A 65-year old male patient with a past medical history of chronic obstructive pulmonary disease (COPD), chronic alcoholic pancreatitis, and squamous cell carcinoma of the oropharynx status post-chemoradiotherapy in 2014 presented to the hospital due to abdominal pain and shortness of breath. His abdominal pain was in the epigastric area, sharp in nature, radiating to the back, associated with nausea, and constipation. The patient had no fever, chills, chest pain, cough, or vomiting.
On physical exam, blood pressure 117/60 mmHg, heart rate of 86 beat per minute, respiratory rate of 16 breaths per minute, temperature 36.6°C, oxygen saturation 95% on room air, the patient was alert and oriented. Heart examination showed a regular rate and rhythm with normal S1 and S2. Lung examination showed decreased breath sounds with dullness on percussion of the left side of the chest. Abdominal examination was significant for epigastric tenderness and hypoactive bowel sounds. Other systems’ examination was unremarkable.
Blood work showed leukocytosis with a WBC count of 14.1X 10E9/L, serum amylase of 2636 U/L, serum lipase of 1199 U/L, serum creatinine 0.68 mg/dl, BUN 13 mg/dL, AST 22 U/L, ALT 25 U/L, alkaline phosphatase 58 U/L, and total bilirubin 0.4 mg/dL. Chest X-ray showed large left sub-pulmonic pleural effusion (Fig ). The patient was started on IV hydration along with pain control and was not given food or water before the surgery. CT scans of the abdomen showed a dilated pancreatic duct and calcifications in the pancreas consistent with chronic pancreatitis. It also showed left pleural effusion and atelectasis of the left lower lung lobe (Fig ). The patient underwent thoracentesis and a chest tube placement that yielded 1 L of pleural fluid. According to Light's criteria, pleural fluid was transudative with significantly elevated amylase and lipase (pleural fluid amylase of 48,466U/L, lipase >33,000U/L, pH of 7.5 triglycerides <10 mg/dl, total protein 1 g/dL, LDH of 58U/L, serum LDH 259 U/L, serum total protein 5.3 g/dL) which raised suspicion for pancreatic pleural fistula. Magnetic resonance cholangiopancreatography was showed findings consistent with acute on chronic pancreatitis along with a complex left pleural effusion (Fig ), peritoneal fluid, and fluid in the anterior pararenal space. It was difficult to delineate the fistula between the pancreas and the pleura due to pancreatic obstruction at the level of the body of the pancreas due to a large stone. The patient then underwent ERCP with endoscopic ultrasound for further diagnosis and visualization of the pancreatic pleural fistula. A trial of stent placement in the pancreatic duct to obliterate the fistula and to decompress the pancreatic duct was conducted in order to allow the pancreatic secretions to follow the lower pressure pancreatic duct to the duodenum rather than to the pleural space. However, a large stone measuring 10 mm was noted in the pancreatic duct obstructing the view as the contrast couldn’t pass the stone; therefore, a stent was not able to be placed (Fig ). The pancreatic duct was 7.5 mm in diameter at the body distal to stone. The head of the pancreatic duct measured 5.7 mm, and the tail of the duct measured 6.2 mm. The surgery team was consulted to evaluate the patient for possible surgical intervention, recommending starting total parenteral nutrition with surgical intervention plan within a week. The patient tolerated total parenteral nutrition well. Repeated chest X-rays prior to removal of the chest tube showed re-accumulation of the pleural fluid. The patient's symptoms of shortness of breath, abdominal pain, and nausea improved throughout his hospitalization. However, due to the obstructing stone in the pancreatic duct that was visualized during the ERCP and the rapid reaccumulating pleural effusion, the patient underwent Frey procedure with open Roux-en-Y lateral pancreaticojejunostomy, splenectomy and cholecystectomy. In the operation, there was a fistula extending from the body of the pancreas to the left diaphragm that traveled posterior to the stomach and along the esophagus via the diaphragmatic hiatus. The fistula was ligated during the surgery. The patient tolerated the surgery well, the chest tube was removed, no more pleural fluid was accumulated. The patient was discharged home with a follow-up visit to receive his vaccination post-splenectomy.


A 42-year-old female with no significant past medical history presented to the ER with a chief complaint of AMS. On arrival, the patient was confused and agitated and required sedation with Lorazepam and Haloperidol. History was provided by the family at the bedside who stated that for the past three to four weeks, the patient had been experiencing generalized malaise, bruising, headaches, chest pain, and abdominal pain. On the day of admission, the patient had chest pain with shortness of breath (SOB). There were no reported fevers or sick contacts for the patient.
Our patient had no previous medical history, surgical history, and no family history of blood disorders. She was married and lived with her husband; the patient took no medications, did not smoke, drink alcohol, or do illicit drugs.
On arrival, the patient had normal temperature, blood pressure 130/77 mmHg, pulse 115 beats per minute, and 20 respirations per minute. On physical exam, she was very confused and agitated. Multiple ecchymoses were noted but the exam was otherwise negative; the neurological exam was non-focal. The patient was noted to have gross hematuria. Labs showed platelets of 13000, hemoglobin 9.9, white blood cells (WBC) 11200, LDH 1669, and bilirubin 2.8. Blood urea nitrogen (BUN) was 14 while creatinine was 0.9 and potassium 3.2. At this time, suspicion for TTP was very high and the patient was transferred to the ICU and started on daily TPE. To rule out infection, computed tomography (CT) head, blood cultures, and spinal tap were performed; all with negative results.
The next morning, the patient’s WBC was 8400, hemoglobin 8.4, platelets 8000, and bilirubin 5.6. The diagnosis at that point was beginning to look more like TTP than anything else. The patient’s overall condition didn’t improve dramatically with the first TPE treatment, but gross hematuria did resolve. ADAMTS13 levels were measured and found to be undetectable during this hospital admission. After recovering during the first hospital admission, the patient was switched to twice weekly TPE, followed by once weekly, and was finally tapered off completely.
The patient relapsed two years later, requiring hospitalization and treatment with TPE. She was also started on rituximab once weekly for a total of four weeks. The next relapse was the following year and was treated with TPE daily for six doses and discharged on 60 mg prednisone, which was tapered off eventually. Five months later, the patient relapsed again and was again treated with TPE for a total of five treatments and rituximab for a total of four treatments. After this treatment, the patient’s ADAMTS13 was detectable for the first time at 34%.
The most recent and last relapse for our patient was over a year ago, which was treated with five treatments of TPE and four of rituximab. After this treatment, her ADAMTS13 level was 46%. Of note, the patient’s ADAMTS13 levels were always undetectable at the times of relapse. Given the improvement after rituximab treatment and the rise in ADAMTS13 levels, it was hypothesized that the patient might benefit from maintenance rituximab therapy. Rituximab every two months was initiated and the patient has not had a relapse in over a year. The ADAMTS13 level rose to 60% earlier this year, only one month after starting the treatment; the most recent level was 65%. The patient has been symptom and relapse-free with no deficiencies in complete blood count (CBC) since starting maintenance rituximab; this to go along with a consistent rise in ADAMTS13 levels.


Our patient was a 77-year-old male with a past medical history significant for type-2 diabetes mellitus, dyslipidemia, and hypertension. He started developing weakness and atrophy, initially involving the musculature of the lower extremities followed by the upper extremities. He also reported a significant weight loss of 40 pounds from his baseline weight. The physical examination demonstrated a significant loss of muscle mass in all muscle groups of the upper and lower extremities. This patient did not exhibit symptoms involving bulbar muscles. The Medical Research Council Manual Muscle Testing Scale was utilized to grade muscle strength for different muscle groups, as listed: neck flexion 4, neck extension 5, arm abduction, extension, and flexion 4, elbow extension and flexion 4, wrist extension and flexion 3, fingers’ extension, flexion, and abduction 3, hip extension 4, hip flexion and abduction 3, knee extension and flexion 4, ankle dorsiflexion and plantarflexion 4. Muscle tone remained normal without any restricted range of motion. Deep tendon reflexes were decreased all over, with a value of 1+/5. Sensory perception was intact on both sides of the body. Initial laboratory workup showed an elevated creatine phosphokinase (CPK): 1,231 U/ L (reference range: 22 to 198 U/L) and myoglobin: 1,787 ng/ml (reference range: 0 to 85 ng/Ml). A detailed autoimmune workup performed showed negative serologic markers of antinuclear Ab, anti-Smith Ab, anti-U1 ribonucleoprotein Ab, anti-Jo-1 Ab, anti-Sjögren's-syndrome-related antigen A Ab, anti-Sjögren's-syndrome-related antigen B Ab, myeloperoxidase-anti-neutrophil cytoplasmic antibody, and proteinase anti-neutrophil cytoplasmic antibody. Thyroid stimulating hormone levels were within the normal range (2.3 mIU/L, reference range: 0.4-4.0 mIU/L).
A right quadriceps muscle biopsy was performed, which demonstrated necrosis and atrophy of muscle fibers and inflammatory infiltrate of eosinophils, plasma cells, and lymphocytes forming multinucleated giant cells consistent with granulomatous myositis (GrM) (Figure ).
This was followed by detailed laboratory and imaging studies to rule out the underlying cause for this GrM, which showed positive testing for elevated titers of AChR Ab (79.50 nmol/L, reference range: <0.02 nmol/L) and striational Ab (titer: 1:320, reference range < 1:120). Computed tomography of the chest did not show the presence of a thymoma. Repetitive nerve stimulation testing for the left ulnar nerve showed a decrement in the amplitude of muscle action potential by 13%, further confirming the diagnosis of non-thymoma-related MG concomitant with GrM. Other causes of GrM were also ruled out. For example, sarcoidosis was ruled out due to the patient’s normal serum levels of angiotensin-converting enzyme and 25-hydroxycholecalciferol levels with no mediastinal or cervical lymphadenopathy evident on chest imaging. Testing was negative for infection with syphilis and human immunodeficiency virus, which ruled out infection as a cause of myositis. Occult malignancy was also ruled out by a negative positron emission tomography scan for increased tracer uptake throughout the body.
The patient was started on treatment with 60 mg of oral prednisone for MG-associated GrM. We were successful in tapering down the dose of prednisone to 5 mg daily with the addition of mycophenolate mofetil 1000 mg twice a day as steroid-sparing medication. Therapy resulted in a moderate improvement in symptoms along with a descending trend in the levels of CPK and myoglobin.


A 50-year-old female presented to the emergency department (ED) with a chief complaint of chest pain. Although she had several risk factors for coronary artery disease, she stated she did not think this was cardiac in nature. Rather, she described the pain as cramping and burning in her chest. It had been going on for two weeks but increasing in intensity. She reported that the spasms worsened after ingesting liquids or solids. Her past medical history included thyroid cancer and gastroesophageal reflux disease (GERD). Surgical history included thyroidectomy and lap band placement several years prior.
She had an appointment for esophagogastroduodenoscopy (EGD) with her gastroenterologist in five days, but her symptoms became so severe so she came to the ED. She was on daily esomeprazole, which normally helped with her reflux but did not anymore. She reported globus, nausea, heartburn, and productive bilious nocturnal coughing but denied odynophagia, palpitations, shortness of breath, fever, chills, vomiting, abdominal pain, diarrhea, constipation, blood in stools, dysuria, or hematuria.
Laboratory analysis was unremarkable. Her vital signs were pulse 69, blood pressure 123/83 mmHg, temperature 36.5° C, respirations 17/min, and pulse oximetry 96%. An abdominal radiograph revealed that the lap band was in nearly horizontal in position, suggesting slippage had occurred (Figure ). The patient was admitted to the hospital for gastroenterology consultation and eventually got her lap band fixed.


A gentleman, 76 years old, was scheduled for catheter ablation of atrial fibrillation (AF) and atypical left atrial flutter in the context of symptomatic left ventricular dysfunction. He reported fatigue and exertional dyspnea, and presented persistent AF on EKG. He had a weight of 68 kg, and a height of 170 cm (BMI = 23 kg/m2), with high estimated thromboembolic risk (CHA2DS2VASc = 4). He was previously prescribed with anticoagulation (Dabigatran 110 mg bid), beta-blocker (bisoprolol 5 mg od), ACE-inhibitor (ramipril 5 mg od), diuretic (furosemide 50 mg) therapy. A 2D-echocardiogram documented left ventricle dilation (LVEDD (end diastolic diameter): 61 mm) with systolic dysfunction (EF (ejection fraction): 38%). A 2D-transesophageal echocardiogram (TEE) showed absence of images referable to atrial and auricular thrombosis. Single-lobe left appendage displayed reduced function with velocity peaks of 25 cm/sec. The left atrial area was 28 cm2. No relevant atherosclerotic plaques were found in the thoracic aorta. Written informed consent was obtained and the patient underwent radiofrequency electrical pulmonary veins isolation plus roof and mitral isthmus ablation lines during systemic intraprocedural heparinization (activation clotting time (ACT)-target: 300–350 s). Electrical cardioversion was also performed due to presence of persistent AF. The total procedural time was 180 min. Dabigatran was temporarily interrupted for 36 h across the procedure and the patient was discharged the next day. After one week he was admitted to the emergency department for sudden dyspnea, being hospitalized for acute heart failure. At admission the EKG showed sinus tachycardia, while chest X-ray depicted bilateral alveolar edema. During hospitalization, after achieving hemodynamic stabilization, the patient suffered aphasia and space-time disorientation with near loss of consciousness. The Angio-CT (computational tomography) showed hypodense lesions in the left cortico-subcortical temporo-occipital area and in the left cerebellar hemisphere as showed in . Carotid and vertebral arteries were free from hemodynamic atherosclerotic plaques. Symptoms completely disappeared after two days and at the 24 h CT scan control, the lesions were stable, in the absence of hemorrhagic transformation. After a few days, the patient complained left limb pain and an acute distal embolism was diagnosed. A new transthoracic echocardiogram revealed a further deterioration of left ventricular ejection fraction (EF: 30%) with no evidence of intraventricular thrombosis and a clearly discernable interatrial septal defect with left-to-right shunt, this was likely attributable to the trans-septal puncture performed during the ablation (). Ultrasonography of the groin region documented in situ not compressible left femoral vein thrombosis (). Non fractioned heparin infusion was administered with complete resolution of both the arterial embolic occlusion and venous thrombosis. After a few days, oral anticoagulation with apixaban was initiated and the patient was discharged. At the six months follow-up, he presented with mild cognitive impairment, which persisted overtime till the last visit.


A 24-year-old man was referred to our outpatient clinic due to ulcerations of palatal mucosa, nasal congestion and epistaxis two months ago. He had an episode of arthralgia that started one week ago.
Also, he had a history of unilateral blepharitis and upper eyelid edema five months ago (). On physical examination, strawberry-like gingival and labial hypertrophy, erosive and ulcerative lesions on the hard palate, a few ulcerative papulonodules on the right elbow, palmar purpuric papules, and nasal crusted hemorrhagic debris were detected () .
Eyelid edema had been resolved with a topical steroid. There was no history of constitutional symptoms. Past medical history and drug history were unremarkable. Laboratory work-ups were as follows: hemoglobin 11.8 g/dL, leukocyte count 8460 /µL with 5.6% eosinophils, erythrocyte sedimentation rate 47 mm/h, C-reactive protein (CRP) 2+ , blood urea nitrogen (BUN) 9 mg/dl (reference, 7-21), creatinin 1 mg/dl (reference, 0.7-1.4), serum immunoglobulin E (IgE) 2300 IU/mL (reference, up to 200), anti proteinase 3 (C-ANCA) 67.5 Au/mL (reference, positive: >18), hematuria [many red blood cells (RBCs) in urine analysis], dysmorphic RBCs in urine sediment, cloxacillin- sensitive staphylococcus aureus growth in nasal culture. Anti-myeloperoxidase (anti MPO or P-ANCA), anti-nuclear antibody, rheumatoid factor levels, liver and thyroid function tests were within normal limit. Stool exam and culture and labial Tzanck smear had no specific findings. Viral serology for human immunodeficiency virus, hepatitis B or C virus was negative. Labial and nasal mucosal biopsy showed focally ulcerated epithelium with marked spongiosis and irregular acanthosis associated with diffuse dense dermal infiltration of inflammatory cells mainly composed of neutrophils, admixed with large number of plasma cells and eosinophils as well as some lymphocytes, histiocytes and few multinucleated giant cells which formed vague granuloma. In addition, foci of vasculitis involving small-sized vessels as well as foci of RBC extravasation were evident (). Direct immunofluorescence study was negative. Also previous eyelid biopsy had been done by an ophthalmologist showed the same pathologic findings without any giant cell formation. Renal biopsy under ultrasound guide was done due to significant hematuria. Serial cortical renal tissue sections showed 23 glomeruli. All glomeruli had fairly normal appearance, except for mild mesangial hypercellularity in 2-3 glomeruli. Basement membranes were thin and regular without spikes and hole in PAS & Jones stains. There was no endocapillary proliferation, crescent formation or fibrinoid necrosis. Some tubules contained RBCs and rare RBC casts. Interstitium and vessels showed no inflammation. Direct immunofluorescence study was negative. Electron microscopic evaluation was not available. Chest and orbital computed tomography (CT) imaging and echocardiography were normal and paranasal sinuses CT scan showed mild mucosal thickening of maxillary sinuses. According to these findings, the diagnosis of GPA without significant renal involvement was proposed.
All mucosal and skin lesions and hematuria significantly improved using oral prednisolone 60 mg daily tapere dose during two months. Topical mupirocin and oral cloxacillin were prescribed for nasal carriage of staphylococcus aureus. Adjuvant therapy was not recommended for him after nephrologist consultation had been done due to non-significant involvement of kidney based on the biopsy. Mild microscopic hematuria was sustained after four months follow-up without proteinuria, dysmorphic RBCs or changing in BUN and creatinine levels.


Patient 1 is a 24 years old female who is not a known case of any medical illness. She was admitted for an elective laparoscopic sleeve gastrectomy with a BMI of 40.6 kg/m2 and a weight of 118.3 kg. The procedure was uneventful. Her post-operative course was uneventful and she was discharged home on the second post-operative day. No anticoagulants were prescribed upon discharge.
The patient was readmitted 17 days post-operatively complaining of upper abdominal pain worsening with diet and associated with nausea and vomiting. Her abdomen was soft and lax with minimal tenderness in the right upper quadrant. An abdominal ultrasound was performed that demonstrated portal vein thrombosis. The patient was treated with therapeutic doses of low molecular weight heparin. The patient improved symptomatically and enoxaparin was bridged with warfarin. The patient was subsequently discharged after achieving a therapeutic INR. The warfarin was continued for a total of 3 months. A repeat abdominal US repeated 6 months later and showed a fully patent portal vein.


Patient 2 is a 38 years old female who is not a known case of any medical illness. She has a history of a laparoscopic band insertion in 2012. The band was removed and the patient underwent a laparoscopic sleeve gastrectomy in 2014. Both these operations were undertaken in other centers and she failed to lose weight. She was admitted for an elective conversion from a sleeve gastrectomy to a minigastric (single anastomosis) bypass with a BMI of 36.05 kg/m2 and a weight of 90 kg. Intraoperatively, dense adhesions were noted between the stomach, liver and spleen. Bleeding was encountered as these adhesions were dissected. This was controlled with clip application. The operation was completed laparoscopically. Post-operatively, the patient’s repeat haemoglobin level on the first post-operative day was 7.9 g/dL dropping from 10.9 g/dL preoperatively. The patient was transfused 2 units of packed red blood cells and was discharged on the third post-operative day. Anticoagulation was not withheld.
The patient was readmitted 11 days post-operatively with complaints of left upper quadrant pain radiating to the left shoulder and neck associated with a fever measured at 38.5 °C. A CT abdomen & pelvis with IV and oral contrast showed thrombosis of the extrahepatic portal vein confluence, superior mesenteric and splenic veins. It also demonstrated a left subdiaphragmatic collection as well as an enlarged and ballooned spleen that was likely infarcted & liquefied. A radiologically guided drain was inserted and the collection drained leading to the resolution of the patient’s symptoms. She was subsequently discharged in good condition after achieving a therapeutic INR level. The warfarin was continued for 3 months. A repeat abdominal US performed 3 months after diagnosis of the portomesenteric venous thrombosis showed a patent portal vein measuring 1.1 cm in diameter.


Patient 3 is a 28 years old male who is a known case of diabetes mellitus. He was admitted for an elective laparoscopic sleeve gastrectomy with a BMI of 40.3 kg/m2 and a weight of 125.2 kg. The procedure was performed successfully with no intraoperative complications noted. The post-operative course was uneventful. The patient was subsequently discharged in good condition on the second post-operative day with no anticoagulation prescribed on discharge.
The patient was readmitted 9 days post-operatively. He presented to the emergency department complaining of recurrent colicky severe central abdominal pain radiating to both lumbar regions. This was associated with nausea but no vomiting, fever or changes in bowel habits. He was tolerating oral fluid diet well. Examination was unremarkable with a totally soft and lax abdomen with no area of guarding or tenderness. A CT scan of the abdomen & pelvis was performed with IV and oral contrast and showed complete thrombosis of the main portal vein, right and left portal veins, splenic vein and superior mesenteric vein. The patient was started on a therapeutic dose of enoxaparin that was bridged with warfarin. He was discharged in good condition after achieving a therapeutic INR. Follow up in the clinic shows that the patient’s symptoms have fully resolved and he is tolerating oral diet well with no abdominal pain, nausea or vomiting.


Following coronary artery bypass grafting, a 73-year-old man presented with symptoms of palpitation, headache, sweating, and blurred vision. His initial systolic blood pressure was over 240 mmHg. The patient's medical history included type 2 diabetes, dyslipidemia, coronary heart disease, prostate cancer and long-standing hypertension that used to be well-controlled with 2 medications; Metoprolol 25 mg twice daily and Valsartan 80 mg daily. However, his hypertension had been harder to control for the past 2 years. His family history was unremarkable except for cases of sudden death for his maternal uncle and aunts. No familial syndromes were noted. Patient was worked up for severe postoperative hypertension with a computed tomography (CT) scan which revealed a small right adrenal mass measured at 31 mm (). Biochemical testing included measurements of urinary fractionated metanephrines which revealed high normetanephrines with a value of 880 nmol/d (N < 240). However, metanephrines (148 nmol/d: N < 275), dopamine (936 nmol/d: N <2570), epinephrines (undetectable: N < 110), and norepinephrines (456 nmol/d: N < 440) were within the normal limits. Plasma free normetanephrines were 2.10 nmol/L (N <1.20) and free metanephrines 0.4 nmol/L (N < 0.48). The patient had normal calcitonin levels. A meta-iodobenzylguanidine (MIBG) scan showed intense and moderate uptake of a 2 × 2 × 3 cm mass of the right adrenal mass with no distant metastases. The patient underwent adrenalectomy and the diagnosis of PHEO was confirmed by histology post-operatively (). PHEO of the Adrenal gland Scaled Score (PASS) was 4, and the ki67 proliferation index was less than one percent.


A 37-year-old woman presented with twelve-month history of intermittent pain over the right upper quadrant of the abdomen and left iliac fossa. She also complained about distension of the abdomen.There was no history of nausea and vomiting. There was no significant medical, surgical history with normal obstetric history. Her menstrual cycles were normal with last menstrual period one week back. She was a farmer from a poor community with overcrowding and poor hygiene. She had a dog in her house for 5 years. On the examination, her vitals were within normal limits. On the examination of the abdomen, there was tenderness over the right hypochondrium with palpable mass. Similarly, there was nontender smooth palpable mass over left iliac fossa not moving with respiration. Her cardiovascular, respiratory, and neurological examinations revealed no abnormality. Her blood picture was within normal limits except raised eosinophil count (15%). Serological IgG antibody test ELISA for Echinococcus was raised (19.5 U/ml). Ultrasonography revealed well-defined hypoechoic heterogenous lesion involving the right lobe of the liver and complex multiloculated anechoic lesion involving left iliac fossa with internal echoes within the lesion suggestive of hydatid disease. CT scan of the abdomen revealed well-defined large complex heterogenous lesion measuring 10 × 8 cm with septations within in 8th lobe of the liver and well-defined lobulated lesion with septation measuring 7 × 6 cm in left iliac fossa suggestive of hydatid cyst (Figures and ). Intraoperative findings of laparotomy revealed multiple loculated hydatid cysts in the liver and left iliac fossa with multiple daughter cysts (). Pericystectomy and excision of hydatid cyst without spillage of content for the liver and left iliac fossa were done, respectively. Postoperative period was uneventful. She was discharged on the 10th postoperative day. She was followed up for twelve months with serial ultrasound abdomen and pelvis scans and clinical examinations.
The patient was treated with albendazole 400 mg two times a day for 3 months to prevent recurrence postoperatively. Patient's blood counts and liver enzymes were monitored during a two-week interval that is given after every month of postoperative albendazole therapy.


A 36-year-old Caucasian man developed end-stage renal disease (ESRD) secondary to recurrent acute kidney injury (from recurrent sepsis due to chronic hip methicillin-resistant staphylococcus aureus osteomyelitis) and contrast exposure. He was on dialysis for 9 years before receiving a deceased donor kidney transplant in February 2015. He had an episode of combined cellular and antibody-mediated rejection in October 2015 due to noncompliance with his immunosuppressive medications which was successfully treated with pulse steroids, plasmapheresis, intravenous immunoglobulin, and rituximab. After that, his baseline creatinine was around 1.5-1.7, last checked in December 2015. He did not follow-up until November 2017 with a creatinine of 2.3. In January 2018, he was admitted with severe nausea, dark-colored urine, and diarrhea. On physical examination, he had elevated blood pressure and 1+ bilateral lower extremity edema. There was no skin rash. Laboratory evaluation revealed acute kidney injury with a creatinine of 11 mg/dL (new baseline 2.3), BUN of 96 mg/dL, metabolic acidosis with bicarbonate of 11 mEq/L, potassium of 5.6 mEq/L, and subtherapeutic tacrolimus level of 1.6 ng/mL. Urinalysis and microscopy revealed dysmorphic RBCs and proteinuria without any evidence of infection. Patient admitted to being noncompliant with his immunosuppressant medication. Transplant ultrasound did not show any obstruction or vascular compromise. Due to worsening acidosis and hyperkalemia, hemodialysis was initiated. Transplant kidney biopsy was performed which revealed mesangial expansion, glomerular crescents (in 75% of the glomeruli on light microscopy sample), severe lymphoplasmacytic tubulointerstitial infiltration, glomerulitis, infiltration of the peritubular capillaries by inflammatory cells (peritubular capillaritis), mononuclear infiltrate along arterial intima (transplant arteritis), and subendothelial expansion and duplication along the glomerular basement membranes (transplant glomerulopathy; Figures , ). Immunofluorescence showed mesangial IgA (Figure ) and C3 deposition, positive C4d along peritubular capillaries. IgM, IgG, and C1q immunofluorescent stainings were negative. Ultrastructural evaluation revealed mesangial immune complex deposits associated with mesangial expansion. Immunohistochemical staining for SV 40 (BK Virus) was negative. The biopsy findings were consistent with crescentic IgA nephropathy along with both cell mediated as well as humoral rejection in the transplanted kidney. Donor-specific antibodies were positive in very high titers in our patient. Further glomerulonephritis workup revealed positive antinuclear antibodies, mildly depressed C3, normal C4 level and negative C-ANCA, P-ANCA, anti-GBM, cryoglobulins, HIV, hepatitis panel, and RPR test. Blood cultures, urine culture, EBV, CMV, adenovirus, BK virus, and influenza virus testing were all negative. There were no vegetations on echocardiogram.
Treatment was initiated with high-dose pulse steroids, thymoglobulin, intravenous immunoglobulin, and seven sessions of plasma exchange. Cyclophosphamide was not used. Despite these measures, the patient never recovered good allograft function and ended up on maintenance hemodialysis.


Patient is a 59-year-old lady who initially presented with bright red blood per rectum. She had a past history of Bartholin cyst removal years ago and subsequently has had rectovaginal fistula with multiple repairs. Worsening pain prompted more workup and imaging that showed the pelvic mass. In January 2017, MRI was done at Mayo Clinic which showed a polypoid mass in the lower rectum/anus with extension through the anterior wall of the rectum. It was associated with enlarged left external iliac lymph nodes and enhancing mass on the left side of the pelvis which extended into the sciatic notch. She had a CT-scan of the chest that revealed scattered bilateral sub centimeter pulmonary micro nodules which at the time were indeterminate. There was no prior dedicated prior chest imaging to compare the differences in some of these nodules.
Biopsy came back as poorly differentiated squamous cell cancer. After a multidisciplinary tumor board discussion and consensus, definitive chemoradiation with fluorouracil and mitomycin was pursued in February 2017 with excellent clinical response with improvement of pain/bleeding.
Scans in May 2017 marked shrinkage in anorectal mass, left pelvic sidewall mass and metastatic external iliac lymph nodes. There was no evidence of metastatic disease in the abdomen. However, enlargement in the previously observed lung nodules was noted. This was now amenable to a biopsy that showed metastatic poorly differentiated squamous cell carcinoma that was consistent with the patient's history of anal primary. There was normal expression of MLH1, MSH2, MSH6, and PMS2; and no expression of PD-L1 (less than 1% of viable tumor cells exhibit membrane staining at any intensity). She was started on doublet chemotherapy with carboplatin and paclitaxel in July 2017. She had initial response followed by later progression noted in February 2018. She was switched to single agent nivolumab in February 2018 which she took till June 2018. Subsequent scans in June 2018 showed significant progression of disease (multiple >20 nodules, growth of existing lung metastases measuring on average more than 2 cm as well as a new liver lesion as measuring 1.6 cm). At the time, given the significant growth and lack of clinical trials but still excellent performance status of the patient, modified DCF (mDCF) was chosen given the data published by Kim et al in Lancet Oncology on 2 July 2018. We repeated the imaging after 2 months of treatment in September 2018, which showed a near-complete ongoing response (>20 lung lesions now indiscernible; liver mass now less than 1 cm). The CT scans done pre-DCF therapy and those done post-DCF therapy are shown in Figures and . The dramatic response may be secondary to prior exposure to immunotherapy, which is increasingly being reported across multiple tumor types where the sequence of immunotherapy followed by chemotherapy is yielding greater than historical responses.


A 53-year-old woman presented with a painless pulsatile left neck mass. MRI study revealed a 2.7 × 3.1 × 3.9 cm well-circumscribed hypervascular mass at left carotid bifurcation. The mass was isointense in T1W (Figure A), heterogeneous hyperintense in T2W (Figure B), and intensely enhancing after gadolinium injection (Figure C). Flow-void sign with salt-and-pepper appearance was also noted (arrow). MR angiography demonstrated a tumor splaying at the carotid bifurcation (Figure D). The findings from the MRI support the diagnosis of a paraganglioma.


A 52-year-old man presented to our Emergency Department with sweats, lethargy and weakness 5 weeks following tissue aortic valve replacement with a 23 mm bioprosthetic valve. His background included severe aortic stenosis, mild left ventricular impairment, atrial fibrillation, hyperthyroidism, obstructive sleep apnoea and autistic spectrum disorder.
Following the original aortic valve replacement, the patient had made a good recovery, although he had been noted to be sweating at times. He maintained a normal white cell count and was apyrexial throughout, and was discharged home on postoperative day 6. Immediately following discharge, he did reattend via the Emergency Department with sweats and back pain, and was diagnosed with a lower respiratory tract infection.
Having re-presented with generalised weakness, lethargy and sweats, clinical examination was unremarkable and bloods demonstrated only a raised C-reactive protein (CRP) of 48. Trans-thoracic echocardiography demonstrated no evidence of endocarditis. Blood cultures taken on admission were initially reported as negative. However, during his admission the patient became pyrexial with worsening sweats and rigors. Urine culture, viral throat swab, CT scanning and dental review revealed no clear source of infection. There was no suggestion of immunocompromise or other risk factors for infection with this unusual pathogen.
Trans-oesophageal echocardiography on day 6 revealed infective endocarditis of the prosthetic aortic valve (see Fig. ). Antibiotic cover was broadened to IV vancomycin, IV gentamicin and oral metronidazole. Pyrexia and inflammatory markers improved following this. IV gentamicin was later stopped on microbiology advice. Four days following admission, one set of blood cultures grew a Gram-positive anaerobe coccus, later identified as Finegoldia magna on day ten. Antibiotic sensitivities were assessed using gradient strip diffusion and are shown in Table .
Intravenous antibiotic therapy was commenced. The decision was taken to resort to surgical intervention with replacement of the infected prosthetic valve. The patient underwent re-do surgery with removel of the infected valve and implantation of another bioprosthetic valve. Culture and sensitivities from the excised valve were negative.
After 19 days of IV vancomycin and oral metronidazole, vancomycin was stopped. Given the excellent oral bioavailability of metronidazole, the decision was taken to treat the anaerobic infective endocarditis with oral metronidazole monotherapy. The patient’s improving clinical condition, improving inflammatory markers and the achievement of source control through surgery supported the decision to switch to oral therapy.
The patient progressed well and was discharged on post-operative day 13 to complete a total 8-week course of oral metronidazole from the date of re-do surgery. Regular monitoring of CRP confirmed continuing improvement. He was discharged from follow-up eight weeks later, with final “test of cure” blood cultures reported as negative. No adverse effects following re-operation and prolonged antibiotic treatment were reported.


A 30-year-old male patient was referred with a diagnosis of an asymptomatic calcified mass located on the right side of the anterior maxilla, lasting 18 months. On the intraoral examination, a mucosal cutaneous fenestration with a mineralized tissue measuring approximately 1 cm in diameter was observed (). A periapical radiograph revealed an image exhibiting discrete radiopacity in the region of upper premolars (). The diagnostic hypothesis was maxillary exostosis.
Incisional biopsy was performed for the removal of the fragment, which then was placed in 10% neutral-buffered formalin and sent for histopathological analysis. The gross examination of the calcified specimen revealed a yellowish tumor with 4 × 4 × 5 mm.
The histopathological analysis revealed structures composed of enamel, dentin, pulp chamber, and cement in the same order of arrangement as that of a normal tooth. A mature tubular dentin and an enamel matrix were also observed ().
After the diagnostic confirmation of erupted peripheral odontoma, surgical removal was performed to reduce the lesion. The patient was free of symptoms after the procedure, and no complications were recorded.


The patient was a 17-year-old woman who complains of recurrent patellar instability in her left knee (5-7 episodes per year in the last three years) and in her right knee (two episodes). Physical examination revealed a full range of motion in both knees. The left knee had mild effusion and a positive apprehension test. On the right knee, no effusion or apprehension was noticed, but a pain in the medial retinaculum was found. The hip internal rotation was asymmetric, being greater in the left hip, so femoral malrotation was suspected. Tibial torsion, assessed by clinical thigh-foot angle, was normal. The preoperative patient-reported score was 19 on the Lysholm scale [] and level 1 on the Tegner scale [].
All patients with recurrent patellar instability are studied with full-length standing anteroposterior (AP) radiography, computed tomography (CT) of the lower extremities, and knee magnetic resonance imaging (MRI) in our institution. CT measurements are summarised in , and an increased femoral internal rotation was the main finding (). Femoral torsion was measured according to the technique described by Jarrett et al. [], with an expected value of 15°. Radiography shows mild symmetric valgus alignment in the coronal plane, a femoral-tibial mechanical angle of 1° of valgus, and a mechanical distal lateral femoral angle (mDLFA) of 86° on the left knee. Knee MRI shows a bilateral lesion of the MPFL and chondral type 2 damage in the lateral facet of the left patella. The trochlea was flat on both knees, which means that it corresponds to a type B according to Dejour's classification [].
According to the classification of patellar instability by Frosch and Schmeling [], the right extremity was a type 2: instability without malalignment as femoral torsion was below 25°. The authors use the upper limit to indicate a femoral derotational osteotomy according to the findings of Kaiser et al. []; therefore, a medial patellofemoral ligament (MPFL) reconstruction alone was planned. The left extremity was a type 3e (instability with femoral malrotation); therefore, a femoral derotational osteotomy was planned.
Our planning was estimating the perimeter of the femur at the level of the osteotomy. For the estimation of the perimeter, in an axial section of the CT scan at the level of the desired osteotomy, we measure the radius in the anteroposterior (2,98 cm) direction and in the midlateral direction (2,66 cm). With these two measurements, we obtained an average radius of 2,82 cm. Then, the perimeter calculation is based on the formula 2∗π∗radius; therefore, the perimeter was 17,7 cm. The perimeter accounted for 360°, so for 17° of correction, a rotation of 0.8 cm should be made.
Both knees were operated on during the same surgery. The first step was to perform the femoral osteotomy; for this, a lateral approach in the thigh was performed. The lateral cortex of the distal third of the femoral shaft was exposed. Two clamps were positioned on both sides of the desired location for the osteotomy, and the bone was marked parallel to the long axis. A transverse bone cut, parallel to the shaft axis, was performed in between the clamps and in the middle of the horizontal line with fluoroscopy guidance. Then, a retrograde femoral nail (T2, Stryker®) was used to fix the osteotomy. To begin, a proximal blocking screw was placed in the dynamic hole, then the distal end of the osteotomy was externally rotated, as planned, using the clamps to apply the force to achieve a distance of 0.8 cm between the horizontal lines at both ends of the osteotomy cut. Immediately after the derotational maneuver, two distal locking screws were placed in the distal end of the nail ().
After the osteotomy was fixed, both the gracilis and the semitendinosus tendons of the left knee were harvested. The left MPFL was reconstructed with the gracilis autograft; it was fixed with two anchors in the patellar side and a biodegradable screw in the femur (BIORCI®, Smith & Nephew).
In the right knee, the MPFL was reconstructed with the semitendinosus of the contralateral knee using the same fixation method described for the left knee. In both knees, MPFL femoral attachments were selected using fluoroscopy as described by Schöttle et al. [], and the grafts were fixed with 45° of knee flexion. In the left knee, while harvesting the hamstring tendon, an accidental partial lesion of the distal medial collateral ligament occurred and was repaired with an anchor.
After surgery, the patient did not present any complications. The rehabilitation protocol was weight bearing as tolerated, with two canes. Full knee extension was encouraged, and active flexion was limited to 60° for three weeks. After 10 weeks, she resumed walking without canes and gained full knee range of motion on the right knee and 100° in the left knee.
The eight-month postsurgery radiographs are shown in and are compared with the preoperative radiographs. It is remarkable how the patella looks medialised compared to the preoperative study (), and as planned, the coronal alignment was not changed () achieving a femoral-tibial mechanical angle after surgery of 0.51° of valgus. At the eight-month follow-up, the osteotomy was completely healed (Figures and ). At the 18-month follow-up, a CT scan was performed, and femoral torsion was 6.4° (). At the 24-month follow-up, the patient-reported outcome was 91 on the Lysholm scale [] and level 4 on the Tegner scale []. The patient had no episodes of patella dislocation or instability complaints after the surgery.


A 69-year-old Japanese man with a history of anal fistula presented with diarrhea, intermittent fever and wasting. He did not have any specific family history. He had moved to a Southeast Asian country 12 years earlier, and he started having persistent diarrhea 2 years later. He came back to Japan to visit an outside hospital and was diagnosed as IBS, because stool culture and colonoscopy were normal, and the parasite infection studies were negative. Four years later, his diarrhea worsened with occasional fever. These symptoms continued to worsen for the subsequent year, and his body weight decreased 6 kg over 6 months. Gastroscopy and colonoscopy were unremarkable. However, computed tomography (CT) study of the abdomen detected jejunal wall thickening. Therefore, he was admitted to our institution for further evaluations.
The patient’s vitals were within normal limits, and his physical examinations were benign. The initial laboratory data were only notable for albumin 3.4 g/dL and C-reactive protein 1.83 mg/dL, but the other routine blood cell count and biochemical investigations, including lactate dehydrogenase, were within normal reference ranges (Table ). Repeat CT of abdomen and pelvis with or without contrast in our institution revealed diffuse abnormal thickening of the jejunum and the ileum with enlarged regional lymph nodes at the mesenteries (Fig. a, b). Repeat gastroscopy and colonoscopy performed in our institution were normal and were the same as the ones at the outside hospital. Subsequent pathological results of the random biopsies from the esophagus to the second portion of duodenum and from the terminal ileum to the rectum showed nothing significant including lymphoid infiltration. However, SBE revealed ceaseless villous atrophy throughout most of his jejunum and ileum without any mass, ulceration or stenosis (Fig. a–d). Histopathology of the SBE biopsy specimens from the jejunum and the ileum showed loss of villous architecture and significant infiltration of small cells in the lamina propria and the submucosal layers (Fig. a). Interestingly, the biopsy specimens from the distal third portion of duodenum did not show any clear endoscopic findings of villous atrophy using SBE but still consisted of the same lymphocyte infiltrate (data not shown). Microscopy under high power magnification revealed that these infiltrating mononuclear cells had relatively low nuclear grade, and the presence of lymphocytes in the epithelial layer were mostly normal (Fig. b). Immunohistochemistry (IHC, Fig. a–c) and flow cytometry (data not shown) showed that majority of such lymphocytes expressed CD3, CD5 and CD7 accompanied by aberrant CD20 expression, but not other B cell or natural killer (NK) cell-markers. In addition, IHC also showed that less than 10% of infiltrating cells expressed Ki-67 (Fig. d). These cells consisted of CD4+, CD8−, CD30−, CD56−, and Granzyme B−. In situ hybridization did not show Epstein–Barr virus-encoded RNA (data not shown). Additional analysis showed elevated soluble IL-2 receptor 1,470 unit/L, but anti-human T lymphotropic virus 1 antibody (Ab) was not detected (Table ). Fluorodeoxyglucose-positron emission tomography (FDG-PET) detected diffuse small intestinal wall thickening and enlargement of mesenteric lymph nodes, but their maximal standardized uptake values were 1.4 and 2.8, respectively, which are considered as low as physiological uptake (Fig. ). In addition, a bone marrow examination showed no infiltration with atypical lymphocytes. Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) of the GI tract was originally diagnosed based on the World Health Organization (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues at the time [], and the findings were considered to be stage II-2 based on the Lugano classification.
Chemotherapy was thus initiated and consisted of pirarubicin, cyclophosphamide, vincristine and prednisolone (THP-COP). However, there was no response in either clinical or histological presentations even after 6 cycles of THP-COP. Afterward, rituximab, an anti-CD20 Ab, was also administered to the patient. However, the symptoms persisted despite this therapy. Subsequently, the WHO classification was revised in 2016–2017, and associated with this, a revised diagnosis of indolent T cell lymphoproliferative disorder (T-LPD) of the GI tract was made for the patient [–]. Serial sections of SBE biopsy specimens revealed that the majority of infiltrating T cells in the tissue were surprisingly CCR4 positive (Fig. ). Subsequently, mogamulizumab, an anti-CCR4 Ab, was administered to treat the patient, and all the patient’s symptoms including diarrhea and fever improved. To date, he is still followed at our institution, and he has remained in clinical remission for more than 2 years.


A 56-year-old woman presented complaining of severe right arm pain of approximately 18 months duration. She was having ulnar-distribution pain involving the medial forearm and extending into the hand (9/10 on a numerical rating scale). She noted declining strength of the right hand and movement-dependent hypoesthesias and paresthesias. She was otherwise healthy and without a family history of hereditary tumor syndromes.
A clinical examination revealed normal wrist and finger extension at Motor Research Council grade 5/5, wrist flexion 5/5, finger abduction 3/5, and thumb abduction and adduction 0/5. There was a pronounced claw deformity of the right hand with intrinsic atrophy.
An MRI of the cervical spine revealed foraminal stenosis at C6-7 and C7-T1 (not shown). Because of the pain in her arm, cervical decompression was recommended, and she underwent an anterior cervical discectomy. Postoperatively, her symptoms did not improve after discectomy, and she instead noted worsening pain. Electrodiagnostic studies demonstrated pronounced fibrillations, decreased voluntary activation, and minimal collateral reinnervation in the abductor digiti minimi and abductor pollicis brevis, consistent with injury to the lower trunk of the right brachial plexus. MRI (Figure -C) and computed tomography (CT) (Figure -E) scans of the right brachial plexus were interpreted as a tumor on the right supra- and infraclavicular brachial plexus that was weakly contrast-enhancing.
Surgical exploration for neurolysis and removal of the associated lipoma was recommended. Intraoperatively, the brachial plexus was embedded in relatively tight connective tissue with a typical lipoma posteriorly. The lipoma was resected, and the plexus was explored extensively, resulting in the removal of the tight connective tissue (Figure -B). At the conclusion of the case, the nerves were slack and widely decompressed but of notably firm consistency. Pathologic specimens showed a mass of mature, uniform adipocytes separated by dense fibrous septa (Figure ), compatible with lipoma. Small nerve fibers were identified in the fibrous septa with antibody staining to S100 (Figure ).
Immediately postoperatively, the patient reported decreasing pain and hypoesthesia. She also noted subjective improvement of the right-hand agility. Unfortunately, she had neurologic worsening a few months after surgery and declined further treatment and imaging.
Reinterpretation
Reevaluation of the MRI of the right brachial plexus demonstrated pathognomonic spaghetti-like fascicular enlargement and fatty displacement on the coronal plane and coaxial cables on the sagittal plane (Figure -C). The lipofibromatous involvement extended from the C5-T1 spinal nerves to the cord level, with the largest proliferation affecting the upper trunk. Imaging did not cover the whole affected extremity so the full distal extent of the LN is not known. Nerve territory overgrowth affected soft tissues and bone. Soft-tissue overgrowth included the extraneural lipoma contiguous to the brachial plexus, a fatty muscle infiltration in various muscles in the latissimus dorsi, subscapularis, and teres major, and the enlargement of the subcutaneous fat around the latissimus dorsi (Figure ). The cervical spine imaging demonstrated asymmetric hypertrophic bony changes of the neural foramen and a circumferential osseous canal of the vertebral artery (Figure ), which is atypical of fusion changes and predated the fusion by history. Other bony abnormalities noted included exostoses of the glenohumeral joint and overgrowth of the coracoid, acromion, and scapula. Although the patient’s hand clearly demonstrated atrophy and not distal hypertrophy, it is uncertain whether other muscles of the upper arm or forearm were involved.


A 57-year-old Egyptian male chef and avid eater of unpasteurized cheese presented with right upper quadrant abdominal pain in March 2018. White blood cell count (WBC) and liver function tests were normal. Ultrasound in the emergency department (ED) revealed a stone in a contracted gallbladder. Cholecystectomy was deferred due to uncertainty if the ultrasound findings were the true cause of his pain. Because of continued pain, he made repeated visits to the ED until November 2018 when he underwent laparoscopic cholecystectomy. Surgery was complicated by severing of an accessary hepatic artery and a bile leak. After an unsuccessful endoscopic retrograde cholangiopancreatography (ERCP) on postoperative day (POD) 2, he was transferred to an academic medical center due to concerns for common bile duct injury.
Upon transfer, ERCP demonstrated a truncated common bile duct, and an angiogram confirmed ligation of the right hepatic artery. White blood cell count was 9600, alkaline phosphatase (ALP) 69, alanine aminotransferase (ALT) 160, aspartate aminotransferase (AST) 79, and bilirubin (TBILI) 4.5. Blood cultures (BCs) obtained on POD 3 were negative. After stabilization and supportive care, he was discharged on POD 5 with plans for drain placement by interventional radiology and definitive surgery by hepatobiliary in 6 weeks to achieve sufficient collateral formation.
On POD 11, the patient developed fevers, abdominal pain, and jaundice. He was found to have a WBC of 27 000, ALP 255, ALT 432, AST 320, and TBILI 10.7. A computerized tomography scan of the abdomen showed a large biloma above the right lobe of the liver extending laterally, medially, and inferiorly. The biloma was aspirated percutaneously and drains were placed. Cultures from the aspiration and from 1 set of BC obtained at admission grew Brucella melitensis. A serum Brucella titer, obtained POD 18, was 1:80. He was initiated on doxycycline, and, because streptomycin is not available in the United States, he was given a short 1-week course of gentamicin [] to avoid renal toxicity while awaiting surgical correction.
On POD 35, he underwent biliary reconstruction with Roux-en-Y hepaticojejunostomy. Intraoperatively, he was noted to have abscess cavities as well as necrotic and purulent material in the gallbladder fossa. Cultures were negative. Postoperatively, he continued doxycycline for 6 weeks and was reinitiated on gentamicin to complete a 2-week course of gentamicin from the time of corrective surgery. His abdominal pain and dyspepsia completely resolved and he remains asymptomatic 4-months later.
Lack of suspicion for brucellosis led to laboratory exposure work-up of 25 employees. Six of seven high-risk exposures received antimicrobial postexposure prophylaxis (1 declined). Per guidelines [], all exposed workers were monitored serologically and for symptoms. None seroconverted or developed brucellosis.


A 67-y/o female (BMI: 51.19) with history of bilateral knee pain, diagnosed with osteoarthritis refractory to medical management, underwent a left total knee arthroplasty using a DePuy Synthes ATTUNE primary porous rotating-platform cruciate-retaining TKA system. Preoperatively, the patient has no instability and standing plain radiography showed a varus deformity with severe tricompartmental arthritis. The distal femur was prepared to accept a size 6 porous femoral component, and the proximal tibia was prepared to accept a size 5 cementless POROCOAT rotating-platform tibial base plate. A 5 mm size 6 tibial polyethylene articular component was placed, and the patella was prepared to accept a 35 mm all-polyethylene anatomic patellar implant. There was good ligament balance in flexion and extension as well as anatomic patellar tracking. Flexion and extension gaps were symmetric. She was discharged without complication.
Radiographs at 6 weeks post-op showed well-aligned TKA (Figures and ). Left knee range of motion was 0–100 degrees, with no flexion/extension mismatch and normal patellar tracking. The patient did report that she had more pain from the current TKA compared to a previous right TKA. Five months postoperatively, the patient reported progressive ambulatory pain. Knee range of motion had decreased to 10-60 degrees. Radiographs showed anterior dislocation of the femoral implant on the tibial polyethylene component (Figures and ). Open reduction verse revision of all components was planned at this time. Intraoperatively, there were no signs of infection, and after arthrotomy, we noted that the tibial polyethylene articular component was found to have rotated 180 degrees, with the anterior aspect of the polyethylene facing the posterior aspect of the knee. The polyethylene component was removed. At this time, it was noted that the posterior cruciate ligament had been avulsed off the posterior tibial plateau. The intercondylar notch of the distal femur and posterior aspect of the knee was then debrided. The polyethylene was replaced with the same size component, 5 mm height size 6 rotating-platform polyethylene. It was placed in an appropriate position and was noted to have good range of motion, ligament balance, and anatomic patellar tracking. The patient was, again, discharged without complication.


We report a 28-year-old HIV-negative Ugandan woman with no known chronic illness. She presented to the eye clinic at Mulago National Referral Hospital (Kampala, Uganda) with a 2-week history of sudden onset blurring of vision in both eyes, especially in the mornings. This was preceded by a 1-week history of low-grade intermittent fevers, sore throat, running nose, and a nonproductive cough. These symptoms were attributed to a viral upper respiratory tract infection that was managed conservatively. She denied any history of headache, neck stiffness, floaters, excessive tearing, or reddening of the eyes but reported itching of both eyes. There was no history of chronic eye disease, trauma, or previous surgery on the eyes. There was a family history of blindness, which affected one of her four paternal uncles and his son who died under unclear circumstances. Notably, both relatives developed sudden loss of vision and had vitiligo-like skin patches.
During this visit, eye examination revealed a best-corrected visual acuity (BCVA) of 6/36 in both eyes and intraocular pressures (IOP) of 10 mmHg and 11 mmHg in the left and right eye, respectively. On slit lamp examination, pigmented keratic precipitates (KPs), posterior synechiae, and vitreous inflammatory cells were noted. She was managed for an acute uveitis for which she was prescribed Maxitrol® eye drops (active ingredients being dexamethasone, neomycin, and polymyxin B) with minimal improvement after 2 weeks of treatment.
Two weeks later, she developed a sudden onset frontal headache which was throbbing in character and associated with reddening, tearing, and pain involving both eyes. She also reported transient episodes of visual loss but denied any history of neck pain, photophobia, phonophobia, loss of consciousness, or convulsions. During this time, she also noticed impaired hearing in the right ear with bilateral tinnitus, especially in quiet places. There was no associated discharge, dizziness, vertigo, nausea, or vomiting. She was initiated on oral prednisolone 10 mg daily for one week with reported improvement in the symptoms. Her vision improved bilaterally though it remained blurred with floaters. Hearing also improved bilaterally, but the tinnitus persisted.
Five weeks after the onset of the symptoms, she developed an intensely itchy and painful papular rash, which was generalised. This healed after a week but left some hypopigmentation around the face and trunk. There was also associated whitening of her scalp hair, eyebrows, and lashes. However, there was no hair loss/balding.
On clinical examination, she had multiple, “chalk-white”, sharply demarcated, nonscaly patches on both sides of the forehead and upper back, discrete scattered macules on the upper and lower limbs, and the torso with intact sensation. Blanching was observed in these patches. Patches of white hair involving the scalp hair, eyebrows, and eyelashes were also noted. She had no evidence of hair loss, and her fingernails and toenails were normal (). The thyroid gland was normal, and she was clinically euthyroid. Other systemic examination was unremarkable. Her vitals were within normal ranges: temperature = 36.7°C, BP = 120/80 mmHg, PR = 79 bpm, RR = 16 bpm, and SPO2 97% on ambient air.
Eight weeks after the onset of her disease, ocular examination revealed a BCVA of 6/36 in both eyes (same as previous), with IOP of 11 mmHg bilaterally. She had normal eyelids, with patches of white eyelashes. Her conjunctivae were normal. Keratic precipitates were noted on the endothelium of the cornea in both eyes. She had posterior synechiae, and the lenses were clear in both eyes. The inflammatory cells and flares were observed in the vitreous. Fundoscopy revealed a “sunset glow fundus”, serous retinal detachment, blurring of the optic disc margin, papillitis, and normal retinal vessels bilaterally (). However, inflammatory cells (“snow balls”) were observed in the vitreous of the left eye.
A diagnosis of VKH syndrome was considered, as possibly a complete VKH syndrome in the chronic stage. Full blood count, erythrocyte sedimentation rate, antinuclear antibody tests, and chest radiograph were normal. The Mantoux test and a venereal disease research laboratory test were all negative. Oral prednisolone was restarted at a dose of 15 mg daily for two weeks in addition to oral azathioprine at a dose of 50 mg daily for one month and possible refills for 6 months. She received counselling regarding her disease and on the irreversibility of her vitiligo and poliosis, which were of concern to her. She continues to attend follow-up clinic visits.


Patient 1 was a 60-year old man, without any past medical history, admitted in the emergency room of a nearby hospital in September 2012 for progressively increasing headaches, fever and confusion. The CT scan revealed no parenchymal abnormality. The cerebrospinal fluid examination showed 400 white blood cells/mm3 (95% lymphocytes), with high protein amount (2.4 g/L), normoglycorachia, and xanthochromia.


A 52-year-old Caucasian man, who had undergone renal transplantation 12 years ago, was admitted in the month of January with a five-day history of progressively worsening non-productive cough, nasal congestion, sore throat, and fever. His past history was significant for polycystic kidney disease, systolic heart failure, diabetes mellitus, hypertension, and morbid obesity. Home medications included prednisone, tacrolimus, mycophenolate mofetil (MMF), warfarin, insulin glargine, aspirin, furosemide, and carvedilol.
On admission, he had a temperature of 38.7 degrees centigrade, a pulse of 103 beats per minute, blood pressure of 166/91 millimeters of mercury and oxygen saturation of 96% breathing ambient air. Laboratory data revealed a white blood cell count of 8,500 cells/mm3 (78% Neutrophils, 8% Lymphocytes, 12.5% Monocytes), hemoglobin of 13.5 g/dL, platelet count of 167,000 cells/mm3, creatinine of 1.4 mg/dL, blood urea nitrogen of 20 mg/dL, and tacrolimus level of 8.8 ng/mL. Chest roentgenogram (CXR) revealed a right lower lobe infiltrate. Intravenous ceftriaxone and azithromycin were initiated for presumed community-acquired pneumonia. A respiratory pathogen multiplex polymerase chain reaction (PCR) (BioFire, Salt Lake City, USA) performed on a nasopharyngeal swab was positive for Adenovirus. MMF was discontinued, and the tacrolimus dose was reduced by 25% to target a trough level of 5 ng/mL. One dose of intravenous immunoglobulin (IVIg) was administered on day three (0.4 g/kg body weight). On day four, he had persistent fevers, worsening transaminitis, and developed acute hypoxemic respiratory failure requiring mechanical ventilation. CXR revealed increased consolidation in the right lower lobe as well as new left retrocardiac opacity. The serum creatinine worsened to 2.43 mg/dL. Adenovirus was isolated from an endotracheal aspirate by shell vial culture, and plasma PCR for Adenovirus DNA revealed a viral load of 188,000 copies/mL (limit of quantitation- 190 copies/ml). Rests of the diagnostic tests were negative for bacterial, fungal, other viral, or any non-infectious etiologies.
Treatment was started with one dose of intravenous cidofovir (5 mg/kg body weight) on hospital day five to treat severe adenoviral pneumonia. On day eight, he was started on 100 mg of oral brincidofovir, which was administered through the nasogastric tube at twice a week dosing. Brincidofovir was acquired from Chimerex through U.S. Food and Drug Administration (FDA) authorization under the Emergency Investigational New Drug program, and it was administered in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. During the rest of the hospital course, he received six more doses of brincidofovir. The patient clinically improved and was extubated on day 19. Treatment was discontinued on day 26 after full clinical recovery, and two consecutive plasma adenovirus PCR assays one week apart were negative. Renal function improved, with serum creatinine returning to baseline range by day 26. He was transferred to a rehabilitation facility on day 39 and was placed back on his admission immunosuppressive medications. The patient remained well on sixty-day follow up. Diagnostic tests, plasma viral loads, and treatment are summarized in Figure .


A 66-year-old Asian female patient, who was a housewife, visited a local clinic for a routine screening colonoscopy in mid-January 2018. During the colonoscopy, a submucosal tumor sized about 4cm was identified at the sigmoid colon (
) and biopsy was performed. The microscopic exam of the biopsied specimen showed an ulcerated lesion with a proliferation of fibroblast-like spindle cells beneath the ulcer, which was insufficient for a definite diagnosis.
The patient was referred to our hospital at the end of January 2018. She presented no specific symptom and physical examination showed no specific finding. She had a history of hypertension and a benign breast mass. She had a positive family history of cancer: her father had gastric cancer, and her uncle had lung cancer.
An abdominopelvic computerized tomography (CT) scan revealed 4.4cm sized well-circumscribed, well-enhanced, round-shaped mass in the sigmoid colon, which was slightly heterogenous inside. No intraabdominal metastasis was identified (
). Chest CT scan showed no intrathoracic metastasis. Carcinoembryonic antigen (CEA) level was 1.33ng/mL, which was within a normal range (0.0–5.0ng/mL). Other laboratory test results were also within normal ranges. Differential diagnosis was 1) gastrointestinal stromal tumor (GIST); and 2) neuroendocrine tumor (NET).
The patient underwent a laparoscopic anterior resection. On laparoscopic exploration, an extruding mass was identified at the anterior wall of the sigmoid colon and no metastasis was observed. The sigmoid colon was mobilized and the inferior mesenteric artery was low ligated. Sigmoid colon resection with end-to-end anastomosis was performed.
On examining the resected specimen, about 4.5 × 4.0cm sized round mass was observed on the surface of the serosa and there was no tumor infiltration to the serosa (
). The tumor was located 7cm from the proximal resection margin and 4cm from the distal resection margin. On sections after fixation, the cut surface showed a yellowish mass (4.2×3.2cm), which was abutting on the circumferential resection margin. The mass was relatively well-demarcated without encapsulation (
). On hematoxylin and eosin (H&E) stain, the tumor was composed of spindle cells with low nuclear atypia, with nuclear palisading growth pattern, and lymphoid cuffing surrounding tumor cells were identified (
). Mitosis was rarely observed (1/50 in high-power field). The remaining mucosa and serosa were grossly unremarkable. The resection margins were free from tumor. Lymph node metastasis was zero in 13 regional lymph nodes. On immunohistochemical analysis, s-100 was strongly positive in tumor cells; otherwise, c-kit, CD34, and SMA were negative (
). Finally, the diagnosis was a benign schwannoma of the sigmoid colon.
The patient recovered from surgery uneventfully and was discharged on postoperative day 5. When she visited the out-patient clinic two weeks after discharge, she did not present any complication. No postoperative adjuvant therapy was performed.


A 59-year-old woman with a past medical history of chronic dysphagia following PEG tube replacement due to thyroid surgery complications approximately 20 years ago presented to the clinic for the establishment of primary care and evaluation for chronic dysphagia as she wished to eat food naturally. She recently moved from Jamaica, where the PEG tube was initially placed soon after the thyroid surgery. Since then, she reported that she has been on PEG tube feeding due to the inability to resume oral feeding. Interestingly, she reported replacing the PEG tube herself through the original track whenever she experienced a tube clog. She reported chewing her food, then pushing it through the tube with a syringe. On examination, we noted a well-healed scar at the base of her neck. The PEG tube was located at the left upper abdomen with no signs of inflammation or drainage at the peristomal area (Figure ).
We also noted a laparotomy scar from a previous surgery for intestinal obstruction. The other physical examination findings were not remarkable. Her laboratory evaluations including complete blood count, chemistry panel, and lipid panel with normal findings; the viral serology for Hepatitis B and C were negative. Ultrasonography of the abdomen revealed no significant findings. Recent esophagogastroduodenoscopy revealed scarring in the hypopharynx as well as single diverticulum in the cricopharyngeus without further advancement of the scope (Figure ).
Subsequently, a diatrizoate swallow study revealed the inability of the passage of contrast out of hypopharynx to the esophagus. She was ultimately counseled to depend on PEG feeding as she was maintaining her nutritional status with PEG feeding, and there were no better alternatives considering the potential worse consequences of surgery.


A 37 year old female patient, a psychiatric nurse, was recommended at 19 years to the outpatient facility specializing in eating disorders (ED). Her anorexia nervosa symptoms appeared at 13 years, and were diagnosed at her first hospitalization after a collapse with seizures (glycemia 2.5) when she achieved her least weight (37 kg/173 cm, BMI 12.36). She has a history of long-term repeated vomiting, diets, meat rejection, vegetarianism, abuse of laxatives, drinking of some bleach, and excessive exercise. Always skeptical about the treatment, she expressed the feeling she can live with the disease because without it she would lose her self-image. During the years of illness, she experienced various psychotherapy and pharmacotherapy (venlafaxin, hypnogen, seropram, prothazin, rivotril, prothiaden, fevarin), and had been hospitalized as an inpatient at the day care of a specialized ED center. When she reached 60 kg/173 cm (BMI 20.05), she felt "fat as a pig", and focused only on weight loss and gradually increasing somatic difficulties. Since 2016, the patient was treated for dyspepsia and malnutrition. Dysmicrobia with dyspeptic syndrome, mycotic upper esophagitis, hooked stomach, stomach evacuation disorder, and duodenogastric reflux were indicated. At that time, galactosemia and SIBO were diagnosed by an immunohistochemical examination of jejujan mucosa samples and jejunal fluid aspirate cultivation. SIBO was manifested by a wide range of symptoms: Bloating, diarrhea, constipation, abdominal pain, and abdominal cramps. Despite various antibiotic treatment regimes, and a low saccharide diet, only mild and temporary symptom alleviation was achieved. Due to the persistent symptomatology which limited the patient in daily activities, and considering the negative impact of the SIBO symptomatology, as well as the negative impact of the low saccharide diet on AN, the FMT was applied (approved by the Ethical Committee of the University Hospital for severe gastrointestinal disorders). There were no known contraindications for this procedure. During the study, the authors monitored her psychiatric symptoms, family dynamics, and specific symptoms with eating disorder examination questionnaire (EDEQ).


A 72-year-old male nonsmoker presented to the emergency department for a 1-month history of intermittent perirectal bleeding and progressive lower limb weakness. His medical history was of diabetes complicated by stage 4 chronic kidney disease (CKD), hypertension, and hyperlipidemia.
Examination revealed prominent bruising over the left flank, a hematoma over the right deltoid, and ecchymoses over the left inner arm, in conjunction with severe anemia (Hb 3.7 g/dL) and a prolonged activated partial thromboplastin time (APTT) (78.6 s), which was not fully correctible on 50% mixing studies (59 s). FVIII levels were low (<1%) and significant levels of FVIII inhibitor were detectable (82 Bethesda Units), establishing the diagnosis of acquired hemophilia A. Platelet and von Willebrand factor (VWF) levels were not deficient (480 × 10
9
/L and >400%, respectively). A malignancy screen with computed tomography (CT) imaging and autoimmune markers were negative.
Additionally, the patient's left calf was noted to be swollen and tense and suspicion of a concurrent DVT was raised.
An urgent abdominal ultrasound examination revealed a retroperitoneal collection, suggestive of a hematoma. A CT scan localized the hematoma to the right psoas muscle with significant subcutaneous flank edema, likely attributable to bleeding (
); the scan also detected significant thrombosis along the patient's left femoral vein (
). The patient was treated with 90 mcg/kg/dose of rFVIIa (NovoSeven), and immunosuppression with prednisolone (1 mg/kg/d) and cyclophosphamide (100 mg/d) was promptly commenced.
Extensive DVT involving the left common and superficial femoral veins and extending up to the left external and common iliac veins was subsequently confirmed with a Doppler ultrasound scan. Once acute bleeding had stabilized, inferior vena cava (IVC) filter insertion was performed on day 5 of admission, with periprocedural administration of rFVIIa. Due to technical difficulties, femoral venous access could not be secured, and the procedure was performed via the right internal jugular vein (IJV). The patient had mild oozing from the IJV insertion site and continued to receive rFVIIa till oozing had completely resolved. Approximately 24 hours after discontinuation of rFVIIa however, progressive bruising was noted over the IJV puncture site, and concerningly, the patient developed acute dysphonia. Urgent endoscopic examination discovered bruising and swelling over the right tongue base and aryepiglottic fold, suggesting extrinsic compression. Imaging revealed an expanding hematoma at the IJV puncture site with associated tracheal deviation (
). The patient was intubated expectantly and received regular rFVIIa doses, with monitoring in intensive care. Administration of rFVIIa was discontinued after 3 days when the neck bruising demonstrated clinical improvement and endoscopy confirmed resolution of airway compression, and extubation was successfully performed 24 hours later.
Despite reduction in inhibitor levels and slight recovery of FVIII levels with prednisolone and cyclophosphamide (
), the patient continued to have intermittent melena and developed a new trapezius hematoma with associated fall in serum hemoglobin, requiring packed cell and rescue rFVIIa administration. Endoscopy did not reveal any gastrointestinal lesions. He was commenced on second-line therapy with Rituximab (375 mg/m
2
per week in four doses), which resulted in gradual normalization of FVIII levels (
) and resolution of active hemorrhage.
At 8 weeks of therapy, prednisolone was tapered, and he was started on low-dose dose enoxaparin (40 mg/d; dose adjusted for stage III–IV renal impairment and increased risk of bleeding). Repeat Doppler ultrasonography of the left leg at 3 weeks following initiation of anticoagulation demonstrated complete resolution of the DVT. Enoxaparin was continued with the intention to complete 12 weeks of anticoagulation, or longer if the patient remained immobile, as immobility was identified as the primary prothrombotic risk factor. However, anticoagulation had to be discontinued after 8 weeks due to acute clinical deterioration and subsequent demise of the patient from a severe hospital-acquired pneumonia. Notably, there was no clinical recurrence of DVT or derangement of APTT values to suggest recurrence of his hemophilia.


A 68-year-old male, heavy smoker with poorly controlled diabetes mellitus type 2 and hypertension, presented to the emergency department with severe left flank pain for a few days. This pain was associated with moderate lower urinary tract symptoms, progressive left lower limb edema and moderate abdominal pain. Patient denies fever, chills, vomiting, diarrhea and hematuria.
His past medical history was unremarkable except for poorly controlled diabetes (last hemoglobin A1C of 13% (normal: 4% and 5.5%) and hypertension. Physical examination revealed a diffusely red, painful and grossly swollen left lower extremity, moderate left costovertebral angle tenderness. Digital rectal examination reveals a large, non-tender, firm prostate gland with no nodular lesions. The scrotal exam was normal.
Laboratory examination revealed white blood cells (WBC) count of 9000/mm3, C-reactive protein of 1 mg/L, random blood sugar 420 mg/dl and creatinine 3.8 mg/dl (baseline was 1.5 mg/dl). Urine analysis showed 1–2 WBC per high-power field, other blood investigations were all within their normal range. Chest X-ray was normal.
Computed tomography (CT) scan of abdomen and pelvis with Per os contrast showed a partially necrotic and lobulated retroperitoneal mass extending along para-caval and para-aortic regions on both sides till the pelvis and enlarged prostate gland measured 60 g (). The left kidney shows severe hydronephrosis.
The patient was admitted to the hospital. A left double J stent was placed. PSA level was >2000 ng/ml. Core needle biopsy of the retroperitoneal mass showed thick fibrous tissue with metastatic high grade and poorly differentiated clusters of neoplastic cells consistent with carcinoma. Immunohistochemical staining reveals strongly positive results for anti-PSA (). These findings were consistent with metastatic adenocarcinoma of the prostate. Prostate biopsy was performed and it showed prostate adenocarcinoma, Gleason grade 8 (5 + 3) ().
Venous ultrasound was performed, it showed deep venous thrombosis encompassing the left distal external iliac, common femoral, femoral, popliteal and posterior tibial veins. Appropriate anticoagulation was initiated.
A bone-scan demonstrated diffusely increased uptake of sternum, lumbar spine and pelvis. Degarelix 240 mg was given initially and then 80 mg/month. The patient was offered docetaxel chemotherapy but he declined. The clinical and radiologic follow-up with abdominopelvic CT scan for 3 months shows no mass progression.


A 70-year-old woman was shown to have a mass, 10 mm in diameter, in segment Ⅷ of the liver during a follow-up evaluation for PBC. Her social and family histories were unremarkable. She had chronic rheumatoid arthritis in addition to PBC, and she was prescribed oral steroids and methotrexate. She had no abnormal physical findings.
Laboratory testing was negative for hepatic virus, and hepatic function was in the normal range, although the antimitochondrial antibody titer was positive. Tumor markers, including carcinoembryonic antigen, carbohydrate antigen 19-9, alpha-fetoprotein (AFP), and protein induced by vitamin K absence or antagonist Ⅱ(des-gamma-carboxy prothrombin [PIVKA-Ⅱ]), were within normal limits. In addition, the ICG 15 min value was 9.8% and the Child-Pugh classification was A at 5 points (Table ).
Abdominal ultrasonography showed a hypoechoic mass, 13.4 mm in diameter, in segment Ⅷ of the liver (Figure ). An abdominal computed tomography (CT) scan showed a mass, 10 mm in diameter, which was slightly enhanced in the early phase and washed out in the late phase (Figure ). On gadoxetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI), the mass was enhanced in the arterial dominant phase and washed out in the late and hepatocyte phases (Figure ).
Based on the preoperative diagnosis of HCC, a laparoscopic-assisted S8 subsegmentectomy was planned. The operation began with laparoscopic right liver mobilization with the camera port and three ports under the right brow arch, followed by right subcostal oblique incision for hepatic resection.
A gray-white solid tumor with a maximum diameter of 9 mm was observed macroscopically (Figure ). The tumor in the liver consisted of a dense lymphocytic infiltration, including multiple lymphoid follicles with germinal centers, microscopically. The interfollicular areas were expanded and filled with small-to-medium lymphocytes without cellular atypia (Figure ).
Immunohistochemical staining revealed that the follicles were CD20 (+), CD79a (+), CD10 (+), and Bcl-2 (−), and the interfollicular area was CD3 (+) and CD5 (+). Taken together, a diagnosis of pseudolymphoma was favored (Figure ).
The postoperative course was good, and she was discharged on postoperative day 7.


A 34-year-old man with dizziness, dyspnea, and inability to swallow referred to the hospital emergency department. The patient presented with dizziness, followed by dyspnea, a gradual feeling of dryness in the throat, an impaired reflex, slow papillary reaction to light, a decreased eye movement, a decreased power of facial muscles and limbs, normal deep tendon reflexes (DTR), lack of focal neurologic signs, and respiratory failure. The patient reported the use of clay cheese in the week before referring to the emergency. In the laboratory evaluation, hematological and biochemical tests such as complete blood cell counts, and also serum level of sodium, potassium, blood sugar, BUN, and creatinine were normal. electrocardiogram, magnetic resonance imaging, and chest X-ray were also reported as normal. The patient was under supportive therapy, respiratory care, cardiac monitoring, and pulse oximetry after transferring to the intensive care unit (ICU).
Based on the clinical suspicion to botulism, diagnostic evaluations, including the evaluation of serum samples, emission, and gastric juice, were done to confirm the BT. The patient’s history showed the use of cabbage salad, vegetables, and clay cheese in the last week. Thus samples of these foods were evaluated regarding the BT. The patient was treated with three doses of antitoxin. Neurology consultation was done a day after hospitalization. After three days, the patient was transferred to the infectious diseases ward. After 11 days and resolving of ptosis, the power of organs reached the normal state (5/5), and the gag reflex was decreased; consequently, he was discharged with medication orders. Four days later, the BT test in blood and cheese samples came positive (toxin B) while it was negative in other food samples.
The patients’ wife also referred to the hospital with asthma and a feeling of dryness in the throat, followed by the inability to swallow, feeling of vomiting, and trouble speaking for four days. During admission to the emergency, the patient was conscious without a fever but, with dysarthria and bilateral ptosis, an impaired gag reflex, slow right papillary reaction to light, a decreased eye movement, a decreased power of facial muscles and limbs, normal DTR, and lack of focal neurologic signs and respiratory failure. This patient underwent supportive therapy and antitoxin prescription. In addition to these two patients, seven other members of the family referred to the hospital with the same signs of botulism to be treated.


This case presents a 44-year-old Hispanic female, with no known past medical history, complaining of an acute onset of intractable hiccups, vomiting, and dysphagia. Physical examination was notable for a thin female appearing much younger than stated age. Cardiac and pulmonary examinations were unremarkable. Skin thickening of the fingers extending proximal to the metacarpophalangeal joints was noted bilaterally. Review of systems was positive for Reynaud’s phenomenon, however negative for finger ulceration, nail pitting, dry eyes or mucus membranes, or skin rash. Vital signs on admission were stable. Laboratory studies were unremarkable except for hyponatremia and hypokalemia, which were replaced appropriately. Patient received supportive IV fluids and anti-emetics. The following day, the patient developed new onset of blurry vision, motor weakness and paresthesias of the bilateral lower limbs. Lower extremities were notable for hypertonia and paroxysmal tonic spasms. Motor strength of lower extremities was 0/5 with intact bilateral upper extremity strength. Upper extremity reflexes were 2+, and knee and ankle reflexes were 4+ bilaterally. Babinski’s sign was present bilaterally. Loss of pinprick and vibratory sensation up to bilateral anterior superior iliac spine was noted. Visual acuity diminished bilaterally with no visual field defects, and extra ocular movement was intact. Other cranial nerves were intact. Brain MRI with and without gadolinium enhancement showed multiple scattered hyper-intense T2 flair foci in the bilateral periventricular white matter. MRI spine revealed multiple enhancing lesions in the spinal cord at the levels of C2–C6, consistent with transverse myelitis.
Extensive laboratory evaluation, which included thyroid function, vitamin B12 level, and folic acid was normal. Serologies and PCRs for common neurotropic viruses (HSV1, HSV2, CMV, EBV, HBV, HCV, VZV, HTLV 1/2, Toxoplasma, and HIV) were negative in blood and in cerebrospinal fluid analysis (CSF). Specifically, CSF analysis revealed lymphocytic pleocytosis but no oligoclonal bands. Immunological tests for anti-dsDNA, anti-cardiolipin, anti-β2GPI, ANCA, and cryoglobulins were negative. However, ANA, anti-RNA polymerase III autoantibodies, Scl-70, and NMO-IgG were all positive. Repeat NMO-IgG was positive. Effectively, preliminary diagnosis of NMO was made based on seropositivity and clinical features consistent with transverse myelitis and area postrema syndrome (see Table ). Our case of NMO diagnosis is based on the revised consensus criteria in 2015, shown in Table .
The patient was started on high-dose intravenous methylprednisolone (1 g daily for five consecutive days) on day. Resolution of visual symptoms occurred within 2 days. Nonetheless, the patient showed marginal improvement in motor symptoms. On day 5 of hospitalization, patient developed abrupt onset of malignant hypertension, as well as acute onset of renal failure with non-nephrotic range proteinuria, requiring urgent hemodialysis. Renal biopsy showed obliteration of the vascular lumen, with concentric fibrinoid necrosis and “onion-skin” hypertrophy, most consistent with diagnosis of steroid induced scleroderma renal crisis. Steroids were discontinued and patient was started on an ACE inhibitor with resultant normalization of blood pressure and slow recovery of renal function. Patient was started on Azathioprine with improvement of motor symptoms; however, it was discontinued due to hepatotoxity. Effectively, it was changed to Rituximab 1000 mg × 2 doses 14 days apart, with gradual improvement in motor functions.
In sum, our patient was diagnosed with both NMO and Systemic Sclerosis based on her history, clinical presentation, MRI findings as shown in Fig. , anti-Scl-70 antibodies, and the presence of serum NMO-IgG antibody. She had a subacute onset of lower extremity paraparesis, dysesthesia, and hyperreflexia, with gradual improvement in motor function over the course of a year. In the interim, she had multiple readmissions for painful paroxysmal tonic spasms which responded to Gabapentin, Baclofen and Carbamazepine.


A 35-year-old female came to the surgical out-patient department of our hospital with chief complaints of intermittent pain in the periumbilical region and right iliac fossa for one year. Pain was not associated with other complaints or menstruation. There was a history of infertility and no other significant past medical history. The physical examination was unremarkable. The routine blood investigations were normal. USG abdomen and pelvis revealed well-defined nodular lesion (1.5 × 1.3 × 1.6 cm) in right iliac fossa, and mild hypoechoic circumferential wall thickening in the terminal ileum and ileo-caecal junction (3–5 mm thick). Contrast enhanced computed tomography (CT) of abdomen revealed well-defined long tubular structure (11 mm in luminal diameter) with lobulated tip along the postero-medial wall of caecum, mild pericaecal stranding and thickened right lateral conal fascia – suggesting of acute appendicitis (). Additionally, multiple cysts were present in both the ovaries. In view of above findings, patient was planned for laparoscopic appendectomy after obtaining written informed consent.
Intra-operatively, there was presence of a 2 cm tumor-like mass at the tip of appendix involving the mesoappendix, while base of the appendix was normal. There was no ascites, peritoneal or omental deposits or any signs of inﬂammation. The entire appendix measuring 2.5 × 2 × 5 cm was dissected and excised (). Caecum and terminal ileal loops were unremarkable. The operative time was 60 minutes with blood loss of 20 ml. The postoperative recovery was uneventful with the hospital stay of 3 days. Gross examination found a firm white nodule measuring 1.5 cm in diameter located at the tip of the appendix. Microscopic examination revealed the presence of endometrial glands, stroma with no signs of inflammation suggestive of appendicular endometriosis. Till the last follow up at 3 months, the patient was symptom-free.


21-year-old male with history of left sided testicular swelling with increased alfa fetoprotein levels (10,789 IU/ml) and beta-human chorionic gonadotropin levels (66.05 mIU/ml) with normal chest radiograph, underwent left high inguinal orchidectomy in December 2016 in the outside hospital. Histopathology report of the specimen was suggestive of “Mixed seminomatous and non seminomatous germ cell tumour with yolk sac tumour (40%), mature teratoma (55%) and seminoma (5%)”. Positron emitted tomography was done 1 month later post orchidectomy suggestive of “Diffuse metabolic activity seen in the left hemi-scrotum is secondary to recent surgery. There is no metabolically active lymph node, pleural, peritoneal, omental, solid visceral, osseous lesion, ascites or pleural effusion. Isolated non metabolic nodule in the left upper lobe and left lower lobe of the lung would need serial follow up”.
Patient was asymptomatic for next 9 months and didn’t come for the regular follow-ups until he developed chest pain, breathlessness and severe back pain ().
Computed tomography of the chest done in September 2017 suggestive of “Area of collapse-consolidation in the periphery of apical-posterior segment of left upper lobe of lung and another well-defined oval radiopacity with sharp borders is noted in superior segment of lower lobe of left lung with left hydropneumothorax- nonspecific morphology probably of benign aetiology”.
Patient was managed with intercostal drainage tube insertion. Tumour markers were done showed Alpha fetoprotein levels (1606 IU/ml) and beta-human chorionic gonadotropin levels (5.40 mIU/ml). The pleural fluid sample was sent for cytology showed no malignant cells. subsequent radiographs were taken which confirmed the resolving pattern. Patient was discharged within 5 days after removal of the intercostal drainage tube.
Within 2 months patient again presented with severe respiratory complaints and repeat computed tomography of chest was done in December 2017 showed “subpleural nodule in left lower lobe of lung is suggestive of metastasis. This study is negative for any significant mediastinal adenopathy, interstitial lung disease or centrilobular opacities with shallow left hydro-pneumothorax”. Patient was again managed with intercostal drainage tube and pleural fluid sent for cytology was negative for any malignant cells.
Patient was again hospitalised 2 times in 15 days with similar complaints for which pleurodesis was also attempted. But in view of recurrent pleural effusions which were not responding to medical management, patient was then subjected to 4 cycles of chemotherapy with Bleomycin, Etoposide and Cisplatin for a period of 2 months. Within a week after completion of chemotherapy patient was again admitted with severe respiratory complaints. The whole-body positron emitted tomography was repeated in February 2018 showed “Appearance of the multiloculated lesion in the pleural space on the left side is a new finding compared to the prior scan and should assumed to be metastatic unless proved otherwise with fluid aspiration cytology or biopsy of the solid component in a nodule. No retroperitoneal adenopathy seen” ().
Patient was subjected to salvage chemotherapy with Etoposide, Carboplatin and Ifosphamide in a period of March to May 2018. Pleural fluid aspiration under sonography guidance was done which again failed to show any malignant cells. ().
Computed Tomography of chest at the end of 5th chemotherapy cycle done, suggestive of “Considerable increased in shift of cardio-mediastinal structures. The rest of the morphological appearance of the loculated/septated left pleural effusion remains more or less unchanged with no significant mediastinal adenopathy or pulmonary metastasis.” ()
Patient was then referred to surgical team for further management. In view of worsening of the symptoms and sequential chest x-rays showing no improvement patient was subjected to “Left Mini-Thoracotomy with complete decortication of the diseased pleura” in July 2018. Histopathology report of this pleural sample was suggestive of “Differentiated teratoma- post chemotherapy pleural metastasis”.
After the procedure sequential chest x-rays were taken each day which were showing resolving pattern of consolidation and pleural effusion with good lung expansion (). Patient is under regular follow up for last 9 months. Patient does not have any complaints, also is gaining weight, normal values of alfa fetoprotein levels (5.88 IU/ml) and beta-human chorionic gonadotropin levels (less than 2 mIU/ml). Computed tomography was done in December 2018 showed “Considerable regression in the loculated/septated left pleural effusion with considerable expansion of left lower lobe of lung and lingula compared to previous scan. No significant mediastinal adenopathy, pulmonary or pleural metastasis and no new lesion is seen” ().


A 66-year-old male patient was examined in July 2018 due to painful swelling with increased consistency in the left paramedian sternum at the level of the manubrium. In November 2018 a chest-abdominal computed tomography documented an infiltrating neoformation of the manubrium of the sternum of 54 × 40 mm (A–C), and an 8 mm neoformation in the left kidney. A biopsy of the kidney confirmed the malignancy of the mass, and a left nephrectomy was performed. In December 2018, due to the increasing of size of the sternal mass (A and B) and more pain, an echo-guided needle biopsy confirmed the metastasis of clear cell renal carcinoma. Firstly, the patient underwent 30 Gy transcutaneous radiotherapy. After radiation, 18F FGD PET-CT showed a hypermetabolic positivity only at the level of the sternal mass and an increased size (108 × 80 × 90 mm). Due to persistent pain, a multidisciplinary team recommended surgery to remove the sterno-costo-clavicular metastasis.