her geographic location she did not visit a healthcare professional. In the 4 months previous to seeking medical attention, she noticed pain around the mass, severe weight loss and recurrent episodes of postprandial vomit, which prompted her family to take her to the emergency room. On clinical examination, a malnourished and dehydrated patient with a giant mass in the upper abdominal was discovered. She reported a slight pain on palpation, but no tenderness was encountered. Blood work was normal and due to the size of the mass, an abdominal contrast-enhanced computerized tomography (CT) was requested. It revealed a huge 40 × 28 × 10 cm3 encapsulated retroperitoneal tumor with a soft tissue density (19–30 UH), and an area containing a calcification (Fig. A). The mass extended from the right flank to the pelvis. It displaced the liver, right kidney and most of the bowel (Fig. A). Yet, it did not invade any adjacent structures and no other masses were observed. Due to these findings and after adequate reanimation, surgery was decided. At laparotomy, a 40 × 30 × 10 cm3 reddish soft consistency retroperitoneal mass was encountered (Figs A and B), the mass was firmly attached to the third portion of the duodenum, right kidney and ureter. Nevertheless, it did not invade any of the latter (Fig. B). No other masses or lymph nodes were encountered. From there surgical decision was straightforward, and complete excision of the mass was performed. During the procedure, an adhesion between the mass and the third portion of the duodenum was tractioned, and a small traumatic duodenal injury occurred. However, due to prompt intervention, the lesion was identified and repaired. A drain was left near the duodenal injury and another one in the retroperitoneal space. After this, the remainder of the procedure continued without any complications (Fig. B).\nPathology reported a DDL, the tumor was composed of mature adipocytes with some enlarged atypical nuclei (Fig. C). In some areas, the liposarcoma transitioned towards a fusiform cell sarcoma and in one area it showed a benign ossification (Fig. C). The resection margins were free of tumor tissue.\nThe postoperative period of the patient was uneventful, 7 days after surgery, the nasoenteric tube was withdrawn and sips of liquids were initiated. Attaining good oral tolerance, antibiotics were withdrawn, abdominal drains were removed and a soft diet was initiated.\nThe patient was discharged in good conditions. On follow-up controls the patient is doing well and is undergoing periodic controls with an oncology specialist (Fig. C).
A 64-year-old man, known for diabetes managed with insulin therapy since 20 years, had a motor car accident leading to a degloving injury and a skin maceration of his central forehead. It was repaired by local flaps in a private hospital. As a complication, he developed a skin necrosis with exposure of bone. The defect size was 17x10 cm. Six weeks after the previous repair he was presented to our center (Figure 1 ).\nConsidering the importance of a reliable coverage after bone debridement and the relative medical condition of the patient precluding a free flap or multiple stages operations, a reconstruction by a big rotation flap was planned.\nBecause of the patient’s systemic fragility, hospitalization with tight control of blood sugar was done 3 days before the operation. The most suitable vessels for the flap were the superficial temporal artery and the postauricular artery on the left side. The arteries were checked with Doppler probe preoperatively.\nAfter debridement and removal of all necrotic tissues and cleaning of bone, the scalp was infiltrated with tumescent solution along the proposed incision line. The incision line was started from the largest side of the defect (right side). Anteriorly, the incision was extended along the frontal hairline. As the incision was going laterally on the right side, the flap was widened up to 2 cm above the hairline. Posteriorly, the incision was lowered down to the middle of the occipital area (Figure 2 ).\nThe incision was stopped before approaching the left ear. Then, the flap was dissected anteriorly and laterally on the left side without injury to the feeding vessels (Figure 3 ). The flap was based on the following arteries: superficial temporal and postauricular arteries. The arteries were checked by intraoperative Doppler to ensure the integrity of the feeding vessels.\nCareful hemostasis was done and a drain was inserted. The wound was dressed and light compression applied over it.\nThe dressing was changed on the 1st postoperative day and a light compression was reapplied again. The tip of the flap was exposed to check the vascularity of the flap. Blood sugar was monitored carefully. The drain was removed after 10 days and healing was excellent. Follow-up was extended up to 3 months (Figure 4 ).
A 21-year-old male soccer player with no history of prior knee injuries presents to the ED via private vehicle complaining of left proximal leg pain and difficulty ambulating. The patient comes to the ED directly from the soccer field where he states he was running and then made an abrupt cut to the left causing his ankle to roll. He then heard a crack from his knee and immediately felt pain along the lateral side of his proximal leg and proceeded to fall. The patient denied any pain while flexing or extending his knee. The patient states that he may have adducted his left leg while in a flexed position when he cut to the left. The patient complained of his left knee feeling “tight.” The patient had no other injuries or complaints and the rest of his physical exam was unremarkable.\nOn examination, the patient appeared in no distress and his vital signs were stable. The patient had 5/5 strength with dorsiflexion and plantarflexion. Both knee joints were stable with Lachman, posterior drawer, varus stress, and valgus stress test. Just by visual inspection, the left fibular head appeared more pronounced when compared to the contralateral side. The patient also complained of severe tenderness upon palpation over the fibular head with varus stress of the left knee. There was no crepitus to flexion or extension of the left knee. Anterior-posterior (AP) and lateral view X-rays of the left knee are shown in . shows the contralateral AP and lateral X-ray views of the unaffected right knee. The AP view of the affected left knee shows that the fibular head was situated with a more lateral prominence and with less tibia-fibula overlap compared to the right knee. On the lateral views, the left fibular head is very slightly more anterior compared to the contralateral knee. The constellation of history, physical examination, and X-ray finding suggested that the patient had a proximal anterolateral fibular head dislocation. Closed reduction of the left fibular head dislocation was performed by orthopedics. Immediately after joint relocation the patient stated that the left knee pain had improved and the “tightness” had resolved. shows post-X-ray reduction films of the left knee and normal anatomical alignment of the left tibiofibular joint. The patient was placed in a knee immobilizer, given crutches, and referred to orthopedics.
A 54 year old male presented to a busy district general hospital medical emergency department with abdominal pains and swelling over the right flank for a two to three week period. More recently he noticed swelling of his right lower leg and thigh. He was a non-smoker, and there was no significant family history of disease. He had no upper or lower gastrointestinal symptoms. There was no change in weight or appetite. There was no history of cardio-respiratory disease, and his exercise tolerance was not limited. He was not able to volunteer any further information as regards to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment, due to an "atrophic left kidney". This was diagnosed by ultrasound of the renal tract. There was no evidence of any other imaging modalities or radiological investigations undertaken to investigate the cause of his atrophic kidney.\nOn further review of his medical notes it was revealed that he had a troublesome childhood with bilateral Perthe's disease, and non-healing venous ulcers on the medial aspect of his right ankle.\nIn 1973, aged 21 years, he underwent skin grafting of a non-healing ulcer. One year later he was re-admitted with recurrence of ulcers in the same region and was then noted to have dilated varicose veins and thrombophlebitis that was treated with crepe bandaging for 2 years. Treponemal serology then was negative. In 1977 he had ligation of the dilated varicose vein that was "feeding the ulcerated part of the leg". In 1979 he was discharged from follow up with complete healing of the leg ulcers.\nOn examination he was noted to have bilateral lower limb varicose veins. Examination of the abdomen revealed large, tortuous, hard, "snake like" palpable veins over the right aspect of his abdomen with overlying bruising. (see photo- figure ). A preliminary diagnosis of probable Thrombophlebitis Migrans was made. A CT scan was performed to exclude underlying intra-abdominal malignancy.\nThe CT scan showed : "A congenitally absent inferior vena cava with collaterals on the anterior abdominal wall and prominent azygous and hemi-azygous veins. Agenesis of the left kidney is noted." (see radiograph- figure )\nUS Doppler revealed : thrombosis in the right iliac and superficial femoral veins.\nHe was commenced on low molecular weight heparin and warfarin. Low molecular weight heparin was stopped when the International Normalised Ratio (INR) was greater than 2.0.
A 47-year-old man presented with a two-month history of constant cough, chest distress, and slight dysphagia. Physical examination revealed that breathing sounds were markedly decreased throughout the right thorax and the sound of the heart had moved to the left side. Chest radiography showed a high-density shadow in the lower two-thirds of the right thorax (Fig a). Enhanced computed tomography (CT) of the chest confirmed a huge mass with fat tissue density in the posterior mediastinum, which extended to the bilateral thorax and compressed the heart, aorta, and bilateral lung (Fig b). Upper gastrointestinal contrast showed an obvious dislocated esophagus, resulting from compression of the mass. Bronchoscopy revealed that the right middle and lower lobe bronchi were distorted, and the lumen had become severely narrowed. Cranial and abdominal CT scans and a bone scan excluded the potential metastasis of the tumor. Blood biochemistry and serum cancer markers were all normal.\nThe patient declined percutaneous needle biopsy; therefore, surgical intervention was applied for diagnosis and treatment. The patient underwent the placement of a stomach tube before surgery. Anesthesia was induced while the patient was in the horizontal position. Using positive-pressure separate lung ventilation to avoid compression of the heart and lungs by the large tumor, the patient was moved to the left lateral position. Vital signs, including arterial blood pressure, central venous pressure, and heart rate, were monitored carefully. After confirmation of stable anesthesia, right posterolateral thoracotomy was performed in the fifth intercostal space. The sixth rib was resected in order to better expose the huge mass. Intraoperatively, a giant, well-capsulated yellow tumor without abundant blood supply was found, which almost occupied the whole right thorax. The tumor had not invaded any other organs, such as the lung, chest wall, or pericardium; only moderate adhesion existed in the thorax. The partial tumor in the right thorax was smoothly removed in two blocks after cutting off the adhesion. The esophagus, phrenic nerve, and inferior vena cava (IVC) were carefully protected during surgery. The esophagus and IVC were then mobilized from the tumor. The tumor of the left thorax was drawn upward through the space between the esophagus and IVC, and the rest of the tumor was gradually separated and successfully removed. The atelectatic right middle and lower lobes obtained good pneumatization when both lungs were ventilated.\nThe gross size of the tumor was 30 cm × 25 cm × 20 cm and it was covered with a capsule (Fig ). The cut surface appeared lobulated in shape and was pale yellow in color. Pathology showed that the tumor comprised abundant atypical adipocytes with dispersed smooth muscle proliferation (Fig ). The final pathological diagnosis was well-differentiated liposarcoma. The patient experienced an uneventful recovery. Postoperative chest radiography showed a normal right lung (Fig ). No sign of recurrence or distant metastasis was detected during 25 months of follow-up after surgery.
A sixteen year old boy presented with one year history of pain, swelling and restriction of left knee joint. Patient's symptoms were insidious in onset, which gradually progressed in its severity. There was no history of antecedent trauma, loss of appetite and fever. Patient does not give history of any definitive treatment taken for his present complains. On examination, the left knee was kept in 15-20° flexion with obvious quadriceps wasting. There was generalized swelling of the knee with fullness in the popliteal fossa. On palpation effusion was present with normal local temperature. There was a diffuse tenderness all around the knee, medial joint line being the most tender site. There was a bony hard slightly movable swelling palpated just lateral to the patella, which was extending to the midline beneath the patellar tendon. Irregular hard swellings could be felt along the margins of medial femoral condyle. Patient had fixed flexion deformity of 15° with further flexion was up to 110°. Instability tests were negative and there was no abnormality upon examination of distal neurovascular status. Plain X-ray of the left knee joint shows a large radiodense body in front of the femoral condyle with irregularity of the posterior articular margin of the medial condyle (). MRI was showing the effusion, synovial hypertrophy and a loose calcific body in front of the femoral condyle pushing over the patellar tendon anteriorly ()\nSurgical management was planned and open procedure was preffered considering the extensive involvement with a large loose body inside the knee joint. Anterior mid-line incision was given and knee joint was exposed by medial parapatellar approach. A large loose body of around 7×4 cm was removed which was lying beneath the patellar tendon and lateral ratinaculum (,). Irregular nodular outgrowths were present along the margins of medial femoral condyle from anterior to posterior, which were thoroughly removed (,). Extensive synovectomy was done in all the compartments. Synovium and the bodies were sent for histo-pathological examination, which confirmed the diagnosis of synovial chondromatosis with papillary hyperplasia of the synovium (,). Post-operatively patient was instructed about knee mobilization and strengthening exercises and followed up at one, three and six months. Patient's range of movement was 0-130° of flexion without pain at three months post-operative period. There was no recurrence at one year after the surgery.
An asymptomatic 17-year-old woman underwent echocardiogram at our laboratory because of heart murmur. Rest electrocardiogram was normal. On family history, her father was affected by HCM. On echocardiography, we found normal volume, mass, and systodiastolic function of the LV. The right ventricle was normal too and no valve disease was detected. Nevertheless, we found two deep recesses in the basal and medium third of the interventricular septum containing luminal blood in diastole with complete obliteration in systole []. No interventricular shunt was found. The septal myocardium appeared to be compact, and no local hypertrophy was found. A cardiac MR confirmed the presence of two clefts of the septal myocardium penetrating more than 50% of the thickness of the septum, the distal one being bilobated [Figures and ]. The surrounding myocardium showed a normal contractility, as normal was the global systolic function of the left ventricle []. Neither areas of fibrosis after gadolinium infusion nor markers of HCM were detected. The patient and the parents were reassured about the benign nature of these findings. Nevertheless, since some authors suggested that LVC may be more prevalent in HCM mutation carriers without hypertrophy[] and given that the father of the patient was affected with a clinically evident form of HCM, a genetic counseling was suggested.\nLVC have been reported both in the general healthy population[] and in HCM mutation carriers without LV hypertrophy.[] They must be correctly identified and diagnosed and should not be misinterpreted as ventricular diverticula or congenital aneurysms. Up to now, there are no robust data on the clinical significance of myocardial clefts in otherwise normal individuals. Nevertheless, on the basis of the existing literature, such individuals should be reassured and LVC should probably be regarded as incidental variants of myocardial structure unlikely to require further investigation and with an overall good prognosis on follow-up.\nThe authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.\nNil.\nThere are no conflicts of interest.
A sixty-four years old female with a past medical as well as family history of type II diabetes and hypertension presented to an outside facility with worsening dyspnea on exertion that had rapidly progressed from her baseline along with troponin elevation. Her electrocardiogram (EKG) did not show evidence for ST elevation. A transthoracic echocardiogram (TTE) was performed which revealed EF 20%-25%, severe inferior hypokinesis, mild apical hypokinesis. She underwent cardiac catheterization for presumed ischemic cardiomyopathy due to reduced EF and troponin elevation, which revealed chronic occlusion of the right coronary artery, subtotal occlusion of the proximal LAD, a severe stenosis in the first circumflex marginal and an occluded second marginal. She was transferred to our facility for coronary artery bypass grafting. TTE on presentation to our facility revealed a severely dilated left ventricle (LV) with eccentric hypertrophy and reduced ejection fraction (EF) of 30%. Thinning and akinesis of the inferior/inferolateral wall was noted with basal and mid inferior wall aneurysm which was likely supplied by an occluded right coronary artery. There was severe functional secondary mitral regurgitation arising from annular dilatation. No thrombus was identified despite administration of ultrasound contrast agent (Figure ).\nCMR was also done within 24 h of the TTE to ascertain viability of myocardium to determine candidacy for surgical revascularization. CMR revealed a severely dilated LV chamber size with large basal inferior wall aneurysm. Post Gadolinium Inversion recovery sequences with high inversion time of 600 ms revealed 3 cm x 1.3 cm mass within basal inferior aneurysm with homogenous black appearance confirming presence of mural thrombus. There were also 2 apical thrombi visualized with largest measuring 1 cm x 1 cm (Figure ). Aneurysmal basal inferior wall was non-viable however myocardium supplied by left anterior descending (LAD) and left circumflex territory (LCX) was noted to be viable. She underwent coronary artery bypass grafting with a saphenous vein graft (SVG) to LAD and a SVG to the first obtuse marginal branch of LCX as well as bioprosthetic mitral valve replacement (MVR). She also underwent resection and plication of posterior left ventricular aneurysm with removal of mural thrombus. She was started on anticoagulation with warfarin post-operatively for the apical thrombi.
A 27-year-old woman with complete soft tissue avulsion of her left forehead was referred to our service 2 years after a severe motor vehicle crash. She underwent in another hospital 17 months before a delayed scalp flap for 30 days to promote granulation subsequently followed by full thickness skin graft over frontal bone. In addition, no attempt to reconstruct the eyebrow was previously performed. At physical examination we noticed absence of frontalis muscle in the implanted region, lack of functioning of the upper eyelid elevator muscle and complete left eyelid ptosis. The patient also had absence of left eyebrow and an extensive scar tissue on the site (). Further evaluations revealed she had traumatic superior orbital fissure syndrome; characterized by ophthalmoplegia, ptosis, proptosis, and hypesthesia in the forehead and upper eyelid regions.( Her vision was impaired due to non-treatment of the syndrome in the other hospital. An electromyography showed inactivity of left upper eyelid elevator muscle.\nWe planned a composite TPF flap with a longitudinal island of scalp () as follows. A coronal incision was carefully marked away from the path of the superficial temporal artery (STA) identified using a Doppler device. The distance between the rotation axis of the flap and the eyebrow defect was marked with a blue marking ink and transferred to the scalp, showing the length of the TPF flap to be harvested. The dimension of the contralateral eyebrow was also transferred to the scalp, showing the region of the scalp, simulating the ipsilateral eyebrow, and an initial incision was carried out at the distal parietal portion of the coronal marking.\nThe island of hair-bearing scalp flap was incised up to the left ear, however, preserving the STA. After wide elevation of the scalp flap at the subcutaneous plane, the STA trajectory was fully visualized and the distal parietal portion of this vessel was ligated distally from hair-bearing island scalp flap. The thin STA, including the hair-bearing island scalp flap, was carefully elevated toward its axis of rotation. An incision was performed on the left eyebrow region and the TPF flap rotated to this area through a subcutaneous tunnel between the base of the pedicle and the eyebrow defect.\nWe adapted the hair-bearing island scalp flap into the incised space of the eyebrow region (when the contralateral eyebrow design was used as a template) and sutured it using nylon threads (Johnson & Johnson, São José dos Campos, São Paulo, Brazil). The scalp donor site was closed directly. A vacuum drain was placed for 24 hours.\nConcurrent with the TPF flap the patient underwent left frontal bone reconstruction using calvarial grafts. The patient was also submitted other surgical procedures, one to reconstruct her left nasal wing with an expanded two-stage forehead flap and another to remove the full thickness skin graft over her forehead with an expanded temporal skin flap.\nNo procedure to restore the upper eyelid functioning was performed because of the severity of the soft tissue damage in patient’s upper third of the face (absence of the frontalis muscle and impaired function of upper eyelid elevator muscle).
A 54-year-old Caucasian female proceeded to our institution with epigastric pain, nausea and vomiting along with pain located around the lumbar area lasting for one week. No previous surgical history or commorbidities existed. Clinical examination did not reveal any palpable abdominal masses or abdominal tenderness and the patient’s vital signs were within the normal spectrum. Blood test detected hypercalcemia (serum calcium: 10.2 mg/dL) and parathyroid hormone level of 111.8 pg/mL. All the findings in conjunction with the clinical presentation lead to the assumption that the patient had primary hyperparathyroidism (PHPT).\nThen, an ultrasound was performed but it was negative for any thyroid or parathyroid abnormalities. Subsequently, the thoracic and abdominal CT revealed a soft tissue in the anterior mediastinum 7 × 1 cm. Additional Tc-99m-MIBI scintigraphy followed, which detected an ectopic adenoma located in the lower anterior mediastinum, on the left of the median line (). Following these, a mid-sternal thoracotomy was finally scheduled.\nDuring the operation, after the thoracotomy, surgeons attempted to detect deep into the mediastinum the parathyroid adenoma according to the preoparative localization. Indeed, the mediastinal mass was detected on the left of the median line, at the anterior mediastinum, in front of the anterior surface of the pericardium and close to the left pericardiophrenic vessels and the left phrenic nerve (). The adenoma was covered by a thin fibrous capsule. When surgeons removed the capsule, a dark red mass of 7 × 2.8 × 1 cm was finally revealed (Figs. and 4 ). The detailful preoperative localization of the present mediastinal adenoma which was in close relation with various anatomical structures of the thorax, reduced effectively the difficulty of the mass excision and the potentiality of accidental surgical injuries which may lead to thoracic bleeding and subsequent obstructive symptoms.\nThen, the operation continued in the usual fashion and a drainage was placed into the left side of the thoracic cavity. The patient was discharged the 5th postoperative day with instructions, when the drainage was finally removed.\nHistology of the mass confirmed the diagnosis of ectopic parathyroid adenoma that was composed predominantly of oxyphil cells arranged in an acinar pattern. Serum calcium level was 2.60 mmol/L and iPTH 17.6 pg/mL 12 h after the operation. Serum calcium and iPTH remained normal after 6 months’ follow-up.
The patient was born by Cesarean section at 39 wk of gestation to a 34-yr-old G3P2001 mother following a pregnancy notable for maternal genitourinary Group B streptococcal infection and the presence of IgM and IgG antibodies to cytomegalovirus (CMV) in maternal serum. The mother had previously lost a 3-yr-old female child diagnosed with twice relapsing acute megakaryoblastic leukemia and concentric left ventricular hypertrophy in the absence of a discernible cardiac defect on transthoracic echocardiogram. Ultrasound of the patient obtained at 21 wk of gestation showed dilated lateral ventricles (11 mm in diameter), indicating mild fetal ventriculomegaly on this otherwise normal evaluation. Subsequent ultrasound evaluations at 27 wk and 34 wk of gestation demonstrated affirmed ventriculomegaly. Head ultrasound obtained shortly after birth revealed continued dilatation of the cerebral ventricles and prominent choroid plexi in the lateral and third ventricles.\nAt 5 mo, the patient came to the attention of our pediatric neurosurgical service after a surveillance magnetic resonance imaging (MRI) of the head showed markedly dilated lateral and third ventricles with prominence of the extra-axial spaces in the frontal and temporal regions, abnormal prominence of the choroid plexus, and two choroid plexus cysts with no evidence of aqueductal stenosis or other intraventricular obstructions (). Initial examination by our team did not reveal clinical signs of hydrocephalus (i.e., full and bulging fontanel, progressive macrocephaly) but was notable for positional plagiocephaly and dysmorphic facial features (flattened left facial structures and asymmetrically set ears). The patient also had sensorineural hearing loss of the left ear and global developmental delay. A diagnosis of hydrocephalus associated with diffuse villous hyperplasia of the choroid plexus (DVHCP) was made. In the setting of no clinical signs of hydrocephalus and subsequent MRI evaluations showing no significant increase in ventriculomegaly, the patient was managed conservatively with surveillance MRIs with a plan to pursue CSF diversion surgery if indicated. The patient experienced delays in attaining developmental milestones: he sat independently at 12 mo, pulled to his knees at 16 mo, and possessed a vocabulary of fewer than 10 words at 21 mo.
A 7-year old boy presented 1 day after injury to the right eye. The left was normal. The right eye vision was 6/18. There were abrasions on the cornea. Anterior chamber had cells of 1+ and flare of 2+. Fundus examination showed a double humped sub-foveal bleed. The retina over the macula looked edematous clinically. OCT done at presentation showed blood between the RPE and ellipsoid layer. The ellipsoid and ELM layer over the blood lost its clear distinction and looked thickened due to edema. OCT line scan showed two areas of breaks in the RPE/BM complex measuring 103 and 185 μm [ pre]. One week later since the sub-macular blood persisted, he underwent pars plana vitrectomy, sub retinal tissue plasminogen activator injection, and SF6 gas replacement of the vitreous cavity using the technique described previously.[] A month later the vision improved to 6/9. The fundus picture and OCT line scan after surgery is shown in . Clinically macular area showed multiple stellate lines under the retina that did not correspond to areas of choroidal rupture. The ELM layer and ellipsoid layer were continuous under the foveola. There was scar tissue between the photoreceptor layer and RPE corresponding to the yellowish gray area under the fovea seen clinically. Full thickness neurosensory retinal defect was seen at the site of retinotomy. The RPE/BM complex break near the retinotomy was plugged with scar tissue. There were some cystic changes in the margin of the retinotomy site. At the site of the rupture corresponding to the area in pre, there was scarring and closure of the BM break like in the previous two cases [ post].\ngives an overview of the cases presented. As has been noted above besides a break in the BM, the SSOCT showed blood between RPE and ellipsoid layer, and variable amounts of edema of the inner retinal layers in all the three acute cases. The gap in the BM ranged from 103 to 465 μm. The inner neuro-sensory retina and the Sattler's and Haller's layers of the choroid did not show any disruption in the acute stage.\nOCT after a month of the injury showed scar tissue involving Sattler's layer, choriocapillary layer, BM, RPE, ellipsoid layer, ELM, and outer nuclear layer in all the cases.
Our case began with a 79-year-old female who called 911 from her home for transport to the emergency department (ED) for evaluation of abdominal pain in her left upper quadrant with radiation through to her back and left shoulder, which had been present for approximately three hours. The patient had a past medical history significant for hypertension and hypercholesterolemia for which she took lisinopril and rosuvastatin daily.\nUpon arrival at the patient’s home, the emergency medical services (EMS) team reported that the patient initially appeared well with normal vital signs but then began to deteriorate. The patient’s blood pressure fell to 61/38 mmHg and she had a near syncopal episode prompting EMS to administer intravenous (IV) fluids and commence transport to the ED for further evaluation.\nWhen the patient arrived to the ED she was awake and alert with mild distress from her pain but interacting appropriately. Her vital signs were blood pressure of 101/56 mmHg, pulse rate of 74 beats per minute and an oxygen saturation of 100% on room air. Her physical exam was positive for tenderness in the left upper quadrant of her abdomen, but it was soft with no guarding or rigidity. She showed no signs of focal neurologic deficit, jugular venous distension, heart murmurs or abnormal lung sounds. Her extremities had equal distal pulses with good capillary refill. Her medical history provided no identifiable explanation for her pain. The patient denied any history of trauma but reported she “did sneeze three times” just prior to the onset of her pain. Given the patient’s symptom of sudden onset abdominal pain with radiation to her back accompanied by hypotension, abdominal aortic aneurysm (AAA) was immediately considered. A bedside ultrasound was then performed but results were limited due to bowel gas present. Initial interpretation of her exam showed no obvious AAA, no definite free fluid and no evidence of pericardial tamponade. In the meantime vascular and general surgery were notified of the case, and since the patient’s blood pressure had remained stable the decision was made to obtain computed tomography (CT) angiography of the abdomen and pelvis to further evaluate for vascular pathology.\nFollowing the CT, the radiologist immediately called with preliminary results concerning for splenic laceration and hematoma with hemorrhagic abdominal and pelvic ascites (). After arriving back in the ED, the patient’s blood pressure decreased to 68/45 mmHg. She was given a one-liter IV fluid bolus and was transfused one unit of packed red blood cells. The CT results and clinical deterioration were communicated to the general surgeon who took the patient directly to the operating room where she was found to have a ruptured spleen in multiple pieces with a large amount of free intraperitoneal blood. A splenectomy was performed and the patient recovered without sequelae. The pathology report revealed splenic fracture with otherwise-normal splenic tissue. Lab results showed the patient’s initial liver functions and hemoglobin were within normal limits and a mononucleosis screen was negative.\nDuring her recovery the patient was questioned concerning any sustained trauma or recent illness. She continued to deny any obvious inciting event but recalled she had three forceful sneezing episodes prior to the onset of her pain. From a clinical standpoint her sneezing episodes were the only identifiable trigger for this patient’s SSR.
A 55-year-old Caucasian woman with a 5-month history of abdominal pain and vomiting was diagnosed as having a probable high-grade ovarian malignancy with a large volume of peritoneal disease. She had a prior ultrasound of her pelvis which demonstrated a 17 cm large irregular solid vascularized mass in her right ovary. Her comorbidities included obesity (body mass index of 33), asthma, and she had previously undergone a laparoscopic cholecystectomy. There was no significant family history. She did not smoke tobacco and she drank alcohol occasionally. She was scheduled for debulking surgery; however, she presented to our emergency department with worsening abdominal pain, vomiting, and diarrhea. A physical examination showed dry mucous membranes, capillary refill < 3 seconds, and jugular venous pressure of 4 cm. Her chest was clear on auscultation with dual heart sounds. Her abdomen was distended with generalized tenderness but no guarding or signs of peritonism. Bowel sounds were present. A computed tomography (CT) scan of her abdomen and pelvis was performed which demonstrated the large right ovarian tumor, peritoneal tumor deposits, and ascites. There was extrinsic compression of her sigmoid colon due to the tumor without radiological signs of large bowel obstruction. There were no other abnormalities of her bowel. She received intravenously administered fluids for rehydration, anti-emetics for nausea, and intravenously administered morphine for abdominal pain. She remained overnight in our emergency department for treatment. She was reassessed the following morning after resolution of her symptoms and was found to be hemodynamically stable and subsequently discharged home.\nShe re-presented 1 week later with similar symptoms and was admitted to hospital for further management. Following multidisciplinary discussion, she was recommended for neoadjuvant chemotherapy prior to surgical debulking. An urgent core biopsy of the mass confirmed the likely diagnosis of ovarian malignancy. This biopsy indicated a high-grade serous adenocarcinoma. The histology and CT findings were consistent with an International Federation of Gynaecology and Obstetrics (FIGO) stage III ovarian cancer. She was appropriately counselled as to the benefits and risks of chemotherapy prior to commencing treatment.\nShe was commenced on a first cycle of the commonly used platinum-based two-drug chemotherapy regime of paclitaxel and carboplatin []. The dose prescribed was a three weekly cycle of paclitaxel 100 mg/m2 and carboplatin 385 mg/body to achieve area under the curve (AUC) of 5 using the Calvert formula. The results of her pre-chemotherapy blood tests were within acceptable ranges. Specifically, her white cell count (WCC) was 10.1 × 109/L (normal 4–11 × 109/L) and neutrophils were 8.5 × 109/L (normal 1.5–8 × 109/L). She reported feeling better 1 day after chemotherapy. However, 3 days following the commencement of chemotherapy, persistent diarrhea developed. Stool cultures were negative for stool pathogens including Clostridium difficile. On the sixth day post-chemotherapy, she became febrile and acutely unwell with severe abdominal pain. Blood tests at the time revealed a WCC of 0.6 × 109/L and neutropenia of 0.1 × 109/L. A repeat CT of her abdomen and pelvis showed a thickened descending colon and rectal pneumatosis with perforation into the mesorectum (Figs. and ). Blood cultures were positive for Escherichia coli and meropenem was commenced. She was transferred to our intensive care unit for hemodynamic support due to septic shock. A surgical assessment was conducted but immediate surgical intervention was deemed inappropriate due to high predicted mortality. She was administered granulocyte colony-stimulating factor but her white blood cell counts did not improve and her respiratory function deteriorated. She died 9 days after the administration of the first dose of chemotherapy. An autopsy was not performed due to the wishes of our patient’s family.
A 79-year-old male with longstanding history of advanced prostate cancer on androgen deprivation therapy presented to his urologist after noticing a mass on the tip of his urethral meatus. A subsequent biopsy of the mass was positive for SCC, and the patient underwent partial penectomy and lymph node dissection that revealed positive right inguinal lymph nodes (three out of seven) revealing pathologic T2N2M0 disease. He received adjuvant chemotherapy by extrapolating data of its benefit when given in the neoadjuvant setting. The standard TIP regimen was not pursued given patient's concern for side effects. The patient proceeded with alternative plan of chemoradiation with 5 weeks of weekly low dose carboplatin and paclitaxel. In addition, he received radiation with a total dose of 5000 cGy over 25 fractions to the right inguinal region. However, the patient developed disease recurrence with nodal involvement nine months later. On restaging CT imaging, the patient was found to have new involvement of the left pelvis. A nodal conglomerate measuring 31×58 mm with central necrotic change was identified in the left inguinal region.\nGiven the patient's age, performance status, and local recurrence of disease, he was started on therapy with chemoradiation with curative intent one month later. Treatment with an additional round of chemoradiation with low dose carboplatin and paclitaxel was given for 5 weeks. He had radiation with a total dose of 5000 cGy over 25 fractions to the left pelvic region. He had stable disease with chemoradiation, but he eventually developed disease progression within a year from the end of chemotherapy. At that point, he was considered for second-line therapy with the PD-L1 inhibitor atezolizumab. After being on atezolizumab for approximately 2 years, he developed biopsy-proven bullous pemphigoid, an immune-mediated toxicity of the skin that has been described with those agents. A restaging scan at approximately 2 years showed near complete response, so patient has been placed on treatment holiday at the time of this report. He was started on prednisone 1 mg/kg per immune-mediated management guidelines and had quick resolution of his blistering symptoms [].
The patient was an 87-year-old female who initially presented to her primary care provider in May 2010 complaining of increasing abdominal pain and tightness in the central abdomen for two days with associated nausea, vomiting, and anorexia. Her physician sent her to the emergency department for further evaluation. The patient's surgical history included an open appendectomy in 1960 and her past medical history was significant for hypertension, atrial fibrillation, polycythemia rubra vera, and epilepsy. Her urological history was unremarkable. According to the patient, she had similar episodes in the past few years where she felt occasional bloating and the feeling that something was protruding into her scar, but that pain usually passed within a day.\nUpon evaluation in the emergency department, the emergency medicine resident noted a “soft, midline mass, which is tender and is incompletely reducible with moderate increase in discomfort.” A healed 5 cm scar was present in the right abdomen. Bowel sounds were increased, and the mass was initially attributed to a ventral hernia causing the patient's symptoms. An acute abdominal series showed no clear signs of obstruction. A CT scan (Figures and ) of the abdomen was obtained due to her age and severity of pain and the report from the on-call radiologist noted the following.\n“The right kidney is no longer within the renal fossa as seen in 2006, but has changed position and is now anterior to the liver. There is resultant severe hydronephrosis involving the renal pelvis and proximal ureter. This abruptly changes caliber on axial image 38 and the ureter is not well seen inferior to this level. There is symmetric renal perfusion. High attenuation material is seen dependently within the dilated renal pelvis, this could reflect blood products or stone material.” Additionally, there was no diaphragmatic hernia noted.\nThe urologist on call was then called at approximately two thirty in the morning to assess the patient, and noted in the documentation the following. “A patient with what appears to be UPJ obstruction on the right side that has developed since 2006 when she had a CT scan demonstrating what looks to be a lower pole crossing the arterial vessel. She has since had progressive obstruction that has pushed her renal pelvis posteriorly and her kidney anteriorly up in front of her liver and has displaced her colon. I am concerned that a full surgical repair would require open surgery in this delicate 88 year old and would suggest that she undergo a cystoscopy and right double-J stent placement to assess how she tolerates that.” Approximately 6 weeks following the emergency department visit, a cystoscopy, bilateral retrograde pyelograms, and right double-J stent placement were performed. This ameliorated her symptoms and the stents were removed in a timely fashion.\nThe patient was seen recently in followup in June 2013 and she stated that she had an additional episode of abdominal pain similar to her presentation to the emergency department and that manual pressure on her right upper quadrant was successful in reducing the mass and relieving the pain. In followup, the right kidney was palpable but nontender. The patient was scheduled for a routine six-month followup to track her progress. Since then, she endorsed self-treating with manual manipulation of the kidney when it has been painful.\nIn November 2013, however, the patient again presented to the emergency department with the same abdominal pain. A noncontrast CT scan was performed and the patient's right kidney had now translocated across the midline to a position anterior to her left kidney with extreme hydronephrosis present (). The following day, a retrograde pyelogram () was performed showing that the right kidney had moved back to the right lower quadrant. A double-J stent was placed to reduce the hydronephrosis and subsequently her symptoms resolved. She is expected to follow up for repeated imaging and stent removal.
A four year old male patient presented with dull pain at the right shoulder region and inability to abduct the right shoulder in 2003. There was no history of trauma at that time. The radiograph showed osteolysis of the scapula, with some thinning of the lateral third of the clavicle, without the involvement of proximal humerus (). Two years later, in 2005, the patient had pain over the right clavicular region. The radiograph showed a fracture of the right-sided clavicle with progressive osteolysis of the scapula but the proximal humerus was normal (). The patient was treated conservatively with a clavicular brace. A complete laboratory work-up was done to rule out any metabolic bone disease and all test reports were normal. There was a clinical suspicion of this case being an example of Gorham’s disease. The patient was advised radiotherapy, but the patient refused the same considering his young age. However, the child was kept on oral calcium and oral bisphosphonate (alendronate 1mg/kg) therapy. The patient did not follow-up till 2010, when the patient presented with a fracture of upper third shaft of humerus following a trivial fall. The radiographs confirmed the site of the fracture with progression of osteolysis with disappearance of the clavicle and involvement of the proximal humerus and thinning of the humerus shaft (,). The patient then underwent an open reduction and internal fixation with a long percutaneous K-wire (,,) and the patient was given a U slab, which was later was converted to a U cast. A biopsy was sent for histopathology, since a sufficient bone tissue could be sampled at this stage. Histopathology showed presence of edematous bony tissue with an abundance of inflammatory cells with a great number of thick-walled blood vessels and no evidence of malignancy. This confirmed the case to be Gorham’s disease. Due to the pin tract infection, the wire was removed after 2 months but immobilization was continued for another month. The fracture united after 3 months (). After two years in 2012, the patient came for a follow-up. The radiographs at this time showed progression of osteolysis with scalloping of the upper two ribs in addition to that of the shoulder girdle. However, the cervical spine was unaffected (). A year later, in 2013, the patient presented back with a wasted shoulder girdle without any active movements at the shoulder and a cervical kyphosis and terminal restriction of movements of the cervical spine (,,). The radiographs confirmed the presence of progressive osteolysis of the proximal humerus, scapula, clavicle, upper ribs and an involvement of the cervical spine (). There was no evidence of any neurological deficit. After this, the patient was lost to follow-up.
A six year-old boy presented with a history of pain and local swelling over his third metacarpal of left hand with five months duration. On physical examination, the lesion was firm and immobile and there was slight tenderness on palpation. Active range of motion of his third metacarpophalangeal joint was slightly restricted, and pain was aggravated with movement. There was no history of trauma. His past medical history revealed nothing significant.\nRadiographic examination of the hand showed a marked increase in diameter along the third metacarpal and widening of the medullary canal. The cortex was uniformly thin and the physis was spared []. The characteristics of the lesion were consistent with aneurysmal bone cyst after biopsy, pathologic examination confirmed the diagnosis of aneurysmal bone cyst. Histologically, ABC was composed of cavernous dilated engorged vascular bed and slit like hemorrhagic spaces surrounded and traversed by fibrous septa containing spindled cells, inflammatory cells and lesser number of osteoclast like multinucleated giant cells that are distributed around hemorrhagic cystic spaces. Typically, osteoid formation with or without osteoblastic rimming was observed. We differentiated this lytic lesion with unicameral bone cyst with histological picture of lesion which was consistent with ABC, However probable multilocular lytic lesion in x-ray also in favor of ABC. En – block resection of the tumor was performed through a dorsal longitudinal incision over the third metacarpal. Autologous fibular strut graft was harvested from left leg. The graft was cut into its definitive shape and inserted into the created bony defect. K- wire was used for graft fixation (1.5m K-wire) []. The hand was immobilized in a short arm cast for three weeks, after that patient received three weeks of physiotherapy consisting of progressive active range of motion exercises. Kirschner wires were removed six weeks postoperatively.\nAt final follow-up two years postoperatively, the patient had gained full range of hand motion with no pain. The patient was satisfied with the functional and cosmetic results. Radiographic examination demonstrated osseous integration of the graft with no signs of recurrence [,].
A 5-year-old girl was admitted to our Emergency Department after an episode of acute interscapular back pain occurring without trauma and followed by bilateral upper and lower limb weakness.\nHer family history included 8 Italian members harboring the same homoplasmic m.3460G > A mtDNA (Table , Fig. ). All the family members presented headache poorly responsive to NSAIDs and, except for the girl and her mother, visual loss due to optic nerve pathology. The patient’s medical history was unremarkable.\nThe patient’s vital signs were normal and stable. At the neurological examination, her cognitive functions were normal. There were no cranial nerve lesions. She was unable to stand and walk and had more severe weakness in the right lower limb. She also presented weakness in her upper extremities and difficulties with fine hand movements, particularly in the right hand. The right patellar and Achilles tendon reflexes were brisk. The plantar reflex was positive in the right foot. She presented impaired bladder control. No alterations in touch or pain sensitivity were present in the trunk and limbs. No concomitant signs of infection or inflammation were present, and no such signs had been reported in the previous weeks. Analyses of CSF pressure, glucose, protein, cell count, viral PCR, and culture were normal, and oligoclonal bands were absent (see timeline of events and treatment in Fig. ). The patient underwent a spinal cord MRI; the results showed hyperintense signal alterations in T2-weighted sequences and restricted diffusion in diffusion weighted imaging (DWI) sequences in the anterior portion of the cervical and dorsal spinal cord, suggesting anterior spinal artery territory involvement (Fig. ). Computed tomography angiography (CT) imaging showed no arterial dissection or other vessel abnormalities. Visual evoked potentials were normal. Somatosensory evoked potentials in both legs showed decreased conduction velocities. Motor evoked potentials showed lower amplitude for cortical derivation, prolonged latency in the upper limbs and normal in the lower limbs. Central conduction time was increased in the upper limbs and normal at the lower limbs. Complete autoimmunity and thrombophilia screening were unremarkable. Testing for anti-AQP4 antibodies was negative, and anti-MOG antibodies were not significantly elevated. A cardiologic consultation and echocardiography identified normal heart and aorta features. At the ophthalmological assessment, pupillary reactions were normal, the fundus oculi did not reveal pathological signs, and color vision was not affected. The visual acuity was 9/10 bilaterally. The digital visual field test displayed a mild defect in the peripheral portion of the visual field that was more evident in the left eye. Optical coherence tomography, visual evoked potential and electroretinograms were normal. As the child grows, her visual function will require careful monitoring, particularly when she reaches the adolescence.\nAfter obtaining written informed consent consistent with the principles set forth in the Declaration of Helsinki, total DNA was isolated from the patient’s peripheral blood according to standard protocols. Full-length mitochondrial DNA sequencing was conducted according to a previously described protocol [], and detected the haplogroup H27 was detected in the proband. The following variants were detected in the homoplasmic state: m.41C > T, m.73A > G, m.263A > G, m.1438A > G, m.3460G > A, m.4769A > G, m.8860A > G, m.11719G > A, m.15326A > G, m.16129G > A, m.16316A > G, m.16519 T > C. Based on the presence of the m.11719G > A and m.16316A > G nucleotide variants, the patient is predicted to belong to the H27 haplogroup. The m.3460G > A transition, a major mutation associated with LHON, was verified in blood-derived DNA of other family members by PCR-RFLP analysis.\nAcetylsalicylic acid was administered at low doses (2.5 mg/kg daily), and high doses of methylprednisolone (20 mg/kg daily) were empirically administered for three days. Although no proven treatments for LHON are available, early treatment with idebenone is thought to limit the progression of the disease; the patient was administered 45 mg of idebenone three times daily. Within two days following this therapy, clinical manifestations improved, and the patient regained bladder control and the ability to ambulate; within one week, the girl also recovered nearly normal strength in both arms. A control spinal cord MRI together with 1H-MRS to study the lactate peak was performed 10 days after hospital admission; the results showed complete regression of the alterations and no abnormal metabolites (Fig. ). The patient continued outpatient rehabilitation, and her motor functions improved, resulting in an almost completely normalized neurological examination after 2 months and preserving these achievements at follow-up one year later.
An 86-year-old woman who had been living with her elderly husband was admitted to our hospital with the complaints of nausea, anorexia, and epigastric discomfort. When she was 73 years old, the patient had undergone laparotomy and right hemicolectomy for resection of colon cancer. Five years after hemicolectomy, she had received hernia repair surgery using mesh for an abdominal incisional hernia. There was no history of abdominal or thoracic trauma. Laboratory tests were all within the normal range. Her electrocardiogram showed no ischemic changes. Although chest X-ray revealed an abnormal gas-filled mass in the left thoracic cavity, the patient had no symptom of dyspnea. Contrast-enhanced computed tomography (CT) scan confirmed herniation of the gastric corpus through the left posterior part of the diaphragm (). We diagnosed adult Bochdalek hernia and planned its surgical treatment. As mesh had been placed under the previous upper abdominal midline incision more than 10 years earlier, dense adhesions between the mesh and abdominal tissues were expected. Therefore, we decided to perform diaphragmatic hernia repair by HALS, considering the patient’s safety. After induction of general anesthesia, the patient was placed in the supine position with her legs apart. The previous midline incision was opened carefully to insert a LAP DISC® (Hakko, Nagano, Japan) for a hand port. Dense adhesions, which had to be divided, were found between the mesh used to repair her incisional hernia and loops of the small intestine. The disk for HALS was placed after complete removal of the mesh. Subsequently, a 12 mm trocar was inserted into the inferior umbilical region for the laparoscope. A 5 mm trocar was also inserted into the left upper abdominal region. Herniation of the gastric corpus into the left thoracic cavity through a hernial orifice in the left posterior diaphragm was confirmed. The gastric corpus could not be pulled back into the abdominal cavity because of adhesions around the hernial orifice. After these adhesions were carefully removed by HALS using Harmonic ACE shears (Ethicon, NJ, USA) (a), the stomach was completely freed and could be returned to the abdominal cavity. A 5 × 3 cm hernial defect with sac was observed (b). We decided to perform simple closure of the defect without mesh reinforcement because the rim of the hernial orifice was relatively strong. Accordingly, the defect was repaired with interrupted nonabsorbable sutures (2-0 Nesporen; Alfresa Pharma Corporation, Osaka, Japan) using a 5 mm port on the left upper abdomen and the surgeon’s left hand via the hand port (c). The midline incision for the hand port was closed without mesh reinforcement. A drain tube was placed under the left hemidiaphragm. The operating time was 244 min and there was no significant bleeding. Her postoperative course was uneventful. The patient was discharged on postoperative day 20. There was no evidence of recurrence at 1-year follow-up.
A 36 year-old female complained of persisted dull pain over epigastric area for 2 months. No associated symptoms such as heartburn, nausea, and vomiting can be elicited. She visited local clinic and a mass was palpated in the left upper quadrant area. At the same time, abdominal sonography showed a huge hepatic mass at left lobe of liver. So, she received examination of abdominal computed tomography (CT) thereafter. Abdominal CT () revealed a huge left hepatic mass with a size of 20 x 15 cm. She was then transferred to our institution for further management. Initial laboratory data were only notable for a decreased hemoglobin level of 9.7 g/dL and a mildly elevated level of alkaline phosphatase (139 U/L). The serum marker of hepatitis B or hepatitis C was also negative. Tumor markers, including serum carcinoembryonic antigen (CEA), CA19-9, and α-fetoprotein level (AFP) were within normal value. Angiography revealed this huge hepatic mass was supplied by left hepatic arterial (). After studies, she received operation for excision of the tumor. The excised liver measured 20 x 15 x 10 cm and weighed 793 gram. The cutting surface showed a huge soft mass of size 14 x 12 x 6 cm with whitish to pinkish surface (). There was no central necrosis in the tumor. The tumor margin was greater than 5 cm away from the nearest section margin. There was no cirrhotic change at the remaining part of liver parenchyma. Microscopically, the lesion was mainly composed of hyperchromatic and mild pleomorphic spindle cells admixed with many reactive plasma cells and lymphocytes. These neoplastic cells formed fascicles, storiform and whorls pattern. They had plump cytoplasm and indistinct cell borders with vesicular or granular chromatin and small nucleoli. Some giant cells and fibrous bands were seen but rare mitotic figures were found within the tumor (). These tumor cells were positive for expression of CD21 and CD35 by immunohistochemical staining and positive for expression of EBER by in situ hybridization (). Therefore, the final pathology was follicular dendritic cell tumor of the liver.
A 30 year old male from mallavalli taluk, Mysore presented with severe deformity and unable to move his both upper limbs since 20 days after fall from height. He was sitting over cement well when he had a sudden onset of generalised seizure. He lost consciousness and fell on the ground. There was no external wound and associated injury in other parts of the body. He was taken to a local osteopath and massaging was done. He was later brought to our center 20 days after injury. On presentation he complained of severe bilateral shoulder pain with difficulty in movements of both the shoulders. Physical examination findings included bilateral flattening of shoulder, restricted and painful movements in all ranges of shoulder joint []. Distal neuro vascular status of both upper limbs was normal. Radiological investigation revealed comminuted fracture neck of right humerus (Neer’s 4 part fracture []) and greater tuberosity of left humerus (Neer’s 2 part fracture) with bilateral subcoracoid dislocation of shoulder []. Closed manipulations under general anaesthesia was unsuccessful in reducing the dislocations (as expected in delayed presentations). Open reduction and internal fixation on both the sides was planned. Deltopectoral approach was taken bilaterally. Joint capsule was divided and fracture site directly visualized. On left side the joint was relocated and the tuberosity fracture was repositioned. K wires were passed securing the tuberosity fracture and were advanced further to transfix the glenohumeral joint, maintaining reduction. On right side, reconstruction of proximal humerus fracture was done using simple plate and screws. The reconstructed proximal humerus was then relocated into the glenoid cavity and transfixed with K wires. Primary rotator cuff repair was done on both the sides. Deltoid muscle was repaired in single layer. Wound was closed in layers. Bilateral U slab immobilization was done []. The shoulders were kept immobilized for 3 weeks at the end of which K wires were removed and shoulder rehabilitation was started. At one year follow up the patient had acceptable range and was able to carry out daily activities. Radiographs showed good union ()
An eighteen years old, unmarried, Saudi female, underwent bariatric surgery. Three months after the procedure, she complained that she had experienced weakness in both lower limbs for a period of 3 weeks. This was associated with a burning sensation, which had started on the soles of her feet and progressively over a period of 2 weeks had increased until it reached her thighs, making it difficult for her to stand and walk without support. Although this weakness did not affect her upper extremities, she eventually became bed bound and dependent on her family members for her daily needs. She also reported that she had suffered from dizziness and intermittent mild blurring of vision. Initially, she also had suffered from vomiting, which had improved with medication. She did not report a history of headaches, double vision, confusion, seizures, difficulty in swallowing or speaking, sphincter dysfunction, or fever.\nPrior to surgery, the morbidly obese patient had a body mass index (BMI) of 41.2 kg/m2 and confined to a wheelchair. The patient was living with her parents and had just completed high school and waiting for acceptance to the university. She had been previously diagnosed with hypothyroidism, for which she was taking 100 μg of thyroxine daily. Other than this, her history and family history were unremarkable, with no history of smoking.\nShe underwent gastric sleeve surgery in July 2014. After surgery, she was primarily on a liquid diet. The patient did not take the prescribed multivitamins and lost a total of 26 kg. At the time of presentation, the patient had normal vital signs. She was conscious with normal language functions and oriented to time, place, and persons, with a mini mental state examination score of 30/30. Her cranial nerves were intact. Concerning her motor system, bulk and tone were normal and symmetrical. However, the lower extremities were tender to touch. Muscle power was 5/5 in upper limbs, proximally as well as distally, and 2/5 in both lower limbs. She had generalized hyporeflexia with bilateral flexor plantar responses. She was found to have hyperesthesia in her soles, feet, and legs. The remainder of her sensory examination was normal. Mild nystagmus was seen in all directions, but finger nose coordination was intact, and there was no dysdiadochokinesia.\nRoutine tests were conducted, including complete blood count, fasting blood sugar, fasting lipid profile, liver function tests, renal function tests, creatine phosphokinase, and urine analysis, which were all within normal limits. Ca++ was 9.6 mg/dl and PO4 was ≤4.25 mg/dl. The Vitamin B12 level was 1803 pg/ml, and erythrocyte sedimentation rate was 36 mm in 1 h. Serum thyroid stimulating hormone was 0.161 (low), T3 was normal, and T4 was 13.91 (high). Her folic acid level was significantly low at 1.3 ng/ml (3.1-20.5 ng/ml, deficient <3.5 ng/ml). The magnetic resonance imaging of the brain and lumbosacral spine was normal. Electromyography and nerve conduction study showed axonal sensory-motor peripheral neuropathy affecting the lower limbs with acute denervation and neurogenic motor unit action potentials. shows the results of the nerve conduction study of the peroneal nerve. reveals that there was significant denervation of the left tibialis anterior muscle.\nOn the basis of these clinical findings and investigations, she was diagnosed as having sensory-motor polyneuropathy secondary to folate deficiency. Folate deficiency is one of the causes of post bariatric surgery polyneuropathies. She was prescribed multivitamin supplements including folic acid and other micronutrients.
A 57-year-old asymptomatic man with no significant past medical history was found to have an enlarged cardiac silhouette on a routine chest radiograph (). Magnetic resonance imaging (MRI) revealed a 9 cm pericardial cyst in the right cardiophrenic angle that was associated with right atrial compression (Figures , , and ). Although the pericardial cyst wall showed contrast uptake, no uptake within the cyst was observed on first-pass or delayed images. There was no compression of the airway or superior vena cava (SVC) and the pericardial cyst had not eroded into the heart. The patient was not at high risk for hydatid cysts and he did not have any history of fever, suggesting that an infectious cause for his pericardial cyst is unlikely. He did not have any history of chest trauma or intrathoracic surgery. The absence of hypertension, hematuria, and a positive family history made a diagnosis of autosomal dominant polycystic kidney disease (ADPKD) unlikely. The patient was scheduled for resection of the pericardial cyst using VATS. Preoperative electrocardiographic findings, complete blood count results, serum creatinine levels, liver function tests, and serum electrolyte levels were normal.\nOn the day of surgery, the physical exam, including heart and lung auscultation, was unremarkable and the vital signs were within normal limits (blood pressure of 119/75 mmHg, heart rate of 83 beats per minute, respiratory rate of 14 per minute, blood oxygen saturation of 97% on room air, and temperature of 36.9°C). A left radial arterial line and two large-bore intravenous catheters were placed. The patient was adequately hydrated with intravenous administration of normal saline. He was transferred to the operating room and placed in the supine position on the operating table. The standard American Society of Anesthesiologists monitors were placed on the patient. The pericardial cyst did not compress the patient's right bronchus or the SVC, and therefore, he was able to tolerate the supine position with no shortness of breath or hemodynamic instability. The patient was preoxygenated and general anesthesia was induced by slow intravenous administration of etomidate 0.2 mg/kg and fentanyl 1 μg/kg. Neuromuscular blockade was achieved by intravenous administration of succinylcholine 1.5 mg/kg. Ephedrine 0.1 mg/kg was administered following induction to minimize the hemodynamic effects of the induction agents and positive pressure ventilation. A 37-French left-sided double-lumen endobronchial tube (DLT) was placed. The patient was placed in the left lateral decubitus position. Anesthesia was maintained with oxygen (fraction of inspired oxygen of 0.6), air, and sevoflurane (1 minimum alveolar concentration). The right lung was collapsed and neuromuscular blockade was induced with rocuronium 0.6 mg/kg. A 1-cm incision was made in the posterior axillary line at the fifth intercostal space. A metal port was placed and a 10-mm, 30° thoracoscope was placed through the incision. A 2-cm incision was made above the fifth intercostal space at the mid axillary line to access the cyst. The pericardial cyst was found to be firm, and had some calcifications on the surface (Figures , , and ). The cyst was easily separated from the pericardial fat. However, it was attached to the anterior chest wall. Following separation of the cyst from the anterior chest wall some bleeding occurred from the distal right internal mammary artery. The bleeding was controlled with Enseal and clips. The cyst was large, and hence, access to the superior aspect of the cyst was difficult. Therefore, an 18-gauge needle was used to aspirate the cyst fluid. Approximately 300 ml of brown, murky, nonodorous fluid was aspirated from the cyst before it was completely resected (). The patient remained hemodynamically stable throughout the procedure and the DLT was removed in the operating room at the end of the procedure. Postoperative pain was managed with an intercostal nerve block using 10 ml of 0.5% bupivacaine and patient controlled analgesia pump using hydromorphone (intravenously, 0.2 mg every 10 minutes). The patient's postoperative course was uneventful, and he was discharged on postoperative day 1 in a stable condition. Cyst fluid cultures were negative.
A 34-year-old African (Zimbabwean) woman in her first pregnancy presented to our institution at 22 weeks of gestation by referral from a local clinic with a diagnosis of OI. She was wheelchair-bound. Her medical history revealed several fractures as a neonate and in childhood, leading to a diagnosis of OI based on clinical presentation and examination. Confirmatory tests were not available in the public health sector. She has been in and out of the hospital since childhood because of these fractures, with various treatments having been received, including plaster of Paris casts and traction. She had a fracture of the right femur at the age of 12 years, which made her wheelchair-dependent. She can, however, stand with support and climb onto and off the wheelchair without assistance. She was abnormally short compared with her siblings and peers. She had normal pubertal development, and her menstrual cycle was very regular. Her sexual debut was at 28 years, and she has had one sexual partner. Her boyfriend, who was married and had five other children, contributed little to her welfare. She was a vendor receiving some financial support from her siblings. She took combined oral contraceptives for 4 years; however, she had stopped taking them because she thought she could not get pregnant. The result of cervical cancer screening with visual inspection with acetic acid and cervicography done in 2016 was negative. She had no family history suggestive of OI. She is educated to form 4 (ordinary level). This current pregnancy, though unplanned, was booked at 18 weeks of gestation. The results of antenatal screening for human immunodeficiency virus and syphilis were negative.\nOn examination, she was of short stature, 120 cm in height, with a triangular face. Her sclera was white. She had poor dentition with grayish discoloration. She had short limbs, with the right lower limb shorter than the left, and deformities were noted on both thighs. She did not have scoliosis or kyphosis. Her chest was barrel-shaped with good air entry bilaterally. She had normal blood pressure and a normal cardiovascular system examination finding. Her abdominal examination revealed central obesity and a bulky uterus of 20-week-size gestation.\nA multidisciplinary team was involved in her management. The team included obstetricians, a maternal-fetal medicine specialist, pediatricians, anesthetists, a psychologist, and midwives. Ultrasound performed by a maternal-fetal medicine specialist showed a fetus with a bowed femur and short humerus. No fractures were noted. The fetal skull was easily deformable. No other malformations were noted. The conclusion was that the fetus had OI, nonsevere disease. Pediatricians counseled the patient about the fetal condition and the possibility of preterm delivery and its accompanying complications. With the limitations of the neonatal unit, the conclusion was an unfavorable prognosis for the neonatal outcome. The patient, however, was willing to continue with the pregnancy.\nShe was subsequently followed closely in the antenatal clinic, with a plan for pregnancy to continue to 37 weeks, with the possibility of early delivery if maternal respiratory compromise occurred. She had an uneventful antenatal period, with serial ultrasound showing satisfactory growth. She had an admission for a urinary tract infection at 29 weeks of gestation and was treated with oral antibiotics with complete resolution. Contraceptive counseling was provided, and she opted for tubal ligation, which was done at the time of cesarean section.\nShe ultimately delivered, by an elective transverse lower segment cesarean section at 37 weeks, a live female fetus with a birth weight of 2100 g. The operation was done with the patient under general anesthesia, and a smooth intubation was done with care so as not to cause cervical spinal fracture. Bilateral tubal ligation was performed. Estimated blood loss at the time of delivery was 500 ml, and 10 IU of oxytocin was administered for delivery of the placenta. The patient was extubated after surgery and admitted to the high-dependency unit and monitored for 24 hours. The baby was admitted to the neonatal unit. She developed a chest infection, which was successfully treated with intravenous antibiotics. The mother was managed for a paralytic ileus on day 2 after surgery. She was discharged on day 7. She developed a superficial surgical site infection, which was managed with daily dressings and oral antibiotics on an outpatient basis. The child is currently seen in follow-up by her pediatrician for OI. She has blue sclera. She was noted to have healed fractures on the left radius and right femur at 6 weeks of age. Initial neonatal x-rays had not revealed any fractures. Bone deformities were also noted. She can now walk with support. The rest of her developmental milestones are normal. No other fractures have been reported since they were originally noted. The parents are not able to afford genetic tests.
The first patient—patient A was a 76-year-old male. He was 1.75 m tall and weighed 75 kg, it means ‘healthy’ BMI of 26.1. He was admitted with the left hemisphere ischemic stroke, which was confirmed by a deep right-sided paresis and aphasia. During the structural assessment on the day of admission to the rehabilitation, the following were observed: a significant weakening of the abdominal and lateral oblique muscles on the right side. Additionally a deep paresis of the right lower and upper limb as well as weakness and decreased postural muscle activity in the torso were also observed. Symptoms of the disease were particularly noticeable as the limited possibilities of taking the vertical position and maintain the posture. It means that before starting the rehabilitation, the patient did not take an upstanding position himself. Due to paresis of the right limb, the patient was not able to move this side independently.\nThe first step during the rehabilitation was an attempt to support the patient in taking the most optimal and active upright position using SPIDER capabilities. This was performed to stimulate the equivalent reactions, mutual control and cooperation of antigravity muscles.\nThe second step was started from the slow and gradual weighting of a right limb with the use of SPIDER device. The appropriate strength of the abductor and external rotators of the hip joint, knee extensor muscles and the three-headed calf were needed to maintain the proper position of the joints during the successive load. It was also expected to resolve the eccentric work of the three-headed calf.\nNext, to improve the work of transporting the numb limb, the device was used to stimulated: right hip flexors, the concentric work of the three-headed calf and the trunk to have shortening ability. SPIDER device was used to actively stabilize the right knee joint as much as possible, in combination with the pelvic stabilization using elastic cords properties. It allowed working on the load phase of the affected right limb.
A 46-year-old man reported experiencing precordial discomfort and was referred to our department. He was a nonsmoker and had no history of any significant illness or surgery. Physical examination and electrocardiography showed no abnormal findings. Chest radiography, however, showed an enlarged right ventricular shadow, leading to a slightly elevated cardiothoracic ratio of 56%. Transthoracic echocardiography showed a mass in the pericardium, 8.0 × 4.0 cm in diameter, that compressed the wall of the right atrium and ventricle (Fig. ); left ventricular function was within normal limits. There was no tricuspid regurgitation or stenosis. Contrast-enhanced chest CT showed a low-density mass in the pericardium that compressed the wall of the right atrium and ventricle and encased the right coronary artery (Fig. , ). The mass was relatively homogenous except for calcified nodules (Fig. ). No clear separation could be seen between the ventricles and the mass. Coronary angiography showed that the mass had some feeding arteries and that the proximal and mid-portions of the right coronary artery were inside of the tumor without any obstruction (Fig. ). No other signs of coronary disease were observed. Laboratory test results, including biochemistry, coagulation, routine hematology, and serum tumor markers, were all within normal limits.\nThe differential diagnosis included sarcoma or primary lymphoma, though the epicardial location would have been atypical for both tumors. Subsequently, biopsy and surgical resection of the tumor were scheduled in order to establish a precise diagnosis and to improve the outflow from the right ventricle and, thus, his symptomatic condition.\nMedian sternotomy was completed under general anesthesia. During surgery, the tumor was determined to be hemorrhagic because incisional biopsy alone caused active bleeding, and cardiopulmonary bypass was initiated. Frozen section led to a diagnosis of a benign vascular tumor, but the right coronary artery was totally encased by the tumor, which was judged to be not amenable to curative resection. Thus, the tumor was resected directly with an electrical scalpel and debulked to the proximity of the coronary artery. The resected tumor specimen appeared as a spindle with a maximum diameter of 6 cm, accounting for approximately 50% of the original tumor volume.\nHistopathologically, dilated venous vessels with smooth muscle layer were widely observed in the fat layer of the pericardium (Fig. –), leading to a diagnosis of venous cardiac hemangioma. On immunohistochemical staining, endothelial markers CD31 and CD34 were positive (Fig. , ).\nThe patient had an uneventful postoperative course and was discharged on postoperative day 7. Follow-up chest CT showed volume reduction of the tumor and improvement of the compression of the right ventricular wall, and no subsequent regrowth (Fig. ). Presently, 36 months after surgery, the patient is living a healthy life without any symptoms.
An 18-year-old female without a relevant medical history presented with an 8-month history of a left foot mass. It started as a small nodule that progressively increased in size over time, and another small mass developed beside it two weeks before the diagnosis. The patient started to develop fever, rigor, and chills. She underwent incision and drainage, and antibiotics were prescribed for her in a primary health care center, which delayed the diagnosis for two more months. On examination, there was a 6×6 cm rounded, firm mass with a mass of approximately 1.5 cm extending from it. The large mass had an open wound with foul-smelling, purulent discharge leaking from the wound with no other masses on the same side. There were no palpable left inguinal or other lymph nodes upon physical examination. Magnetic resonance imaging (MRI) of the left foot showed a large exophytic, lobulated, and enhancing mass, measuring 6 × 4.5 × 6 cm with central necrosis in the soft tissue of the midfoot. The lesion also involved the flexor hallucis and abductor hallucis muscles (). Six core punch biopsies were taken from the large and small masses and confirmed a diagnosis of CCS. Computed tomography (CT) of the abdomen and pelvis was also performed, which revealed multiple enlarged lymph nodes in the left inguinal region and on the left side of the abdominal aortic bifurcation. Lung and abdominal CT were performed and showed no signs of distant metastasis. The patient underwent a left below-the-knee amputation as a palliative procedure. Pathological examination of the amputated specimen showed a grossly fungating mass with focal surface ulceration in the medial side of the foot. The mass was partially necrotic and grossly infiltrated the tendons but did not reach the underlying bone.\nOn microscopic examination of the lesion, a biopsy showed deep dermal infiltration of loosely cohesive, round to oval malignant cells exhibiting eccentric nuclei with a high nuclear to cytoplasmic ratio, abundant clear to eosinophilic cytoplasm, and abnormal mitosis (mitotic count, 25/10 HPF) (Figures and ).\nA panel of immunohistochemical markers was used for testing. The malignant cells were positive for Vimentin, S100, HMB45, MART-1, and CD99 and negative for the remaining markers.\nWritten consent and the approval of the patient was obtained before writing this report. Ethical approval was also obtained from the Unit of Biomedical Ethics at King Abdulaziz University Hospital.
A 57-year-old female with a past medical history significant for polyarteritis nodosa on immunosuppression with Azathioprine presented to the neurosurgery clinic with progressive right lower extremity shooting pain, numbness and tingling over the past six months. On physical examination, she had trace weakness in right lower extremity dorsiflexion as well as right foot eversion with diminished vibratory and pin-prick sensation in her bilateral lower extremities. Magnetic resonance imaging (MRI) of the thoracic and lumbar spine with and without contrast was obtained which demonstrated a cystic lesion in the conus medullaris spanning the thoracic 11 to thoracic 12 levels (Figure ). This cystic lesion was first identified nine years prior in 2011 (Figure ). At the time of initial imaging in 2011, the patient did not have any neurologic complaints nor did she have any appreciable neurologic deficits on physical examination. A dedicated MRI of the thoracolumbar spine was obtained in 2011 due to an indeterminate finding observed in the conus medullaris on abdominal MRI for workup of abdominal pain. At the time of initial identification in 2011, due to the lack of neurologic symptoms and neurologic deficits, the cystic mass was determined to be an incidental finding with no further intervention pursued. On MRI thoracic spine with and without contrast at the time of her subsequent presentation in 2020, she was found to have interval enlargement (Figure ) of the previously imaged conus medullaris cystic mass (Figure ) which had enlarged over the course of the past nine years. Electromyography (EMG) and nerve conduction velocities (NCV) were obtained with normal results, making a peripheral neurologic process such as neuropathy or radiculopathy less likely. Based on the interval increase in the size of the conus medullaris lesion in the setting of normal EMG and NCV, it was felt the etiology of her current symptomatology and neurologic examination findings were related to the cystic conus medullaris lesion. She subsequently underwent a thoracic-11 to thoracic-12 laminectomy with cyst fenestration. She tolerated the surgery well and was discharged on postoperative day 3. Her pathology was negative for neoplasm and read as “minute fragments of glial tissue with no significant histopathological abnormality.” At the time of her four-week follow-up, her right lower extremity pain, numbness, tingling and strength had all significantly improved. Her four-month follow-up imaging remained pending at the time of submission of this article.
A 30-year-old woman was admitted to the emergency department at 23 week of her second pregnancy for non-specific abdominal pain. She was known for previous minor abdominal surgery including mesenteric cyst excision and vesicoureteral reflux surgery in childhood followed by laparoscopic adhesiolysis 10 years later. She had no fever and no vomiting or constipation history. Biological tests including RBC, WBC, C-reactive protein, bilirubin, pancreatic enzymes and serum lactates were also still normal during 48 hours of observation. The initial imaging investigations by abdominal and pelvic ultrasound showed no intra-abdominal abnormalities and the plain abdominal x-ray at 48 hours revealed only some very slightly dilated small bowel loops. The foetus status in ultrasound was normal.\nPersistence of pain not relieved with strong analgesics conducted to laparoscopic exploration despite the absence of biological or radiological abnormality. Laparoscopy revealed massive necrotic lesions of the small bowel with rare viable segments in discontinuity. After conversion to laparotomy multiple segmental resections were performed, potentially viable bowel segments were closed by stapling and abdomen was left open with vacuum assisted dressing in the aim to asses the viability of remaining bowel after 24 and 48 hours (figures , ). The vacuum abdominal closure was done using a negative pressure therapy system ([NPWT] V.A.C.® Therapy™, KCI Inc.) with 125 mmHg continuous negative pressure. At the second and third surgical look some intestinal segments required subsequent additional resections. Eventually, after 48 hours of open abdomen management, the intestinal continuity was restored leaving 110 cm of viable small bowel. Abdominal wall was primary closed without aponeurotic defect (figure ).\nDuring the two days where the abdomen was left open, optimal foetal and mother conditions were maintained by intensive care procedures including sedation, mechanical ventilation, liquid resuscitation, adapted parenteral nutrition and pharmacologic tocolysis by hexoprenaline.\nThe patient left the intensive care unit on 9th postoperative day. Complete recovery requires in-hospital and ambulatory nutritional support for short bowel syndrome. Pregnancy was uneventfully carried to full term vaginal delivery.
The index case was an approximately 50-year-old man who presented to the General Hospital. He had sustained a minor head injury and lacerations to the right side of his face and right arm as a result of falling while walking in the street. The patient had obvious chorea of his head and all four limbs which he said had caused him to fall. The patient insisted that falling in the street was unusual as he had learned how to live with his abnormal movements over the preceding ~20 years, and that despite his movement disorder he was usually able to maintain his balance.\nThe patient had been an accomplished athlete in his youth and hoped to pursue a career as a professional golfer. These aspirations were abandoned with the onset of his involuntary movements. At the time of presentation, the patient was gainfully employed as a gardener and enjoyed fishing. There was no evidence of a deterioration of performance in either work or hobbies, such as impaired ability to organize and perform specific tasks.\nCranial nerve examination revealed no abnormality apart from mild chorea of his face and his tongue. Speech was normal. His head and all four limbs displayed marked chorea. Tone was mildly reduced, and deep tendon reflexes were normal. Sensory examination was normal, and there was no evidence of cerebellar dysfunction.\nThe patient appeared to be oriented in time, place, and person. Based on his ability to interact and hold a conversation, his cognitive skills appeared consistent with his seventh grade education. This impression was supported by his physical presentation (personal hygiene, clothing), mood, language usage, and his ability to give an apparently appropriate detailed medical history, however, formal cognitive testing was not performed. Since the patient appeared stable, and a formal psychological testing battery was not available at this time, we did not pursue further cognitive investigations. The patient died within a year of this evaluation; the cause of death remains unknown.
A 17-year-old boy slipped in the bathroom and landed on his gluteal region. When he got up, he developed pain in his lower abdomen and noticed bleeding from his rectum along with mass. He was taken to a nearby hospital where they documented it to be prolapsed omentum which was digitally repositioned and was referred to our centre. On examination at our centre, about 3 h after his fall, he had tachycardia but was haemodynamically stable. Abdomen examination revealed tenderness in the lower abdomen with no peritoneal signs. There were no bruises or evidence of pelvic fracture. A rectal examination showed only blood and no mass was seen. A magnetic resonance imaging (MRI) of the abdomen was performed which picked up a tear in the rectum about 2–3 cm just above the peritoneal reflection with the omentum plugging it [Figure and ]. On repeated questioning, he denied any history of rectal instrumentation or abnormal sexual activity. He had no history of constipation or rectal prolapse. He had undergone an appendectomy many years ago. He was administered broad spectrum antibiotics and taken up for a diagnostic laparoscopy during which a 3 cm transverse tear in the left anterolateral aspect of the rectum just at the peritoneal reflection with the omentum plugging it was seen with minimal contamination []. There were no underlying faecoliths. The omentum and pelvic cavity were irrigated with copious saline. The tear was repaired in two layers [] using four ports, one 10 mm and three 5 mm. A covering loop sigmoid colostomy was added. He made an uneventful post-operative recovery. Eight weeks later, a rectal contrast study was done which showed no leak of contrast, and a sigmoidoscopy to rule out an underlying pathology was normal following which his colostomy was closed. Three months following the last surgery, he is doing well. He was repeatedly questioned but denied any direct trauma, in any form that could have caused the injury.
A 12-year-old boy with acute abdominal pain presented to our hospital. The patient reported that pain sometimes occurred postprandially, and that the frequency and severity had worsened over the previous six months.\nA physical examination revealed normal findings, with the exception of the right upper abdominal tenderness. The laboratory data were within normal limits (data not shown). Contrast-enhanced CT in axial view in the first radiologic examination showed a circular solid mass-like lesion (30 × 23 mm in diameter) adjacent to the liver that was heterogeneously enhanced with a small luminal structure in the center (). The association between the mass and the gallbladder was not clearly visualized. A careful US examination led us to suspect that the mass was part of the gallbladder. The patient's abdominal pain improved on the day after admission and fasting. The second US examination revealed that the mass-like lesion was actually the thickened wall of the body and fundus of the gallbladder, which contained several small cysts and a small lumen in its center. It was continuous to the expanded normal gallbladder wall (). The first CT was reconstructed in coronal and sagittal views, and the lesion was recognized as a thickened wall of the entire gallbladder (Figures and ). Magnetic resonance cholangiopancreatography (MRCP) revealed small cysts with an orderly alignment and high-intensity signals in the thickened wall of the body and fundus of the gallbladder, which was regarded as the pearl necklace sign of AMG (), and showed no other abnormalities (e.g., pancreaticobiliary maljunction [PBM]). He was finally diagnosed with AMG and underwent laparoscopic cholecystectomy. The macroscopic observation of the resected gallbladder revealed thickening of more than distal half of the body. The wall of the middle of the body was far more thickened than that of the peripheral area, causing slight stenosis (). The histopathological findings of all areas of the thickened wall contained Rokitansky–Aschoff sinuses (RASs) combined with hyperplasia of the smooth muscle and collagen fibers (), which were compatible with AMG, and no malignant or premalignant findings. Based on these findings the lesion was classified as combined segmental and fundal type. The postoperative course was uneventful, and the patient has been doing well over a one-year follow-up period.
A 35-year-old Turkish man who had fallen on his flexed right knee while walking on ice was referred to our hospital. He suffered from pain and inability to move his right knee. Physical examination revealed prominent swelling and tenderness over the right patella. Plain radiographs showed comminuted displaced inferior pole fracture of the patella (Figure ). His extremity was immobilized initially through a cast brace, and he was then operated under general anesthesia on the following day. During the operation, it was found out that the patellar fracture could not be reduced and repaired. Because of this, a partial patellectomy was performed and his patellar tendon was sutured to the patella with No. 2 polydioxanone (PDS) sutures and augmented with a cerclage wire (Figure ). A long leg cast was then applied and we advised our patient to move using two crutches and bear no weight for three weeks. At the end of the third week, he was started on active and passive ranges of motion exercises of the knee.\nOn his follow-up visit after six weeks, he was already able to flex his right knee by about 100°. After that period, however, he started to experience a gradual decrease in knee movement. Two months after the operation, the active flexion of his knee was only 60°. A lateral radiograph of his right knee showed extensive ossifications at the resected part of the patella and calcifications in the patellar tendon (Figure ). Excision of the ossifications and implant removal were thus planned. During that period, he felt a sudden pain over the tibial tubercle of his right leg while he was descending the stairs. He was unable to extend his right knee.\nPlain radiographs revealed the presence of patella alta. As a result, another operation was performed. It was then discovered that the patellar tendon with ossification throughout its length was avulsed from the tibial tubercle. The cerclage wire from the previous surgery was removed and ossifications were excised. Patellar tendon was fixed to the tibial tubercle with four suture anchors (Figure ). After three weeks of knee immobilization with a long leg cast, a gradually increasing range of knee motion rehabilitation was applied. Full weight-bearing was allowed after six weeks. At the end of the fifth postoperative year, the range of motion of his right knee was 90° flexion and full extension without any pain (Figure ).