A 66-year-old male was admitted to the ICU with complaints of chronic weakness, fatigue, myalgia, weight loss, and left flank pain. Comorbidities included hypertension, active smoking, and diabetes mellitus. Patient denied nausea, vomiting, diarrhea, hematemesis, melena, hematochezia, chest pain, or any shortness of breath. He had a recent admission due to community acquired pneumonia and was treated with antibiotics. He denied using any illicit drugs or alcohol abuse. He was retired truck driver by profession.
On examination, he was an elderly man with clinical signs of dehydration. Blood pressure was 155/56 mm Hg, heart rate was 67/min, respiratory rate was 16/min, was afebrile, and his oxygen saturation was 99% on room air. He had mild epigastric tenderness without guarding or rigidity. He had bilateral lower extremities chronic skin changes with scaling. The rest of the physical examination was normal.
Significant laboratory data revealed serum sodium 124 mEq/mL, potassium 5.1 mEq/mL, chloride 89 mEq/mL, bicarbonate 15 mEq/mL, blood urea nitrogen 44 mg/dL, creatinine 1.5 mg/dL, glucose 85 mg/dL, calcium 10.3 mg/dL, total protein 7.3 g/dL, and albumin 2.9 g/dL. The rest of the liver function tests were within normal limit. Anemia was with hemoglobin 9.4 g/dL, hematocrit 27%, platelets 558 k/μL, and white blood cells 10.4 k/μL. Arterial blood gas analysis showed pH of 7.36, pCO2 of 25 mm Hg, pO2 of 90.3 mm Hg, and oxygen saturation of 97% on room air. There was an anion gap of 20, lactic acid of 4.8 mmoles/L, serum lipase of 1540 U/L, amylase 406 unit/L, gamma GT 25 unit/L, INR 1.3, and lactate dehydrogenase (LDH) 950 unit/L; cancer antigen CA 19-9 was 49.9 μ/mL, TSH was 2.25 mIU/L, serum acetone was negative, and alcohol level was less than 10 mg/dL. His serial cardiac enzymes were negative and he was ruled out for an acute coronary event.
Chest X-ray (CXR) revealed bilateral patchy airspace disease. CT of abdomen and pelvis without contrast showed diffuse enlargement of the pancreas with homogeneous attenuation and without significant peripancreatic inflammation () with extensive retroperitoneal and pelvic adenopathy (). A para-aortic lymph node measuring 2.1 cm and retrocaval lymph node measuring 1.6 cm were seen. A soft tissue density surrounding the right common iliac vessels likely representing confluent adenopathy was seen. His echocardiogram showed an ejection fraction of 67%; right ventricle systolic pressure was estimated to be 44 mm Hg. Pericardium was normal without any pericardial effusion. There was no significant valvular abnormality.
Initially, the patients EKG showed 2nd degree Mobitz type I block which later progressed to complete heart block. Patient was given atropine without improvement; hence, a temporary pacemaker was inserted. He was started on IV hydration and IV antibiotics and all his electrolyte abnormalities were corrected. His temporary pacemaker lead came out and it was removed. Subsequently he became unresponsive, was orally intubated, had four cardiac arrests, and was successfully resuscitated. Due to persistent pulmonary infiltrates he underwent fiberoptic bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsies which showed evidence of pneumocystis jiroveci pneumonia (PJP). He was started on Bactrim for the treatment of PJP. He tested positive for HIV and was found to have a CD 4 count of 12/uL.
A temporary pacemaker wire was again placed after ruling out the relevant reversible causes of third degree heart block. A repeat echocardiogram was unchanged from prior one. CT-guided biopsy of the para-aortic lymph nodes was consistent with diffuse large B-cell lymphoma with marked tumor necrosis (). Immunoprofile showed that CD 20 (), CD 79a, and CD 10 were positive. CD 43, CD 3, CD 7, and BcL were negative. Upper endoscopy for percutaneous endoscopic gastrostomy placement showed gastric polyps which revealed gastric lymphoma on biopsy.
Patient was not a candidate for chemotherapy or ART due to critical condition. Hospital course was complicated by acute respiratory distress syndrome, acute kidney injury, and eventual death a month into his hospital stay.


A previously healthy 44-year-old woman presented to the emergency room with early satiety, abdominal bloating, and epigastric discomfort that had started about a week ago. A right upper quadrant ultrasound was done followed by a CT of the abdomen and pelvis. The CT scan showed a partially visualized large pericardial effusion. There was no lymphadenopathy. She then underwent an echocardiogram that showed a hyperdynamic left ventricle systolic function with large circumferential pericardial effusion with tamponade physiology. She was taken to the cath lab where a pericardiocentesis was performed and a total of 730 cc of sanguineous fluid was drained and a pericardial drain was placed. The pericardial fluid contained 65,000 WBCs with the differential showing 6% polys, 1% lymphocytes, 1% monocyte, and 92% highly atypical lymphocytes with moderate basophilic cytoplasm, rare punched-out vacuoles. Most of the large atypical cells had a vesicular nuclear chromatin, some with large nucleolus and marginated chromatin. A hematoxylin and eosin (H&E) stained cell block preparation is shown in . This in addition shows numerous apoptotic debris and histiocytes with ingested debris. In tissue sections of BL, the histiocytes and apoptotic debris constitute the starry-sky appearance. Similar findings were seen in this cell block preparation, providing morphological evidence of a highly proliferative lymphoma. Flow cytometry further confirmed the presence of a large B cell population comprising 94% of lymphoid gated events. They were kappa light chain restricted (strong expression of light chains) and were CD19 and CD20 positive. They aberrantly expressed CD10. They did not express CD5 and CD23. FISH was positive for the t(8;14). Overall, the diagnosis was consistent with BL. Diagnosing BL is a challenging process, especially in bodily fluids. However, as shown before [], the presence of atypical lymphocytes with characteristic morphologic features and confirmatory flow cytometry is very helpful in establishing the diagnosis. FISH for t(8;14) is virtually confirmatory of BL.
For staging she underwent a bone marrow biopsy that was negative for lymphomatous involvement and a CT of the torso that showed a decreased, now small pericardial effusion, stable small bilateral pleural effusions, a 2.6 cm lobulated soft-tissue lesion in the right atrium, and no evidence of lymphadenopathy. A cardiac MRI revealed on T1- and T2-weighted images a broad base, isointense structure measuring 3 cm × 1 cm arising from the lateral wall adjacent to the atrioventricular junction of the right atrium (). Connected to this broad based mass, there was an isodense, circular, well-circumscribed mass measuring 1 cm in diameter protruding from the broad based mass into the right atrium. Late gadolinium enhancement images showed heterogeneous enhancement of the broad base mass and no enhancement of the circular mass. Her LDH at presentation was normal and an HIV test was negative. A lumbar puncture revealed no evidence of lymphoma cells in the CSF. The patient was treated with four alternating cycles of modified CODOX-M and IVAC in combination with rituximab [] and achieved a complete remission. A follow-up cardiac MRI showed no evidence of the right atrial mass. She remains in remission to this date, more than 5 years since her initial presentation.


A three-year-old boy presented to the emergency room with fever and decreased level of consciousness. Two days earlier, he had been admitted to another hospital with a presumed diagnosis of enteritis, and ceftriaxone therapy had been administered. A physical examination revealed fever, weakness, and nuchal rigidity. His medical history showed that he had been vaccinated four times with 13-valent pneumococcal conjugate vaccine (PCV-13) and that his father had been treated for meningitis when he was seven years old. A laboratory investigation revealed the following: a peripheric blood leukocyte count of 52.000/mm3, an absolute neutrophil count of 48.000/mm3, a platelet count of 413.000/mm3, and a C-reactive protein (CRP) level of 214 mg/L (normal range: 0–5 mg/L). Furthermore, the results of the CSF examination showed 200 polymorphonuclear leukocytes/mm3, an elevated protein concentration of 214 mg/dL, and a low glucose level of 1 mg/dL along with a simultaneous blood glucose level of 126 mg/dL. However, the CSF pyogenic culture and blood cultures remained sterile, and his cranial magnetic resonance imaging (MRI) was also normal. Ceftriaxone and vancomycin were administered for 14 days because his meningitis had only been partially treated with antibiotics before his admission. He was then discharged after full clinical and laboratory improvement.
Ten days after being discharged, the patient was readmitted with fever and neck stiffness, and he underwent another CSF examination that revealed 10 leukocytes/mm3, an elevated protein level of 209 mg/dL, and a low glucose level of 0 mg/dL. Additionally, his white blood cell (WBC) count was 27.200/mm3, and he had an absolute neutrophil count of 23.500/mm3 along with a CRP level of 91 mg/L. Moreover, the CSF polymerase chain reaction (PCR) test for M. tuberculosis was negative. Treatment with ceftriaxone and vancomycin was then restarted. The CSF culture revealed Streptococcus pneumoniae (serotype 19F) which was susceptible to both ceftriaxone and vancomycin, and a repeated lumbar puncture showed a cell count of 10 leukocytes/mm3, a CSF protein concentration of 41 mg/dL, a CSF glucose level of 35 mg/dL, and a simultaneous blood glucose level of 85 mg/dL, but the CSF pyogenic culture was sterile. Hence, the antibiotic therapy was given to the patient for four weeks.
Cranial and temporal bone imagings were performed to determine the predisposing conditions for RBM, and temporal bone CT showed decreased aeration in the right middle ear and mastoid cells, a dysmorphic structure of the semicircular canals, vestibular dilatation, and the cystic appearance of the cochlea, all of which are consistent with Mondini dysplasia (Figures and ). Furthermore, an audiometric screening was consistent with severe sensorineural hearing loss in the right ear and normal hearing in the left ear.
Surgical repairment of the dysplastic inner ear was planned, but it could not be performed because of parental refusal.


In 2002, a 52-year-old asian woman underwent a lumpectomy for a stage II infiltrating duct carcinoma over her right breast, followed by four cycles of adjuvant chemotherapy combining doxorubicin, cyclophosphamide and paclitaxel. Chemotherapy was followed by locoregional radiation. Eight years after the completion of chemotherapy, she found a slowly growing mass on her right neck. Positron emission tomography (PET) scan revealed large infraclavicular mass and a supraclavicular adenopathy of the right neck and right nasal cavity. Analysis of biopsy samples from both masses was consistent with metastatic carcinoma and suggestive of the metastasis having arisen from primary breast cancer. She underwent removal of the right supraclavicular and infraclavicular masses at that time, followed by adjuvant chemotherapy. However, one year after the surgery, she found a mass in the upper outer quadrant of her right anterior chest wall. Ulceration and bleeding of the mass caused her to have a painful disability of her right upper limb. A computed tomography (CT) scan (Figure a) revealed a mass in the upper outer quadrant of her right anterior chest wall. The mass had invaded the ribs and pleura. Magnetic resonance imaging (MRI, Figure b) revealed that the mass involved the axillary artery and occluded the right lateral subclavian vein and axillary vein.
After a multimodality assessment followed by discussion of the available options and risks, benefits and subsequent rehabilitation associated with forequarter amputation with the patient and her family, she consented to the amputation. Surgery revealed a fungating tumor in the anterior superior chest wall that involved the right first, second and third ribs. This tumor had adhered to the right lung as well as the brachiocephalic-subclavian vein and artery.
During surgery, the patient was placed in a semi-lateral position on her left side with her right side elevated at a 45° angle. An incision was made over her sternocleidomastoid muscle, about 3 cm above where the muscle attaches to the clavicle. The incision was directed posteriorly around the posterior triangle of the neck, around the scapula, under the axilla(including an extension along the latissimus dorsi muscle to allow for proper flaps) and then anteriorly around the mass and across the chest wall. Subcutaneous dissection was done along the anterior border of the manubrium and the right first, second and third ribs. For the scapulectomy, an incision was made in the patient’s back, and the teres major and latissimus dorsi were dissected to obtain negative margins. The latissimus dorsi muscle was completely transected from the chest wall and scapula and a flap was created under the teres major. The intercostal continuous dissection was carried up to the neck. The platysma muscle was divided, and the sternocleidomastoid muscle identified and divided to identify the jugular vein, carotid artery and vagus nerve. Once the neck musculature was divided and the carotid sheath contents isolated, the posterior scalene muscle was clearly identified. Then the posterior scalene muscles were divided sequentially while the brachial plexus branches were identified. At that time, the phrenic nerve was traced and dissected from the tumor, and the brachiocephalic vein and internal jugular vein that had adhered to the tumor were ligated. The remaining attachments of the structures to the tumor and arm were dissected, including the subclavian artery and first ribs. Then the chest wall was covered and reconstructed with the free forearm flap. Histological analysis of the metastatic mass showed features of primary breast cancer of intracytoplasmic mucin and glandular formation in hematoxylin and eosin staining (Figure a). Immunohistochemical staining revealed apositive finding of mammaglobin (Figure b), gross cystic disease fluid protein 15 (Figure c) and P63 (Figure d).
The patient experienced no wound complications during follow-up after surgery. To date, she has been followed up on for three years after surgery and has shown no signs of malignancy (Figure ).


A 66-year-old female was admitted to Kagawa University Hospital (Kagawa, Japan) with the complaint of intermittent epigastric pain that was exacerbated by fasting. The patient had a history of hypertension and obstructive sleep apnea syndrome. Physical examination upon admission revealed no anemia (via conjunctival pallor examination), jaundice or pulmonary abnormalities. On palpation, the abdomen of the patient was soft and flat, with no areas of tenderness. Furthermore, pretibial edema was not observed and superficial lymph nodes were not palpable. Serum concentrations of the tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, were within the normal ranges (<5 ng/ml and 0–37 U/ml, respectively). However, X-ray examination indicated reduced gastric distension, as well as deformation of the stomach, which exhibited a leather bottle-like appearance (). In addition, the lower gastric body demonstrated luminal narrowing and increased rigidity, with a depressed lesion (longest diameter, 20 mm) at the posterior wall of the gastric antrum and abdominal computed tomography revealed thickening of the antrum. No lymphadenopathy was observed. Additionally, endoscopy revealed an ulcerative lesion covered by a white necrotic substance on the posterior wall of the antrum () and severe luminal narrowing, with poor distension of the lower gastric body. The upper gastric body, however, demonstrated good extension when compared with the middle and lower gastric bodies. The folds of the gastric antrum were flexible, stretched smoothly, and crossed one another, resulting in a waffle-like appearance on the greater curvature of the upper gastric body (). Analysis of biopsy specimens from the ulcerative lesion revealed a poorly differentiated adenocarcinoma containing signet ring cells, however, adenocarinoma was absent from biopsy specimens obtained from the abnormally crossed folds. Due to the morphological changes that occured in the gastric folds, creating the waffle-like appearance, it was determined that cancer cell invasion of the upper gastric body was likely, and a total gastrectomy was performed. The resected specimen revealed the wall thickening and crossing folds of the gastric body () that were previously observed by endoscopy. Microscopic examination revealed that cancer cells had spread throughout the upper gastric body and had infiltrated the vessels in the submucosa, predominately into the muscularis propria, and marginally into the serosa (). Immunohistochemical examination revealed positive staining for MUC5AC and MUC6 (gastric marker mucins) and negative staining for MUC2 and CD10 (intestinal marker mucins), indicating gastric-type mucin expression. The final diagnosis, according to the Japanese Classification of Gastric Carcinoma (), was T4aN3aM0, clinical stage IIIC advanced gastric cancer. The patient was discharged 17 days after surgery without complications and commenced three cycles of S-1 adjuvant chemotherapy (80 mg/day, days 1–28) for 16 weeks. Following three courses of chemotherapy for 16 weeks, treatment was terminated due to patient fatigue. The patient has survived and is without disease recurrance 14 months after surgery.


A 73-year-old female was admitted to the First Affiliated Hospital of Nanchang University (Nanchang, China) with a one-month history of upper abdominal distension and discomfort on September 5, 2003. In addition, the patient had a history of diabetes mellitus and hypertension. A computed tomography (CT) scan demonstrated extensive thickening of the mucosa and a mass on the posterior wall of the stomach that was indicative of gastric lymphoma. Gastroduodenoscopy (GIF-2T240; Olympus Corporation, Tokyo, Japan) and biopsy identified the mass to be a malignant gastric lymphoma (), and the initial laboratory analyses revealed the following data (normal ranges are provided in parentheses): Cancer antigen 125, 109 kIU/l (0–35 kIU/l); white blood cell count, 3.7×109/l (4–10×109/l); red blood cell count (RBC), 3.06×1012/l (3.5–6×1012/l); and lymphocyte percentage, 15.4% (18–58.4%). The patient received six courses of chemotherapy as follows: 375 mg/m2 rituximab on the first day; then a CHOP chemotherapy regimen consisting of 750 mg/m2 cyclophosphamide (CTX), 1.5 mg/m2 vincristine (VCR), 60 mg/m2 Pharmorubicin on day 1 and 30 mg prednisone on days 1–5, in total each course of chemotherapy lasted for three weeks, for a total of six courses. This was then followed by daily radiotherapy at 1.8 Gy for 20 days.
The patient was admitted to the hospital for a second time on November 25, 2010. The patient received a gastroscopical examination and was identified to be positive for Helicobacter pylori using the rapid urease test method, indicating that an ulcer was present on the fundus of the stomach (); the ulcer was confirmed by performing a biopsy (). Furthermore, a CT scan revealed a small hypointensity in the middle of the chest, close to the right lung, as well as an enlarged spleen. Thus, the patient was discharged and received treatment for H. pylori infection and the ulcer (20 mg omeprazole twice daily, 1000 mg amoxicillin twice daily, 500 mg clarithromycin twice daily and 1000 mg bismuth citrate four times daily, for 8 weeks).
The patient was admitted to the hospital for a third time on June 1, 2011. Gastroscopical examination identified a probable ulcer, with gastric lymphoma, on the fundus of the stomach that was determined by biopsy to be a chronic superficial inflammation of the fundus. The patient was negative for H. pylori and the ulcer was in the same position as in the previous examination. A CT scan demonstrated that the wall of the antrum was thickened and nodules consistent with pulmonary emphysema were present in the middle of the right lung. The patient was discharged and once again received treatment for the ulcer (orally, with 20 mg omeprazole, twice-daily, for 8 weeks).
The patient was admitted to the hospital for a fourth time on May 28, 2012. Gastroscopical examination indicated the presence of an ulcer, with gastric lymphoma, on the fundus of the stomach () that was confirmed by performing a biopsy (). Furthermore, a CT scan demonstrated a thickened gastric wall and nodules in the middle of the right lung. The patient was again discharged and received additional anti-ulcer treatment (orally, with 20 mg omeprazole, twice-daily, for 8 weeks).
The patient was admitted to the hospital for a fifth time on April 7, 2013. Gastroscopical examination and biopsy identified an ulcer on the fundus of the stomach, with gastric lymphoma, and a CT scan demonstrated a thickened gastric wall that was similar to the previous examination and infection of the right lung. Once more, the patient was discharged and received anti-ulcer treatment (orally, with 20 mg omeprazole, twice-daily, for 8 weeks).


A 77-year-old female was admitted to the First Affiliated Hospital of Nanchang University on October 22, 2009, reporting a six-month history of upper abdominal distension, discomfort and intermittent diarrhea. Furthermore, the patient had a history of Parkinson’s disease, cholecystectomy, diabetes mellitus and hypertension. A CT scan demonstrated irregular thickening and stiffness in the wall of the antrum, as well as partial intrahepatic metastasis and bilateral pleural effusion with a small number of ascites. Gastroscopy (GIF-2T240; Olympus Corporation) indicated gastric cancer of the antrum (), however, biopsy of the lesion determined it to be a distal gastric diffuse large B-cell lymphoma (). The initial laboratory analyses revealed the following data (normal ranges are provided in parentheses): RBC, 2.39×1012/l (3.5–6×1012/l) and hemoglobin, 55 g/l (110–150 g/l). The patient received the following oral chemotherapeutic regimen: 375 mg/m2 rituximab and 5 mg dexamethasone on the first day; 0.5 mg VCR on the third and sixth days; 30 mg prednisone on the third and seventh days; and 750 mg/m2 CTX, 1.5 mg/m2 VCR and 50 mg/m2 Pharmorubicin on the tenth day.
The patient was admitted to the hospital for a second time on November 26, 2009, and received the following chemotherapeutic regimen: 375 mg/m2 rituximab on the first day; then a CHOP chemotherapy regimen consisting of 750 mg/m2 CTX, 1.5 mg/m2 VCR, 60 mg/m2 Pharmorubicin and 15 mg prednisone. CHOP chemotherapy was administered twice-daily from the second day onwards. Symptoms associated with the ulcer were relieved by regular anti-ulcer treatment prior to discharge. This included ondansetron to prevent vomitting, recombinant human granulocyte colony-stimulating factor to accelerate white blood cell production and omeprazole for anti-ulcer treament, until the symptoms subsided.
The patient was admitted to the hospital for a third time on December 24, 2009. Gastroscopical examination identified a lesion in the antrum and pyloric obstruction, while a biopsy of the lesion demonstrated inflammation. The patient received a third round of CHOP chemotherapy, followed by daily radiotherapy at 1.8 Gy for 22 days. Thus, radiotherapy was administered daily from February 8–17, 2010 and from March 8–19, 2010.
The patient was admitted to the hospital for a fourth time on October 31, 2011, with a two-year history of repeated epigastric pains and a one-week history of nausea with vomiting. Gastroduodenoscopy identified an ulcer in the pyloric canal (), which was confirmed by performing a biopsy (). A CT scan demonstrated a thickened gastric wall and nodules in the middle of the right lung. The patient was discharged and received anti-ulcer treatment (orally, with 20 mg omeprazole, twice-daily, for 8 weeks).
Between September 2012 and April 2013, the patient was admitted back to the hospital three times for abdominal distension, nausea and vomiting. Gastroscopical examinations were performed each time and identified an ulcer at the same location (), as determined by biopsy (). The patient received treatment to alleviate the symptoms (ondansetron to prevent vomitting, recombinant human granulocyte colony-stimulating factor to accelerate white blood cell production and omeprazole for anti-ulcer treament, until the symptoms subsided), and was discharged each time.


A 39-year-old woman with a history of schizoaffective disorder and bilateral pulmonary emboli being treated with rivaroxaban presented to the Emergency Room (ER) with a two-week history of vaginal bleeding. She endorsed fatigue, dyspnea, and lightheadedness while associating abdominal cramping and low back pain with the bleeding. She reported no prior history of abnormal menstrual bleeding. She stated she was transitioned from warfarin to rivaroxaban approximately six weeks ago, with hospital records indicating a switch to rivaroxaban 20 mg daily was made due to concerns of noncompliance with warfarin monitoring. Physical exam revealed blood pressure 122/70 mmHg, heart rate 70 beats per minute, respiratory rate 18 breaths per minute with an oxygen saturation of 99% on room air, and a temperature of 36°C. Pelvic exam showed fresh blood and clots in the vaginal vault, and after they were cleared, bleeding from a closed external cervical os was identified. Laboratory investigations showed her hemoglobin on presentation was 6.8 g/dL, 4.0 g/dL below routine lab work done one month before, platelet count 266,000/μL, prothrombin time 14.1 seconds, activated partial thromboplastin time 27.2 seconds, and INR 1.3.
In the ER she was started on intravenous fluids and given 1,800 units of intravenous activated prothrombin complex concentrate (PCC) per ER protocol to attempt reversal of anticoagulation. Upon admission hematology was consulted and their recommendations of holding rivaroxaban, transfusing two units of packed red blood cells, and discontinuing the PCC therapy, due to the increased risk of thrombosis, were followed. Through the next day the patient continued to bleed with her hemoglobin dropping from 8.3 g/dL after the initial transfusion to 5.7 g/dL and attempts to stop the bleeding with 2.5 mg intravenous conjugated estrogen recommended by gynecology were unsuccessful. By the third day of admission the patient had received an additional 4 units of packed red blood cells with improvement in the hemoglobin to 8.3 g/dL. Notably, repeat coagulation studies revealed a prothrombin time of 14.6 seconds, activated partial thromboplastin time of 21.5 seconds, and INR 1.3 (). Gynecology attempted balloon tamponade which was also unsuccessful in inducing termination of bleeding. After discussing the implication to her fertility, the patient was agreeable to a dilation and curettage with endometrial ablation the next day which resulted in cessation of bleeding and stabilization of hemoglobin to 9.5 g/dL after one additional unit of packed red blood cells was transfused. She was discharged on fondaparinux with hematology follow-up.


A 73-year-old man was admitted to our department with a diagnosis of an asymptomatic 57 mm fusiform abdominal aortic aneurysm (AAA). Medical history was notable for obesity, hypertension, chronic depressed (<30%) left heart dysfunction, and chronic obstructive pulmonary disease. Preoperative computed tomography angiography (CT-A) highlighted the presence of a hostile proximal aortic neck characterized by a reversed tapered shape (Figures and ) plus an acute β-angle > 60° (). In the operating room, a 28 mm transrenal bifurcated EG (Endurant-Medtronic; Santa Rosa-CA; USA) was implanted through conventional bilateral groin cut-down. Final angiography confirmed the complete exclusion of the AAA with no evidence of proximal or distal endoleak, as well as the visualization of the renal arteries (Figures and ). The immediate postoperative serum creatinine level was 1.48 mg/dL (range, 0.6–1.3 mg/dL; preoperative level: 1.36 mg/dL). Twelve hours later, a progressive reduction of urine output was noted; at that time, serum creatinine level increased to 3.26 mg/dL, configuring a grade 4 AKI according to the Aneurysm Renal Injury Score (ARISe) []. Angiography was performed immediately thereafter: it showed the occlusion of the renal artery, bilaterally. During the same procedure, we attempted an endovascular revascularization of the renal arteries, which failed because of the acute onset of a high-response atrial fibrillation causing hemodynamic instability and acute respiratory distress. The patient was transferred in the intensive care unit and started a temporary renal replacement therapy and amiodarone (Cordarone-Sanofi-Aventis; Milano-Italy) intravenously. Four days later, hemodynamic stability was recovered; at that time, a percutaneous left transbrachial approach was used to catheterize selectively the renal arteries () with a 0.014′′ guidewire (Stabilizer-Cordis, Miami Lakes, FL, USA) coupled with a 4F vertebral catheter (Cordis; Miami Lakes-FL; USA). A 5 × 15 mm bare metal stent (Genesis-Cordis; Miami Lakes-FL; USA) was used bilaterally with the complete restoration of renal flow into the parenchyma (). The subsequent postoperative course was uneventful: the urine output improved progressively. He was discharged on day 6 postoperatively on acetylsalicylic acid (Cardioaspirin-Bayer; Milano-Italy) 100 mg/die ad infinitum. He was last seen eighteen months later; the patient is still alive, asymptomatic, and the follow-up CT-A confirmed the complete exclusion of the aneurysm without endoleaks and the patency of the renal stents with preservation of visceral flow (Figures and ). At that time serum creatinine level was 1.38 mg/dL.


A 10-year-old boy with newly diagnosed metastatic medulloblastoma status after subtotal resection presented to follow-up neurooncology clinic for routine evaluation after recently being discharged from the hospital 5 days after surgery. The surgery itself was without complications despite being a subtotal resection. He was placed on perioperative dexamethasone (4 mg IV Q6 hours, equaling 0.458 mg/kg/day). The maximum dose of dexamethasone was chosen given the extent of his disease and concern for tumor associated edema, given that he had a subtotal resection. Ranitidine was added for gastrointestinal (GI) prophylaxis. Given that his course was uneventful, tapering began around discharge, and his dexamethasone dose was adjusted to 3 mg PO TID at discharge.
At the follow-up appointment, postoperative day 10 from surgery or 5 days after discharge, he reported transient abdominal distension with flatulence for the last 2 days, without any report of pain, nausea, vomiting, dysmotility, constipation, diarrhea, hematochezia, or melena. Review of past medical history and family history was noncontributory. The patient was on no other additional medications other than the dexamethasone and ranitidine. On physical exam, he was noted to be tired appearing with normal vital signs without any signs of abdominal distension and/or tenderness. Over the course of the visit he suddenly became pale and lost consciousness. Emergent head CT revealed no evidence of intracranial hemorrhage; however complete blood count revealed hemoglobin of 4.2 gm/dL. Despite rapid delivery of blood products the patient died in the intensive care unit less than 2 hours after presentation. An autopsy performed postmortem demonstrated extensive intraluminal blood within the intestinal tract without evidence of peritoneal hemorrhage or metastatic disease. There were extensive ulcerations in the duodenum on gross and microscopic analysis confirming the cause of death to be due to his gastrointestinal ulcer secondary to dexamethasone therapy.


A 65-year-old male with a history of gastric bypass surgery and left inguinal hernia repair presented with left groin swelling for two months without any other associated symptoms. He initially presented with an enlarged, palpable, nontender left inguinal lymph node measuring 1 × 1.5 cm. Initial laboratory results, including complete blood count, complete metabolic panel, lactate dehydrogenase, and beta-2-microglobulin, were normal. Computed tomography scan detected multiple lymph nodes in the mesenteric, aortocaval, and right common iliac lymph nodes measuring up to 13 mm in diameter. At the time of surgical evaluation, the inguinal lymph node had regressed and was difficult to pinpoint by physical exam. He, therefore, underwent excisional biopsy of a mesenteric lymph node visible on the CT scan (). The follicular center site stained positive for PAX5, CD20, CD10, Bcl-2, and B-cell lymphoma 6 (Bcl-6), indicating follicular lymphoma. However, CCND1 unexpectedly highlighted CD5 positive mantle zone cells in neoplastic follicles, which suggested a MCLIS () component. FISH analysis demonstrated 35% of t(11; 14) and 65% of t(14; 18) in tested nuclei that are mutually exclusive. Further workups including bone marrow biopsy and aspirate were unremarkable. Colonoscopy with random biopsies was negative with no evidence of malignancy although esophagogastroduodenoscopy was not feasible due to his recent gastric bypass surgery. The patient has been followed up for 2 years since initial diagnosis of composite lymphoma and is currently under active clinical surveillance without any signs of disease progression.


32-year-old woman with CD localized in the small intestine and in the ileocaecal valve has been treated in the conventional manner (azathioprine (2,5 mg/kg), mesalamine (2 g a day) and budesonide (9 mg a day)) for half a year. The diagnosis of the disease was confirmed by colonoscopy, abdominal ultrasound examination, and microscopic evaluation of samples taken during colonoscopy. In the past, the patient was treated by a gynecologist because of chronic ovaritis. She was not suffering from any other diseases and the results of laboratory tests showed only moderate anemia and elevated CRP (C-reactive protein) level.
Due to the ineffectiveness of such therapy (abdominal pain, diarrhea), she received biological treatment, anti-TNFα (tumour necrosis factor α), infliximab (at a dose of 5 mg/kg) intravenously at weeks 0, 2, and 6 with the outcome of clinical and endoscopic remission. Treatment with azathioprine (2,5 mg/kg) and mesalamine (2 g a day) was continued during the remission period.
After three years, the disease course worsened. In addition to the severity of diarrhea and abdominal pain, an increasing abdominal circumference has been observed, as well as swelling of lower extremities. The patient returned to the gynecologist for consultation. Due to suspicion of ovarian cancer with concomitant ascites, numerous laboratory tests were performed, which showed a decrease of total protein, including albumin fraction, and an increase of CRP and CA125 level, which exceeded the normal upper limits more than five times (). Abdominal ultrasonography and CT (computed tomography) showed the presence of ascites, as well as wall thickening with features of inflammation in the jejunum (). Apart from the so far used mesalamine and azathioprine, budesonide was included to the treatment at a dose of 9 mg a day. Additional tests were performed to rule out infection, tuberculosis, kidneys and liver diseases. Within two months there was no improvement in general condition of the patient. Ascites was still present, as well as abnormal serum levels of albumin, CRP, and CA125. Due to persistence suspicion of malignancy, a diagnostic laparoscopy was ordered by gynecologist and oncologist, preceded by a transvaginal ultrasound scan, to clearly exclude ovarian cancer coexistence. During laparoscopy, the fluid was collected from the abdomen to the laboratory and microbiological evaluation. The test results indicated a transudate, with no signs of infection. A negative result of diagnostic laparoscopy influenced the decision to intensify Crohn's disease treatment. The patient received anti-TNFα, adalimumab (at a dose of 160, 80, and then 40 mg every other week). The choice of the drug was dictated by patient preference, because of everyday business activity. After a period of 14 weeks, clinical remission was achieved, as well as recovery of ascites and normalization of CA125 serum level (). Maintenance treatment with adalimumab was continued for one year. The clinical remission has been maintained for 18 months so far since the completion of biological treatment.


The patient is a 54-year-old, outdoors-oriented woman who began to experience intermittent pain, paresthesias, and numbness of the right arm followed by the same symptoms in the bilateral lower extremities. Within 4–6 weeks she was only able to walk with assistance and then limited to a wheelchair. The patient had an MRI of her brain and spinal cord to rule out multiple sclerosis (MS). Her brain MRI revealed some white matter lesions that were not consistent with MS, but her C-spine MRI showed a significant C5-6 herniated nucleus pulposus and severe canal stenosis at C5-6, which flattened her spinal cord. This pathology was thought to be the cause of her symptoms. Subsequently, she underwent a C4–C7 anterior cervical discectomy and fusion surgery. The surgery appeared successful as she was ambulating independently within 1 week of surgery and hiking long distances at altitude within 6 weeks. At this point, the patient did not have any physical limitations or deficits. Four months after surgery, she began to experience unexplained pain, paresthesias, and numbness in her right foot/ankle. This quickly became an uncontrollable pain requiring axillary crutches and then a wheelchair. After three months of the foot/ankle pain, she was seen by Physical Medicine and Rehabilitation and was then enrolled in a comprehensive rehabilitation program for presumed CRPS. Even though she was treated with opioid and neuropathic pain medications (morphine sulfate, gabapentin, pregabalin, and amitriptyline), she was unable to participate fully in physical therapy due to pain. During month 4 of foot/ankle symptoms, the pain worsened and she was no longer able to bear weight on her right lower extremity and the foot/ankle began to exhibit classic signs and symptoms of CRPS including allodynia, edema, erythema, hyperhidrosis, decreased range of motion, and dystonia [, ]. Electrodiagnostic studies showed no evidence of large fiber neuropathy, distal tibial neuropathy, or right lower lumbosacral radiculopathy. However, testing revealed significant asymmetry in skin temperature, 29.0 Celsius on the left lower extremity and 26.3 Celsius on the right, along with asymmetrically prolonged onset latency difference of 484 msec on the right. She was expedited to the Department of Pain Management for sympathetic blocks, but it was decided to pursue early neuromodulation instead. Approximately 4-5 months after the onset of symptoms she underwent successful SCS trial. The trial was performed in the pain management clinic and consisted of the placement of one percutaneous lead with eight electrodes introduced via a 14 gauge modified Tuohy needle and entering the epidural space in a parasagital approach at L2-3 under fluoroscopy. Lead location in the posterior epidural space was confirmed with lateral fluoroscopy. The trial lead was advanced carefully to the T11-12 disc space under intermittent fluoroscopy (Figures and ). The patient reported incredible pain relief and that she was ambulating around her home for the first time in months. The paresthesias over her most painful areas allowed her to regain the majority of previously lost function and she began to take fewer medications. Because of the unqualified success of the trial, she went on to successful implantation in the operating room of two percutaneous leads with eight electrodes placed in the same area as the trial. For the implantation, two leads were used instead of one in order to maximize potential programming ability (Figures and ). She reported that the induced paresthesias allowed her to stand and ambulate around her home for the first time in months and to begin actively participating in intensive physical and occupational therapy. After placement of the SCS, the patient was seen for physical and occupational therapy sessions of one hour each, five times per week, for four weeks. Sessions were then decreased to one hour each, three times per week, for fourteen weeks. Physical therapy consisted of graduated weight-bearing, gait training, stepping and balance drills, recumbent cycling, standing elliptical, and lower extremity strengthening exercises. Occupational therapy consisted of desensitization, treatment with IFC (inferential current), and graded motor imagery. The patient was seen regularly by a Physical Medicine and Rehabilitation physician to monitor symptoms and adjust medication and functional therapy as needed. She was also seen regularly in the Department of Pain Management for follow-up of the SCS and an IFC unit was prescribed for home use as well. In addition, the patient was seen weekly by a behavioral health psychologist (also within the Department of Pain Management) for cognitive behavioral therapy and biofeedback. Finally, the patient participated in an 8-week course of mindfulness-based stress reduction. Ten months after the onset of symptoms she is no longer taking pain medication, is independent in all ADLs, and has returned to work.


A 41-year-old man presented to the emergency room with complaints of fever, diarrhea, and fatigue for 24 h.
On examination, he was restless and his systolic blood pressure, pulse, respiratory rate, and temperature were 50 mmHg, 143 beats/min, 30 breaths/min, and 38.2°C, respectively. Oxygen saturation could not be recorded because of peripheral circulatory failure. Marked cyanosis was evident in the facial region, peripheral limbs, and penis. Lung auscultation revealed coarse breathing sounds.
Arterial blood gas analysis was suggestive of remarkable lactic acidosis with a serum lactate level of 11.5 mmol/L. His white blood cell and platelet counts were low at 1,600/μL and 36,000/μL, respectively. Serum urea nitrogen (36.1 mg/dL), creatinine (4.8 mg/dL), and C-reactive protein (25.6 g/dL) levels were elevated. Coagulation was prolonged (prothrombin time, 50.9% of normal), and fibrin/fibrinogen degradation product levels were higher (98.9 μg/dL). Furthermore, his procalcitonin and endotoxin levels were markedly elevated (457 ng/mL and 548 pg/mL, respectively).
Computed tomography (CT) revealed left-sided sinusitis, bilateral lung consolidation, and an increased bilateral concentration of perirenal fat tissue. Lesions requiring debridement or drainage were not evident. His spleen was small, with a size of 38 cm3 on CT.
The observed cyanosis rapidly developed into purpura within 1 h after arrival. Samples of blood, sputum, stool, and urine were obtained for microbiological evaluation, and imipenem/cilastatin (IPM/CS) was administered as empirical therapy (0.5 g q12h). His blood pressure continued to decline despite the infusion of large amounts of fluid and catecholamines. His carotid pulse was nonpalpable 1 h after the arrival. Recovery of spontaneous circulation was achieved after 2 min of resuscitation. Narrow QRS sinus tachycardia without ST-T changes was apparent on electrocardiography. Decision to introduce veno-arterial ECMO (V-A ECMO) was made because of prolonged hemodynamic catastrophe.
Approximately 12 h after admission, his pulse pressure was undetectable and no cardiac contraction could be discerned by ultrasonography. However, we maintained blood pressure and oxygenation at adequate levels with the aid of V-A ECMO. PMX therapy was induced at emergency room for elevated endotoxin level, but it was not effective because there was no improvement of vital signs in this case.
Three days after hospitalization, cardiac function began to improve, and the ejection fraction improved to 40% by day 5. However, poor lung oxygenation persisted (PaO2/FiO2 ratio was 50). To maintain oxygenation of the brain and coronary arteries, we changed the support protocol from V-A ECMO to veno-venous ECMO (V-V ECMO).
Notably, the creatine kinase (CK) MB levels were 261 IU/mL on day 1 of hospitalization; these levels gradually increased to a peak of 417 IU/mL on day 3 before gradually decreasing. These variations and the elevated CK levels influenced the CK-MB/CK ratio, which was 1%–2% during the hospital stay.
A diagnosis of PF due to invasive H. influenzae infection was made on the basis of the isolation of β-lactamase-negative ampicillin-resistant (BLNAR) H. influenzae from the patient's sputum and blood. Minimum inhibitory concentration values for antibiotics were as follows: ampicillin, 2 μg/mL; cefaclor, 16 μg/mL; cefotiam, 4 μg/mL; cefpodoxime proxetil, 8 μg/mL; and cefozopran, 16 μg/mL.
On day 9 of hospitalization, infection-induced necrotic tissue in the limbs was clinically suspected (Figure ), necessitating bilateral above-knee amputation and right forearm and left upper arm amputation. As the necrotic areas were not clearly demarcated, the stumps were not closed and daily debridement and irrigation was performed.
The patient's respiratory condition gradually improved thereafter, and he was successfully weaned from V-V ECMO on day 12 of hospitalization. However, on the same day, the patient developed left pneumothorax complicated by persistent air leakage, which resulted in empyema caused by methicillin-resistant Staphylococcus aureus. He improved enough to leave the catecholamines once, but septic shock due to empyema was rekindled. As oxygenation was too bad to perform isolated lung perfusion, surgical lavage and drainage were impossible. Daily lavage from a chest tube was performed, but he eventually succumbed to sepsis caused by the empyema on day 34 of hospitalization.


A preterm female infant (weight 2780 g) was delivered by caesarean section at 341/7 weeks, because of fetal distress, from a 38-year-old mother with measles pneumonitis. She was born on the sixth day after her mother's exanthema necessitated noninvasive ventilation because of severe pneumonia (). The mother's medical history indicated that she had not been vaccinated for measles. The infant showed no clinical signs of the disease at birth.
Cord blood and serum samples were collected from the infant and mother soon after delivery. An enzyme-linked immunosorbent assay (ELISA) indicated the presence of measles-specific IgM and IgG antibodies in the mother. The infant's serum was positive by ELISA for the presence of measles specific IgM but negative for the presence of IgG antibodies. However, RNA extracted from cord blood mononuclear cells for use in a measles virus-specific reverse transcriptase-PCR assay indicated the presence of measles virus (). On her first day of life, the infant received IVIG (400 mg/kg) and 50,000 IU of vitamin A orally. The initial thrombocyte count shortly after birth was 228,000/μL and had dropped to 44,000/μL after 4 days (). Thrombocytopenia was further improved after a second dose of IVIG (400 mg/kg) and 50,000 IU of vitamin A orally with the addition of 10 mm3/kg thrombocyte transfusion. The newborn was discharged in a good condition on the tenth day.


A preterm female infant (weight 2630 g) was delivered by vaginal route from a 27-year-old mother with measles pneumonitis at 36 weeks, on the fourth day after her mother's exanthema had disappeared. The mother's medical history indicated that she had received only 1 dose of the measles vaccine, and the serum obtained 1 month previously was negative by ELISA for antimeasles IgG and IgM. The infant showed no clinical signs of the disease at birth.
Cord blood and serum samples were collected from the infant and mother soon after the delivery and serum specimens were tested for the presence of measles-specific IgM, IgG, and RNA as described above. The infant's serum was positive by ELISA for both measles-specific IgM and IgG antibodies. Measles-virus-specific RNA extracted from cord blood mononuclear cells was positive as well ( and ). The infant was treated with IVIG (400 mg/kg) and 50,000 IU of vitamin A on the first day of life and discharged in a good condition on the seventh day.


A 27-year-old G2P0010 at 33 weeks and 4 days by last menstrual period was brought in by Emergency System to the hospital on June 25th 2014, with complaints of severe abdominal pain of 1 hour duration. Patient was without medical or surgical history and had a termination of pregnancy before. Abdominal pain was generalized, 10 out of 10 in severity, and associated with vomiting. She denied any diarrhea, vaginal bleeding, or leakage of amniotic fluid. She had recently migrated from the Dominican Republic in May 2014 with no record of prenatal care.
On examination, patient was in visible pain with elevated blood pressure, maternal tachycardia, and bilious emesis. An abdominal examination revealed generalized tenderness with guarding and rebound and a fundal height of 34 cm. The fetal heart rate was category III with absent variability and repetitive late decelerations. A vaginal examination revealed a bulging pouch of Douglas with the presenting part deep in the pelvis: a short, firm, and closed cervix displaced anteriorly behind the pubic symphysis.
On the way to the operating room limited bed side sonogram revealed fetus in cephalic and a questionable placental location. A tentative diagnosis of uterine rupture versus concealed placental abruption was made proceeding with immediate abdominal delivery.
At the time of laparotomy, meconium stained amniotic fluid was seen upon entry to the peritoneal cavity. A fetus was located outside of the endometrial cavity covered only by the uterine serosa on the right side with a placenta attachment to the serosa of the uterus. The left ovary was unremarkable in appearance and an anatomical distortion of the right adnexa was appreciated. A large opening was noted on the posterior aspect of the serosa where the amniotic fluid was leaking.
An incision was made on the protruding serosa and a viable female infant was delivered via cephalic presentation with Apgar score of 9/9 at 1 and 5 minutes with weight of 2362 g. The uterus and placenta were exteriorized after delivery due to massive bleeding and distortion of the anatomy (). On further inspection of the placenta, it was noted to invade the pouch of Douglas and lower part of the sigmoid colon and the right uterine serosa.
A massive hemorrhage protocol was initiated and an emergency back-up team was called. A general surgical consult was requested due to involvement of bowel. The decision was made to proceed on hysterectomy and removal of the placenta tissue due to continuous bleeding. The patient underwent supracervical hysterectomy and excision of the placenta tissue occupying the right side of the pelvic floor. Adhesiolysis from the sigmoid colon was performed by surgery with minimal damage to the serosa.
Intraoperatively, the patient received 6 units of packed red blood cells, 4 units of fresh frozen plasma, and one unit of platelets. Estimated blood loss was 3000 mL. The patient was then transferred to the ICU for further observation and extubated the following morning.
She was discharged home with the baby on day 4 after surgery. There was no evidence of anomaly documented in the baby. Mother and baby are doing well and currently being followed up closely.
A pathology report revealed that placenta with a segment of trivessel umbilical cord marked old infarct at fetal and maternal surfaces. Attached to the maternal surfaces are fibrous tissues with smooth muscle and dilated vessels. Focal endovasculopathy with luminal occlusion, focal amnion with squamous metaplasia with an attached stretched ovary and fragment of mostly chorionic villi.
The uterus was described as intact and weighed 300 g measuring 9.5 cm in length, 11 cm from cornua to cornua and 6 cm anterior posterior diameter with thick endometrial, decidual changes and focal autolysis, no chorionic villi or trophoblast are seen in the endometrium.


A 47-year-old Chinese man presented to our department of ophthalmology with a 26-month history of left upper eyelid painless swelling and redness. The patient was initially treated with a course of intravenous penicillin, which resulted in temporary regression of the lesion. There had been no similar family history of this appearance to date.
Physical examination revealed that the left lacrimal gland was obviously swelling, which presented with a soft, non-tender and smooth-surfaced. Slit lamp examination was otherwise unremarkable aside from bilateral mild cataract. Laboratory testing revealed a white blood cell count of 9.8 × 10-9/L with 15.91% eosinophils(normal range: 0.05-0.5 × 10-9/L). Serum concentration of immunoglobulin E was elevated at 469.3 IU/ml, and immunoglobulin G4 was 295 mg/dL (normal range less than 135 mg/dL). Magnetic resonance imaging (MRI) examination of the orbit showed that the left lacrimal gland was lobulated with a distinct margin and was predominantly isointense on T1-weighted images. Isointense on T2-weighted images and an obvious heterogeneous enhancement on contrast-enhanced MRI images are shown in Figure . The chest radiograph was normal and no cervical lymph nodes were palpable.Next, we advised the patient to be hospitalized and the patient underwent an orbital biopsy. Intraoperative frozen sections were reported as being consistent with a benign tumor. Therefore, a complete resection was undertaken. Perioperatively, it was noted that the mass had a smooth and homogeneous grey appearance. There was no evidence of haemorrhage or cystic change and borders were indistinct. A definite diagnosis of KD was made by histopathology and immunohistochemical examinations of representative resected specimens after surgery. Histopathological examination demonstrated follicular hyperplasia with evidence of reactive germinal centres. Eosinophilic infiltration, involving the interfollicular areas, and proliferation of post capillary venules were observed. These features enabled a diagnosis of KD to be made (Figure A). Further analysis, including immuohistochemistry, was undertaken, demonstrating positive staining for CK, Vimentin, CD3, CD4, CD20, CD21, CD117, CD5, CD8, CD23, IgG and IgG4 (30 per high-power field) and negative staining for CD10 and CD34 (Figure B), the ratio of IgG4 to IgG positive cells was about 35%. The pathological diagnosis, which was also the final diagnosis, was KD. However, some ophthalmologists questioned whether the histological and immunohistochemical findings were also had common features with IgG4-related disease, which is a newly recognized fibro-inflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 concentrations.
The patient was discharged following his surgery and treated with prednisone 25 mg/day for four weeks and then reduce by 5 mg/day every two weeks. His symptoms gradually improved. Serum immunoglobulin E (IgE) levels dropped to 125.9 IU/mL and eosinophil’s decreased to 0.69 × 109/L after 2 months. There was no sign of recurrence in twelve months of regular follow-up.


A 35-year-old woman at 24 weeks and 4 days of gestation was admitted following 7 days with epigastric pain, postprandial vomiting, and abdominal bloating causing nutritional problems. Her history included appendectomy, RYGB 4.5 years earlier, two pregnancies before the RYGB, and laparoscopy for suspected internal herniation. Abdominal and obstetrical ultrasonography, gynaecological examination, and upper endoscopy were normal. Subacute internal herniation was suspected.
During surgery the patient was hypotensive and positioned flat and rotated to her left. A 12 mm Visiport was applied to access the abdominal cavity, 7-8 cm above the suspected fundus uteri palpated at the umbilical level. This corresponded with the anticipated level of the uterine fundus level based on age of gestation. The trocar was directed towards the subhepatic region. Intraabdominal CO2 gas pressure was set to 12 mmHg. Following trocar insertion, intra-abdominal fluid was observed and suspected to be induced by herniated intestines. The camera tip could be palpated in the right subcostal area below the costal margin. A 5 mm trocar was introduced at the right side superior to the first trocar. A fetal foot was then observed. Gas was exufflated and the trocars were closed. The obstetricians attended and a laparotomy extending high up in the epigastrium was made to mobilize the uterus with the trocars in the intrauterine position. The trocar entry points were identified below the tubes, more than 5 cm from the uterine fundus. The trocars were removed following placement of purshing sutures (3–0 absorbable, Vicryl). A cesarean section was not considered a viable option. A few extra sutures were made. The enteroenteroanastomosis was identified posterior to the high located and downward mobilised uterus. No pathology was identified.
Postoperatively, atosiban was given for 48 hours. Betamethasone, anti-D immunoglobulin, prophylactic cefuroxime, metronidazole, and an epidural catheter were administered. The patient remained at the intensive care unit for 2 days and subsequently 15 days at the unit for high risk pregnancies. She felt the abdomen less distended and could eat. Repeated obstetrical ultrasonographic examinations were normal during the hospital stay.
At gestational week 30, the patient experienced acute lower abdominal pain. Obstetrical ultrasonography pictured fluid in the upper uterine wall. A dissected rupture could not be excluded and a cesarean section was performed. The uterus was explored without any defects. The 1800 gram infant was in well shape. The 1 minute APGAR score was 7 and the 5 minute score was 6. However, the infant deteriorated with respiratory problems and pulmonary hypertension and was transferred to the University Hospital Neonatal Intensive Care Unit. Seven weeks later the infant was discharged home. Five months old the child has developed normally and seems healthy.


A 60-year-old man was brought to the emergency room due to family concerns of rapid cognitive decline. Two weeks prior to hospitalization, he had an elective dental procedure. Immediately following the procedure, he complained of headaches, nausea, vomiting, intermittent fevers, and chills. He received one dose of intramuscular ceftriaxone, and daily oral amoxicillin and metronidazole. A week before admission to our hospital, he became progressively weaker, less communicative, and repeated non-sensical words.
Medical history was remarkable for arterial hypertension and dyslipidemia. Medications were simvastatin, amlodipine, and olmesartan.
Upon arrival to our hospital, he was afebrile, with normal blood pressure, heart rate, and oxygen saturation. He was alert and oriented only to person. Language was non-fluent. He was unable to follow commands, had moderate neck stiffness, moved all his extremities spontaneously, and responded appropriately to nociceptive stimuli.
Blood work showed hemoglobin (Hgb) of 12.5 g/dl, white blood cells (WBC) of 8.3 k/UL (85% granulocytes), and a platelet count of 263 k/UL. Basic metabolic profile (BMP) liver enzymes, albumin, bilirubin, and urine drug screen were unremarkable. Cranial computed tomography (CCT) was unremarkable. Empirical intravenous (IV) acyclovir 600 mg every 8 h, ceftriaxone 2 g every 12 h, vancomycin 1000 mg every 12 h, and metronidazole 500 mg every 6 h were started. A lumbar puncture (LP) showed an opening pressure of 7 cm of cerebrospinal fluid (CSF), 278 red blood cells (RBC), 84 WBC (88% lymphocytes), glucose content of 59 mg/dl, and a protein content of 91 mg/dl. CSF PCR for Herpes simplex virus (HSV) type 1 and 2, Epstein Barr virus (EBV), Cytomegalovirus (CMV), Enterovirus, Lyme antibodies, and cultures were negative. Antimicrobials were discontinued after a total of 7 days.
He was admitted to the Neuroscience Intensive Care Unit (NICU). As his mental status further deteriorated, he was electively intubated for airway protection. EEG showed continuous spikes in the left frontal region. He received IV lorazepam 2 mg, and was started on IV levetiracetam 1000 mg every 12 h. He was placed on continuous video EEG monitoring for 48 h with no evidence of epileptiform activity. Magnetic resonance imaging (MRI) of the brain with gadolinium showed multiple rounded lesions throughout the brain parenchyma predominantly involving the white matter of both cerebral hemispheres, basal ganglia, midbrain, and pons. Some of these lesions showed enhancement in a linear and nodular pattern (Figure ). Transesophageal echocardiogram (TEE) showed no sources of emboli or vegetations. Serial blood and urine cultures were negative. A presumptive diagnosis of ADEM was made, and he received methylprednisolone IV 1000 mg daily for 5 days and IV immunoglobulin (IVIG) 0.4 g/kg/dose daily for 5 days were concomitantly started. Due to the lack of improvement with IVIG, he received five sessions of plasma exchange (PLEX) every other day, starting the day after the last dose of IVIG. Despite this, the patient remained unresponsive to verbal stimuli, with preserved pupillary and oculocephalic reflexes and minimal arm flexion on noxious stimuli on the left arm. His exam remained unchanged during his hospital stay. Fourteen days after admission, a repeat MRI of the brain showed more disseminated white matter lesions (Figure ). Stereotactic brain biopsy showed numerous cluster differentiation (CD) 68 positive cells with focal CD3 (T cell) and CD20 (B-cell) perivascular inflammatory infiltrates. The affected parenchymal areas contained both CD4 positive and CD8 positive T cells with the latter in greater number. Luxol Fast Blue stains showed demarcated areas of coalescencing perivenous demyelination with relative sparing of axons with some axonal swellings (Figure ). Simian Virus (SV) 40 polyoma virus, Gram, and AFB stains were negative. Although the PAS stain without diastase was negative, the PAS with diastase stain showed questionable rod-shaped bacteria. He received another 6-day course sulfamethoxazole with trimethoprim 800–160 mg every 12 h. Subsequent bacterial PCR for tissue and serum were negative for Tropheryma whipplei. He then received IV Cyclophosphamide 750 mg/m2/dose. H. Subsequently, one dose of Rituximab 375 mg/m2/dose was given without significant change in his overall neurological condition. He was discharged after 30 days from the hospital to a long term acute care hospital where his condition remained unchanged, in a persistent vegetative state after 6 months, when this report was initially submitted.


A 20-year-old Caucasian man was diagnosed with SLE due to polyarthritis, pleuritis, positive antinuclear antibodies (ANA on human epithelial type 2; HEp-2), positive anti-double-stranded DNA, and biopsy-proven class IV glomerulonephritis. Methylprednisolone 1g/day was started for 3 days followed by prednisone 1mg/kg/day. After 1 month, active renal disease persisted and methylprednisolone treatment was repeated. Thereafter, severe pancytopenia and cytomegalovirus infection were identified. Gancyclovir was administered for 21 days with good response. Cyclophosphamide was started (500mg) but after 1 month he presented daily fever and neck pain. At physical examination, an anterior painful cervical prominence was observed; heart rate 122 beats per minute, temperature 37.9°C and oxygen saturation 91%. Piperacillin/tazobactam was started as pulmonary infection was detected. After 2 days, he developed hypoxemia, vascular shock, severe anemia, lymphopenia (300/mm3), and high C-reactive protein (137.5mg/L; normal range, NR, <5). Thyroid ultrasound revealed well-defined hypoechogenic clusters in both lobes. Thyroid-stimulating hormone (TSH) was suppressed, free thyroxine (T4) >74.6pmol/L (NR 7.7 to 18), total T4 189pmol/dL (NR 90 to 154), total triiodothyronine (T3) 1.15nmol/L (NR 1.23 to 1.85), and high serum thyroglobulin (650.6μg/L). A purulent substance was obtained on fine needle aspiration (FNA) and drained. Cytological analysis showed only pus cells. Thyrotoxicosis due to infectious thyroiditis was established. Fungal culture of thyroid abscess was partial positive. Amphotericin B and fluconazole were introduced. Tracheal and blood cultures were negative. Another thyroid ultrasound revealed persistent small clusters. He underwent drainage again but no secretion was obtained. He died at day 6 of hospital admission with suppressed TSH, lower free T4 but still high (47.6pmol/L) and low total T3 (0.60nmol/L). A culture of thyroid secretion took 10 days to reveal Aspergillus fumigatus, identified by septate hyphae, <5μm in thickness, with branching at acute angles, 2 days after his death.


A 39-year-old otherwise healthy Caucasian man presented with spontaneous, sudden onset headache associated with diplopia lasting for several minutes. The most severe headache episode lasted for about 15 minutes. During admission, the intensity of his headaches diminished significantly along with an improvement of the diplopia. On admission, he was awake, alert and fully oriented with fluent speech. He had no focal neurological deficits except nuchal rigidity. He was a non-cigarette smoker with no significant past medical history. A CT of his head without contrast revealed subtle subarachnoid hemorrhage within the interpeduncular and ambient cisterns (Figure A and B). A subsequent lumbar puncture was found positive.He was admitted to our neurosurgical intensive care unit (N-ICU) for observation and further work-up. Coagulation studies were normal. Further evaluation with magnetic resonance imaging and angiography of his head and neck and 4-vessel cerebral angiogram did not show any abnormalities (Figure A-C). Venous phases of the cerebral angiograms showed a Type C variant of the basal vein of Rosenthal (BVR) draining into the superior petrosal sinus on the left side and a small and hypoplastic Type A variant of BVR on the right side (Figure D and E). His symptoms improved and eventually he was discharged 1 week after the bleeding. On follow-up at 2 years, no neurological deficit and no recurrent subarachnoid hemorrhage were observed.


A 44-year-old otherwise healthy Caucasian man, the older brother of the first case, presented with the spontaneous acute onset of headache. He denied loss of consciousness, vision loss, nausea, vomiting, numbness, weakness and dizziness. On neurological examination, he was awake, alert and fully oriented with fluent speech. In the presence of slight nuchal rigidity, he had full motor strength with no sensory deficit in all four extremities and his cranial nerves were intact. He was a non-cigarette smoker and had no significant medical history except inguinal hernia repair. Family history was positive for P-NSAH in younger brother (Case 1), which was diagnosed 1.5 years previously. A non-contrast axial CT scan of his head revealed a perimesencephalic SAH pattern and no evidence of intraventricular or intraparenchymal blood and hydrocephalus. The center of bleeding was located anterior to the midbrain (Figure A and B).He was admitted to our N-ICU for observation and further work-up. No abnormality was found in the coagulation studies. He underwent additional multiple imaging studies, including CT and CT angiography of his head and neck (Figure A and B) and 4-vessel cerebral angiograms. None of these diagnostic studies showed the source of this SAH. Venous phases of the angiograms showed a Type A variant of BVR on the left side and a Type B variant of BVR on the right side (Figure C and D). His symptoms continued to improve and eventually he was discharged 1 week after the bleeding. A repeat cerebral angiogram 3 months later again confirmed the absence of any aneurysm and vascular abnormality. On 1 year follow-up, his neurological examination was normal and no recurrent subarachnoid hemorrhage was observed.


In 2010, a 41-year-old Caucasian Australian man presented with a 2-week history of urinary incontinence and the inability to walk. Difficulty climbing stairs and occasional foot drop had been present for approximately 3 years prior to presentation. He was also found to have Pseudomonas pneumonia, chronic sinusitis, and a small scrotal abscess. Since 1995 he had spent an extensive period in an Aboriginal community in the Great Sandy Desert and was in contact for a total of 4 to 5 years with them. HTLV-1 infection is typically endemic in these Aboriginal populations. He was significantly integrated into the community, undertaking initiation practices such as circumcision and distal urethral hypospadic incision and had unprotected sexual relationships with Aboriginal women. In the Aboriginal community he used intravenous amphetamine and occasionally intravenous heroin with shared needles for several years in addition to cannabis and infrequent ecstasy use. He has had a long history of alcohol abuse with periods of proactive abstinence.
An examination revealed right foot drop, bilateral leg weakness, some quadriceps wasting, bilateral hypertonicity of his lower limbs with sensory impairment reaching his upper thoracic region, and bilateral tingling and numbness in his upper limbs. Urinary incontinence was found to be due to neurogenic bladder dysfunction. Nerve conduction studies ruled out peripheral neuropathy and myopathy. His cranial nerve function was normal. His spinal cord magnetic resonance imaging (MRI) was normal, showing no evidence of cord lesions or compression. Brain MRI showed nonspecific white matter foci related to perivascular spaces and no signs of demyelination.
HTLV-1 antibodies were detected in his serum and CSF. Polymerase chain reaction was positive for HTLV-1 DNA and negative for HTLV-2 DNA in both whole blood and CSF. CSF analysis also revealed oligoclonal bands, elevated protein and a moderate, nonspecific lymphocytosis with no malignant cells or other abnormalities present. Blood investigations revealed a slight normochromic normocytic anaemia, elevated erythrocyte sedimentation rate (ESR; 53 to 108mm/hour over hospital stay) and rouleaux formation, all supporting chronic disease. His red-cell thiamine was normal. His estimated glomerular filtration rate was normal; liver function tests showed elevated gamma-glutamyltransferase and alanine aminotransferase with a normal blood ammonia level. Melioidosis, Rickettsia, human immunodeficiency virus, hepatitis B, hepatitis C, Brucella, Strongyloides, Venereal Disease Research Laboratory test (syphilis) serology were all negative, as was Mycobacteria microscopy and culture. Increased polyclonal production of immunoglobulin light chains with normal free light chain ratio was present. Flow cytometry of whole blood revealed expansion of CD4+ lymphocytes with a normal CD4+/CD8+ ratio. Predominant left-sided, chronic sinus disease was detected on computed tomography (CT). His sputum culture produced moderate Pseudomonas growth. A chest X-ray in addition to chest, abdominal and pelvis CT were normal apart from a few prominent retroperitoneal lymph nodes of less than 8mm.
On presentation he was started on high dose methylprednisolone (500mg per day for 5 days), resulting in a marked improvement in functionality. His function continued to improve to the point where he could walk into the consultation room on a single elbow crutch. Oxybutynin therapy was initiated to control bladder overactivity and his constipation treated with Coloxyl® (docusate sodium). Six months after initial presentation he presented with complaints of spasticity hindering functionality and sleep. MRI findings at this point again showed no abnormalities. Baclofen was prescribed reducing spasticity and improving functionality. His condition slowly improved until he was walking independently with an ataxic gait and self-catheterising once per day. His myelopathy appeared to have stabilised. On the most recent examination in February 2014, progressive improvement of his myelopathy was noted. He was able to walk 50m without a crutch. Neurological examination revealed pathologically brisk reflexes (3+ in the knees and ankles), plantar flexion, nine beats of ankle clonus on right and six on left. Oxybutynin for bladder control was ceased but he continued to self-catheterise once per day. In addition to baclofen 10mg twice per day, he received, amitriptyline to help him sleep and reduce cramps, as well as Coloxyl® to manage his constipation. He now lives relatively independently.


A 75-year-old Chinese Han woman, height: 160cm and weight: 55kg, presented with a simple pertrochanteric hip fracture below the lesser trochanter with a fracture classification AO: A1.3. A closed reduction with traction was achieved. While implanting an INTERTAN™ nail (INTERTAN™, 125°, 18.20-cm long, 10-mm diameter, Smith & Nephew, Andover, MA, USA) at the usual insertion point, we came across severe resistance. We tried to ream the medullary canal wide enough for insertion, but no relief was achieved. After careful analyses of the radiographs, during the intraoperative procedure, an excessive curvation of the femoral shaft was found in the coronal and sagittal planes. In response to those anatomical specificities, we adjusted the point of entry approach and reamed the medullar canal. With these improvements, a relatively smooth implantation was achieved (Figure ).


A 64-year-old Chinese Han obese woman, height: 158cm and weight: 78kg, sustained a multifragmentary pertrochanteric hip fracture with two intermediate fragments (AO : A2.2). We opened the proximal femur and inserted a 12.5mm entry reamer to the greater trochanter. A less-than-ideal placement was achieved with the interlocking screw constructs. We tried to hammer the main pin deeper to get the perfect position for the interlocking screw constructs. During the procedure, an iatrogenic fracture of the proximal femur developed (Figure A, B). Based on our experience with obese patients we maintained a vertical trajectory approach during the nail insertion and fully reamed the medullar canal of the proximal femur, especially those with the anatomical specificity of an extremely narrow proximal canal (Figure C, D, E).


An 83-year-old Chinese Han woman, height: 177cm and weight: 70kg, sustained a multifragmentary pertrochanteric hip fracture with one intermediate fragment (lesser trochanter detachment) (AO of A2.1). After viewing the X-ray images during the preoperative measurement of the femoral medullary cavity, we did not expect there would be difficulties in implanting the INTERTAN™ nail (10mm in diameter) (Figure A, B). After a routine proximal canal reaming, we manually implanted the main pin and encountered resistance. We then hammered the main pin into the ideal position and performed the remaining steps. When the final X-rays were taken to confirm the positioning of the nail, an iatrogenic fracture of the distal femur was observed. The original short TRIGEN INTERTAN™ nail and the capturing locking screw were removed. Subsequently, a longer INTERTAN™ nail was implanted to fix the iatrogenic fracture (Figure C).


A 40-year-old Chinese Han woman, height: 175cm and weight: 72kg, sustained an intertrochanteric hip fracture (simple, oblique) (AO : A3.1). The reduction of the fracture was difficult due to comminuted fractures between the extended fragments. With the correction of multiple stresses by traction and manipulative reduction, as a result of the fractured fragments, the implantation of the main nail was inserted with difficulty. After the insertion of the integrated interlocking screw, we locked the distal slot. During the drilling, an iatrogenic fracture developed around the distal locking slot. An open reduction followed using a locking fixation plate to manage the femoral shaft fracture.


We evaluated a 41-year-old male presented in September 2004 with an episode of acute gastrointestinal bleeding, which was associated with non-Hodgkin's lymphoma with a nonspecific subtype. The patient lived in a remote rural area in Northern Brazil and was promptly transferred to a larger center to receive chemotherapy to avoid a surgical procedure.
He was treated with three cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), without clinical response. He was then submitted to a surgical procedure and the anatomopathological study of retroperitoneal lymph nodes was consistent with an infiltrative carcinoma. The immunohistochemical analysis revealed chromogranin and synaptophysin expression and also a Ki 67 < 3%. At this moment carcinoid related markers (glucagon, gastrine, prolactin, PTH, 5HIAA, and chromogranin A) were normal. Because of the discordance between the two biopsies regarding the histological diagnoses, a review was performed in both specimens and there were no histological findings suggesting lymphoma and the hypothesis of a low grade neuroendocrine tumor was confirmed. The patient was then treated with octreotide acetate until clinical recovery and then, without any clinical or radiological evidence of disease, treatment was withheld.
On December 2010, he presented with an abdominal obstructive syndrome and an exploratory laparotomy was necessary. The anatomopathological diagnosis of mesenteric lymph nodes resected was consistent with areas containing marginal zone B-cell lymphoma/chronic lymphocytic leukemia and neuroendocrine tumor. Bone marrow was not infiltrated and FDG-PET showed exclusively abdominal nodal disease which was compatible with lymphoma involvement. At this time he was treated with 6 cycles of CHOP and octreotide was started again. CT scan obtained at the end of the treatment showed complete response.
The patient's family history was revealed to be very unusual. He referred to the fact that his father had a prostate cancer and the majority (if not all) of the 9 father brothers presented lymphoma or leukemia. These data could not be double checked since these individuals have died many years before in very distant places, but the fact that the patient was able to describe a very unique type of cancer in his relatives, many of them with low level of literacy, suggested that the presence of lymphoproliferative disorders is quite common in the paternal side of the family.
At clinical examination the patient lacked any cutaneous stigma of neurofibromatosis, but one of the CT scans revealed the presence of various neurofibromas in the thorax and abdomen (). He was tested for NF1 gene mutations and chromosomal abnormalities encompassing part of the long arm of chromosome 17 containing the NF1 gene.
The coding region of the NF-1 gene was analyzed by real-time polymerase chain reaction (RT-PCR) and direct sequencing. Multiplex ligation-dependent probe amplification (MLPA) was also performed to detect the number of mutant copies (deletions and duplications).
The following alterations were met: duplication of NF1, TRAF4, and MYO1D. Additionally, fluorescence in situ hybridization (FISH) using probes (RP5-1002G3 and RP5-92689) flanking the NF1 gene in 17q11.2 and CEP17 for 17q11.11.1 was performed. There were three signals (RP5-1002G3conRP5-92689) in the interphases analyzed and two signals (RP5-1002G3conRP5-92689) in 93% of cells. Those findings suggest that the patient had a tandem duplication of 17q11.2.
On June 2013, the patient was submitted to routine laboratorial tests and a blood workout displayed a lymphocytosis (7500 lymphocytes/dL). The immunophenotype of the lymphocyte population was mostly composed of a clonal mature B lymphocytes positive for CD19, CD5, CD20, and CD23. These findings were consistent with CLL. FISH analysis displayed a del 17p13.1 ().
The patient has been in treatment with octreotide acetate, without signals of disease progression. CLL is under clinical surveillance and the patient is out of treatment in the moment.


The patient (#ID 9 in Table ) was an 89-year-old woman. She got married when she was 24 years old. Her husband was killed in World War II when she was 25 y. Thereafter, she obtained a hairdresser’s license and ran a barber shop with her nephew. When she was 84 years old, she had difficulty in financial management and closed her barber shop. When she was 86 years old, she had a difficulty in maintaining hygiene in the house. Since her room had become verminous, a public health nurse forced her to be placed in N2. Her MMSE score was 7. After living in N2, she had nothing to do and just spent her time watching TV.
Taking into consideration her past life history, we prepared hair mannequins for her rehabilitation. A session was made up of occupational therapy of hair dressing using hair mannequins, together with reality orientation and the reminiscence approach. We performed one hour per day per week for 3 months. At the beginning, she told us that she had never seen hair mannequins when they were initially presented to her. After administration of donepezil 5 mg/day combined with the rehabilitation, her attention was stimulated. After 2 months, she recalled that she had been a hairdresser and indulged in reminiscence about her past life. She claimed that other patients were lacking in sanitation. Although her MMSE score was not increased to 8, her Vitality Index increased dramatically from 1 to 6, with increases for all subscores. A physical therapist reported that she had become active and had more attention for the rehabilitation program of standing and gait. After three months, she was finally discharged from N2 and returned to her home and started to live together with her nephew.


A 40-year-old male was admitted to hospital because of chest discomfort and dyspnea. The patient had been well until 2 weeks before admission, when chest pain, cough, and dyspnea had developed. Two days before admission, he could not lie down because of dyspnea. He went to another hospital and a chest X-ray (CXR) was performed. A CXR revealed a widened mediastinum and he was referred to the emergency department of our hospital.
On examination, he appeared dyspneic and presented with orthopnea. His vital signs were normal. He had no peripheral lymphadenopathy. Laboratory tests on admission showed elevated lactate dehydrogenase level of 744 IU/L. The result of arterial blood gas was normal (). A CXR revealed a widened mediastinum and left pleural effusion () and contrast enhanced computed tomography (CT) of the chest showed a mediastinal mass of 12 cm by 7 cm, compressing the trachea (). A CT revealed no lymphadenopathy other than the mediastinal lesion.
On hospital day 2, tracheal intubation was performed by an anesthesiologist as the patient was considered to have high risk of suffocation. During the tracheal intubation, the left main bronchus was obstructed completely by a mediastinal tumor and the patient suddenly developed hypoxia. Oxygen was administered through a tracheal tube but the oxygen saturation was approximately 80 percent. ECMO was introduced immediately to recover oxygenation, placing a venous cannula in the right common femoral vein and an arterial cannula in the jugular vein. A CXR after intubation revealed atelectasis of the left lung (). After introducing ECMO, oxygen saturation was maintained at about 98 percent and PaO2 was 96 mmHg (). Percutaneous fine needle biopsy of the mediastinal tumor was performed and the patient was transferred to the intensive care unit. A provisional pathological diagnosis was LBL. On hospital day 3, chemotherapy, comprised of adriamycin, vincristine, cyclophosphamide, and prednisolone (CHOP), was started to debulk the mediastinal lesion.
After chemotherapy was started, the mediastinal mass shrank rapidly and oxygenation gradually improved. He was weaned from ECMO on hospital day 8 and weaned from the mechanical ventilation and was extubated on hospital day 28. He did not have any respiratory sequelae after extubation. The final pathological diagnosis was T-LBL. Chromosomal analysis did not reveal any abnormalities and an FISH of the BCR-ABL was negative. Acute Lymphoblastic Leukemia 202 protocol without imatinib (Japan Adult Leukemia Study Group) [] was started on hospital day 23 followed by subsequent consolidation chemotherapy. A CXR performed on day 71 shows disappearance of atelectasis and shrinkage of the mediastinal tumor (). A contrast enhanced CT of the chest performed on hospital day 87 confirmed partial remission of the tumor (). The patient's sister was confirmed as having full-matching HLA and the patient was referred to another hospital to receive allogeneic hematopoietic transplantation.
Adverse reactions during clinical courses were febrile neutropenia, acute kidney injury, which did not require renal replacement therapy, and right femoral subcutaneous abscess caused by cannulation for ECMO. He did not develop bleeding or thromboembolism as complications of ECMO.


A 65-year-old nonhypertensive, nondiabetic woman presented with generalized itching since 2 years. She developed rash on body with photo aggravation. She complained of muscle weakness for the last 2 years for which she had been taking treatment from a local doctor without being investigated. She had difficulty in standing from sitting position and climbing up the stairs as well as combing her hair. There was no history of dysphagia, oral ulcers, or joint pains. She had vaginal discharge for the last 6 months and vaginal bleeding of few days duration. She was nonalcoholic and nonsmoker. She achieved menopause 15 years back.
Examination revealed presence of heliotrope rash around eyelids characterized by periorbital, confluent, and violaceous erythema (). Poikilodermatous changes were evident on V-area of neck and dorsolateral aspects of bilateral forearms and shins (). Trunk was relatively spared. On examination of hands, hyperkeratotic lesions were seen predominantly involving the centre of palms (mechanics hands) along with presence of Gottron's papules on proximal interphalangeal joints. Muscle power was 2/5 in both upper and lower limb proximal muscle groups. Per vaginal examination revealed an exfoliative growth extending into upper half of vagina. On per speculum examination, growth was seen in vagina protruding out of external cervical os. About 5 × 6 cm mass at the level of cervix with involvement of bilateral parametrium just short of pelvic wall was felt on per rectum palpation. Clinical findings strongly suggested the possibility of underlying genital tract malignancy, so oncology consultation was sought.
Results of investigations showed Hb = 7.8 g/dL, total leukocyte count = 5.25 × 103/μL, LDH = 960 IU/L, s. creatine kinase = 18 IU/L, and negative ANA and anti-dsDNA.
Skin biopsy was consistent with DM (). Cervical biopsy showed keratinizing squamous cell carcinoma (SCC). CT scan of pelvis showed heterogeneous enhancing mass (69 × 89 mm) in the region of cervix and proximal part of vagina and distal part of body of uterus. Few soft tissue density lesions at both iliac fossa regions were seen. Chest X-ray and bone scintigraphy were normal. Thus, a diagnosis of keratinizing SCC uterine cervix stage III A with paraneoplastic DM was made for which she was started on PTF (Paclitaxel, Cisplatin, 5-Fluorouracil) chemotherapy followed by external beam radiation therapy. She was started on oral steroids (prednisolone 1 mg/kg), topical steroids, antihistamines, emollients, sunscreens, and physiotherapy for the treatment of dermatomyositis. After 6 weeks of treatment, she reported improvement in her muscle power without much improvement in dermatological manifestations. She also developed anagen effluvium secondary to chemotherapy and prominent follicular pluggings on scalp and forehead. After that, the patient was lost to follow-up.


A 10-year-old preadolescent girl incidentally presented with subclinical hypothyroidism. Ultrasonographic (USG) examination of the neck revealed the absence of a normally located thyroid gland (). Physical examination revealed that a 2-3 cm mass at the tongue base (). Thyroid function tests were in the normal range for free T3 and free T4 with elevated TSH of 32 IU/ml, compatible with subclinical hypothyroidism. A radionuclide thyroid scintigraphy with Technetium-99m (Tc-99m) pertechnetate showed two foci of intense uptake; one at the base of the tongue and the other in the infrahyoid region consistent with dual ectopic thyroid. There was no uptake of thyroid in the usual thyroid location in the neck (). The patient had congenital dual ectopic thyroid and subclinical hypothyroidism, so she was started on replacement therapy.
Literature Review and Discussion
The thyroid gland develops in the floor of the primitive foregut between the first and second pharyngeal pouches from the endoderm. It descends in the anterior neck to the level of the trachea and is connected to the tongue base by the thyroglossal duct. The thyroglossal duct normally involutes or sometimes develops into a pyramidal lobe which is contiguous with the thyroid isthmus. Most congenital thyroid anomalies result from defective migration to the pretracheal position or incomplete obliteration of the thyroglossal duct. Ectopic thyroid tissue involves aberrant embryogenesis of the thyroid gland during its passage from the floor of the primitive foregut to the pretracheal position. Prevalence of ectopic thyroid gland is about 1 per 100.000-300.000 people and 1 per 4.000-8.000 patients with thyroid disease (). 90% of undescended thyroid tissue occurs at the base of the tongue and is called lingual thyroid (). Ectopic thyroid is a congenital anomaly that may present at any age and is usually detected during puberty or pregnancy because the increased demand for thyroid hormone elevates the TSH levels (). Ectopic thyroid tissue may be also seen in the mediastinum, trachea, lung, porta hepatis, duodenum, esophagus, heart and breast ().
Congenital hypothyroidism may be due to thyroid dysgenesis (ectopia, hypoplasia and agenesis) or occurs in eutopic glands. Subclinical hypothroidism is a clinic presantation of congenital hypothyroidism. In congenital hypothyroidism, thyroid scan with either Tc-99m perthecnetate or I-123 permits a precise characterization of the aetiology, which is important for genetic counselling and clinical management ().
Lingual thyroid usually presents as a nodular mass at the base of the tongue in the midline. The lingual thyroid is four times more common in females than in males (). Most patients with ectopic thyroid tissue are usually asymptomatic and thyroid hormone levels are normal. Major symptoms are dysphagia, dyspnea, foreign body sensation and bleeding (). Hypothyroidism may be seen in up to 33% of cases (). Clinically, any disease that affects normal thyroid glands can also affect ectopic thyroid tissue (). Hyperplasia or inflammation may be also seen in lingual thyroid. Lingual thyroid should be evaluated with biopsy in cases of suspicious adenoma or carcinoma ().
Dual ectopic thyroid tissue is very uncommon and has been reported in 43 cases in the search of the Pubmed in English literature. The age range of these patients was between 4 and 71 years and it was more common in females than males. The first lesion is usually seen in the lingual or sublingual region and the second lesion is subhyoid, infrahyoid or suprahyoid (). Dual ectopic thyroid tissue with a normally located thyroid gland has been reported in only two cases in literature (,).
All the functional ectopic thyroid tissue can be determined by Tc-99m pertechnetate, I-131 or I-123 scintigraphy. To confirm diagnosis, scintigraphy is the most important method as it is a sensitive and specific imaging modality in the differential diagnosis of midline neck masses (). Lateral views should be taken in addition to the anterior view to ensure that other ectopic thyroid tissue is not overlooked. The lateral views are helpful in the determination of anatomical position, and also useful in the detection of multiple ectopic thyroid tissues (). Ectopic thyroid tissue may mimic thyroglossal duct cyst, but thyroid scintigraphy can differentiate these entities. Thyroglossal duct cysts do not contain enough ectopic follicular thyroid cells, so they are not detectable with scintigraphy (,). Uptake by salivary glands and nasal mucosa may cause physiological false positive scans, whereas meningioma, dacro-cystitis, prosthetic eye, sinusitis, dental pathologies and sialo-adenitis may show Tc-99m pertechnetate uptake and cause pathological false positive scans (15). For a decision of complete excision of lingual thyroid or transplantation of the ectopic tissue, thyroid scintigraphy is the method of the choice for the visualisation of normal thyroid tissue ().
The normal thyroid gland is absent in about 70% of patients with lingual thyroid (). Therefore, if surgical removal of the ectopic tissue is planned, the normal thyroid gland should be evaluated. In the case reported here there was no normal thyroid gland either on scintigraphy or in US examinations. Ectopic thyroid may be confused with oropharyngeal salivary gland. In our case the patient had subclinical hypothyroidism, there should be a thyroid tissue that produces thyroid hormone and so two foci of tissue are thought to be thyroid tissue.
There is no consensus on the optimal therapeutic strategy for ectopic thyroid tissue because of the rarity of this clinical entity. Surgical treatment is planned according to the age, size, local symptoms, functional thyroid status and complications. Complete surgical resection may be preferred if malignant transformation is suspected. Otherwise, follow up is suggested due to mass enlargement or complications in cases of asymptomatic and euthyroid status. For hypothyroid cases with mild symptoms, levothyroxine replacement may be effective and provides mass reduction (). In the case reported here, the patient wasn’t operated and it was decided to follow-up the patient with levothyroxine replacement therapy due to elevated TSH unless any symptoms or complications occurred. In cases of subclinical hypothyroidism and nonvisualization of the thyroid gland by USG, thyroid scintigraphy is indicated for ectopic thyroid tissue.
Conflicts of Interest
There are no conflicts of interest.