A 32-year-old male presented to our department with an alleged history of accidental acid ingestion seven months back. Initially, his Esophagogastroduodenoscopy (EGD) was suggestive of Zargar grade III esophageal injury and he was managed conservatively. Over the subsequent six months, he developed progressive dysphagia and was able to swallow liquids only (grade IV). His barium swallow and EGD were suggestive of long-segment esophageal stricture (~10 cm) starting around 24 cm distal to incisors (Figure ). Endoscopic dilatation was attempted but with subsequent failure, he was planned for surgery.
Thoracoscopic esophagectomy was carried out in the prone position with the use of a double-lumen endotracheal tube (DLT) for intubation. Three ports were placed on the right side of chest: a 12-mm port in the seventh intercostal space (ICS) in the mid-axillary line; two 5-mm ports in the ninth and fifth ICS along the posterior axillary line, respectively. After deflating the right lung using a DLT, we encountered periesophageal adhesions in the mid and lower esophagus with a dilated upper thoracic esophagus. Thoracoscopic mobilization of the esophagus was done under vision using blunt and sharp dissection. The plane of resection was kept close to the esophagus taking care to avoid injury to both the trachea and bronchus; however, the azygous vein was divided for easy mobilization (Figure ). After the complete mobilization of the esophagus, a 28 Fr intercostal drainage tube was placed through the 12-mm camera port and rest of the port sites were closed.
For the abdominal phase, the patient was placed supine with legs split and in a slight reverse Trendelenburg position. A 12-mm port was placed infraumbilical, two 5-mm ports were placed in the right and left mid-clavicular line at the level of umbilicus, and another assistant port (5 mm) in the left flank region. The left lobe of the liver was retracted using a Nathanson liver retractor. The lesser sac was opened, and the greater omentum was divided preserving the gastroepiploic arcade. The stomach was mobilized completely after dividing the left gastric and gastroepiploic vessels (Figures , ). The esophageal hiatus was opened, and communication established with the thoracic esophagus.
On the left side of neck, an oblique ‘J’-shaped incision was made anterior to the sternocleidomastoid muscle; esophagus was dissected and divided at the lower cervical level. An upper midline incision was given, and a gastric tube was prepared with multiple fires of linear cutting stapler with green cartridge. The specimen was retrieved through a small supraumbilical incision (Figure ), and the gastric conduit was pulled to the neck with help of a nasogastric tube fixed to the gastric tube. Hand-sewn side-to-side cervical esophagogastric anastomosis was done using polydioxanone (3-0) interrupted sutures. Neck wound was closed after approximating strap muscles and placing a Jackson-Pratt drain. Feeding jejunostomy (FJ) was done using the Witzel technique. After securing hemostasis, abdominal closure was done. The total blood loss was ~200 ml with an operative duration of six hours.
His postoperative course was uneventful with the FJ trial feed being given on postoperative day (POD) 1 and gradually increased which he tolerated well. Neck drain was removed on the second POD. An oral contrast study performed on the fourth POD showed no evidence of extravasation of contrast, following which he was started on liquids. Abdominal drain was removed on the fifth POD and he was discharged on the sixth POD on an oral semisolid diet. At the end of three months, he was eating comfortably and had no cough, gastroesophageal reflux or hoarseness of voice.


An eight-year-old girl presented to the clinic suffering from tenderness and bleeding from a vast necrotic sacro-gluteal mass. She had small pigmentation at this region since birth but it was asymptomatic and diagnosed as a naevus. However, at the age of eight years, the lesion aggressively progressed and became painful and haemorrhagic. No family history was significant. The patient went to a dermatologist who prescribed local steroids without any benefits. The lesion continued to become larger and more painful. No signs of puberty were present at time of presentation and no trauma other than sitting for long periods during school was reported.
Currently, the lesion became around 12 cm at the longer diameter and 10 at the shorter one with irregular episodes of mild bleeding which prevented the patient from her normal life. Current physical examination found a murmur on auscultation over the mass and Doppler ultrasonography showed a fast and high-flow lesion which speculated to be AVM. Magnetic resonance imaging (MRI) showed non-capsulated superficial soft tissue mass in the sacro-gluteal region. The mass was large and measured approximately (12*10*4) cm. It consisted primarily within the skin and subcutaneous fat in the retro sacro-gluteal region and encased the coccyx. There was no extension to the pelvis or to the spinal canal. The mass was heterogeneous in signal intensity with numerous dark flow voids. Contrast enhanced images showed multiple enhancing abnormal blood vessels. It was consistent with an arteriovenous malformation (AVM). Otherwise, the pelvic structures were within normal limits (). Brain MRI was normal, and no medications were used to treat the lesion.
The patient had financial difficulties and the surgery and embolization was postponed for another six months. During the six months, the lesion grew rapidly and became necrotic with occasionally heavy haemorrhage. Haemoglobin (Hb) reached as low as (5 g/dl) and the mass reached (15*15*2 cm) in size (). No sign of puberty was noticed during this period. When was possible, AVM embolization before surgery was indicated. Under general anaesthesia, the right common femoral artery was accessed, 4 French sheath was inserted against flow, and selective digital subtraction angiography (DSA) of infrarenal aorta the right and the left iliac artery was conducted. Huge AVM was noticed in the pelvis originating simultaneously from the right, and left internal iliac arteries and median (middle) sacral artery. Selective embolization was conducted by injecting polyvinyl alcohol (PVA) particles (). After 24 h excisional surgery was performed through an elliptical incision and the lesion was removed successfully with no complication ().
Pathology showed scattered arteries and veins with variable walls’ thickness with channels connecting them without capillary beds and only small quantities of normal tissue were observed in the lesion. Anti-biotic therapy and daily compressive dressing at plastic and reconstructive surgery department were performed. Day 10 after surgery, mild bleeding recurrence was noticed during the dressing and was managed by simple suturing. Post-operative MRI was conducted and showed a remnant of small nidus () which was managed again by repeating selective embolization under general anaesthesia of the collateral artery of the left internal iliac artery and the median (middle) sacral artery was completely bolted (). No bleeding was noticed during the following dressing and the lesion was left to heal by second intention as counseling from the plastic and reconstructive surgery department saw this more adequate for this case (). Five- and ten-month follow-up showed no evidence of recurrence of the AVM, and the child resumed her normal life and went back to school. She was happy, playful, and back to her normal weight and her quality of life has improved.


A 37-month-old male infant from Guangxi Province, China, with recurrent pneumonia since infancy exhibited recurrent pain in the right abdomen and fevers for 3 months. The abdominal pain was related to eating food, but not to physical activity and position. His maximum temperature was 40 °C. Physical examination revealed lymphadenopathy in the left neck and hepatomegaly. His abdomen was soft with normal bowel sounds; however, the liver and spleen were palpable 4 cm below his costal margins. The rest of the physical examination was unremarkable. Routine blood tests revealed 16.9 × 109/L leucocytes, 9.5 × 109/L neutrophils, 4.4 × 109/L lymphocytes, 0.22 × 109/L eosinophils, and 90 g/L hemoglobin. Blood was observed in his stool. The serum albumin and C-reactive protein levels were 29.0 g/L (40–60 g/L) and > 192 mg/L (< 10 mg/L), respectively. Erythrocyte sedimentation rate was 28 mm/h (≤15 mm/h). Serum aspergillus galactomannan antigen was 0.826 (normal<0.5). His CD4+ T-cell count was 1078 cells/μL (normal: 410–1590 cells/μL), while the percentage of his natural killer cells was 18.10% (normal: 9–15%). Serum immunoglobulin (Ig) M was slightly elevated, while IgG and IgA were normal. Serum aspartate aminotransferase, alanine aminotransferase, and creatinine levels, as indicated by the blood test, were all normal. Anti-nuclear and anti-HIV antibodies and INF-γ autoantibody were all negative. Blood and stool cultures were negative. Chest computed tomography (CT) showed disseminated patchy exudates throughout the left upper and lower lobes of the right lung. Contrast-enhanced CT of the whole abdomen showed hepatomegaly, intestinal wall thickening in the ascending colon, and mesenteric lymphadenopathy in the cecum with (Fig. a-b). Colonoscopy showed a cobblestone pattern (non-ulcerated mucosa separated by ulcers) with erosion, ulcer, polypoid lesions, and lumen deformation from the colon to the cecum (Fig. a-b). Crohn’s disease and intestinal tuberculosis were also considered based on the presence of multiple ulcers on colonoscopy and on the clinical manifestations. Therefore, the patient was treated with mesalazine and thalidomide for 1 week but without clinical improvement. After 5 days, the colon biopsy revealed mucosal ulceration and ulcers in the colon and massive infiltration of the mucosa and submucosa by the engorged macrophages. A large number of fungal spores were observed in the interstitial space and the macrophages (Fig. ). Subsequently, the patient underwent ultrasound-guided liver biopsy. Histopathological examination of the liver tissue revealed granulomatous inflammation (Fig. a), while periodic acid-Schiff staining revealed aggregates of macrophages engorged with numerous yeast-like organisms 2–4 μm in diameter. These yeast-like organisms were spherical to oval and had a transverse septum (Fig. b). After 2 weeks, the bone marrow culture confirmed T. marneffei, and the diagnosis of disseminated Talaromycosis involving the liver, colon, lymph nodes, and bone marrow was made. Intravenous voriconazole (12 mg/kg every 12 h) was administered for 4 weeks. Subsequently, the liver size reduced and was palpable 1 cm below the costal margin. Voriconazole was then administered orally (7 mg/kg twice a day). Unfortunately, 1 month later, his abdominal pain and fever recurred, and he presented with reduced urine output. Abdominal radiograph showed bowel perforation, pneumoperitoneum, and intestinal obstruction (Fig. ). Hence, an emergency exploratory laparotomy with intestinal resection, anastomosis, and a colostomy was performed. During the surgery, we observed the pebble sign with erosion in the ileocecal intestinal cavity. The lesion segment was approximately 8 cm long. The ileocolic junction was narrow and obstructed, and the adjacent intestinal ducts were edematous and thickened. Postoperative pathology indicated the presence of T. marneffei. Whole-exome sequencing was performed using the patient’s and his parents’ peripheral blood. A heterozygous missense mutation in exon 17 of the STAT3 gene (c.1673G>A, p.G558D) was found in the patient but not in his parents (Fig. ), indicating that the mutations were de novo.
The patient’s condition improved after the administration of intravenous voriconazole and antibiotics for 10 days and oral voriconazole for 7 months. Seven months after the surgery, repeat colonoscopy showed good recovery of the stoma located at the ileocecum, within 35 cm from the anal verge (Fig. c). No relapse was observed during the 18-month period of antifungal treatment.


A 49-year-old right-handed AA woman with a past medical history of rheumatoid arthritis, schizoaffective, and conversion disorder presented to the UAB emergency room for evaluation of altered mental status after a witnessed seizure. There was no travel or contact exposure to COVID-19, and she had no symptoms of COVID-19. At presentation, she had an axillary temperature of 99.5F, respiratory rate of 16 breaths per minute, heart rate of 120 beats per minute, blood pressure of 180/95 mm Hg, and oxygen saturation of 94%. The neurologic exam revealed lethargy and disorientation to time and place. There was no meningismus. CT head was normal. Continuous video EEG monitoring was obtained to rule out subclinical seizures and status epilepticus. Scalp EEG showed severe background slowing in the delta range and the presence of multiple seizures (approx. 4-6 every hour over the next 8 hours) emanating from the midline and left fronto-central regions (Figs. , ). Initially, there was a clinical correlate of facial twitching, head version to the right followed by a bilateral tonic–clonic seizure. In the latter half, there was electroclinical dissociation with the presence of only electrographic seizures. The patient was intubated and treated with multiple doses of lorazepam, levetiracetam, and propofol for sedation. Soon after arrival, the patient was tested for SARS-CoV-2 by reverse-transcriptase polymerase chain reaction (RT-PCR) through a nasal swab specimen, which returned negative. MRI brain without contrast was unrevealing for any definitive pathology. Her clinical course over the next 36 hours improved significantly with resolution in status epilepticus, and she was extubated within 48 hours. A lumbar puncture was not performed due to rapid improvement, and excellent response to levetiracetam. However, within 72 hours of the initial presentation, the patient developed fever, shortness of breath, myalgias, and nonproductive cough. A repeat nasal swab RT-PCR for SARS-CoV-2 returned positive. She remained stable with a mild cough and was eventually discharged home on levetiracetam and recommendation to self-quarantine.


A 73-year-old right-handed AA woman with a known history of hypertension, diabetes mellitus, and chronic kidney disease presented to the UAB emergency room with shortness of breath, lower extremity edema, and confusion. There was no travel history, and contact exposure to COVID-19 was unknown. A significant past neurological history included surgical repair of left frontal skull base ethmoidal encephalocele and ventriculoperitoneal shunt for hydrocephalus. At presentation, she had an axillary temperature of 90.6 F, heart rate of 44 beats per minute, blood pressure of 86/71 mm Hg, and oxygen saturation of 100%. There was no focal neurological weakness or neck stiffness, but she had diminished level of consciousness. Chest X-ray demonstrated patchy bilateral opacities and serum procalcitonin (0.53 ng/mL) was elevated. She was admitted to the intensive care unit with the diagnosis of sepsis. The next morning, bedside nurses noticed persistent face and arm myoclonus with worsening altered mental status that was concerning for status epilepticus. Scalp EEG showed marked background voltage attenuation and slowing, continuous 0.5-0.75 Hz bilateral independent periodic discharges (BIPDs) over the left and right hemisphere that evolved to form recurrent (approx. 5/hour) discrete seizures emanating from either right or left fronto-central-parietal regions (Figs. , ). Myoclonic status epilepticus with coma (MSE) was diagnosed and treated with intravenous levetiracetam, lacosamide, phenytoin, and midazolam to achieve burst suppression for 48 hours. Four days later, the EEG improved significantly with the cessation of epileptiform activity. However, clinically she worsened with multi-organ failure requiring blood transfusions and hemodialysis. Tracheal aspirate tested positive for SARS-CoV-2 by RT-PCR. Due to a strict isolation policy to minimize the spread of COVID-19, the MRI brain was not obtained. Instead, a CT brain and perfusion study were performed a day after the MSE ceased, and it did not reveal any abnormality that could explain the etiology of MSE. For the next 3 weeks, multiple attempts to arouse or extubate failed after sedatives were held. Care was withdrawn, and the patient expired.


A 60-year-old man with a past medical history of HIV and anal HPV was referred from his primary care provider to the urology office for an abdominal mass that was found incidentally upon abdominal ultrasound (US) after being admitted to the hospital for an acute kidney injury from dehydration. The patient denied any weight loss, abdominal pain, umbilical discharge, hematuria, or voiding difficulties. He is a former tobacco smoker with a 15-pack-year smoking history and has a history of alcohol abuse. Upon presentation, his vital signs and physical examination were unremarkable. Labs were also unremarkable, and his urinalysis was pertinent for a urine pH of 6 with minimal proteinuria and microscopic hematuria. A retroperitoneal US showed a 7.0-cm midline pelvic mass located just superior to the urinary bladder (Figure ).
We performed a subsequent CT scan of the abdomen and pelvis, which demonstrated a 7.3-cm multiseptate anterior bladder wall lesion (Figure ).
The patient underwent cystoscopy, which ruled out a bladder mass, diverticulum, inflammation, or stones and concluded a mass effect on the anterior bladder wall from a suspected urachal mass with no communication between the mass and the urinary bladder. Eventually, the patient underwent an elective open excision of the lesion which was found to be a sizeable urachal cyst. Because of the adherent nature of the mass to the surrounding tissue, the procedure was combined with partial cystectomy. A pathological examination from the tissue taken from the urachal cyst showed mucinous cystadenocarcinoma favoring urachal origin (Figure ). The patient was, therefore, diagnosed with Sheldon stage I, Mayo stage I, and T1N0M0 urachal mucinous cystadenocarcinoma. He is doing well and is disease-free at follow-up 10 months after the diagnosis.


A healthy 7-year-old boy was referred from the dental emergency department to the pediatric dentistry department in the Dental School of Casablanca.
Clinical examination revealed that the child has a flexible splint on maxillary teeth and a coronal restoration with a glass ionomer on 11. Radiographic examination showed incomplete root formation of both central incisors (Nolla's stage 8: short root with thin walls) ().
Based on the emergency file, it was found that the boy fell at school three weeks ago. Clinical examination revealed that 11 had a lateral luxation with an uncomplicated crown fracture and 21 had a subluxation. Emergency treatment was carried out on the same day and consisted of reattaching the tooth fragment, repositioning 11 with a gentle digital pressure, and stabilizing it with a flexible splint. One week after trauma, the patient presented with intraoral swelling on 11 revealing necrosis. Consequently, an endodontic treatment was performed and the root canal was instrumented using hand files with gentle irrigation using 0.5% sodium hypochlorite. Calcium hydroxide was placed as intracanal medication. The access cavity was sealed with glass ionomer cement.
For this purpose, the apexification technique using calcium hydroxide has been initiated on 11. The calcium hydroxide was renewed once resorbed. A regular clinical and radiological follow-up was carried out to check the vitality of 21 ().
6 months after the beginning of the treatment of 11, a radiolucent lesion in the periapical region was detected on 21 (). The negative response to the cold test confirms a pulp necrosis. Considering the immaturity of the tooth and the thinness of the root and after obtaining parental consent, the optimal treatment option was revascularization.
An access cavity was made under a dental dam. Once the canal orifice was well cleared, gentle root canal irrigation was performed with 2% sodium hypochlorite followed by irrigation with saline solution. Subsequently, the root canal was dried with large paper points until they were removed without evidence of fluids and filled with calcium hydroxide used as intracanal medication. A temporary restoration using a glass ionomer was placed.
The patient was recalled 4 weeks after the first appointment. The tooth was clinically and radiologically asymptomatic. In the second session, the tooth was anaesthetized, accessed, and irrigated gently with 2% sodium hypochlorite followed by saline solution to remove calcium hydroxide. The bleeding was induced with manual K files introduced into the root canal and placed at 2 mm beyond the working length. Once the blood clot was formed, MTA was placed in the cervical third of the root canal (). The access cavity was sealed with a glass ionomer.
Three months after performing revascularization treatment, visible root edification was observed (). The 6-month follow-up showed an important thickening of the root with calcification of the canal. Concerning 11, a renewal of the calcium hydroxide was carried out once the material has been absorbed ().
After 24 months of apexification treatment on 11, we noticed the formation of an apical barrier which was easily crossable with a file (). To solve that problem, the apical third was sealed using MTA and then the coronal two-third with warm gutta-percha. Concerning 21, the canal was almost completely obliterated with an extension of the root ().


A 53-year-old male patient was admitted with cough and blood in the phlegm and found to have an occupancy lesion (3.4 × 3.1 cm maximal section) in the left lower lobe on plain CT (Fig. A,B). The symptoms and mass images indicated lung neoplasm.
The patient had undergone a dual-source CT scan (Definition, Siemens, Munich, Germany). The patient was scanned approximately 16–20 sec after contrast agent was injected. The scanning range was defined from the plane of the thoracic inlet to the diaphragmatic plane. The collimator thickness was 0.6 mm, and the reconstruction thickness was 1 mm.
CT angiography showed that the mass was an ASA originating from the descending aorta with a saccular aneurysm in the basal segments of the left lower lobe (Fig. C–E, Video ). The patient was diagnosed with anomalous systemic arterial supply to normal basal segments of the left lower lobe. The distal left pulmonary artery ended and was distributed in the lingual and dorsal segments, while the pulmonary artery basal segments were absent and substituted by the ASA (Fig. E–G).
DICOM data were transferred to an intelligent/interactive qualitative and quantitative analysis (IQQA) 3D reconstruction system and its web based workstation (EDDA Technology, Princeton Junction, NJ, USA). The differences in the density of the contrast agent, as well as the measurements such as axis direction and vessel diameter, are automatically identified to distinguish between arteries and veins. The data are analysed, calculated, segmented, and rendered to describe and interpret the morphology and spatial position of the structures. And the IQQA 3D-reconstructed images of vessels, bronchi and segments are merged into a single 3D image. The location and course of the ASA was identified (Fig. G, Video ). Based on the course of the bronchi and blood vessels, the spatial conformation of the bronchial tree and accompanied arterial tree is reconstructed, and the stems of venous branches threading between are intersegmental veins, which manages the automated the lobulation, segmentation and subsegmentation (Fig. G–I, Video ). Then, we had got a clear imaging profile of the disease. The patient recovered from cough and blood in the phlegm with medical treatment denied further invasive therapy to cure this disease although being informed high-risk prognosis of conservative measures.
Permission was obtained from the patient to publish this case study.


A woman requested prenatal diagnosis because of advanced age (36 years old) and fetal evidence of cystic hygroma with increase nuchal translucency (value 7.6) observed at 13th week of gestation. After chorionic villus sampling, the analysis of cytotrophoblasts (direct analysis) showed a normal male karyotype.
At the same time Array CGH analysis on placental biopsy was performed and a segmental trisomy of 17p chromosome was evidenced: arr[GRCh37] 17p13.3p12(1693_13611322)x3 (C). FISH study on the cytotrophoblasts revealed that the trisomic 17p region was translocated on one chromosome 15p (D). In the maternal karyotype a balanced translocation between 17p and 15p was identified (E) and confirmed by FISH (F). FISH with β-satellite probe showed a positive signal on the p-arm of the derivative 17 and the absence of signal on the derivative chromosome 15 (G). In 9% of the examined metaphases, association with acrocentric p-arms was observed.
At 15th week of gestation, the pregnancy was interrupted and persistence of cystic hygroma was observed.
The woman had two previously pregnancies: the first, a twin pregnancy, was spontaneously aborted in the first trimester (no cytogenetically investigated); the second one had ended in a phenotypically normal female. In both cases karyotype analysis was not performed.


A nine-month-old girl, with unremarkable clinical history, presented to our emergency room with a history of three days of fever and one day of diarrhea. Three hours prior to admission to our center, the patient suffered a generalized tonic-clonic seizure that lasted almost 30 minutes. It required two doses of IV diazepam, one dose of phenytoin, and endotracheal tube protection of the airway. The patient then presented cold extremities and liquid stools. It was necessary to reanimate with 50 cc/kg of normal saline initially and then another 50 cc/kg of crystalloids prior transfer to our center. The patient was admitted to our center in evident hypovolemic shock, with mydriatic pupils, no spontaneous movements despite no sedation, and absence of brainstem reflexes.
Laboratory findings showed metabolic acidosis (pH: 7.18; pCO: 26.5 mm Hg; HCO: 10 mEq/L; lactate: 2.4 mmol/L), pancytopenia (hemoglobin: 10.8 g/dL; leucocytes: 2,320/mm3, neutrophils: 33%; bands: 16%; lymphocytes: 46%, and platelets: 122 x 103/mm3), negative C-reactive protein, normal renal function test and electrolytes, elevated hepatic transaminases (AST: 888 U/L; ALT: 232 U/L), and coagulopathy (PT: 39%; ATTP: 73.2 s).
CNS computed tomography (CT) revealed severe diffuse cerebral edema, collapsed lateral ventricles, thigh basal cisterns and, no clear differentiation between gray and white matter (Figure ).
Cefotaxime and acyclovir were started empirically. Cerebral spinal fluid (CSF) analysis revealed HHV-6 using BIOFIRE® FilmArray® Meningitis/Encephalitis Panel (bioMérieux, Marcy-l'Étoile, France). Neurologic death was declared five hours later. CSF and bloodstream cultures were negative. Autopsy was carried out and reported herniation of cerebellar tonsils, cerebral edema, and meningoencephalitis due to HHV-6 by polymerase chain reaction.


A 45-year-old female presented with complaints of progressive dysphagia for six months. Outside endoscopy showed a friable and fixed lesion in the esophagus suspicious of malignant growth and biopsy was taken. She was referred to us after the biopsy came out to be negative. We did a repeat endoscopy that revealed an impacted denture in the mid-esophagus. On eliciting the history, the patient recollected that she lost her denture 13 years back and was unaware that she swallowed it. On examination, the patient was hemodynamically stable. X-ray and CT scan revealed a crescent-shaped hyperdense foreign body in the esophagus at the level of aortic arch T4 (Figure ).
Under general anesthesia, the patient was positioned in left lateral side and thoracotomy was made in the right fifth intercostal space. The impacted denture was identified and removed by esophagotomy (Figures -).
The esophagotomy site was closed with interrupted 3-0 polydioxanone (PDS) sutures and reinforced with viable pedicled muscle flap from right fifth intercostal space (Figure ).
The patient had an uneventful postoperative recovery. Oral intake was started on postoperative day three and the patient was discharged on postoperative day seven.


The patient was a right-handed man aged 74 years who had a right-hemispheric ischemic stroke in the thalamus, capsula interna, and right temporal lobe 7 years before participating in the experiment. Acutely after stroke, he had mild motor deficits in the left leg (National Institutes of Health Stroke Scale leg item score 1/4). At the time of this experiment, he had minor limitations of sitting and standing balance (Berg Balance Scale 54/56); he also had mild limitations in motor function (Fugl-Meyer Motor Assessment lower extremity subscale 29/34) and strength (Motricity Index lower extremity subscale 88/100) of the affected left side. The patient walked independently without a walking aid (Functional Ambulation Categories 5/5), with a comfortable walking speed of 1.0 m/s and a step length of 0.56 meters as measured by the 10-Meter Walk Test. The patient had slight risk of falling (Dynamic Gait Index 19/24). He reported numbness in the feet and a feeling of wearing socks when he was not. In addition, a mild cognitive impairment was present (Montreal Cognitive Assessment 25/30).


A 36-year-old man was born with a spinal cord lipoma at the L5-S1 level. Soon after birth, he underwent surgery for spinal lipoma removal. He could walk whilst dragging the left foot, and he performed bladder expression (Credé maneuver) during childhood. The modality of urination was changed to CIC at the age of 10 years, owing to vesicoureteral reflux. He underwent cystometrography at 27 years of age: his bladder compliance was as low as 9.0 mL/cmH2O with a capacity of 100 mL. Anticholinergic agent administration was initiated. Consequently, his bladder capacity increased to 200 mL, bladder compliance improved, and urination interval lengthened. Regarding evacuation, he started transanal irrigation at the age of 27 years, and his QOL improved. He could experience erection and ejaculation. His SHIM scores were 3-3-3-2-2, indicating mild-to-moderate erectile dysfunction, and his force of ejaculation was weak.
He got married at the age of 28 years but failed to father a child for 2 years thereafter. Consultation with a fertility specialist and sperm examination showed a sperm quantity of 0.6 mL, density 10 300 000/mL, and motility rate 43.7%; all three parameters being lower than normal according to the World Health Organization criteria. Furthermore, the sperm deformity rate was 24.4% (Fig. ). The sizes of the patient’s right and left testes were 11.7 and 7.3 cm3, respectively. The sizes are small compared to the standard. It was concluded that natural conception would be difficult; thus, the patient underwent several rounds of ICSI. After undergoing fertility treatment for 4 years, his wife conceived and gave birth to a healthy baby.


A 63-year-old Japanese man (SIMC-Uro #9950, a unique non-sequential patient control number in the department of SIMC-Uro) visited a nearby clinic due to intermittent macroscopic hematuria. He had a medical history of mild diabetes mellitus and surgery for colon carcinoma. Physical and laboratory findings were within normal limits except for the presence of microhematuria at the clinic. Cystoscopy revealed a broad-base non-papillary bladder tumor. CT (#1, ) and MRI (#1, Fig. a) revealed a bladder tumor invading perivesical tissue without apparent metastases (cT3N0M0). He underwent transurethral resection of the tumor, and the pathological diagnosis of the resected specimen was UC that invaded sub-epithelial connective tissues (pT1).
He was referred to the SIMC-Uro for the treatment of the invasive bladder tumor. Physical and laboratory findings were within normal limits (height 175 cm, body weight 63 kg). CT #2 at the SIMC detected no metastatic lesions. He underwent three cycles of monthly neoadjuvant chemotherapy using gemcitabine (1000 mg/m2, day 1, 8, and 15) and cisplatin (70 mg/m2, day 2), followed by radical cystectomy and extended pelvic lymph node dissection. Histopathological examination detected high-grade UC in the previously resected portion even after chemotherapy, however, no cancer cells were detected in the surgical margin of the bladder specimen (pT1N0).
CT #5 which was performed 266 days after radical cystectomy, identified cancer recurrence in the pelvic wall (50 mm in length) which invaded to the rectum (Fig. b). Transrectal biopsy of the tumor pathologically conformed the recurrence of the bladder cancer. Irrespective of two cycles of salvage chemotherapy using the same regimen as the neoadjuvant setting, the recurrent tumor significantly enlarged to 81.6 mm (CT #5, Fig. b).
The patient initiated treatment with tri-weekly 200 mg of pembrolizumab injection 283 days after radical cystectomy. Of note, 9 days before the treatment, the case complaint of any symptom except for mild diarrhea (Grade 1 in Common Terminology Criteria for Adverse Events version 4.0 []) due to unknown reason. At the day of Grade 1-diarrhea, laboratory data including serum CK level (CK: 34 [normal range: <244] U/L) (Fig. ) and ECG (#1, Fig. a) were normal. From 12 to 34 days after the administration of pembrolizumab, he serially complained of Grade 3-diarrhea, Grade 3-erythema multiforme with pruritus and left ptosis with diplopia. Our comprehensive management team consisting of pharmacists, nurses, cardiologists, and neurologists intervened to this case. Laboratory examination showed significantly elevated CK (3385 U/L), its isozyme CK-MB (62.5 [<5] n/mL), and troponin I (372.1 [<26.20] pg/mL) (Fig. ). ECG#2 findings revealed a right bundle branch block (Fig. b) which was not observed at ECG#1 (Fig. a) although echocardiographic findings were normal. Overall, the ECG, echocardiography, and laboratory data suggested that he had mild myocardial damage with conduction disturbance. MG and myositis due to autoimmune abnormality was unlikely, because their specific examinations including repetitive stimulation test, tensilon test, electromyography as well as serum measurements of anti-AChR Ab and anti-MuSK Ab were all negative. Consequently, the case was diagnosed as MG with myositis with systemic myopathy involving the cardiac and facial muscles as an irAE.
He initiated 60 mg/day of prednisolone (1 mg/kg, Fig. ). Serum levels of CK, CK-MB, and troponin I as well as symptoms including diarrhea and ptosis gradually improved. Although the conduction disturbance remained in the remote period, the patient demonstrated no impairment of ventricular function throughout the whole observation period. Interestingly, the tumor significantly shrunk to 25 mm only 49 days after the single pembrolizumab administration (CT #6, yellow arrowheads in Fig. c). The dose of prednisolone was gradually reduced to as shown in Figure . The tumor remained small without any treatment after a single injection of pembrolizumab as of 201 days after the pembrolizumab injection (CT #8, Fig. d). Prednisolone was withdrawn 362 days after the pembrolizumab injection (321 days treatment with prednisolone). Consequently, irrespective of severe neuromuscular complications, this case accomplished putative pathological complete remission by the single injection of pembrolizumab.


The patient was a 36-year-old male with a history of cardiac arrest five years earlier, status post-implantable cardioverter-defibrillator (ICD) placement with no other comorbidities; the patient was not on chronic medications. He presented to the emergency department (ED) in the morning complaining of palpitations and three ICD shocks overnight. He admitted that he had consumed six drinks containing vodka the night before, but stated that he only drank sporadically and rarely had more than one drink. The patient reported palpitations prior to the ICD shock and mild chest pain after the shock delivery but denied shortness of breath, dizziness, and loss of consciousness.
In the ED, his initial vital signs were as follows: blood pressure of 120/75 mmHg, heart rate of 85 bpm, afebrile, and oxygen saturation of 98% on room air. He was initially placed on telemetry, which showed atrial fibrillation with heart rate in the 90s; however, subsequent 12-lead electrocardiogram (EKG) demonstrated that he had spontaneously converted into sinus rhythm, with no ST deviations and a QTc of 461 msec. Additionally, there were no significant laboratory abnormalities (complete blood count within normal limits, Mg 2.2 mEq/L, K 3.6 mEq/L, Ca 9.6 mg/dL, Phos 3.4 mg/dL, Cr 1.0 mg/dL); troponin was negative (ref <0.3 ng/mL), and urine toxicology was negative for cocaine, amphetamines, barbiturates, cannabinoid, and opiates. The ethanol level was not checked.
While in the ED, the patient was found to have multiple episodes of non-sustained ventricular tachycardia (VT) on telemetry, which resulted in another ICD shock. He was admitted to the cardiac care unit (CCU) and given lidocaine bolus 100 mg, followed by continuous infusion at 2 mg/min. The patient continued to have multiple episodes of VT and ICD shocks, and amiodarone infusion was started at 1 mg/min after an initial bolus of 150 mg. Because of the increasing distress and agitation, the patient was sedated and intubated. Despite dual anti-arrhythmic therapy, he continued to have VT storm and received approximately 25 ICD shocks in two hours, followed by ventricular fibrillation (VF), requiring external defibrillation and cardio-pulmonary resuscitation for eight minutes. A review of the patient’s EKG during VT storm indicated premature ventricular complex (PVC) triggering polymorphic VT with the R-on-T phenomenon (Figure ).
Given his unstable condition, a short-term external circulatory assist device was placed, and he was started on extracorporeal membrane oxygenation (ECMO) support. He continued on amiodarone drip and temperature control through the ECMO circuit thermoregulator. No recurrent episode of VT/VF or ICD shock was noted after the ECMO placement. Initial transthoracic echocardiogram (TTE) showed severe global hypokinesis (ejection fraction of 20%), with no regional wall motion abnormality. The patient initially required norepinephrine and dobutamine for pressor and inotropic support but was able to wean off vasopressors, sedation, and he tolerated ECMO flow reduction overnight. A Repeat TTE performed one day later showed normal left ventricular function, making ECMO withdrawal possible on the third day of admission. The patient was subsequently extubated and found to have no residual neurological deficits. Amiodarone was discontinued, and he was started on oral flecainide 100 mg twice daily and diltiazem 120 mg daily for PVC suppression. He remained in sinus rhythm, with no ventricular ectopy on telemetry.
A cardiac MRI was performed and showed no late post-gadolinium myocardial enhancement suggestive of myocardial fibrosis and no evidence of structural abnormalities; however, the test was suboptimal because of artifacts. A cardiac positron emission tomography (PET) scan demonstrated a structurally normal heart, with no evidence of infiltrative or inflammatory disease. As there was no ventricular ectopy on telemetry for several days, it was determined that an electrophysiology study would likely not be beneficial. The patient was discharged after 10 days of hospital course.
Of note, the patient had experienced an earlier similar episode of VT/VF cardiac arrest at 31 years of age. At that time, the patient had drunk at least four drinks with vodka the night before the event, which he described as an unusual amount of alcohol for him. After his wife had witnessed him collapsing in the morning, EMS had been contacted, and he had been successfully resuscitated after seven defibrillations and a total on-scene time of approximately 38 minutes. Subsequent in-hospital workup had shown normal coronary arteries on angiography and a structurally normal heart with an ejection fraction of 60% on TTE. One of the patient's EKGs demonstrated a prolonged QTc (540 msec), and he had an ICD implanted for secondary prevention prior to discharge. As an outpatient, he had a genetic test for long QTc syndrome, reportedly inconclusive. No history of early coronary disease, cardiomyopathy, or sudden death in his family had been reported. He has two children who also underwent a genetic test for long QT, both negative. Upon follow-up, the patient’s EKG had shown a QTc of 428 msec. The patient had never experienced an episode of ICD shock until his presentation to ED five years later.


A term born, second born, non-breech presentation, caesarean section, seven-month-old girl was referred to the hospital with a diagnosis of a congenitally dislocated right hip. She had a negative family history for hip disease. Limited abduction of the right hip compared to the healthy contralateral side, asymmetrical skin folds of the right thigh and a positive Galleazi sign (shorter right limb) indicated a resting dislocated (Ortolani) right hip. Anteroposterior X-rays of the pelvis and ultrasound examination of the right hip confirmed the diagnosis (Figures , ).
Clinical signs of instability were positive. The right hip was reduced using the Ortolani manoeuver and was redislocated when the hip was abducted less than 45 degrees. Restricted abduction of the right hip compared to the contralateral side, indicating adductor muscle contraction, was evident. Due to the relatively delayed diagnosis, the patient was placed in longitudinal skin traction for one week, in order to stretch soft tissues around the hip joint and to facilitate closed reduction.
The knee-extension-hip-dislocation manoeuver
After one week in traction, the patient was examined under general anaesthesia. The right hip reduced with maximal abduction (about 80 degrees) using the Ortolani manoeuver. From this position of maximal abduction (and while in 90 degrees of flexion), the right hip was gradually brought to the minimally abducted position, where the femoral head was still stably seated in the acetabulum (about 45 degrees of abduction). In the clinical setting, this is the position the surgeon would prefer to immobilize the hip in order to treat the patient.
The question is, is the hip joint really stable with hip abduction at 45 degrees and hip flexion at 90 degrees, irrelevant of the position of the knee? And what would be the preferred treatment? A below-knee hip spica, an above-knee hip spica or a Pavlik harness?
At this point, knee extension started. At 60-80 degrees of knee flexion, some resistance was felt, while the examiner was applying force on the tibia. After a few more attempts to extend the right knee, the right hip re-dislocated (Figure ).
An arthrogram of the right hip followed. Anatomical reduction of the femoral head in the acetabulum (femoral head in contact with the acetabular floor) was confirmed with hip flexion at 90-100 degrees and hip abduction at 45 degrees (Figure ).
Percutaneous adductor tenotomy was performed in order to release tension of the contracted adductors of the right hip. As expected, adductor tenotomy facilitated hip abduction. However, hip dislocation with knee extension was unaffected by adductor tenotomy, indicating involvement only of the hamstrings on the knee-extension-hip-dislocation manoeuver. Immobilization with a hip spica in the arthrographically determined the position of stable reduction (hip flexion 100 degrees, hip abduction 45-50 degrees) followed. Extension of the hip spica below the knee, with the knee in 90 degrees of flexion, was decided to avoid the adverse effect of knee extension on hip stability (Figure ).
The patient was followed in regular intervals. After a few weeks, the nucleus of ossification of the affected hip appeared. The hip spica was removed after four months according to the clinical examination and the radiographic appearance of the hip. The development of the right hip improved significantly. Complete resolution of acetabular dysplasia was manifested at the age of four years (Figure ).


A 95-year-old man presented for a tender lesion on the right thumb that had been present for more than two weeks. His medical history included numerous nonmelanoma skin cancers, no family history of melanoma, no other skin lesions or rashes of concern, and no associated unexplained fevers or weight loss. He appeared well developed, well nourished, and in no apparent distress.
On the palmar aspect of the right thumb metacarpophalangeal joint was a 4.5×3.5 cm keratotic indurated flesh-colored nodule with a central keratotic core (Figure ). It did not appear to be mobile, but did have a suggestion of deep extension. It was tender to palpation, with moderate pain severity, and could not be drained. Examination of the right epitrochlear and right axillary lymph node region was unremarkable.
Radiography of the right hand excluded bone involvement (Figure ). A 3-mm punch biopsy was performed and showed well-differentiated squamous cell carcinoma with regressing keratoacanthoma-type features, with deep biopsy edge involved.
The patient and his son declined the proposed treatment with amputation due to his age. They accepted the proposed nonsurgical treatment with intralesional methotrexate. After an alcohol and Techni-Care skin preparation using an antiseptic from the Care-Tech Laboratories, Inc. (St. Louis, MO), the tumor borders at four quadrants and central portion were injected with 12.5 mg/mL each of intralesional methotrexate, for a total of 2 mL, 25 mg of methotrexate. The treatment was repeated twice more at an interval of one month.
The tumor showed considerable regression after the first injection, and had completely disappeared at three-month follow-up. The patient tolerated the procedure without any difficulty or adverse effect.


A 22-year-old African-American male with a medical history of childhood asthma presented to the ED after suffering from a witnessed syncopal event. The patient had prior consumption of marijuana throughout the evening. He denied the use of any other medications. The presentation history was provided by the patient's girlfriend. She stated that she had witnessed the patient experience a syncopal event while on the couch. The patient recalled falling asleep on the couch and, upon waking, had found his girlfriend standing over him. There was no postictal confusion state and no report of seizure-like activity. Upon arriving at the ED, the patient went into a spontaneous, transient pattern of ventricular tachycardia and torsades de pointes with pulse (Figure ). After these rhythms were aborted with amiodarone and magnesium, the patient converted into new-onset atrial fibrillation. Further inquiry into the patient’s family history revealed that two of his maternal aunts had internal cardioverter defibrillators (ICDs) implanted for unknown reasons. There was no other history of ICD, Brugada, or sudden cardiac death in the family. Notable workup in the ED revealed detectable but trivial serial troponins, no electrolyte abnormality, and urine drug screen (UDS) positive for cannabis. A 2D echocardiogram showed preserved left ventricular ejection fraction (LVEF) of 64% without structural and functional abnormality. An electrocardiogram (EKG) was obtained, which showed classic type II “saddleback” morphology in V2 (Figure ). A second EKG was obtained with anterior leads V1 and V2 elevated approximately 2 cm on the chest, over the second intercostal space, resulting in classic Brugada type 1 pattern (Figure ). The patient was monitored in the ICU, and care was taken to maintain potassium levels above 4 mmol/L and magnesium above 2 mmol/L, and to maintain core body temperature below 38 °C. The patient underwent ICD placement and was cautioned against further use of marijuana; he was also prescribed several other medications and advised on certain conditions as per The Brugada Foundation guidelines [].


A 34-year-old male presented with complaints of swelling on the anterior aspect of the left knee joint for three months. It was insidious in onset and slowly progressively increasing in size. The swelling has been associated with moderate dull aching pain for 10 days. Swelling had ruptured 20 days before presentation with pus discharge, but there was no decrease in the size of the swelling. He gave no history of fever or any complaints in movements at the knee joint. The patient was diagnosed as a case of myasthenia gravis, and he has been on steroids since then. The patient had also developed steroid-induced diabetes mellitus. The patient was admitted with complaints of weakness of bilateral upper and lower limbs. On examination, there was a swelling on the right knee joint about 15 × 10 cm in size in the subcutaneous plane, and also three more swellings of maximum size 4 × 5 cm in the subcutaneous plane in the anterolateral aspect of the upper two-third of the right leg (Figure ).
The patient was evaluated. Fine needle aspiration cytology (FNAC) of the swelling showed sheets of neutrophils in a necrotic background. There were many fungal organisms, which are slender, septate hyphae with variable branching bulbous ends. A few of the hyphae had pigmentation morphologically suggestive of PHM (Figure ).
Periodic acid-Schiff (PAS) stain had highlighted the fungal organism. MRI of the right lower limb was done, which showed a hypoechoic cystic loculated lesion in the subcutaneous plane with no involvement of the underlying knee joint (Figures , ).
The patient was optimized for surgery and excision of the right knee and right leg swellings was done under spinal anesthesia (Figure ).
The postoperative period was uneventful, and the patient was discharged. Surgical site healed well and sutures were removed on postop day 7.


The patient was a 45-year-old woman who was referred to a nearby clinic for fever of 38 °C, cough, and nasal discharge. Although she was treated with antibiotics, her signs were not improved. Chest X-ray and computed tomography showed a 61 × 45-mm anterior mediastinal tumor (Fig. ). Positron emission tomography scan showed 1.8-fold greater uptake than the maximal standardized uptake value in the tumor. A blood test revealed a serum immunoglobulin G level of 239 mg/dL (normal range 870–1700 mg/dL), serum immunoglobulin A level of 24 mg/dL (normal range 110–410 mg/dL), and a serum immunoglobulin M level of 26 mg/dL (normal range 46–260 mg/dL). She was referred to our hospital for further examination and treatment for the anterior mediastinal tumor and hypogammaglobulinemia. The histopathological diagnosis of a CT-guided biopsy specimen was type AB thymoma based on the World Health Organization classification, leading to the diagnosis of Good syndrome.
While undergoing diagnostic workup, the patient developed sudden deafness that was treated by corticosteroids. She then became febrile with worsening liver function, showing a serum aspartate aminotransferase level of 127 U/L and a serum alanine aminotransferase level of 132 U/L. She developed serum cytomegalovirus antigenemia, and altogether, the findings were diagnosed as cytomegalovirus hepatitis due to hypogammaglobulinemia. She received 15 g of immunoglobulin and ganciclovir with subsequent improvement in her liver function, with normal serum levels of aspartate aminotransferase and alanine aminotransferase. Her serum cytomegalovirus antigenemia was undetectable 2 weeks after initiation of antiviral therapy.
After her cytomegalovirus hepatitis improved, the patient underwent surgical resection for thymoma. Because she was immunocompromised, we performed a video-assisted left anterior thoracotomy with an 8 cm skin incision instead of a median sternotomy to minimize the risk of a perioperative infection (Fig. ). We administered immunoglobulin twice before surgery, and thymectomy was performed 3 months after the diagnosis of cytomegalovirus hepatitis. The postoperative course was uneventful without signs of infection, and the patient was discharged 10 days after the surgery. Macroscopically, the tumor was encapsulated grayish-white mass with a size of 80x42x63mm (Fig. a). Pathological diagnosis showed type AB thymoma (Fig. b).
The patient remains alive without recurrence of thymoma for 26 months. Her hypogammaglobulinemia has persisted, and she has undergone regular administration of immunoglobulin therapy (Fig. ). She has not developed signs of infection since the immunoglobulin therapy was initiated. Sudden deafness was not improved by corticosteroids. Six months after thymectomy, the cochlear implant was performed for deafness.


A case of a seventy-two-year-old male patient is reported, diagnosed with coronary artery disease and referred for CABG. Coronary-angiogram revealed triple vessel disease. Echocardiogram showed no regional wall motion abnormality (RWMA) and Ejection-fraction (EF) of 55%.
LIMA--Mid-LAD (Left Internal mammary artery-to-mid Left anterior descending artery), RSVG--Distal-LAD (Reverse-saphenous vein-graft-to-Distal Left anterior descending artery) and RSVG-Ramus anastamoses were completed off-CPB, using Octopus(Maquet-Acrobat-i. OM-10000) tissue-stabilizer with 200 mmHg negative-pressure. The other coronaries were diffusely diseased, non-graftable.
Post-grafting, electrocardiogram (ECG) was unremarkable, hemostasis was done. Sternal wiring was done. ST-segment elevation was noted in the lateral-leads on ECG, associated with hemodynamic deterioration. Sternal-wires were cut and chest was re-opened immediately. A large hematoma was noted near the Ramus-graft. CPB was established emergently. Examination revealed the hematoma, measuring about 5X6cm, present at the site of application of the octopus stabilizer during the RSVG-Ramus anastamosis, covering the part of the LV between LAD and Ramus.
A nick was made in the epicardium to evacuate the hematoma and limit the dissection. Rapid re-accumulation of blood was noted underneath the epicardium. Repeated attempts were made to take plication-sutures over the epicaradium but were unsuccessful.
The epicardial-membrane was opened which revealed a bare-area of the myocardium (), with multiple bleeders, at the sites of thebesian-vessels. Heart was arrested with cardioplegia.
Once arrested, attempts were made to seal the bleeding-areas with Fibrin-sealant but were unsuccessful. A large autologous-pericardial free-patch was prepared and the myocardium was covered (), using Cyanoacrylate-glue. Sufficient contact-time was given. Aortic cross-clamp was removed. Patient was weaned off CPB with intra-aortic balloon-pump. Post-operative echocardiogram revealed minimal pericardial-effusion, RWMA of antero-lateral walls, EF of 45%. Patient was discharged on ninth postoperative day, and examined a week later. Follow-up echocardiogram showed an EF of 50% and RWMA had reduced. The patient continues to be followed up.


We present a clinical case report of a 34-years-old pregnant woman with a single gestation. An omphalocele enclosing mixed intestinal and hepatic content was diagnosed at 12th week (). Nuchal translucency (NT) was normal, and no other associated malformations were observed. Parents were counseled regarding ultrasound findings. A genetic study was performed including karyotype, 60K Array-CGH, and methylation-sensitive multiplex ligation probe analysis to analyze the presence of epigenetic and genetic changes related to BeckwithWiedemann Syndrome (BWS), all of them being negative. Parents were informed of the results, as well as the impossibility of ruling out BWS completely despite the negativity of the genetic study, since this disorder is caused by epigenetic defects and there are up to 20% of cases whose diagnosis is clinical and therefore postnatal. After counseling, the patient decided to continue with gestation. The 20th week anomaly scan showed no associated malformations, except a slight upward and leftward heart displacement due to diaphragm elevation. Cardiac function and ductal venosus flow were normal throughout gestation and the heart was structurally normal. At 21st week, the omphalocele content became only hepatic (). Ultrasound controls were scheduled every 4 weeks to rule out the early onset of fetal growth restriction, as well as other disorders. At 37th week the estimated bag size was 51 × 56 mm (). At 38.3 weeks, the patient was admitted because of mild but regular contractions. At 38.4 weeks, a caesarean section was performed with the coordination of the Obstetrics, Neonatology, Anesthesiology, and Pediatric Surgery teams. The newborn weighed 3540 g, Apgar Test 9-10, and pH 7.32 (). A thermal bag covering the whole baby's body was placed immediately after the birth to maintain body heat and reduce the risk of infection (). The baby was admitted in the Neonatal Ward for management and surgical preparation in the first hours, starting parenteral nutrition after childbirth. At 48 hours of age, he underwent surgery to repair the omphalocele. A primary closure of the herniation was the first choice, though mesh repairing using a synthetic patch was considered depending on the intra-abdominal pressure (IAP), which was monitored throughout the intervention. As IAP was 15 cm H2O, a primary closure of the defect was performed (Figures , , and ). The newborn evolved favorably. He remained relaxed with a rocuronium perfusion for 72 hours after surgery. Analgesia with fentanyl and midazolam perfusion was administered for 6 days after surgery. Intubation was removed 5 days after surgery. Oral feeding was introduced from the 6th day of the procedure. Parenteral nutrition could be removed after 15 days of life. Intestinal transit was normal on the 6th day of surgery. At 3 months of age, two inguinal hernias were diagnosed and repaired surgically (). During the exploration of the left herniated sac, the ileocecal junction and the appendix were observed inside. Due to this abnormal location, a prophylactic appendectomy was performed (). Currently, the baby is one year old and he is alive and well ().


A 65-year-old Chinese man, who was diagnosed with laboratory-confirmed COVID-19, was admitted to our isolation ward on 25 March 2020. A week before his admission, he flew in from Brazil airport, and was subsequently under home isolation. On 23 March 2020, he presented with fever (38.8 °C), chills and myalgia. Viral RNA test from throat swab was positive. The cycle threshold (Ct) values of open reading frame 1ab (Orf1ab) and nucleocapsid (N) genes by RT-PCR assay were 20 and 21, indicating a high viral load. Computed tomography (CT) scan showed bilateral pneumonia. Laboratory findings showed high lactic acid level (2.1 mmol/L) and C-reactive protein (CRP, 48.8 mg/L), and low white blood cell count (1.96 × 109/L), indicative of severe COVID-19. Oxygen and atomized inhalation of recombinant human interferon-ɑ2b was given at admission. CP from two convalescent patients was intravenously given, and hydroxychloroquine (Shanghai Zhongxi Pharmaceuticals, Shanghai, China) was orally administrated for a week (0.2 g, three times a day).
As shown in Fig. , on day 4 after CP transfusion, the lactic acid and CRP levels remained high (2.1 mmol/L and 73.23 mg/L, respectively). The arterial oxyhemoglobin saturation (SaO2) decreased to 86%, while the OI decreased to 76 mmHg, and mechanical ventilation was administered. His temperature returned to normal and the OI ascended above 300 on day 11, after which the ventilator was withdrawn. On day 11 after CP transfusion, the RNA test remained positive in throat swab, and CT revealed severe pulmonary lesions (Fig. ).
In addition, no apparent side-effects were found during CP transfusion and hydroxychloroquine treatments.


A 27-year-old male, without any family history of the cancer events, suffered from left posterior thigh pain for more than two years. The pain had an insidious onset and became more intense gradually. He denied any pre-existing neurological diseases and trauma at the painful region. He had visited several doctors where possible diagnoses like intervertebral disc herniation, lumbar-sacral radiculopathy, myofascial pain syndrome and fibromyalgia were given. Various kinds of therapies had been applied, such as local injection, lumbar traction, heat thermal modalities, manual exercise and acupuncture. At his worst moment, oral Ultracet (tramadol 37.5 mg/acetaminophen 325 mg) and pregabalin 75 mg were needed for pain control. In recent few months, the pain had made it difficult for him to sit on the chair. He was then referred to our clinic for better management.
During physical examination, neither tenderness nor numbness was noted at the paraspinal and gluteal areas. No radiating pain was elicited by lumbar flexion, extension, rotation and lateral bending. The straight leg raising test yielded a negative finding. The Tinel sign was positive when compressing his left posterior mid-thigh, and the numbness and tingling sensation were radiated to the lumbar, gluteal and leg regions. The muscle power and sensation (light touch and pinprick) were intact at both lower limbs. We performed an ultrasound examination of his left posterior thigh. A solid mass sized 2 cm × 1.7 cm × 2.2 cm with clear posterior enhancement was noted on the track of the sciatic nerve (Figure ). There was a well-defined border between the mass and surrounding tissues (Figure ).
We did not visualize a significant increase in power Doppler signals inside or adjacent to the mass. The magnetic resonance imaging (MRI) with contrast of gadolinium was arranged, revealing a well-defined mass located in the posterior compartment of the left thigh. The mass, abutting the left sciatic nerve, revealed a pattern of heterogeneous hyperintensity on T2-weighted imaging with the rim of fat around it (Figure ). Following the administration of contrast media, multiple ring-like enhancement indicating intraneural fascicular bundles was observed within the mass (Figure ). The nerve fascicles from the sciatic nerve were deviated medially to the mass (Figure ).
Under the impression of a neurogenic tumor, he received neurolysis and the pathology confirmed it to be a schwannoma. After the excision, the patient did not have any neurological deficit and achieved a complete relief of the pain.


A 48-year-old man with past history of CHD, who has been treated with O2 at home for several years because of dyspnoea and cyanosis, was admitted to our hospital on 8 April, 2020, due to exacerbation of dyspnoea and central cyanosis for 2 days. During physical examination, we heard harsh systolic murmur in upper left sternal border and generalised coarse crackle in both lungs. Mild clubbing was visible on examination of the limbs. His habitual history indicated that the patient was a cigarette smoker. We noticed a history of diagnosed but untreated CHD because of inability to afford treatment expenditures at the time of diagnosis.
At admission, the patient’s vital signs were as follows: BP:130/80 mmHg, PR:86 beats per minute, RR:23 per minute, T:39.5, and SPO2:70% in room and 86% by nasal cannula. First VBG was PH:7.45, PCO2:47, HCO3:33, and second VBG after initial treatment with O2 and inhaler was PH:7.47, PCO2:37, and HCO3:27. Electro Cardio Gram (ECG) showed increased right ventricular forces (tall R waves in V1); right atrial enlargement (prominent P waves in V1); right ventricular hypertrophy; and undetermined axis (Fig ). Other initial laboratory findings were WBC:11,100 (per μl), Hb:18.2 (g/dl), PLT:195,000 (per μl), lymphocyte count: 968 (per μl), CRP:52 (mg/L), LDH:542U/L, and procalcitonin (PCT):0.7 ng/ml (normal range: less than 0.5 ng/ml). Liver and kidney tests were normal.
After that, chest X-Ray was taken and with regard to patchy infiltration, he was susceptible to COVID-19 infection. Hence, chest CT-scan and Polymerase Chain Reaction (PCR) were requested. Chest CT-scan revealed bilateral lung involvement, ground glass appearance, and peripheral round opacity all in favour of COVID-19. Furthermore, distinct cardiomegaly and dilation of pulmonary trunk were observed (Fig a,b). Moreover, patient’s nasopharyngeal swab tested positive for 2019-nCoV PCR assay. Patient was managed by a team of physicians including fellowship of adult CHD, anesthesiologist, and infectious diseases specialist.
Patient received high-flow oxygen and common antiviral protocol of COVID-19. In addition to high level of procalcitonin and suspected concurrent bacterial pneumonia, he was also treated by an infectious disease specialist with antibiotics. Despite mentioned treatments, his dyspnoea progressed and SO2 decreased to 68% in the second day of hospitalisation, and patient was referred to ICU where he received prophylactic heparin to prevent thromboembolism, but due to absence of severe respiratory distress, we decided not to intubate him. On the third day of hospitalisation, due to the lack of appropriate therapeutic response and the disproportionate low level of oxygen saturation, limited portable echocardiography was performed and showed: situs solitus; D-looped ventricle; normal continuity of inferior vena cava to right atrium; moderate left ventricle enlargement and systolic dysfunction, severe right ventricle enlargement and systolic dysfunction and severe right ventricular hypertrophy; large sub-aortic ventricular septal defect (3.2 cm) with extension to inlet and bidirectional shunt (predominantly right to left); overriding of aorta; pulmonary artery originate from right ventricle with thick and dome-shaped pulmonic valve; relative dilation of pulmonary trunk (3.9 cm) and acceptable pulmonary branches (Fig ). All of these findings were highly suggestive of tetralogy of Fallot anomaly.
Consequently, with regard to these findings, we added diuretic and angiotensin-converting enzyme inhibitors to his medical regimen with adequate dosage which led to partially improved condition and increased blood oxygen saturation (89%). Fourteen days after the start of treatment, due to the relative improvement of the repeated chest CT-scan findings and his general condition, the patient was discharged with the necessary medication instructions and recommendations. Furthermore, we advised him to undergo a complete follow-up echocardiography and the necessary diagnostic and therapeutic measures.


Against a background of hypertension and migraine, a 54-year-old man was admitted to our hospital after 2 months with palpitations and activity-related presyncope, as well as influenza-like symptoms. Chest X-ray was normal. Electrocardiography showed type-II atrioventricular-block. A tentative diagnosis of myocarditis was made. Figure gives a timeline presentation of the successive diagnoses and the main clinical investigations.
Three weeks later, magnetic resonance imaging (MRI) showed oedema and multiple nodular structures in the heart. Positron-emission tomography (PET) revealed uptake by mediastinal lymph glands, some minor activity in the heart, and focal marked uptake in the right thyroid lobe, suspicious of malignancy. Chest computed tomography (CT) reported minor (10 mm) hilar lymph glands and multiple 2–5 mm nodular lesions in the lungs. Endomyocardial biopsies showed fibrosis and granulomas, in the absence of infectious agents (Figs. a, b), prompting a diagnosis of cardiac sarcoidosis. An automated defibrillator was implanted and corticosteroid therapy (prednisone) initiated.
Three months later, a fine needle aspirate of the thyroid was reported as suspicious for papillary thyroid carcinoma (PTC). A thyroid lobectomy was performed 27 days later, and pathology showed a BRAF-positive encapsulated PTC measuring 28 mm with diffuse necrosis. Four days postoperatively the patient developed signs and symptoms of meningoencephalitis. There were 39 leukocytes per μl cerebrospinal fluid (CSF) (with both polynuclear and mononuclear cells). Blood samples revealed the same findings as during the preceding 3 months; granulocytosis and minor lymphopenia. Antiviral therapy was started, but CSF and blood cultures 2 days later yielded growth of C. neoformans (Fig. c), identified at species level using matrix-assisted laser desorption/ionization-time-of-flight mass spectrometry (MALDI-TOF MS).
Fluconazole (800 mg) was given intravenously before the patient was transferred to a central hospital and the antifungal regimen was then changed to amphotericin B (a daily 200 mg infusion) plus flucytosine (1500 mg infusion twice daily); one month later parenteral treatment was terminated and flucytosine (1500 mg × 2) plus fluconazole (400–800 mg daily) were given during the next month, to be followed by fluconazole (400 mg) until 1 year. Cerebrospinal fluid was sampled weekly, showing sparse growth of C. neoformans after 1 week while later samples were without growth but with decreasing quantity documented by a C. neoformans antigen test until 2 months later. Neurological status including cognitive function was noted to be fully normalized at 1 month after onset of the cryptococcal infection. There were no signs of cryptococcus infection at follow-up 15 months after diagnosis, and antifungal treatment was terminated.
A retrospective fungal staining of the thyroid specimen revealed widespread cryptococcal elements in the PTC (Figs. d-f). In addition, cryptococcal elements were observed intravascularly (Fig. g), in the few non-necrotizing granulomas located in the thyroid parenchyma (Fig. h) as well as in an adjacent lymph gland (Fig. i). The types and distribution of cells within the thyroid granulomas (Fig. h) were typical for those commonly seen upon histopathologic examination of granulomatous inflammation, with centrally located giant cells and epitheloid histiocytes dominating, and with lymphocytes only at the outer rim []. Notably, there was no evidence of cryptococci in the heart sarcoid sections (Figs. a, b) or in non-cancerous thyroid parenchyma adjacent to areas with tumour transformation (Figs. d, g, h).
At the 2-year follow-up there was no evidence of thyroid cancer relapse, but the cardiac sarcoidosis was still active.


We report the case of a 63-year-old male patient who was diagnosed in July 2016 with a highly differentiated functional NET of the ileum and synchronous liver metastases. An overview of the entire course of disease (including information on staging, key diagnostics, therapies and response) is provided in Table .
When presenting at our outpatient unit, the patient was suffering from hot flushes and diarrhea (up to 10x/day) since several years. An ultrasonography performed by the family practitioner of the patient had revealed several echogenic liver lesions. We conducted a multi-slice computed tomography (CT) revealing multiple liver lesions (initial hepatic tumor load 50%) and a tumor formation of the ileum. A subsequently performed DOTATOC- positron emission tomography/computed tomography detected SSR positivity for the primary tumor but multiple SSR negative liver metastases (Fig. ). In further work-up, serum Chromogranin A concentrations were significantly elevated (29,924 μg/L) and urinary 5-hydroxyindoleacetic acid (5-HIAA) excretion was increased to 10,915 mg/24 h (Fig. ). Immunochemical analysis of a biopsy taken from a liver metastasis revealed a strong expression of both synaptophysin and chromogranin, ultimately proving the presence of a NET differentiation. The proliferation rate according to antigen KI-67 was 10% to 15%.
We initiated SSA therapy (octreotide depot 30 mg LAR every 28 days) in August 2016. Follow up CT imaging in November 2016 revealed progressive disease showing a tumor progression occurring in the liver (tumor load up to 60%). To prevent a potentially life-threatening small bowel obstruction, the patient was admitted to laparascopic right-sided hemicolectomy along with right sided ureterolysis. Intraoperatively, peritoneal lesions were detected that turned out as metastases of a neuroendocrine tumor (antigen KI-67 17%), confirming peritoneal carcinomatosis. In this clinical constellation (progression and unresolved flushing under standard dose SSA), we decided to administer SSA therapy in a shortened schedule (octreotide depot 30 mg LAR every 21 days) trying to increase its efficacy. Nevertheless, the patients’ general condition deteriorated, and the patient was suffering from flush symptoms, diarrhea (up to 6x/d) as well as significant weight loss (-3 kg body weight within 4 months). CT-imaging 4 months later revealed further hepatic tumor progression (Fig. ) along with an elevation in chromogranin A and 5-HIAA concentrations (Fig. ).
IFNα has been associated to anti-secretory effects in patients with NET, relieving clinical symptoms of carcinoid syndrome. Moreover, it exerts antiproliferative effects in NET providing tumor control in many patients .[ We therefore decided to initiate an add-on therapy with IFN taking into consideration that alternative therapy options such as Peptide receptor radionuclide therapy[ or everolimus[ were not considered eligible due to negative SSR expression in liver metastases or, in case of everolimus, the lack of anti-secretory effect and minimal cytoreductive ‘potential. Locoablative procedures (Transcatheter arterial chemoembolization, Afterloading, Selective internal radiation therapy) could not be performed based on the high hepatic tumor burden. Due to its better tolerability in comparison to “conventional” IFN, pegylated IFNα2a was administered (135 μg/wk), although it is not specifically approved for NET yet.[
Following this treatment, the patient fully recovered from signs of hypersecretion and demonstrated an impressive tumor response in CT imaging 4 months later (Fig. ). Chromogranin A and 5-HIAA concentrations immediately decreased after initiation of therapy as demonstrated in Figure . Moreover, the treatment was well tolerated, and the positive effect was not associated with any adverse events. Subsequent staging examinations until December 2019 showed a sustained partial remission and stable peritoneal carcinomatosis. Thus, 30 months after initiation of IFNα-treatment, the patient is in excellent conditions with sustained tumor response and free from any symptoms related to hormone hypersecretion.


We present a 32-year-old transgender female with a past medical history most significant for depression with suicidal ideation who presented to our hospital due to an acute onset of nausea and lethargy with one episode of nonbloody emesis. Symptoms were not alleviated or exacerbated by any specific factors. The patient had purchased a Cerbera odollam plant on the internet a week prior. She ingested one seed of this plant, as this was suggested to be the lethal dose, with the intent of ending her life. The exact time between ingestion and presentation was unknown. On transportation to the hospital, the patient had a documented heart rate in the 30s with a junctional rhythm and therefore received a total of 10 vials of Digibind (digoxin immune fab). She denied any headache, visual disturbances, chest pain, palpitations, shortness of breath, abdominal tenderness, diarrhea, or constipation.
On presentation, the patient's vital signs were blood pressure of 105/74 mmHg, heart rate of 106 beats per minute, respiratory rate of 18 breaths per minute, body temperature at 98.6 degrees F, and oxygen level of 98% on room air. Physical examination was noted as within normal limits. Labs performed included urine toxicology which was positive for cannabis, digoxin level 0.2 ng/mL, salicylate level less than 1.7 mg/dL, acetaminophen level less than 2 mcg/mL, blood alcohol content of 0, lactic acid 1.2 mmol/L, white blood cell count 8,100/μL, hemoglobin 13.9 g/dL, hematocrit 40.8%, platelets 165/μL, sodium 138 mmol/L, potassium 4.3 mmol/L, chloride 103 mmol/L, bicarbonate 29 mmol/L, blood urea nitrogen 10 mg/dL, creatinine 0.84 mg/dL, glucose 107 mg/dL, total bilirubin 0.5 mg/dL, AST 37 U/L, ALT 61 U/L, and alkaline phosphatase 48 U/L.
Electrocardiogram (ECG) was performed shortly after arrival. This demonstrated a significant decrease in heart rate as compared to presenting vital signs. The heart rate had decreased from 106 beats per minute to 36 beats per minute (). Therefore, the patient was monitored on telemetry, and repeat ECGs were ordered. Her heart rate remained in the 30s to 40s, and the rhythm was sinus bradycardia. Upon consultation, the toxicology team recommended to monitor electrolytes and heart rate closely while continuing intravenous fluids. If there were any conduction blocks or further slowing of heart rate, 5 more vials of Digibind could be given. She had a hypoglycemic episode with blood glucose of 60 mg/dL. She was administered dextrose and responded appropriately.
The following day, the psychiatry team was consulted, and the patient was diagnosed with gender dysphoria and depressive disorder and noted to have narcissistic and borderline personality traits. The patient met the criteria for inpatient psychiatric hospitalization once medically cleared.
The toxicology team continued to follow the patient during her hospitalization. Being that her digoxin level was only 0.2 ng/mL and she had normalized potassium levels, her likelihood for toxicity was low. Given her lab work and improving heart rate on ECGs (Figures and ), the team was reassured that the patient's clinical status was improving. The patient's home hormonal therapy was started, and she was admitted to the psychiatry unit.


This is a 44-year-old Caucasian male patient with past history significant for hyper-IgE syndrome (confirmed with positive STAT3 mutation) and recurrent infections since childhood, including soft tissue infections (i.e., abscesses and osteomyelitis), recurrent pulmonary infections (i.e., empyemas), and recurrent staphylococcal infections. The patient presented to the infectious disease clinic with a progressively enlarging skin lesion on the lateral aspect of the right knee for two months. The lesion initially started as a quarter-sized ulcerative lesion associated with intermittent purulent discharge and pain upon palpation. Initial evaluation showed a temperature of 36.6°C (97.7°F), blood pressure of 108/60 mmHg, pulse of 80 beats/minute, and respiratory rate of 16/minute. On examination, the patient was well-developed and well-nourished. Cardiopulmonary and abdominal examinations were unremarkable. Upon skin inspection, the patient had several scars on his neck and back (), reflecting prior staphylococcal furunculosis previously treated with linezolid. Remarkably, the patient also had onychomycosis (), affecting several fingernails and toenails.
The primary skin lesion on the lateral aspect of the right knee () was measuring 7.5 cm × 6.0 cm × 0.1 cm. The wound bed was mostly filled with eschar with very little granulation tissue. The surrounding skin was edematous, erythematous, and with mildly tender edges but did not show signs of cellulitis. Debridement was attempted during the initial encounter, but it was limited by the thick eschar tissue. Subsequently, wound culture and a punch biopsy of the lesion was obtained. The patient was scheduled for a follow-up visit with the wound clinic. Beyond the primary skin lesion, the patient denied having fever, chills, recent trauma, and animal/tick bites. He also denied a history of malignancy, chemotherapy, or hematopoietic stem cell transplant.
He has previously been treated for an HPV infection and had a negative hepatitis C and HIV screening tests recently. The patient had a recent travel to Hawaii one month prior to his initial encounter. During the trip, he indulged in aquatic sports, including swimming in pools and the ocean. Of notice, the patient has recently started a marijuana dispensary business, which also involved growing Cannabis plants. He reported that his marijuana plants were “damaged by mold” after which he had to bring new plants to grow.
Initial workup showed a hemoglobin of 14.2 g/dl, white blood cell count of 3.9 k/ul (neutrophils: 56%, lymphocytes: 19%, monocytes: 15%, and eosinophils: 8%), and platelets of 214 k/ul. ESR and CRP were both within normal range at 13 mm/hour and <0.1 mg/L, respectively. Liver studies showed an ALT of 29 IU/L, AST of 35 IU/L, alkaline phosphatase of 57 IU/L, total bilirubin of 0.5 mg/dl, albumin of 5.0 g/dl, globulins of 3.4 g/dl, and total protein of 7.4 g/dl. Upon reviewing immunoglobulins levels, the patient was noticed to have a markedly elevated IgG (1,838 mg/dl, normally <200) and IgE levels (4,242 mg/dl, normally <300).
A punch biopsy of the right knee lesion was performed during the initial encounter and showed an ulcer and abscess in the upper and mid-dermis. The deep dermis also exhibited suppurative inflammation within the subcutaneous tissue. Most remarkably, digestive PAS stain revealed the presence of numerous fungal hyphae consistent with deep fungal infection (). Gram stain appeared negative for bacterial organisms.
Wound cultures from the right knee lesion obtained on initial encounter isolated Fusarium species on the aerobic culture. However, no bacterial growth was observed on Gram stain or anaerobic cultures. Wound swab culture was repeated four days later which again identified Fusarium species as well as coagulase-negative staphylococci, likely representing cross contamination.
Sabouraud dextrose agar (SDA), the standard media for most fungi, was used to cultivate the organism. The VITEK-2 automated system was utilized for fungal identification, and Fusarium species were identified. In addition, lactophenol cotton blue (LPCB) wet-mount preparation was used for direct visualization of fungal structures, and structures consistent with Fusarium species were recognized (e.g., Fusarium species hyphae) under slide preparation. Further identification of the fusarial subspecies was attempted; however, the cost of processing was prohibitive.
Since Fusarium species were isolated from wound culture, the patient was initiated on antifungal therapy with posaconazole (300 mg once daily) for a planned treatment duration of 4–6 months. Since azole antifungals are notorious for hepatotoxicity, patient's liver functions were monitored monthly and were persistently normal. In addition, serum levels of posaconazole were monitored during the treatment duration to ensure achieving the target therapeutic range (>0.7 mcg/ml). Wound debridement was attempted; however, it was initially limited by the thick eschar tissue ().
One week after initiating treatment, granulation tissues started to appear, and serosanguinous discharge continued to show on the ulcer base (). On subsequent visits, surgical debridement was performed until almost all eschar tissue was removed. A month later, the ulcer showed hypergranulation with sloughing around the periphery, without eschar tissue or serosanguinous discharge (). The patient continued to be free of symptoms during that time and was able to tolerate posaconazole without adverse events. After six weeks of treatment, the ulcer had reduced to 6.0 × 4.0 cm with visible pink granulation tissue at the base; no further surgical debridement was needed. By the third month, the wound healed completely leaving pale and raised scar tissue at the site of the lesion (). The patient continued on posaconazole for a total of four months before it was discontinued.


A 33-year-old female was referred to our clinic, after accidental exposure to alexandrite laser (750 nm) during a hair removal session (without the use of protective goggles). Pulse duration was 3 ms and energy density was approximately 10 J/cm2. The patient did not report any symptoms during her first ophthalmologic examination and her best-corrected visual acuity (BCVA) was 20/20 in both eyes. Anterior segment examination was unremarkable and intraocular pressure was 13 mmHg in both eyes. However, fundus examination of the right eye revealed a spot on the parafoveal region presumably caused by laser-induced burn. Optical coherence tomography (OCT) revealed focal disruption of inner/outer segment junction of photoreceptor and outer plexiform/nuclear and retinal pigment epithelium (RPE) focal deformations. Topical steroid eye drops and non-steroid anti-inflammatory eye drops were prescribed four times and once daily, respectively. Tapering doses of steroid eye drops were administered during a four-week period.
A month and a half later the patient complained of blurred and reduced vision from her right eye. Upon clinical examination, BCVA in the right eye was 20/40, the anterior segment was normal and the intraocular pressure was 14 mmHg. Clinical examination in the left eye was unremarkable. Fundus examination demonstrated sub-retinal haemorrhage and edema (Figure ) whereas fluorescein angiography (FA) confirmed the presence of neovascular membrane (Figure ).
The patient was treated with a total of three intravitreal anti-vascular endothelial growth factor (anti-VEGF) (aflibercept) injections at one-month intervals.
Follow-up examinations were conducted one day, one week, one month, three months and one year post injections. During the last follow-up examination, the patient’s BCVA was 20/20 and the fundus examination revealed a chorioretinal scar with peripheral hyperpigmentation, without the presence of hemorrhage nor edema (Figure ). OCT demonstrated a pigment epithelium detachment with a hyperreflective area without the presence of sub-retinal fluid or retinal edema, while OCT angiography showed that the neovascular membrane remnants had no signs of activity (Figure ).


A 58-year-old man was found with some liver lumps by abdominal ultrasonography in his regular physical check-up in May 2019. Without any symptoms before, he went to the hospital for further examination. A test of tumor markers showed that serum CA125 was 1,898 U/ml and the CA199 level was out of the upper limit of detection (>1,000 U/ml). A computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen both indicated multiple liver lesions and a pancreatic tail mass at a size of 3.9 × 2.6 cm. He was referred to the Department of General Surgery and underwent a laparoscopic liver biopsy. Intraoperative findings showed multiple scattered nodules on the surface of the liver, whose diameters were <2 cm. Pathology showed metastatic ductal adenocarcinoma. Given these findings, his final diagnosis was pancreatic adenocarcinoma with multiple liver metastases (cT2N+M1, stage IV). The next-generation sequencing of his tumor showed an intermediate tumor mutation burden with 5.65 mutations/megabase and microsatellite stable (MSS) status. The immunohistochemistry (IHC) data of the tumor tissue of this patient indicated the positive expression of PD-L1 protein (30%), and the tumor proportion score (TPS) was 20% and combined positive score (CPS) was 30 (). Additionally, deleterious alterations occurred in CDKN2A, KRAS, TP53, and VEGFA genes. There were not any applicable targeted drugs for these gene mutations.
With his content, he was eligible for a clinical trial about the combination of doublet chemotherapy (gemcitabine 1,000 mg/m2 and nab-paclitaxel 125 mg/m2) and toripalimab (a novel PD-1 inhibitor, 240 mg) for the first-line treatment of metastatic PDAC conducted by our department. Therefore, he received gemcitabine 1,700 mg and nab-paclitaxel (Abraxane) 200 mg at day 1 and day 8, along with toripalimab 240 mg at day 1 every 3 weeks. After 2 cycles of the combination therapy, his metastatic liver lesions almost disappeared with an evaluation of partial response (PR) by Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 (). Surprisingly, he did not suffer any serious side effects except mild nausea and loss of appetite (grade 1, CTCAE 3.0), which was self-cured at the interstitial period of therapy. The treatment continued and repeated CT scans after four cycles showed shrinkage of the primary lesion but an increase in the number of the liver metastases. However, the CA199 level plummeted from 8,015 to 553.7 U/ml after the first four cycles of treatment (). He was still asymptomatic but had grade 1 myelosuppression, which was successfully treated with a recombinant human interleukin-11. Through multidisciplinary therapy (MDT) and communication with the patient, we thought that it was highly possible for him to have radiological pseudo-progression and suggested he continue the therapy regimen. As we expected, the subsequent two-cycle treatment brought new clinical benefits to this patient, which in turn confirmed the previous diagnosis of pseudo-progression. The patient's continuous PR is still ongoing at the time of this report (eight cycles after the initial of the combination therapy). Primary and metastatic lesions were significantly decreased or shrank to nearly invisible status as the last evaluation showed, and the level of CA 199 has maintained within the normal for a long period but a little increase at the last test (). All treatment-related adverse events (TRAEs) of this patient throughout the clinical course were listed in . The most serious TRAEs he had was grade 2 leukocytopenia, which was recovered under drug intervention before the next cycle treatment. Overall, he did not suffer any grade 3 or higher toxicities and maintained good tolerance. With a history of hypertension and type II diabetes, the patient also kept his blood pressure and blood glucose under good control.


A 46-year-old female, known case of rheumatoid arthritis on Methotrexate, hypothyroidism on Levothyroxine and unremarkable surgical history referred to otolaryngology clinic as a case of recurrent epistaxis from the left nostril for the last 7 months, required nasal packing at one point. Her chief complaint was associated with hyposmia, nasal discharge, nasal obstruction, left ear fullness, mouth breathing and dryness. She had intentional weight loss, insignificant for the past 3 months, decrease in appetite with on and off night sweat.
She had unremarkable family history except of a sister with breast cancer. She had no known allergies. Upon examination, 0-degree rigid nasal scope was used and showed an easily bleeding swelling. It was blocking the left nostril with difficulty in determining its origin whether it was from the septum or lateral nasal wall. Scope through the right side revealed patent nasal cavity and clear nasopharynx.
Urgent sinus computed tomography (CT) revealed large left nasal cavity heterogeneous and hypodense mass with faint central enhancement. It was seen extending to the left maxillary sinus, with opacification of anterior ethmoid air cells and left frontal sinus and widening of the left osteo-meatal unit. It showed as well severe thinning of the left maxillary sinus wall, nasal septum and inferior left orbital wall with possible dehiscence of the left lamina papyracea, bilateral cribriform plate and medial part of the fovea ethmoidalis with clear nasopharynx ().
Patient underwent examination of nasal cavity under general anaesthesia and debulking of the left nasal mass. Left nasal cavity was completely obstructed with a nasal mass, its origin could not be identified precisely but it was seen below and separable from the middle turbinate. It seemed attached to the inferior turbinate and to the nasal septum. Multiple biopsies were taken for histopathology and debulking was done using a debrider and forceps until reaching the nasopharynx which was clear from any obstruction. After that, left middle sac was opened through the natural ostium which revealed some secretions.
On histological examination, the biopsy showed monotonous population of plump to spindle cells with indistinct cytoplasm and oval dark stained nuclei, arranged in solid sheets and short fascicles. These are alternating with less cellular areas showing myxoid change and perivascular hyalinization ( A&B). The tumor shows few mitotic figures. The tumor cells are positive for EMA (. C) and Bcl-2 (. D) with focal immunoreactivity to SMA, while being negative for S100, desmin, myogenin, myo D1, CD99, SOX-10 and CD34. The morphological features and the immunoprofile of the tumor are highly diagnostic of monophasic synovial sarcoma.
Patient was later admitted for full workup of left nasal high grade spindle cell sarcoma. Sinus magnetic resonance imaging (MRI) revealed left nasal cavity mass expanding to the left frontal recess with extension to the left frontal sinus. The lesion showed involvement of the left lamina papyracea and cribriform plates with small extension to the left intra-orbital compartment. The lesion showed an intermediate to low signal lesion in T2 weighted images with heterogeneous enhancement. It was approximately 3 × 2.7 × 3.5 cm in dimension (). Neck CT showed prominent upper cervical lymph nodes. Chest, abdomen and pelvis CT were clear from any metastasis.
Patient’s clinical, radiological and pathological results were discussed in tumor board. Final decision with medical oncology, radiation oncology, and neurosurgery was to go for surgery followed by adjuvant chemoradiotherapy.
Rheumatology advised to stop Methotrexate 2 weeks prior to surgery.
Patient underwent anterior cranial tumor resection, bicoronal craniotomy and skull base reconstruction, by otolaryngologist and neurosurgeon.
Left external ethmoidectomy, left medial maxillectomy, bilateral sphenoidotomy were performed with ligation of the left anterior ethmoidal artery. lamina papyracea and periorbital fat on the left side were resected. The nasal septum was separated from the face of sphenoid and maxillary crest with anterior preservation of the nasal caudal septum. Bicoronal incision, pericranial flap and frontal craniotomy were performed. Resection of the tumor from the anterior cranial fossa was done; at the area from the right cribriform plate to the left fovea ethmoidalis, anterior to the level of crista galle and posterior to the face of sphenoid. Left temporalis muscle fascia was harvested to reconstruct the left periorbital area.
Post operation, patient was kept intubated in the intensive care unit (ICU). CT done day one post operation and revealed no intracranial haemorrhage (). Patient was extubated and taken to a regular ward. Then, patient developed central diabetes insipidus and was treated by one dose of desmopressin. Family noticed behavioral changes in the patient and new brain CT revealed right frontal hemorrhage and edema that improved with time.
Patient was discharged home on second week after surgery with normal facial nerve and vision, healing wound and stable condition. She was given follow up with otorhinolaryngology, neurosurgery, medical and radiation oncology and rheumatology.
Patient completed full course of adjuvant radiotherapy with monthly follow up in otorhinolaryngology clinic for debridement and examination.
Seven months post-operation, findings using 30 degree rigid scope revealed healed nasal cavity mucosa except for a small frontal area with no water discharge.
Patient is following on regular basis with new MRI every 4 months with no evidence of local recurrence ().


A 47-year-old woman visited our medical facility due to a perianal bulge and recurrent attacks of pelvic pain. Thirty-six months earlier she had presented with chronic pelvic pain and had undergone en bloc resection of a right ovarian angiomyxoma. The patient underwent an exploratory midline laparotomy under general anesthesia. Upon entering the abdominal cavity, a large soft pelvic mass was noticed filling the pelvis, which was adherent to the right adnexa right ureter, and right pelvic side-wall. Fortunately, the mass was not infiltrating the adjacent organs; therefore, it was successfully resected along with the right adnexa after a careful pelvic side-wall dissection. The uterus, left adnexa, and upper abdomen were all normal.
A review of the operative pathology slides prepared 36 months earlier revealed hypocellularity, spindle-shaped myxoid cells without atypia, and intermediate thickened wall vessels with few mast cells. Immunohistochemical marker analysis tests were positive for estrogen receptor, progesterone receptor, and desmin and locally positive for SMA as well as for CD34. The tumor had a myxoid stroma and variable-sized vascular channels with different kind of cells, include ovoid, stellate, and spindle-shaped cells with scant cytoplasm [].
Current initial clinical assessment showed a partially defined, partially firm mass in the right perineal area. Cross sectional imaging revealed a large pelvi-abdominal, elongated, relatively homogenous hypo attenuated mass extending from the perineum to the mid abdomen. It measured 24 × 18 × 16.5 cm in the maximum dimensions, extending from the right ischioanal, ischiorectal fat planes and traversing the right levator ani muscle. Inferiorly, it had a mass effect on the rectum anteriorly and to the left as well as on other pelvic organs, mainly the urinary bladder (). The mass demonstrated iso intense signal intensity as compared to muscle in T1 weighted imaging (WI) sequences and relative brightness in T2WI; furthermore, alternating linear low signal bands (swirl appearance) were noted within the mass. There was no definitive restriction in the diffusion weighted image sequence. Post contrast images demonstrated heterogeneous enhancement throughout with linear hypointensity “swirled” signals (). The current imaging findings were pathognomonic for AAM and continued imaging follow up was final plane in accordance with patient preference.