An 80-year-old woman presented after a referral from her GP with chest pain and vomiting after mild exertion. Chest pain was reported to attenuate with rest after 30 minutes. Approximately three cups worth of vomit was produced, some redness in coloration, but was attributed to red wine consumed at lunch. Her chest pain was of band distribution around the chest, came on after vomiting, was six out of ten in severity and settled after resting for a few hours. Relevant negatives included no shortness of breath, calf-swelling, altered sense of taste, or change to exercise tolerance. Of note, the patient reported a similar episode of pain three months prior, with the family practitioner concluding this was musculoskeletal pain. Her past medical history was significant for rheumatic fever aged six, and an un-repaired hiatus hernia. She was on no regular medications, with no known drug allergies. Her family history was significant for diabetes mellitus on the paternal side, and ischemic stroke on the maternal side. Generally, she maintains activities of daily living well, with a normal walking tolerance of over two miles. On examination, there was a collapsing pulse, with no other significant findings.
Based on this initial history and presentation the following differential diagnoses were considered. Firstly, MI due to chest pain and vomiting present in history, rendering it vital to exclude. Stable angina due to chest pain on exertion, which was relieved after rest. Pulmonary embolism due to sudden onset of chest pain, however, no association of shortness of breath. Rheumatic heart fever due to the past medical history of rheumatic fever as a child. Rheumatic heart fever was mentioned in her history and was an important background for the cardiovascular examination. Collections of granulomatous tissue can lead to a build-up of fibrotic connective tissue on the heart valves.
Her blood results showed a Troponin T (968 ng/L). All other blood results were within normal ranges. Electrocardiogram (EKG or ECG) showed ST-segment elevation in leads V3 and V4 with no further identifiable pathology. Figure shows the full ECG on admission.
Her echocardiogram was performed after her initial management was completed. Figure shows an image of the echocardiogram on admission. It showed akinesia of inferior, posterior, and infero-lateral segments with a severely akinetic apex. Left-ventricular ejection fraction (LVEF) was 25%, with aortic regurgitation present. Her coronary angiography was performed after her initial management was completed and demonstrated normal arteries throughout with some mild plaques.
Based on the presentation, our patient was started on aspirin, fondaparinux, omeprazole, ticagrelor, bisoprolol, isosorbide mononitrate, atorvastatin, ramipril, and spironolactone.
Medical management was decided as per hospital trust guidelines which stratifies patients based on GRACE score, with our patient being deemed high risk (calculated six-month mortality risk of 24%). Our patient also reported having symptoms of heart failure (in particular, peripheral edema) shortly after admission which was the rationale behind the use of spironolactone. The patient was stable and discharged before a definitive diagnosis was made, with a follow up in an outpatient clinic.
Definitive diagnosis
After the results of the coronary angiogram showed normal arteries throughout, our differentials changed significantly. Whilst the ECG indicated STEMI, the results of the coronary angiogram did not align with this. On consideration, transient coronary ischemia would lead to ECG changes, however, not enough plaques seen for this to be likely. Pericarditis could explain a clear coronary angiography, but the absence of ECG changes indicates otherwise. Syndrome X was a potential differential due to angina symptoms with normal coronary vasculature, however, unlikely given the repeat echocardiogram. On balance, the most likely diagnosis given all evidence was TC.
Our patient was followed up in an outpatient clinic to assess her response. At follow-up, our patient's repeat echocardiogram demonstrated significant improvement, with a left ventricular ejection fraction of 64% (improved from 25% on admission). Figure shows an image of the echocardiogram at follow-up. The seemingly transient nature of the reduced cardiac function solidifies the diagnosis of TC in contrast to other differentials.
Also known as stress cardiomyopathy, or broken-heart syndrome, TC is a condition where there is a transient weakening of the heart muscle, often triggered by emotional stress.


A 65-year-old man presented with a mass on the left upper eyelid that had been progressively enlarging for the past seven years. A pedunculated growth measuring 20 12 mm was observed involving the medial half of the eyelid. The mass was huge, with superficial ulceration and a red base with sharply defined indurated borders. It was pedunculated, such that the base of the mass was smaller than its superficial appearance. He was diagnosed with papilloulcerative squamous cell carcinoma [Figure 1]. The mass was excised with a 4-mm free margin, creating a defect measuring 8 6 cm, which was reconstructed using a Cutler–Beard flap. The flap was prepared from the inferior eyelid below the tarsus to ensure compatibility with the superior eyelid defect by making a horizontal incision 4 mm below the lid margin. The bridge flap was advanced to the superior eyelid below the eyelash margin. The anterior and posterior lamellae were sutured separately to the levator muscle and the rest of the orbicularis muscle by placing the flap on the defect area [Figure 2(a)]. Second stage surgery was performed four weeks postoperatively to separate the superior and inferior eyelids and reconstruct the upper lid margin [Figure 2(b)].
Histopathological examination revealed no atypia; rather, the lesion was composed of papillae with vascularized connective tissue covered by acanthotic epithelium. Hence, it was diagnosed as ulcerative squamous cell papilloma [Figure 3]. A repeat examination with repeat tissue cuts and histopathology slide preparation performed by another pathologist did not change the diagnosis. The patient was followed-up for 30 months and no recurrence was observed.


A 46-year-old man with a history of dog-bite in his left hand 3 months ago, developed fever (body temperature > 38 °C), visual disturbance, and dyspnea at rest. He had been otherwise healthy without significant medical history. He was tachycardic, and coarse crackle and diastolic heart murmur (Levine III) was audible. Laboratory test results were as follows: white blood cell count, 10,500/μL (59.8% neutrophils); hemoglobin level, 11.7 g/dL; brain natriuretic protein level, 689.2 pg/mL; and C-reactive protein level, 9.0 mg/dL (Table ). Chest X ray showed pulmonary congestion and bilateral pleural effusion. Brain magnetic resonance imaging revealed no lesion in optic nerve and brain. Transthoracic echocardiography revealed moderate-to-severe aortic valve regurgitation and vegetation of 17-mm in size (Fig. ). Seven days later, blood culture yielded Coagulase-negative staphylococci in one of four culture bottles. Although diagnosis of IE was not definitive according to Duke criteria [], history of dog bites, his clinical course, and imaging studies suggested Staphylococcal IE. Following administration of cefazolin 6 g/day and gentamycin 3 mg/kg/day for a week, the patient underwent aortic valve replacement and resected aortic valve was negative for Staphylococci. A week following surgery, however, microorganism grew in two bottles of preoperative blood culture. This microorganism was cultured on blood agar, and gram staining of the colonies showed Capnocytophaga-like gram-negative bacilli (Fig. ). 16S ribosome DNA sequencing both from blood and from resected heart valve identified C. canimorsus. Disk diffusion test revealed that the isolate was susceptible to almost all antimicrobial agents and did not produce β-lactamase (Table ). The protocol of the disk diffusion test was as follows: A Brucella HK agar plate was seeded with a lawn of C. canimorsus using sterile cotton swabs. For the plate, antibiotic disks containing 10 IU of penicillin G, 10 μg/10 μg of sulbactam/ampicillin, 10 μg/100 μg of tazobactam/piperacillin, 30 μg of ceftriaxone, 10 μg of meropenem, 10 μg of gentamycin, 30 μg of amikacin, 5 μg of levofloxacin, 30 μg of minocycline, 250 μg of sulfamethoxazole/trimethoprim, 15 μg of clarithromycin, 2 μg of clindamycin were used with BD Sensi-Disc (BD Bioscience Co., USA) and dispensed on the agar surface. Both plates were incubated at 30 °C overnight and the diameter of each zone was measured in millimeters to evaluate susceptibility or resistance using the comparative standard method.
Based on these results and symptoms, empirically selected combination of gentamycin and cefazolin was converted to ceftriaxone 4 g/day. The patient completed a total of 4 weeks of ceftriaxone. The patient has been doing well for 12 months after hospital discharge.


The patient is a 60-year-old married Caucasian female who presented in the emergency room of a local community hospital with a chief complaint of acute memory loss. She reported being confused for the past two days. She had no recollection about what has transpired the past two days but she remembered the details of her identity and that of her family. She could not recall the circumstances that brought her to the hospital, the name and dosages of her medications, her work history, or her medical history. The patient denied abusing any illicit drugs. She could not identify neighbors who visited her. She reported to her husband that she was not anxious about the memory loss. Her husband was worried that she was having a stroke, so they rushed to the hospital emergency room. Patient struggled to remember details of her morning routine, including her jogging at the neighborhood park, despite the husband's insistence that they ran together. Further inquiry revealed that the patient had attended a recent psychiatric appointment. She had an appointment with her outpatient psychotherapist three days before the acute onset of the memory loss. During that appointment, they discussed the person who physically assaulted her five years ago. She was a teacher in a special education residential program where one of her students with autism spectrum disorder punched her repeatedly on the head until she lost consciousness. Since the assault, the patient has been complaining of nightmares and flashbacks about the attack. She became socially withdrawn.
After the psychotherapist appointment, there was an exacerbation of the patient's anxiety symptoms leading up to the memory loss. There was no loss of consciousness during this period. During the psychiatric evaluation, the patient became tearful when discussing the details of the beating she endured. She described vivid memories of the assault as well as unpleasant dreams related to it. She gets particularly anxious whenever she sees a person that resembles her attacker. She could not perform the task of immediate retention and recall. The patient only identified one out of three words after five minutes. She could not complete her other cognitive examinations because she became tearful and requested to end it. The patient's husband reported that she was diagnosed with post-traumatic stress disorder (PTSD) three years ago and has since been treated with sertraline 100 mg per day. She is also being treated with cognitive-behavioral therapy.
This is reportedly the first time she has experienced memory loss. She denied any symptoms of depression, suicidal thoughts, mania, delusions, or hallucinations. There was no family psychiatric history. Mental status examination described a patient who looked appropriate to her stated age, dressed in a hospital gown, and was calm and cooperative with good eye contact during the psychiatric evaluation. There was no agitation observed. Her speech was coherent and goal-directed. She appeared anxious and restless; her mood was "stressed" and her affect was anxious. The patient was ambulating with good muscle strength. There was no aphasia or apraxia. Preliminary work-up showed a complete blood count with a white blood cell count of 5,900 cells per cubic millimeter of blood. Her basic metabolic panel showed serum sodium of 144 mEq/L. The urine toxicology screen was negative for illicit drugs. Magnetic resonance imaging (MRI) of the brain without contrast, magnetic resonance angiography of the circle of Willis, and a computed tomography (CT) scan of the head without contrast showed no acute intracranial pathology. After 48 hours, her cognitive symptoms improved. There was no anti-coagulation treatment. Her vital signs were stable and her 24-hour electroencephalogram monitoring showed no abnormalities. She was discharged home after 48 hours of observation with no cognitive problems.


In December 2017, a 73-year-old man was admitted with a 2-month history of pain in the back and right side of the chest associated with dry cough, and inability to walk for the last 1 month. He had a 50-year history of smoking and a 10-year history of chronic obstructive pulmonary disease (COPD). He was diagnosed with pulmonary TB based on positive T-spot and Xpert tests and computed tomography (CT)-scans at a local hospital 1 month ago. He was administered anti-TB treatment for 1month, comprising the empirical therapy using pyrazinamide, isoniazid, rifampicin, and ethambutol. Recent signs were weight loss and severe asthenia.
To investigate the patient's pain, a chest CT scan was performed, which revealed significant osteolytic destruction at the bodies of the 5th and 6th thoracic vertebrae and thoracic pedicles, with swelling of the soft tissue (Fig. ). It was suspected that the destruction and the swelling of soft tissue were due to TB with cold abscess, resulting in paraplegia. The chest scan showed tiny, wide spread, and discrete pulmonary opacities with bilateral pleural effusion, and a lump in the lower lobe of the left lung which was suspected to be an inflammatory granuloma. There was 1 thoracic aortic aneurysm (116 mm × 40 mm) at the aortic arch, 1 saccular pseudoaneurysm (67 mm × 32 mm) in the descending thoracic aorta, another saccular pseudoaneurysm (69 mm × 34 mm) in the descending thoracic aorta above celiac trunk, 1 abdominal aortic aneurysm (112 mm × 33 mm) and 1 right iliac aneurysm (58 mm × 16 mm) (Fig. ). A diagnosis of multiple tuberculous mycotic aneurysms of the aorta was made. Considering the patient's condition, surgery was not attempted. He underwent endovascular repair using microcore stent graft at our hospital. The surgical procedures are described below. The femoral artery was incised, and a microcore stent was implanted into the diseased artery. The stent covered a region ranging from the proximal normal aortic docking site to the site above the celiac trunk. The stent was not allowed to cover the openings of the major branches of the abdominal aorta, such as the celiac trunk, superior mesenteric artery, and bilateral renal arteries. Since the aneurysm involved important branches of the abdominal aorta, provisional observation was necessary. The procedures were performed under general anesthesia. Hemodynamic imaging of the distribution of wall pressure of blood flow in the aorta using computational fluid dynamics (CFD), was obtained before and after surgery in accordance with the preoperative and postoperative CT images in the digital imaging and communications in medicine (DICOM) format. On post-operative day 14, the patient was discharged without complications. The patient was in a stable condition and was transferred to a local hospital for continuation of the treatment of pulmonary infection and TB. Following transfer to the local hospital, the respiratory function deteriorated due to aggravated pulmonary infection. The patient was admitted to the Intensive Care Unit for type 1 respiratory failure and pulmonary infection. However, he could not survive the infection.


Case 1: A 10-year-old girl was diagnosed as having scoliosis with Prader Willi syndrome at the age of 2 years. She received brace treatment from 5 to 10 years of age. We performed stage 1 posterior short fusion from T7 to L1 for her preoperative major curve Cobb angle of 100 degrees and angle of trunk rotation (ATR) of 40 degrees at the age of 10 years. Preoperative height was 117 cm, weight was 25.3 kg, and body mass index (BMI) was 18.5 kg/m2. Her height was equivalent to that of a child of 6 years and 8 months. Although, Y cartilage was closed, Risser grade was 0 and she was before the first menstruation before the first surgery. So, we judged that there was a possibility of growing yet, and 2 staged surgery was applied. Surgical time was 205 minutes and blood loss volume was 150 g. Postoperative Cobb angle of the major curve improved to 63 degrees. Postoperative ATR improved to 13 degrees. Postoperative brace therapy was continued to prevent a crank shaft phenomenon. We performed stage 2 posterior spinal fusion from T2 to L4 for her preoperative major curve Cobb angle of 89 degrees and ATR of 28 degrees at the age of 14 years. Preoperative height was 122 cm, weight was 38.8 kg, and BMI was 26.0 kg/m2. Her height was equivalent to that of a child of 7 years and 7 months. Risser grade was 3 before the second surgery. Surgical time was 316 minutes and blood loss volume was 800 g. Postoperative Cobb angle of the major curve improved to 46 degrees. Postoperative ATR improved to 20 degrees. Thoracic height (T1-12) had improved from 160 to 206 mm and spinal length (T1-S1) had increased from 240 to 320 mm at the final follow-up (Fig. ). Forced vital capacity improved from 0.5 L before surgery to 0.88 L afterwards. Preoperative SRS-22 domain scores were 4.2, 4.0, 2.0, 4.4, and 3.65 for function, pain, self-image, mental health, and subtotal, respectively, which were ameliorated at the final follow-up at 4.0, 4.6, 2.8, 4.2, and 3.9, respectively. There were no severe perioperative complications.


Case 2: An 11-year-old boy was diagnosed as having scoliosis with 22q11.2 deletion syndrome at the age of 4 years. He received brace treatment from 4 to 11 years of age. We performed stage 1 posterior short fusion from T7 to L1 for his preoperative major curve Cobb angle of 77 degrees and ATR of 27 degrees at the age of 11 years. Preoperative height was 114 cm, weight was 17.0 kg, and BMI was 13.1 kg/m2. His height was equivalent to that of a child of 6 years and 1 month. Risser grade was 0 before the first surgery. Surgical time was 132 minutes and blood loss volume was 100 g. Postoperative Cobb angle of the major curve improved to 52 degrees. Postoperative ATR improved to 16 degrees. Postoperative brace treatment was continued to prevent a crank shaft phenomenon. We performed stage 2 posterior spinal fusion from T3 to L4 for his preoperative major curve Cobb angle of 81 degrees and ATR of 28 degrees at the age of 15 years. Preoperative height was 123 cm, weight 24.6 was kg, and BMI was 16.2 kg/m2. His height was equivalent to that of a child of 7 years and 7 months. Risser grade was 0 before the second surgery. Surgical time was 279 minutes and blood loss volume was 1330 g. Postoperative Cobb angle of the major curve improved to 48 degrees. Postoperative ATR improved to 22 degrees. Thoracic height had improved from 148 to 211 mm and spinal length had increased from 275 to 352 mm at the final follow-up (Fig. ). Forced vital capacity improved from 0.38 L before surgery to 0.62 L afterwards. Preoperative SRS-22 domain scores were 3.8, 4.2, 3.0, 2.2, and 3.3 for function, pain, self-image, mental health, and subtotal, respectively, which were improved at the final follow-up at 3.4, 4.2, 4.4, 3.4, and 3.85, respectively. There were no severe perioperative complications.


A 64-year-old Japanese male patient was referred to our hospital to treat a floating mass of the right atrium (RA). He had undergone resection of sigmoid cancer 5 years previously and endoscopic submucosal dissection for early carcinoma of the esophagus 5 years ago. Follow-up computed tomography (CT) revealed a mass of 45 × 27 mm in the RA with a low-density area (Fig. a). Preoperative transthoracic echocardiography revealed a round mass with low echo density on the free wall of the RA (Fig. b), which was suspected to represent thrombosis, cardiac myxoma, or a relapsed metastatic tumor. Although the patient was asymptomatic, we planned to urgently resect the floating mass on the next day of admission due to the risk of pulmonary embolism.
The operation was performed under cardiopulmonary bypass. Normothermic cardiopulmonary bypass was established by using an arterial cannula to the ascending aorta and two venous cannulas into the superior vena cava and inferior vena cava with a left ventricular venting cannula through the right upper pulmonary vein. The ascending aorta was cross-clamped, and the heart was arrested with cold blood cardioplegia.
We observed a large, pedunculated, relative tense mass of 45 mm × 27 mm in size on the free wall of the RA. We could directly identify a small part of the mass protruding though the RA wall, which was connected to the RA wall (Fig. a). We resected the tumor with at least 1.5 cm of the RA wall as a margin (Fig. b). We added tricuspid annuloplasty with Carpenter Edwards Physio-ring II of 28 mm (Edwards Lifesciences Corporation, CA, USA) for tricuspid valve regurgitation, and the left atrial appendage was closed with a 40-mm AtriClip (AtriCure, Inc., Mason, OH, USA) and bilateral pulmonary vein isolation by an AtriCure (AtriCure, Inc.) to prevent thrombosis formation due to chronic atrial fibrillation. The operation time, cardiopulmonary bypass time, and aorta cross-clamp time were 317 min, 145 min, and 88 min, respectively. The patient recovered uneventfully and returned to home on foot on the 13th day after the operation.
The postoperative pathological diagnosis of the mass was compatible with a primitive neuroectodermal tumor (PNET), a form of Ewing’s sarcoma. Atypical cells with small oval nuclei propagated into a micropapillary form with a partial rosette formation (Fig. a, b); some parts contained necrotized tissue. These cells were positive for CD56 (Fig. c), CD99 (Fig. d), and Vimentin and negative for S-100 and Desmin. No malignant cells were observed in the margin of the resected RA wall. Although the sarcoma was completely resected, he was transferred to another hospital to undergo adjuvant postoperative chemotherapy and radiotherapy to improve the prognosis by preventing subclinical micrometastasis. The final diagnosis was primary cardiac Ewing’s sarcoma because no primary lesions were observed in other organs on fludeoxyglucose-positron emission tomography (FDG-PET) or bone scintigraphy, which was performed in another hospital. As the adjuvant postoperative chemotherapy protocol, he received VDC-IE therapy as follows: VDC with vincristine 1.4 mg/m2 (d1), doxorubicin 37.5 mg/m2 (d1–2), and cyclophosphamide 1200 mg/m2 (1d); and IE with ifosfamide 1.8 g/m2 (d1–5) and etoposide 100 mg/m2 (d1–5). VDC-IE therapy was the same as that for born-derived Ewing’s sarcoma. He had experienced no recurrence as of 2 months after the operation.


An 8-month-old girl presented with signs and symptoms of obstructive jaundice, with an associated history of progressive abdominal distension. Serology revealed elevated liver ductal and transaminase enzymes with conjugated hyperbilirubinaemia, normocytic anaemia, thrombocytopenia and coagulation abnormalities. The liver function test results were as follows: total bilirubin 92 µmol/L (normal range 0–21), conjugated bilirubin 86 µmol/L (normal range 0–6), gamma-glutamyltransferase (GGT) 967 U/L (normal range 1–39), alkaline phosphatase (ALP) 1117 U/L (normal range 124–341), alanine transaminase (ALT) 134 U/L (normal range 3–30) and aspartate transaminase (AST) 99 U/L (normal range 0–79). The haemoglobin level was 9.0 g/dL (normal range 10.5–13.7), mean corpuscular volume (MCV) 84.4 fl (normal range 70.0–86.0) and platelets 21 ×109/L (normal range 180–440).
The abdominal ultrasound (US) demonstrated a bulky, homogeneous, soft tissue mass in the head of the pancreas that was isoechoeic to the remainder of the pancreatic gland, with associated findings of dilated intrahepatic bile ducts and ascites. Internal flow was demonstrated within this lesion on colour flow Doppler assessment.
This was followed by a computed tomography (CT) scan of the abdomen and pelvis with an intravenous and oral contrast agent. Computed tomography imaging showed an ill-defined, enhancing mass in the head of the pancreas (–), with a poor plane of separation from the D2 segment of the duodenum ( and ). Enhancing soft tissue, which extended beyond the confines of the pancreatic head into the region of the porta hepatis, was also noted and appeared to be contiguous with this mass ().
The parents of the child decided to use a traditional healer, which delayed the magnetic resonance imaging (MRI) of the abdomen by 2 months. The MRI abdomen demonstrated a pancreatic mass of poorly defined margins that had progressively enlarged and extended across tissue planes ( and ). There was encasement of the superior mesenteric artery as demonstrated by the sandwich sign ( and ), peripancreatic extension into the porta hepatis () and also intrahepatic bile duct dilatation ().
The differential diagnosis based on the imaging studies was that of a pancreatic vascular neoplasm; however, with the laboratory evidence of Kasabach–Merritt phenomenon (KMP), this prompted the diagnosis of a pancreatic KHE. Given the large size and peripancreatic extension of this lesion, a differential diagnosis of pancreaticoblastoma was considered; however, these lesions are typically heterogeneous in appearance with both cystic and solid components and often have calcifications.
After correction of the patient’s thrombocytopenia, an open biopsy of the pancreatic mass was taken at laparotomy. Macroscopy of the specimen demonstrated a dark brown wedge of tissue measuring 10 mm × 5 mm× 6 mm. Microscopy () showed representation of pancreatic parenchyma, which contained a lobulated spindle cell neoplasm with slit-like vascular spaces, extravasated red blood cells, haemosiderin and hyaline globules. There was no necrosis and mitotic activity was 0 in 10 consecutive high-power fields (HPFs). No plasma cell infiltrate, no gland or acinus formation and no squamous corpuscles or nests were noted. In the presence of adequate controls, the following immunohistochemical stains were performed: cytokeratin AE1/3 negative, strongly positive CD34 and negative human herpes virus 8 (HH8). Based on the above findings, the anatomical pathology report was indicative of a KHE.
Although the ascites had improved, the jaundice and thrombocytopenia had become progressively worse. The patient was treated with interferon alpha but subsequently demised.


A 55-year-old man was admitted to the Cardiovascular Surgery Department of our hospital for CAA detected by transthoracic echocardiography (TTE) during a routine physical examination. He had neither symptoms of cardiac diseases such as dyspnea, fatigue, chest distress, angina or arrhythmia nor family history of any cardiac diseases. Physical examination showed his heart rate was 78 beats per minute and the blood pressure was 138/80 mmHg. Grade III to-and-fro murmur was heard over the second intercostal space of his left sternal margin. Electrocardiogram (ECG) showed normal sinus rhythm and no significant ST segment or T wave anomaly was found. Chest radiography showed enlarged heart shadow. TTE demonstrated a giant right coronary artery (RCA) aneurysm with a fistula to the right atrium (RA), and the ejection fraction(EF) was 58% (Fig. a, b). The diameter of the RCA increased to 11 mm. Coronary artery computed tomography (CT) revealed a giant aneurysm (65*48 mm) originating from the distal RCA and draining into the RA (Fig. a, b, c). The results of coronary angiography (CAG) were consistent with the above results. Considering the giant size of the aneurysm, surgery was needed as early as possible. Median sternotomy revealed tortuous and dilated RCA, as well as aneurysmal dilation behind the right ventricle (Fig. a, b); a lateral branch originating from the acute marginal branch (AMB) of the RCA and being connected to the aneurysm. Since the surgery aimed to decompress the aneurysm and eliminate the shunt at the fistula site, identifying the entrance and exit vessels of the aneurysm was of vital necessity. To reduce the blood flow and pressure by closing the entrance vessel as much as possible without causing myocardial ischemia, the proximal RCA was ligated first. But intraoperative ECG monitoring showed elevated ST segment, ischemic myocardium and weakening contraction of the right ventricle (RV). Hence, the ligation was performed 3 cm from the distal end of the AMB. After ligation, no elevated ST segment was found. Transesophageal echocardiography (TEE) showed no ventricular wall motion abnormalities and the aneurysm was obviously downsized. The fistula was then occluded through the RA under TEE guidance in the absence of CPB. First, the guide wire and catheter sheath were inserted and passed through the fistula. Then a 30 mm atrial septal defect (ASD) occluder (BEIJING HUAYISHENGJIE) was applied to achieve maximal closure of the aneurysm (Fig. ). TEE showed the size of the aneurysm was obviously reduced, and no blood flow signals, ventricular wall motion abnormalities or shunt were observed (Fig. c, d). The patient was discharged 9 days after operation. Aspirin and clopidogrel were administered for 3 months. The follow-up revealed favorable outcomes and an improved quality of life for the patient.


A 62-year-old female with a medical history of asthma was brought to the emergency department for a week-long symptom of shortness of breath. According to her medical records, she had been diagnosed with pulmonary embolism and deep vein thrombosis 13 years ago. She was then treated with enoxaparin and warfarin and was later discharged with regular followed up in our cardiovascular outpatient clinic. Asthma was diagnosed based on an obstructive airflow limitation accompanied by positive bronchodilator test and symptoms. Her asthma was controlled by long-term medications of Symbicort Turbuhaler, two puffs twice per day, and Spiriva Respimat, two puffs per day according to the Global Initiative for Asthma (GINA) guideline. This time, she suffered from shortness of breath for a week and returned to our emergency department. Physical examinations showed tachycardia (101/min) and hypertension (173/104), and her body temperature was 37.4 °C. Her D-dimer level was 2.64 mg/L and troponin level was within the normal range. Electrocardiography revealed sinus tachycardia, and S1Q3T3 pattern was recorded (). No abnormal findings were obtained in her chest roentgenography. Computed tomography angiography revealed thrombosis in bilateral pulmonary arteries, corresponding to massive pulmonary embolism (). Rivaroxaban was administered but her symptoms did not improve. As a result, she had received catheter-directed thrombolytic therapy by Ekosonic Endovascular System (EKOS, BTG International Ltd., London, United Kingdom), together with recombinant tissue plasminogen activator infusion. Symptoms thereafter were relieved and followed-up pulmonary artery angiography showed decreased thrombus burden and a drop in the pulmonary arterial pressure from 68 to 40 mmHg. We surveyed the etiology of recurrent pulmonary embolism including the following: Tumor markers, antinuclear antibodies, anticardiolipin antibodies, anti-beta 2 glycoproteins, lupus anticoagulant, protein C, protein S, antithrombin III, and the results were all were within normal ranges. Abdominal ultrasound also revealed no abnormality. The patient was then discharged under long-term medication of rivaroxaban. Written informed consent was obtained from the patient for this study.


A 28-year-old, previously healthy man was referred from the ward of otolaryngology, to our thoracic surgery department with dysphagia, muffled voice and swelling at his right sub mandibular area, 3days after blunt neck trauma. He complained from, dyspnea, dysphagia and pain in his right sub mandibular area and neck following falling of heavy bag (50 kg cement pocket) on his posterior neck. On clinical examination, swelling and subcutaneous emphysema was seen on admission day. He had not any dizziness or visual disturbance and his consciousness was normal. The first day after accident he had not serious symptoms except a moderate pain and tenderness on his neck but the pain and tenderness were increased, 24 h latter.
He did not have a history of a mass in his neck, dental abscess, diabetes mellitus, alcohol consumption, smoking or drug abuse.
On Physical examination: he had a blood pressure of 80/60 mm Hg, a Pulse rate of 130 beats/min, axillaries temperature of 38.4 °C, Respiratory rate was 35 breaths/min with dyspnea.
Tenderness, edema, erythema and emphysema was seen in the anterior of neck, but it was more significant in the right angle of mandible and para tracheal rather than the left.
Breath sound was diminished in both lungs fields but significantly was decreased in the right lung. The remainder of the examination revealed no pathologic findings.
Direct laryngoscopy, flexible bronchoscopy and esophagoscopy were performed showing no particular lesions.
Laboratory tests showed an elevated white blood cell (WBC) = 30,600/μl, low Hemoglobin (10.6 g/dL) and was elevated platelet count = 587 000 mm3. BUN = 50 mg/dL, Cr = 2.2 mg/dL, Na = 140 mg/dL, K 4 mg/dl, BS = 110 mg/dL, Urinalysis was normal but urine volume was diminished. An arterial blood gas analyzed in ICU with o2 supplement was as follows: pH = 7.36, PaO2 = 80 mmHg, PaCO2 = 50 mmHg and SaO2 = 90%. The electrocardiogram was normal, HIV test also was negative. Empirical broad-spectrum intravenous antibiotic (imipenem 500 mg/8 h + Ciprofloxacin 400 mg/12 h iv. and Metronidazole 500 mg/8 h) were prescribed till the results of culture to be obtained.
Coronal and sagittal reformat view of chest CT-scan of neck and thorax showed: pneunomomediastinum and emphysema in cervical space, extensive subcutaneous emphysema of the neck, around the right ramus of the mandible and right masseter muscle that extended into the left side of the neck and tracked down into the anterior mediastinum (). Computed tomography of thorax also revealed bilateral pleural effusion, more predominant at right side with loculated empyema. Cervical and dorsal vertebrae were intact (). The diagnosis of DNM was made on base of clinical findings, severe cervical and pharyngeal infection and radiologic features of mediastinitis on CT scan.
First, a 32 F chest tube was inserted in the right pleural cavity, approximately 500cc pus was drained and a sample for antibiogram was obtained. The patient was resuscitated and prepared for surgery after adequate urine output was achieved.
A right poster-lateral thoracotomy, through 5th intercostals space with endo tracheal general anesthesia was performed. During thoracotomy loculations and infected septa were disrupted and evacuated, the pleura of the upper esophagus was excised and the para esophageal planes opened until distal of esophagus. The mediastinum entirely was exposed, extensive debridement of necrotic tissue, irrigation of the paravertebral and para esophageal planes was done.
At the same time the neck was explored, copious amounts of pus and necrotic tissue in the submandibular triangle, parotid region, retro mandibular and pharyngeal spaces as much as possible was removed and irrigated with normal saline adequately, drainage was attained with three latex Penrose drains in different locations, then the patient was transferred to ICU with oral intubation. During18day of the post-operative period, the patient’s status despite of given high dose, antibiogram matched antibiotics and revealed signs of sepsis and pulmonary insufficiency. Bed side echocardiography showed pericardial thickening and effusion, repeated chest CT scan showed loculated empyema in left side with pericardial thickening (7 mm) without massive effusion or tamponade (). According to this finding, a left postero-lateral thoracotomy, through the 5th intercostal space was done and all of the loculations and infected spaces in pleura and mediastinum were evacuated.
Extensive debridement of the empyema and necrotic tissue was done in the same manner as on the right side, pericardia tap revealed serous fluid which was sent for culture. Recovery was relatively rapid after the 2th operation, the General condition improved rapidly, temperature became normal on the 4th post-operative days and the patients was extubated on the 6th post operative days.
The total duration of management was longed 29days. We also performed an upper gastrointestinal endoscopy but there were no pathological findings. Smear and culture from the pericardia tap were negative.
Culture of pleural fluid, revealed Poly microbial infection we did not perform a tracheotomy and weaned the patient from ventilator on 24th admission day then liquid diet began. The patient had no complaints of hoarseness or dysphagia after extubation.
Before the patient’s discharge from the hospital, a control thoracic CT-scan showed relatively complete resolution of the pneumomediastinum and subcutaneous emphysema.
Outcome was favorable, during 90 days follow up, the pericardial thickening became normal and no evidence of tracheal stricture was seen. A written informed consent was obtained from the patient for possible publication including images for this case report.
SCARE compliance: this work has been reported in line with the SCARE criteria [].


A 63-year-old female diabetic patient, presented in January 2017 (day 0) to the Dermatology out-patient department of Khabat Hospital with a dystrophic great left toenail, showing yellowish white to creamy discoloration () of 9 months duration (-9 months). There was a history of probable onychomycosis of her left great toenails (not verified by mycological examination) three years previously (-3 years), that was completely cured after treatment with antifungal clotrimazole cream 1% (fugidin cream, SDI) used 3times/day for exactly 2 months. The associated predisposing factor for this case was diabetes mellitus, and at the time of sampling, the patient had undergone diabetic medication. No history of any underlying disease was reported.
Nail scrapings and clippings from different parts of the affected toenail were collected on day 0 after proper sterilization of the affected area with 70% alcohol and transported in folded dry-square of paper envelope to the mycology research laboratory, Biology Department at Zakho University, for direct microscopic examination and culture processing []. On direct KOH examination, septate filaments and arthroconidia were observed. At +3 days Portions of the nail scrapping and clippings were cultured on two sets of Sabouraud's dextrose agar (SDA) medium (CM41; Oxoid, UK): two replicates of SDA plates with antibiotics and cycloheximide [Sigma-Aldrich] (500 mg/l), and one slant of SDA contained antibiotics but without cycloheximide, and incubated at 28 °C for at least 3 weeks. Mycological diagnosis was performed at +21 days. A mesh-like brown globose gymnothecia composed of network of thick-walled hyphae (reticuloperidium) related to onygenalean fungi were grew on both sets of media. No other fungi were detected on both sets of media. The onygenalean fungus was identified as a member of Auxarthron based on cultural and microscopic characteristics (, ) according to relevant references [, , , , ]. For accurate identification of the isolated causal agent, DNA sequencing, was performed.
Fungus genomic DNA was extracted from isolated fungus using liquid nitrogen and EZ-10 Spin Column Fungal Genomic DNA Mini-preps Kit, BIO BASIC INC., Canada, and the genomic DNA was analyzed by electrophoresis on 1.5% agarose gel. The ITS region on rDNA was amplified by using universal primers ITS1- (5'-TCCGTAGGTGAACCTGCGG-3') and ITS4 (5'-TCCTCCGCTTATTGATATGC-3') (MWG-Biotech AG, Germany) which yielded a PCR product size of 582bp. The PCR reaction was done in a GenAmp9700 thermal cycler (Perkin-Elmer) under the following conditions: an initial denaturation set up at 94 °C for 5 min. was followed by 35 cycles of denaturation at 94 °C for 1 min., annealing at 58 °C for 1 min. and extension at 72 °C for 1 min., with a final extension step of 72 °C for 10 min.
The sequencing was done at Macrogen company, Korea and the sequence was deposited in GenBank with accession no. MK112622 (ITS). For phylogenetic analysis, sequences of amplified product was retrieved in FASTA format from GenBank. Phylogenetic analyses using the Neighbour-Joining (NJ) method [] was performed with the MEGA v. X computer program []. The phylogenetic tree was constructed using the Kimura two-parameter distance model [] with the ‘pairwise deletion of gaps option’. The robustness of branches was assessed by bootstrap analysis with 1000 replicates.
At +22 days, the patient was treated with oral itraconazole 200 mg daily for 2 consecutive months. At +82 days, the patient visited the dermatology out-patient department, her toenail was not completely cured, yet, the direct microscopy and culture of nail clippings were negative for fungal elements. At +82 days, oral itraconazole (200 mg/day) in combination with topical fugidin cream (clotrimazole cream 1%) (SDI), twice per day were given for a further 2 months. The patient did not visit the dermatology out-patient department of Khabat hospital after the last therapy, for that reason the patient medical condition cannot be confirmed.


A 86-year-old woman presented with ventricular extrasystoles one year prior to her current admission. She was started on amiodarone 400 mg five days per week. Her thyroid hormone levels were normal prior to initiation of amiodarone. Six months prior to admission, she started complaining of cold intolerance, with bradycardia and myxedema accompanied by occasional vomiting and a change in her mental status. Upon admission, her blood pressure was 170/80 mmHg, body temperature 32.6ºC, and heart rate 40 bpm. The physical examination revealed stupor, myxedema, loss of the outer third of the eyebrows, and macroglossia. Bilateral lower limb edema was noted on examination. The remainder of the examination was non-contributory. Laboratory tests showed T4 0.2 mcg/dL (normal values: 4.5-12.5), thyroid stimulating hormone (TSH) > 60 mUI/l (normal values: 0.4-4), sodium 127 mEq/l, creatine phosphokinase (CPK) 736 U/l, glutamic oxalacetic transaminase (TGO) 122 U/l, lactate dehydrogenase (LDH) 954 U/l, and cholesterol 382 mg/dl. Blood gas analysis showed pH 7.35, paO2 49 mmHg, and pCO2 67 mmHg. Abdominal X-rays were consistent with the presence of paralytic ileus, and the ECG showed sinus bradycardia (40 bpm), QT 0.68 seconds, low voltages, and diffuse disorders of repolarization. Treatment with IV thyroxine hormone (250 mcg), hydrocortisone, and ventilatory support were initiated. During the hospitalization the patient developed refractory hypotension, and she died on day 2 from cardiac arrest nonresponsive to resuscitative efforts.


A 72-year-old woman was admitted to the emergency department for memory loss, behavioral disorders, and confusion for two weeks. Her medical history was positive for primary hypothyroidism treated with 100 mcg thyroxine daily which she had discontinued three months prior to admission. Upon arrival, her blood pressure was 150/90 mmHg, heart rate 50 bpm, and body temperature 35.1ºC. On physical examination the patient was confused and agitated. Myxedema, loss of the outer third of the eyebrows, and bilateral lower limb edema were noted on examination. Deep tendon reflexes were prolonged. Laboratory tests revealed T4 0.1 mcg/dL, TSH 80 mUI/l, and normal liver and renal function tests. White blood cell count, hemoglobin, and electrolytes were within the normal range. The patient received IV thyroxine and hydrocortisone with clinical and laboratory improvement.


A 86-year-old woman was admitted to the emergency department for evaluation of altered mental status. Her medical history was positive for systolic hypertension, obesity, and dyslipidemia. One year prior to her present admission, the patient presented with weight gain, loss of appetite, and labile mood. During the two weeks prior to admission, she became progressively more non-verbal with occasional confusion and daytime sleepiness. On the day of her admission she was nearly unresponsive. Her blood pressure was 160/60 mmHg; heart rate was 45 bpm, and body temperature 34ºC. Physical exam revealed drowsiness, myxedema, dry skin, and bilateral lower limb edema. The remainder of the physical exam was unremarkable. Laboratory tests showed T4 0.1 mcg/dl, TSH 55 mU/ml, cholesterol 285 mg/dl, triglycerides 119 mg/dl, aspartate aminotransferase (AST) 71 UI/l, alanine aminotransferase (ALT) 20 UI/l, CPK 1374 UI/L, LDH 612 U/L, sodium 117 mEq/L, potassium 2.7 mEq/l, calcium 7.19 mg/dl, and phosphorus 2.6 mg/dl. Antinuclear and antithyroglobulin antibodies were negative, but antithyroid peroxidase was 1/160. Sinus bradycardia was noted on the ECG. The patient was given IV thyroxine and hydrocortisone. On the second day of admission, the patient was clinically improved, and her blood tests showed improvement of the thyroid functions.


A 67-year-old man with a previous medical history of COPD and chronic liver disease was admitted to the emergency department for the evaluation of coma. Upon admission the patient was unresponsive. His blood pressure was 80/60, heart rate was 45 bpm, and body temperature was 33ºC. Laboratory tests showed undetectable T4 and TSH over 100 mU/ml. His hemoglobin was 7.8 g/dl, total cholesterol 203 mg/dl, and LDL 101 mg/dl. Electrolytes, renal, and liver tests were within the normal range. The patient received IV thyroxine and hydrocortisone. On the third day of admission, the patient was clinically improved and repeat laboratory tests showed T4 1.28 ng/dl and TSH 48.9 mU/ml.


An 88-year old woman presented with epigastric pain, nausea and hematemesis for the last 2 days, with a recurrent history of gastro-oesophageal reflux disease (GERD), and progressive food intolerance. She had a history of well-controlled hypertension, diabetes, and chronic obstructive pulmonary disease. Physical exam upon arriving to the emergency department revealed mild abdominal distention, with non-radiating epigastric pain, without rebound tenderness or guarding, while the rest of the physical exam was normal. EKG did not reveal any abnormalities. A chest X-ray showed clear lungs with a left thoracic opacity suggestive of a large hiatal hernia. The patient was admitted and an emergency upper gastrointestinal endoscopy was performed due to persisting hematemesis, showing a mild gastritis with a bleeding mucosal lesion in the migrated stomach body that was treated with hemoclips. Barium upper gastrointestinal series showed half the gastric body above the diaphragm in the right thorax, while the Gastro-oesophageal junction was also herniating into the chest, suggesting a type III mixed PEH. The distal oesophagus had a tortuous appearance, suggestive of a probable motor dysfunction. An abdominal CT-scan with IV injection confirmed the diagnosis of incomplete gastric volvulus associated with the PEH (). Due to persistent epigastric pain and food intolerance, the patient underwent a laparoscopic trans-hiatal hernia and volvulus reduction, with total excision of the hernia sac. Due to the patient’s advanced age and comorbidities, we performed a posterior cruroplasty without mesh reinforcement, associated to an anterior gastropexy with continuous running suture with barbed non-resorbable 2/0, anchoring 3/4th of the stomach to the anterolateral abdominal muscles (). The patient tolerated the procedure very well, and had an uneventful 5 days hospital stay with progressive mixed diet. She was seen at 1 and 6 months of follow-up, confirming the complete resolution of her abdominal symptoms, without GERD and a much better food tolerance.


A 91-year old man presented with severe dyspnea and fever for the last two days. He had a history of controlled diabetes and hypertension with heart failure. He notes persisting vomiting and food intolerance for the last 7 days. Physical examination revealed absent sounds on both lungs, with a normal abdominal exam. CXR revealed bilateral pleural effusions, left pneumonia, with a left thoracic opacity, suggestive of a large PEH. He was admitted on midcare cardiology unit for medical conservative treatment including a nasogastric decompressive tube, resuscitation and intra-venous antibiotics. A thoraco-abdominal CT-scan showed a giant paraesophageal hernia with complete intramediastinal gastric volvulus (). After a multidisciplinary discussion, the surgical volvulus reduction was approved and consented, as the mainstay treatment for the patient. The family members were informed and consented on the potential risks and benefits of the procedure. The patient underwent an emergency laparoscopic abdominal trans-hiatal hernia reduction, including complete sac excision, which was tightly adherent to the posterior mediastinum and needed a perioperative guided gastroscopy for the oesogastric junction, with placement of a decompressive nasogastric tube. After complete gastric reduction and sac excision we performed a posterior cruroplasty reinforced with two ePTFE large strips (). We also performed an anterior gastropexy with continuous barbed running suture 2/0. A mediastinal drain 16Fr. was placed. The patient was hemodynamically stable throughout the whole procedure. The patient was placed in the intensive care unit, where he was stable without any surgical complication since both drain and abdomen were clear. Unfortunately, he developed an acute respiratory failure syndrome, and died on the 4th post-op. day.


The patient was a 70-year-old female with continued right knee rigidity and pain after right TKA. Four years earlier, she was diagnosed with severe osteoarthritis of the right knee at another hospital, and TKA was performed uneventfully. She recovered well in a couple of months after surgery, but then she began to feel sharp pain in the medial part of the right knee, and as a result experienced a decrease in the range of motion (ROM). In subsequent years, her right knee pain and rigidity gradually increased. Treatments including non-steroidal anti-inflammatory drugs, cortisone injections and physical therapy provided little or no relief. At our clinic, she reported severe pain on the medial genicular region with any movement of the lower limbs and even at rest. The visual analogue scale (VAS) score for her knee pain was 6/10. Her right knee motion was limited to several degrees. Other medical history involved hypertension, hyperlipidemia, and diabetes mellitus for about 10 years.
On physical assessment, the patient’s BMI was 24.7 kg/m2. She walked into the ward with an antalgic and stiff gait. The skin over the right knee surgical incision was well-heeled. There was slight atrophy of her right calf or quadriceps. Tenderness was found at the medial knee compartments but not the lateral compartment. Tinel’s sign was positive, whereas the varus stress test, valgus stress test and patellar compression test were all negative. Active ROM was 0° to 60° (Fig. a and b).
Plain radiographs demonstrated that the prosthesis was well-placed and well-fixed without any signs of periprosthetic fracture, implant loosening or osteolysis (Fig. a). Magnetic resonance imaging (MRI) was not performed. A diagnosis of arthrofibrosis after TKA was established and neuroma was also highly suspected due to the positive result of Tinel’s sign.
The patient then underwent right knee exploration, adhesiolysis and spacer exchange under general anesthesia. Antibiotic prophylaxis was administered for perioperative infection prevention, and the tourniquet was set at 250 mmHg. The surgery was performed through the previous surgical approach, which was a 15-cm standard midline skin incision. After blunt separation of subcutaneous tissue, we observed several thickening neuroma-like tissues distributed in both superior and inferior parts of the medial genicular area (Fig. a and b). Based on the innervation around the knee, these abnormal tissues may have been derived from the infrapatellar branch of the saphenous nerve or the medial femoral cutaneous nerve. Some nerve branches were scarred and adhering to surrounding blood vessels and fat in clumps. Subcutaneous neurectomy was performed and the specimen was then sent for pathological examination. The joint capsule was incised through the medial parapatellar approach. Exploration showed hyperplasia of the synovium, and the presence of a large amount of fibrous tissue around the prosthesis, patella and intercondylar fossa. The femoral component and tibial tray showed stable fixation. These findings corresponded to the stiffness of the patient’s physical examination, and confirmed the diagnosis of arthrofibrosis. Adhesion lysis was performed to remove the intracapsular scar tissue (Fig. c). Synovectomy was performed to remove hyperproliferative synovial tissue. The polyethylene spacer was replaced by a new one of a suitable size, with a thickness of 11 mm. The incision was then closed, and the patient’s immediate postoperative course was uneventful.
The patient resumed knee exercise 2 days after surgery and recovered uneventfully. She had complete resolution of right knee pain without pain medication (VAS 2/10 on 2nd postoperative day, VAS 0/10 at 2 weeks postoperatively). The ROM of the right knee was 0° to 110° on the 2nd postoperative day (Fig. c and d) and increased to 0° to 120° at 2 weeks postoperatively. The post-revision radiograph revealed similar fixation and placement as pre-revision (Fig. b). The pathological report showed that the specimen contained fat, blood vessels, collagen and nerve tissue, and the arrangement of nerve fiber bundles were haphazard, which was considered to be a traumatic neuroma (Fig. ).
At 3-month follow-up, the patient was mobilizing well without obvious claudication and the ROM of the right knee was 0° to 120°. There had been no recurrence of her right knee pain.


A 40-year-old woman was referred to the facial plastic surgery clinic with a seven-month history of asymptomatic left-sided facial lipoatrophy. She was otherwise healthy with no significant medical or surgical history and was not taking any medications. The lipoatrophy presented over the course of 2 weeks and remained stable for 6 months preceding presentation. There was no history of trauma, infection or previous surgery in the area. She did not receive treatment prior to presentation to clinic. The patient reported no previous lipoatrophy, HIV infection, or autoimmune disease. Her family history was unremarkable for these as well.
The area of fat loss was asymptomatic but socially disfiguring (Fig. ). On examination, there was almost complete loss of the malar fat pad over the malar eminence as well as a concave deformity extending temporally towards the lateral canthus. She was clinically assessed to have moderate to severe concavity of one or more facial regions, observable prominence of bony landmarks, and possible visibility of underlying musculature. These features were consistent with grade 4 facial lipoatrophy according to the scale developed by the Facial Lipoatrophy Panel []. Investigations, including a full autoimmune workup, were unremarkable. Lipoatrophy of this severity would be unlikely to resolve spontaneously. No other specialists were consulted in the investigation of this presentation.
The patient provided consent to treatment and the publication of this case report. She received two treatments of autologous fat grafting both of which were done using a Coleman type approach []. The area to be treated was marked with the patient standing. For the harvest of fat, 20 mL of 1% lidocaine with adrenaline was injected into the periumbilical area followed by initiation of sterile technique. Using a Coleman liposuction harvesting cannulae, a total of 15 mL of fat was harvested in an atraumatic fashion through a small periumbilical stab incision. The fat was not centrifuged, but was simply transferred directly into 1 mL leur lock syringes for injection, discarding any fluid that accompanied the fat. An infraorbital nerve block was performed with local anesthetic injection into the depressed area. The face was prepped and draped in a sterile fashion and a small stab incision was performed in the mid cheek subunit at the intersection of a vertical line drawn down from the lateral canthus and a horizontal line drawn from the ipsilateral alae. A total of 8 mL of fat was injected into the soft tissues of the cheek and various levels ranging from the supraperiosteum to the subcutaneous plane. Both umbilical and facial stab incisions were closed with a single fast absorbing gut suture. This procedure was repeated 1 year later as a second stage of treatment to improve the correction of the facial deformity. The patient reported no adverse effects from treatment and was satisfied with the results (Fig. ). She continues to be followed annually though future treatments are not anticipated.


A 13-year-old girl from a remote village of Bandung presented with urology emergency to our hospital with gross hematuria and difficult and painful micturition since 2 years before admission. She had flank pain as well since a year ago. There was no history of cough, night sweats, malaise, or swelling of abdomen or extremities. The patient had lost her weight for about 2 kg in a month. There was no history of TB disease previously, but her grandmother was diagnosed as having pulmonary TB and was on TB treatment.
Physical examination showed undernourishment, body weight 27 kg (–2 to –3SD), and height 137 cm (–2 to –3SD). On examination, her general appearance was moderately ill and blood pressure was within the normal limit and had no fever. There was pain on the left flank. Urine microscopy showed leukocyturia, hematuria, and positive acid-fast bacilli, and M. tuberculosis was detected in rapid-test molecular test (gene Xpert). Laboratory findings showed serum creatinine 0.80 mg/dL, serum urea 12.0 mg/dL, serum sodium 142 mEq/L, serum potassium 4.5 mEq/L, serum calcium 5.61 mg/dL, Hb 10.6 g/dL, hematocrit 33.9%, WBC 11,800/mm3, platelet count 663,000/mm3, differential count 0/2/0/73/19/6, and quantitative CRP 1.2 mg/dL, and anti-HIV, anti-HCV, and HbsAg were negative. Ultrasound of kidney-ureter-bladder showed bilateral kidney pelvocaliectasis, and diuretic renogram revealed an important difference in renal function, i.e., the uncorrected glomerular filtration rate (GFR) of the right kidney and the left kidney was 32.5 mL/min/1.73 m2 and 5 mL/min/1.73 m2, respectively. Chest radiograph showed active pulmonary tuberculosis; Mantoux test showed positive result (an induration of 17 mm). Based on those findings, we diagnosed complicated urinary tract infection, left ureteral stenosis, bilateral hydronephrosis, and stage 3 chronic kidney disease (CKD). Left kidney nephrectomy and bladder augmentation were performed by a pediatric urologist, and then the anatomopathological result was pyelonephritis tuberculosis (tuberculoma).
The patient was treated with four antituberculous drugs in the intensive phase which she had completed and then continued with two antituberculous drugs in the continuation phase. She experienced antituberculous drug-induced hepatotoxicity (ADIH) after two months of antitubercular treatment (ATT). We diagnosed the patient as complicated urinary tract TB, stage 3 chronic kidney disease (CKD), and ADIH. The patient was given ethambutol and streptomycin with an adjusting dose according to GFR and was given calcium carbonate, folic acid, and vitamin D orally. The child gained 2.5 kilograms during this time period, and her condition improved significantly ().
The patient was discharged in good condition, without any hypertension and other chronic kidney disease symptoms; the GFR was 29.5 mL/min/1.73 m2 and bladder capacity was 250–300 mL after having bladder augmentation. Her quality of life was gradually getting better according to the pediatric symptoms checklist for quality of life (Peds-QL) of patient age 13–18 years old, and she can perform some daily activity well without any physical and psychological disturbances.


A 33-year-old woman first consulted at the Division of Cardiology because of presyncopal symptoms, which started just after the delivery of her first child, with acral pallor, associated with pain, tremor and weakness in the lower limbs. In addition, she presented gastrointestinal symptoms with epigastric pain and nausea, accompanied by slow palpitations and significant orthostatic hypotension. During one of the episodes an electrocardiogram showed a nodal rhythm with a ventricular rate of 50. The Holter revealed a clear relation between the symptomatology and these episodes of nodal rhythm. Seven years later, after a routine blood test she showed an impaired fasting glucose of 110 mg/dl and was referred to the Division of Endocrinology. During a thorough anamnesis, the patient reported worsening of the symptoms and an increase in their frequency, with the appearance of pulsating headaches, dizziness, tinnitus and high blood pressure (up to 225/117 mmHg) followed by hypotension (63/36 mmHg on average). As far as her medical history is concerned, she had undergone a thyroid lobectomy with isthmusectomy, with a histological finding of a nodular hyperplasia. There was no family history of endocrine disease. However, her mother had hypertension and her father passed away due to a brainstem stroke. She has two healthy children.
With the clinical suspicion of PCC, an analytical study was requested, which showed: urine adrenalin 367 mcg/24 h (<20 mcg/24 h), urine noradrenalin 81 mcg/24 h (15–80 mcg/24 h), urine metanephrine 2490 mcg/24 h (25–312 mcg/24 h) and urine normetanephrine 816 mcg/24 h (60–757 mcg/24 h). Magnetic resonance imaging (MRI) () revealed bilateral adrenal masses, 37 mm right and 49 mm left. It showed a hyperintense signal of both adrenal glands on fat-suppressed T2-weighted, without the presence of fat. After gadolinium administration these masses enhanced avidly and heterogeneously with regions of no enhancement due to cystic changes. Restricted diffusion (b = 800) of hypercellular areas was well demonstrated by ADC map as areas of low intensity signal. In oppossite, cystic components demonstrated high signal. 123I-MIBG SPECT/CT () showed increased uptake foci in both adrenal glands, with right predominance, compatible with the diagnosis of bilateral PCC. No extraadrenal tumour localization was detected.
The patient underwent a laparoscopic bilateral adrenalectomy. The bilateral tumours were circumscribed but unencapsulated, and partially surrounded by a bright-yellow ring of normal adrenal cortical cells. Both tumours also showed a fleshy, tan cut surface, with haemorrhage areas, measuring 4.5 cm and 5.4 cm maximum diameter in the right and left glands respectively ().
Genetic testing was carried out. The patient presented the pathogenic variant NM_017849.3: c.86delG (p.Arg29Leufs∗52) in heterozygosis in the TMEM127 gene. This variant has not been reported in available databases (i.e., dbSNP146, 1000 Genomes, ExAC and GnomAD). This consists of the deletion of one nucleotide in exon 2 of TMEM127 which alters the reading pattern and leads to the appearance of a premature stop codon. Given that this premature stop codon is 7 nucleotides upstream the next exon-junction complex, it is unlikely that this new transcript is down regulated by nonsense-mediated decay []. This result suggests a susceptibility to PCC with an autosomal dominant inheritance pattern (OMIM #171300). Given these findings, a genetic study was performed on her children, aged 9 and 6, who were proved to be carriers of the same variant in the TMEM127 gene. Plasma and urine catecholamines and metanephrines and total body MRI showed normal results in both children. However, given the risk of developing PCC or other tumours (renal carcinoma) [], they will require surveillance. In addition, her mother tested negative for the variant.
One year after the adrenalectomy the patient is asymptomatic, with normal metanephrine excretion. As expected, she has primary adrenal insufficiency and is being treated with hydrocortisone.


We report the case of a 54-year-old Asian male who has suffered from paranoid schizophrenia for 2 years and who has multiple persecutory delusions and paranoid ideation. This patient compulsorily admitted to a psychiatric ward on the basis of extremely disturbing behaviors once. Oral Haloperidol has been prescribed at that time, and the patient responded to the medication very well. The daily dose of oral Haloperidol was increased to 15 mg since one year ago.
Due to the use of a high-dose, first-generation antipsychotic drug, extrapyramidal syndromes afflicted the patient. According to the patient's self-statement, akathisia symptoms—restlessness, anxiety, and agitation—began to appear 1 month after he started taking the antipsychotic drug. Moreover, other symptoms of tardive dyskinesia—including tongue protrusion, lip smacking, excessive eye blinking, repeated bending and straightening of the fingers and elbows, involuntary shaking of both feet—occurred suddenly at the time of the fourth months of his antipsychotic drug treatment and worsened gradually. The patient reported that he felt enchanted and was unable to control his body movements. As a result, Trihexyphenidly 5 mg twice a day for oral use was prescribed to treat the patient's extrapyramidal symptoms.
The patient also reported that flexion contracture occurring over his fourth and fifth fingers on both hands appeared since six months after he started taking the antipsychotic drug. The symptoms worsened very quickly to the point that he was unable to straighten his fourth and fifth fingers (Fig. ). Because of the rapid loss of the function of his hands, he came to our orthopedic clinic for help. Physical examinations revealed that his fourth and fifth fingers were numb and his palmar muscle was characterized by marked atrophy. The EMG revealed bilateral UN with chronic axonal degeneration at the elbow level (Table ). McGowan classification was performed to evaluate the severity of the ulnar nerve injury, and the patient was diagnosed with a grade 3 injury on his left hand and a grade 2 injury on his right hand.
We examined the patient's past medical history to determine the cause of the bilateral UN. The patient reported body mass index (BMI): 20.54, no prior history of smoking, diabetes, gouty arthritis, trauma history, other joint pain or joint deformity as symptoms of rheumatoid arthritis. The patient was also free of hypothyroidism or a related metabolic disease, and he had no special family history. With the regard to medication use, he only took the drug prescribed by the psychiatric clinic. The patient had retired 6 years ago from selling clothing; he did not carry heavy loads or do other labor since then.
X-rays of the lateral and cubital tunnel view and anterior-posterior (AP) view revealed no obvious bone spurs and fractures, and no lumps or soft tissue masses were found upon palpation of the patient's elbows. A physical examination revealed a normal range of motion of elbows, negative Tinel signs at the wrists and positive at the elbows. However, we observed that the patient's elbow involuntarily underwent bending and straightening. After a surgical exploration for the neurolysis, we examined the ulnar nerve and its peripheral region. Neither the presence of nerve compression by adhesion band or nerve swelling was found. We strongly suspected that the bilateral UN in this patient was a complication caused by tardive dyskinesia, induced by antipsychotic drugs. As a result, psychiatrist consultation was done for making medication assessments and adjusting the patient's medications.
According to the patient on the telephone access, numbness of the palms continued to regress over the following 6 months after Haloperidol discontinuation and the anterior transposition of the ulnar nerve. However, the remaining ulnar claw deformities need longer time to observe the outcome. Regression of the involuntary movements, including repeated bending of the elbows, and shaking of both feet, was noted from the patient but was incomplete.


A 45-year-old Caucasian female born to first-cousin parents, with two healthy children and without any relevant family history record of prior diseases, was seen at the service of Dermatology (University of Coimbra, Coimbra, Portugal) in March 2014 because of persistent extensive, skin-colored, exuberant, and disfiguring warts in both feet and hands since the age of 10 years (). Warts were refractory to treatment with keratolytic agents, cryosurgery, and excision, with minor improvement after treatment with acitretin in association with topical 50% urea cream. Apart from this, the patient did not describe recurrent infection-related episodes or diseases, except for past medical history of measles and mumps during her infancy and varicella infection during her first pregnancy, which all resolved without complications. Of note, such past history of infections is not rare among the patient age-matched Portuguese population since vaccination for these diseases was introduced in the Portuguese national vaccination program years after she was born (1969): in 1974 for measles, in 1987 for mumps, and in 2004 for varicella (–). In fact, outbreaks of measles and mumps have been reported in Portugal until the late 80s to mid-90s, with peaks of >10,000 cases per year (, ).
In addition, she referred allergic rhino-conjunctivitis treated with cetirizine and fluticasone, and chronic polyarthralgias in the absence of impaired functionality. Serological studies were negative for (severe) infections, including HIV 1/2, HTLV-1, and syphilis. In turn, she showed IgG antibodies for ubiquitous pathogens including CMV >250 arbitrary units (AU/ml) (positive threshold >6AU/ml) and Epstein–Barr virus VCA = 192 U/ml (positive threshold >20 U/ml) and EBNA = 24 U/ml (positive threshold >20 U/ml) in the absence of serum IgM antibodies for these pathogens (0.06 AU/ml; positive threshold >6AU/ml). Slightly increased serum IgG levels (IgG: 1,430 mg/dl), associated with normal IgA (278 mg/dl), IgM (67 mg/dl), C3 (1.4 g/L), C4 (0.33 g/L), and C1 inhibitor (0.318 g/L) serum levels, were detected. In addition, anti-neutrophil and anti–double-strand DNA autoantibodies were negative, while antinuclear autoantibodies were weakly positive. Screening for immunological alterations by flow cytometry () (–) using the EuroFlow Primary Immunodeficiency Orientation Tube (PIDOT) (, ) showed an absolute defect of CD4-expressing T-cells (<0.01 cells/μl), with normal total T-cell, CD8+ TCRγδ− T-cell, and NK-cell (absolute) numbers, associated with consistently increased B-cell counts vs. age-matched normal reference values. Importantly, (TCRαβ+ TCRγδ−) DN T-cells were significantly expanded (). Signs/symptoms associated with primary immunodeficiency other than persistent warts in the feet and hands were not observed either at presentation or during the subsequently 5-year follow-up period. Of note, peripheral blood (PB) monocytes and DCs showed no cell surface expression of CD4.


The patient was a 56-year-old female who had had oral and lip ulcers for one month and a rash for ten days. The dermatological examination found widespread erosions and shallow ulcers in her oral cavity, tongue, lips, conjunctiva, and genital and anal areas (). Polymorphous eruptions including erythema, vesicles, angry red papules, and plaques were observed on her trunk and distal extremities. She also presented with hair loss, and papules and scars on her scalp. She had dyspnea and dry cough for two weeks. Pulmonary function test showed mixed-type ventilatory defect. Chest X-ray and computed tomography (CT) showed collapse of the bilateral middle and lower lobes of the lungs with ground glass opacity, which suggested bronchiolitis obliterans. A solitary mass of 10 × 8 × 8 cm in size was found in her abdomen by ultrasonic examination; CT and positron emission tomography (PET) scans showed a hypermetabolic mass behind the head of the pancreas ().
Indirect immunofluorescence revealed the deposition of IgG in the intercellular spaces of rat bladder epithelium. Western blot demonstrated anti-epidermal antibodies in patient serum that recognized the antigens of envoplakin, periplakin, desmoglein 3, and linker regions of plakin-family proteins (). ELISA results for antidesmoglein 3 antibody (MBL – Japan) were 108 (positive). Direct immunofluorescence from patient skin lesion showed deposition of IgG in the intercellular space of the epidermis.
Histological examination of the skin lesion biopsy demonstrated typical intraepidermal acantholytic blisters, individual keratinocyte necrosis, interface vacuolar degeneration, and diffuse lymphocyte infiltration throughout the dermis.
The histological examination of the removed tumor showed several areas with storiform arrangement of spindle cells, intermingled with some small lymphocytes. The chromatin was vesicular, with small nucleoli and mild to moderate variation in nuclear size. Morphological examination suggested a diagnosis of follicular dendritic cell sarcoma. As the tumors were located in the retroperitoneum and in the presence of many blood vessels in the background along with occasional fibrous bands, an underlying hyaline-vascular Castleman's disease was suggested. However, the typical follicles were rare.
CD20 and CD21 showed serpentine trabecular meshwork as well as nodular meshwork penetrated by multiple blood vessels. The storiform areas showed positive for CD35, S-100, vemitin, weakly positive to CD21 and CD68. These follicular dendritic cells are obviously outside the follicles ().
Final diagnosis was follicular dendritic cell sarcoma, aggravated by hyaline-vascular Castleman's disease associated with PNP.
The new criteria established for treatment were followed., Surgical resection of the tumor was performed immediately after confirmed diagnosis. Intravenous immunoglobulin (IVIg) was used before, during, and after the surgical procedure. Methylprednisolone 48 mg was administered daily and the dosage was gradually decreased over the next three years.
Skin lesions improved in two months and oral ulcers in six months. After 12 months, indirect immunofluorescence decreased gradually from 1:128 positive to undetectable. The value of ELISA for antidesmoglein 3 antibody decreased from 108 to negative. However, the patient still presented dyspnea and dry cough. Her second chest X-ray suggested that bronchiolitis obliterans still persisted.,


A 41-year-old female with extensive medical history including hypertension, lupus nephritis, anti-phospholipid antibody syndrome, coronary artery disease, and previously treated Libman-Sacks endocarditis presented to the ED with persistent lightheadedness for one week and two days of recurrent nausea and vomiting with decreased oral intake. She had a pertinent surgical history of coronary artery bypass graft and aortic valve replacement secondary to the endocarditis. She was anticoagulated on warfarin and required hemodialysis. The patient also reported two days of right calf pain that occurred only when ambulating. She did not complain of chest pain, back pain, or abdominal pain.
The patient’s initial vital signs included a blood pressure of 171/91 millimeters of mercury, heart rate of 92 beats per minute, respiratory rate of 18 breaths per minute, and oral temperature of 37.1 degrees Celsius. Her oxygen saturation was 99% on room air. On initial evaluation in the ED, the patient appeared in no distress and was alert and oriented to person, place, and time. She answered questions appropriately, and her neurologic examination showed no focal weakness or sensory deficits. Lungs were clear and cardiac exam was noted as regular rate and rhythm without murmur. The patient’s abdomen was soft, non-tender, and non-distended. Her lower extremities were warm and well perfused with normal range of motion and no swelling or calf tenderness. Her peripheral pulses were intact and symmetric bilaterally.
Based on her history and physical examination, the treating physicians were most concerned for an acute viral process or foodborne illness. Nonetheless, given her complaint of right calf pain in the context of a chronic pro-coagulant state, they decided to evaluate for a DVT in the right lower extremity. The patient underwent a POCUS two-point compression examination of the right leg, which showed normal compression of the right femoral and popliteal venous systems. However, an abnormal intraluminal echogenic signal was seen in the right femoral artery, which had the appearance of an intimal flap.
Color Doppler was used to confirm differential flow on either side of the flap (). The ultrasonographers proceeded to interrogate the abdominal aorta, and a dissection flap was noted in the transverse view (). A computed tomography (CT) angiogram of the chest, abdomen, and pelvis with run-off to the lower extremities was then performed, which showed an intimal flap starting in the distal abdominal aorta and extending into the right common iliac, external iliac, and superficial femoral arteries ().
A subsequent review of the patient’s medical chart showed that she had been admitted to our institution one month prior for acute coronary syndrome and had been taken to the cardiac catheterization suite three times during that hospitalization. The hospital record noted that she was canalized in her femoral region three times, twice via her left femoral artery and once via her right femoral artery. The patient was assessed by the vascular surgery team in the ED. Their impression was this dissection was iatrogenic given her history of recent catheterization, and they recommended strict blood pressure control and admission. Given her extensive and complicated cardiovascular history she was ultimately admitted to the cardiac intensive care unit. Her blood pressure medications were adjusted, and she was discharged home three days later.


A 15-year-old female with a past medical history of only autism spectrum disorder presented to the ED with complaints of a laceration to her right fourth digit and an abrasion to her right third digit, which were sustained approximately 22 hours prior to ED arrival. Before presenting to the ED, the patient’s mother attempted wound care at home; however, secondary to persistent bleeding, the patient was brought to a referral urgent care center that then subsequently sent the patient for evaluation at our ED.
On evaluation in the ED, the patient was at her baseline mental status as per her parents. Physical examination revealed a weight of 53 kg, a temporal temperature of 37 degrees Celsius, a heart rate of 71 beats per minutes, a respiratory rate of 18 breaths per minute, a blood pressure of 114/86 millimeters of mercury, and a room air oxygen saturation of 99%. She was noted to have a small abrasion to the medial aspect of the distal phalanx of the right third finger and a one-centimeter, elliptical-shaped laceration to the medial aspect of the distal right fourth finger that was actively bleeding. She had full range of motion of all 10 of her digits with a capillary refill of less than two seconds on each digit. Of note, her neurologic exam revealed the patient to be at her baseline status as per her parents, awake and alert, and she was symmetrically moving all of her extremities equally. The remainder of her examination was normal.
Due to continued bleeding of the laceration despite other attempts at hemostasis, and after a discussion with the patient’s parents regarding increased risk of infection because the wound had been open for 22 hours, they consented to have the laceration repaired with sutures in the ED. Because of the patient’s baseline mental status, secondary to her autism spectrum disorder, the decision was made to perform the laceration closure under procedural sedation.
For the procedural sedation, there was no IV access available; thus, the medication was administered intramuscularly. It had been greater than four hours since her last meal, she was able to fully flex and extend her neck, and she was able to fully open her mouth. We chose ketamine as the sedating agent for the procedure at a dose of 3–5 mg/kg, which is in the accepted intramuscular dosage range. Consent for the sedation was obtained from her parents after a full discussion regarding the risks and benefits of sedation and the known adverse effects of ketamine.
Ketamine was administered via a one-time dose intramuscularly at the start of the procedure; the total dose given was 150 mg, which was less than the recommended intramuscular dose. The patient was on a cardiac monitor with pulse oximetry and end-tidal carbon dioxide monitoring throughout the procedure and remained normoxic. The laceration on the right fourth digit was repaired using an aseptic technique after cleansing of the site, with four 4-0 chromic gut sutures. No local anesthetic was administered. As the last stitch was tied and nine minutes after ketamine was administered to the patient, she was observed to have generalized tonic-clonic seizure of one-minute duration that self-resolved without any administration of further medications. Medical toxicology was consulted and advised to evaluate the patient for a new onset seizure, as ketamine was not known to induce seizure activity.
The patient remained postictal in the ED, IV access was established, and a complete blood count, basic metabolic panel, and a computed tomography (CT) without contrast of the head were ordered. Blood analysis was noted to be within normal limits for our institution’s normal value range. The head CT was read by radiology to show mild colpocephaly of the ventricles with uncertain significance as there was a present corpus callosum and no evidence of transependymal resorption or other white matter findings.
Pediatric neurology was consulted, and the case, laboratory findings, and imaging findings were discussed. Pediatric neurology recommended inpatient admission if the patient maintained altered mental status and to otherwise follow up with them for an electroencephalography (EEG) due to new onset seizure. The patient continued to be hemodynamically stable in the ED and maintain her airway, but did not return to her baseline and had one episode of vomiting despite observation for four hours after the observed seizure activity. At that time, she was admitted to the pediatric step-down unit for further evaluation and monitoring.
While in the pediatric step-down unit, the patient gradually returned to her baseline mental status; she had no further seizure activity throughout her stay and required no administration of any medications. She was evaluated by neurology and underwent a one-hour EEG, which showed no areas of focal slowing, epileptiform discharges, or electrographic seizures. The patient was discharged in stable condition after undergoing the EEG to outpatient follow-up on a course of cephalexin for empiric antibiotic coverage of her repaired finger laceration. The patient was then lost to follow-up.


A 35 year-old Chinese woman was admitted to our hospital 2 days after an acute onset of right-sided weakness and expressive dysphasia. She had no history of infection and vaccination within 6 weeks and no other medical history. Her family had no hereditary diseases. The patient was a salesclerk in a bookstore. She never drank alcohol or smoked. A systemic examination was unremarkable. A neurological examination revealed motor aphasia, facial asymmetry, 2/5 strength in the right upper and lower extremities, and a positive right-sided Babinski reflex.
A brain MRI showed a tumor-like lesion with surrounding edema in subcortical of the left parietal lobe highly suggestive of glioma. The mass lesion demonstrated hypodensity on the brain MRI T1 image, hyperintensity on the MRI T2 image, and hypointensity on the fluid-attenuated inversion recovery (FLAIR) sequences. A gadolinium-enhanced MRI showed a solitary 2.8 cm × 2.6 cm × 4.1 cm irregular peripheral enhancement mass with a central non-enhancing area centered in the left parietal lobe (). Magnetic resonance angiography displayed mildly abnormal with no sign of vasculitis; slight narrowing was observed in the bilateral siphon carotid arteries, bilateral anterior cerebral arteries, and the M1 segment of the left middle cerebral artery. All of the preoperative laboratory findings were within the normal limits: white blood cells were 8.76 × 109/L (normal, 4.0–10.0), red blood cells were 3.93 × 1012/L (normal, 3.5–5.1), hemoglobin was 116 g/L (normal, 115–150), and hematocrit was 0.361 (normal, 0.350–0.450). Electrolytes with glucose level, liver function test, arterial blood gas, renal function test, and thyroid function test were within the normal limits. The venereal disease research laboratory (VDRL) test was non-reactive. High-grade glioma was provisionally diagnosed and the patient underwent a mass resection. However, the histopathological examinations revealed a lymphocytic inflammatory infiltrate in the vessel walls and perivascular zones, and parts of the vessels demonstrated necrotic wall damage and contained hyaline microthrombi, consistent with central nervous system vasculitis (). Immunohistochemical examinations showed negative staining for Ki-67, P53, GFAP, IDH1, R132H, 01igo-2, MBP, Map2, NeuN, Syn, and CD68. After surgery, the patient's consciousness deteriorated, and a postoperative MRI scan demonstrated flaky extensive perifocal edema that could not be explained by glioma ().
Once vasculitis was diagnosed, an evaluation of its secondary causes was pursued. Acute phase reactants, for example, the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and a complete blood count (CBC) were normal. Serum antineutrophil cytoplasmic antibody (ANCA), antinuclear antibodies (ANAs), anticardiolipin antibody (ACA), antidouble-stranded DNA, rheumatoid factor (RF), cyclic citrullinated peptide (CCP), complement 3 and 4, immunoglobulin G (IgG), IgM, and IgA remained within the normal ranges. Whole-body fluorodeoxyglucose-PET (FDG-PET) and several serum tumor markers (CA724, CYFRA21-1, CEA, CA242, CA125, NSE, AFP, SCC, CA199, CA153, HCG) suggested no evidence of metastatic tumors. Lumbar puncture showed the patient's intracranial pressure was 390 cm H2O (normal range, 80–180) and a cerebrospinal fluid analysis identified lymphocytic predominant pleocytosis (64 × 106 cells/L, local reference range, 0–8 × 106), elevated protein (0.82 g/L, local reference range, 0.15–0.45), and normal glucose levels (3.40 mmol/L, normal range, 2.3–4.1). Other CSF findings included IgG (39.00 mg/L, normal range, 0–34), IgG index (3.63, normal <0.85), IgG synthesis (151.2 mg/24 h, normal <7.0), and oligoclonal bands (positive, normal negative). The results of cytologic studies and bacterial and viral cultures were all negative. Additional testing of the CSF and serum including antibodies in various paraneoplastic neurological syndromes, myelin basic protein (MBP), MBP antibody (MBP-Ab), myelin oligodendrocyte glycoprotein antibody (MOG-Ab), and aquaporin 4 antibody (AQP4-Ab) were all within the normal ranges. Further examinations revealed no evidence of metabolic, autoimmune, or infectious disorders, systemic vasculitis, or neoplastic malignancy. PACNS was diagnosed.
The patient was commenced on intravenous methylprednisolone 1 g daily, halved every 3 days and tapered over 12 days to prednisone 60 mg daily via oral administration with 2.5 mg/week reduction, and the treatment was maintained for approximately 6 months. A repeat brain MRI after 6 months of treatment showed that the lesion had nearly disappeared with the resolution of edema and mass effect (). At a 1 year follow-up, the patient could walk independently.


In March 2018, a 69-year-old woman with a history of follicular thyroid cancer presented with a 3-month history of masses in the left chest. She had undergone total thyroidectomy with right-sided cervical lymphadenectomy in our department and pathological diagnosis was thyroid follicular carcinoma (Fig. ), followed postoperative iodine treatment 10 years previously. Two years later, the patient developed a recurrence of left cervical lymphadenopathy, for which she underwent left-sided modified radical neck dissection. Unfortunately, she experienced left supraclavicular and left axillary lymph node recurrence in 2013 and underwent a third operation to resect these metastatic lesions. Following the third iodine treatment, levothyroxine tablets were administered. Physical examination revealed solid, mobile, painless masses in the left chest wall, the largest of which was approximately 4 × 3.5 cm in size below the left clavicle; a medium-sized mass was found in the second intercostal space that was 2.5 × 2 cm in size, while the smallest mass was in the third intercostal space and measured 1 × 1 cm in size (Fig. ). Ultrasonographic examination revealed hypoechoic lymph nodes. A computed tomography (CT) scan of the chest showed lymph node enlargement in the subclavian area, and no pulmonary metastases were found (Fig. ). Systemic and local scans were performed 48 h after oral administration of iodine-131 5 mCi. There was no significant radioactive concentration in the thyroid region, but there was radioactive concentration under the left clavicle and increased point-like radioactive uptake in the left chest wall. Left subclavian and thoracic lymph node metastases of thyroid carcinoma were considered (Fig. ). Cranial and abdominal CT and whole-body bone scans did not reveal further pathology. Laboratory test results revealed normal T4 and T3 levels, a thyroid-stimulating hormone (TSH) level of 2.5 pg/ml (normal < 10 pg/ml) and serum thyroglobulin (TG) level of 3000 ng/ml (normal < 50 ng/ml). Ultrasound-guided core-needle biopsy of the mass revealed a follicular thyroid neoplasm with lymph node metastases. The results of immunohistochemical analysis indicated TTF, CK7, and thyroglobulin positivity and partial CD56 positivity, while the test for CgA was negative; the Ki67 index was 5%(Fig. ). Mass resection was performed on the left chest wall under local anesthesia. Multiple lymph nodes were found to be enlarged under the left clavicle, and a total of 4 lymph nodes were removed, with a maximum diameter of 3 cm. The second intercostal lymph node was 1.5 cm in diameter, and the diameter of the third intercostal lymph node was 0.6 cm (Fig. ). The final pathological diagnosis was thyroid follicular carcinoma metastasis to the lymph node (Fig. ). Following a I131 radiotherapy session, TSH suppression therapy was continued. Nearly 1 year after the last operation, the patient’s serum thyroglobulin level has remained below the threshold, and no local recurrence or distant metastasis has been observed.


A 36-year-old woman, G2P1, with an uneventful pregnancy, presented to Peking Union Medical College Hospital at 22 weeks’ gestation for routine second trimester screening. An ultrasound examination showed the presence of an oral cystic lesion, which measured 18 × 15 × 15 mm. The lesion was posterior to the mandible and attached to the inferior aspect of the tongue in a male fetus (Fig. ). No blood flow signal was observed through the cyst. The lesion appeared to be moving in conjunction with the tongue and did not protrude outside of the oral cavity. The fetal mouth was slightly open for the duration of the ultrasound scan and was shown by 3-dimensional ultrasound in the surface render mode (Fig. ). Color Doppler flow imaging (CDFI) showed fetal deglutition, with clear evidence of transoral amniotic fluid flow around the cyst and in the upper respiratory tract (Fig. ). This indicated patency of the fetal airway. The fetal stomach was visualized and the amniotic fluid volume was in the normal range. This finding indicated normal swallowing function and the cystic mass did not obstruct the esophagus. A sonographic anatomical survey showed no other anomalies of the fetus. Follow-up ultrasound examinations at 24 and 31 weeks of gestation showed a progressive reduction in size of the lesion and absence of polyhydramnios. Tiny internal septation was observed (Fig. ). However, the cyst eventually disappeared in follow-up examinations at 35 and 37 weeks of gestation.
A male newborn who weighed 3480 g was delivered vaginally, with Apgar scores of 10 and 10 at 1 and 5 minutes, respectively. The neonate had no feeding or breathing difficulty. No major oral cyst was identified in the tongue at a clinical and instrumental examination. We followed the child to 6 years after birth. The child had normal growth and development, with no recurrence of the cyst. The study was approved by the Institute Research Ethic Committee of Peking Union Medical College Hospital. The patient has provided informed consent for publication of the case.


A 66-year-old Japanese man was consulted to our hospital due to the progression of dyspnea and edema in both legs, which had occurred during the previous week. He presented with a low-grade fever (37.4°C by armpit), productive cough and hypoxemia with 94% of percutaneous oxygen saturation in ambient air. He was an ex-smoker (10 cigarettes per day, for 6 years from 20 to 25 years of age), but had no particular past medical history or medications.
The results of the initial laboratory tests showed hypoproteinemia, proteinuria, and moderate renal insufficiency (Table ). On electrocardiography, low voltage and a long QT interval were detected (Fig. A). A chest x-ray showed dilation of a cardiac shadow and bilateral pleural effusion (Fig. B). Echocardiography revealed left ventricular hypertrophy (mean wall thickness, 15 mm) and granular high echoic spots at the septum (Fig. C). His plasma brain natriuretic peptide level was increased (448.7 pg/mL; normal range, < 18.4 pg/mL). These typical observations are compatible with amyloid cardiomyopathy.
Percutaneous kidney and endoscopic gastric mucosal biopsy demonstrated Periodic acid-Schiff (PAS)-positive hyalinization in most of the glomeruli and surrounding vessel walls in the kidney (Fig. A and B) and submucosal stroma (Fig. C). These lesions were congophilic, which is considered a signature of systemic amyloidosis. Immunofluorescent (IF) staining revealed strong deposition of IgA and lambda (λ) chain in mesangial regions, but little IgG and IgM deposition, and no kappa (κ) light chain deposition (Fig. D). The amyloid deposits were composed of randomly oriented fibrils with a mean diameter of 10 nm on electron microscopy (Fig. E). Bone marrow aspiration biopsy showed little atypical plasma cell infiltration (<1%) and no evidence of myeloma.
The patient was referred to hematology for further treatment and moved to another hospital for the administration of chemotherapy using melphalan and dexamethasone. After 15-month follow-up from the initial diagnosis, the patient was still alive.


Black female patient; nine years and three months old. She had progressive weight
gain and a decrease in growth velocity, which had been observed since the age of
5 years old. Height1.14 m, Z score -3.24 (below the percentile <2.5); weight
37 kg, Z score 1.33 (>P97); Body Mass Index (BMI) 28.6 kg/m2, Z
score 3.2 (>P97); acanthosis nigricans, hypertrichosis, hunch back, globular
abdomen without purplish stretch marks. Developmental stage in puberty according
to the Tanner Scale M3 P5. Bone age of six years and ten months old, with a
chronological age of nine years and three months old. No history of
glucocorticoid use.
Laboratory tests () showed that she
had ACTH-dependent hypercortisolism and no apparent pituitary adenoma in three
1.5 MRI studies. Additionally, the patient participated in a dynamic study when
she was 9, 10 and 12 years old. Bilateral inferior petrosal sinus sampling
(BIPSS) was indicated. The exam was performed three years after the first
consultation, and the result was unclear due to technical problems. At 12 years
of age, the patient was submitted to a 3-RM MRI in the dynamic study. In the
post-contrast (gadolinium) sequence, the sagittal section, and the T1 sequence,
a contrasting hypocaptive image was found. It was located in the adenohypophysis
on the left side’s cavernous sinus. It measured 2.9 × 2.6 × 2.5 mm, which is
compatible with microadenoma ().
Details of this technique can be found in . A transsphenoidal surgery was performed on the patient when she
was 12 years and 11 months old and because she had enough adrenal to be
diagnosed with hypercortisolim, she took prednisone for two years. Even after
she was cured of hipercortisolism, she did not grow enough. Recombinant growth
hormones were then prescribed, even though the growth hormone test was
responsive after glucagon stimulation (). She thus became officially defined as having idiopathic short
stature. The patient’s final height was 1.41 m, which did not reach the family
target of 1.61 m.


White female patient; nine years and nine months old. She had been overweight
since the age of six, and her mother noticed a decrease in growth one year
before the consultation. She also exhibited depression and anxiety. She used
prednisone until she was seven years old because of bronchial asthma. Height
1.34 m, Z score -0.49 (=P31); weight 59 kg, Z score 3.18 (>P97); BMI 32.9
kg/m2, Z score 3.54 (=P99); Moon facies, Tanner development stage
of M2 P3, generalized obesity with fat deposition in supraclavicular fossa,
cervical acanthosis nigricans, fine axillary hairs, thin purplish stretch marks
in abdomen and breasts. Bone age of a nine or ten-year-old, with a chronological
age of nine years and six months old.
ACTH-dependent hypercortisolism was confirmed (). The 1.5T MRI with a dynamic study did not demonstrate the
presence of adenoma at the age of 9 years and 10 months, but in the 3T MRI with
a dynamic study performed at age 10 years and 3 months, the sagittal cut in the
T1 TSE sequence after the administration of (gadolinium) contrast, demonstrated
the contrast-enhanced, oval-shaped image of the anterior adiphyophysis measuring
3 × 2.3 × 2.1 mm. Details of the examination technique are described in . The patient underwent
transsphenoidal surgery at ten years of age. She continued to grow with
transient diabetes insidious and central permanent hypothyroidism. She presented
insufficient adrenal in the postoperative period, which is compatible with the
cure for hypercortisolism. She used prednisone for two years. She showed
progressive weight loss. Her final height was 1.51 m, with a family target of
1.59 m. She presented new weight gain without the recurrence of
hypercortisolism.