A 23-year-old man presented to our outpatient clinic with painless gradually enlarging mass in the upper part of the neck below the angle of the mandible. Computed tomography (CT) scan of the lesion demonstrated homogenous, hyperdense, lesion at the mandibular angle which was diagnosed as aparaganglioma. The patient underwent surgery by the ear, nose, throat (ENT) surgeon through submandibular approach. After manipulation of the lesion, severe pulsatile bleeding was encountered and a vascular lesion was suspected. Thus two vascular clamps were placed proximal and distal to the lesion and the bleeding was controlled. Vascular surgeon was consulted immediately. A CT-angiogram revealed a huge (8×4 cm) aneurysm located at the distal part of the right internal carotid artery (ICA) aneurysm which was injured (). Anticoagulation and wide spectrum antibiotic was administered to the patient and he was admitted to the intensive care unit (ICU) with the clamps in place ().
After 2 days with available neurovascular surgeon for possible intracranial bleeding control and craniotomy, the patient was transferred to operating room. The previous skin incision was re-opened and the two gauzes were removed (). Appropriate irrigation was performed and the two vascular clamps were removed one by one. There was just small back bleeding from distal neck of right ICA aneurysm, therefore packing by means of small segments of fibrillar Dacron was done for 15 minutes and bleeding was stopped completely. Then Dacrons were covered with muscle flaps and the wound was closed in layers. The patient had an uneventful postoperative course and was discharged on the 6th postoperative day. The postoperative angiogram revealed the patent internal carotid artery without any aneurysm residue. The patient received anticoagulation for 3 months and was followed in outpatient clinic. After 1 year of follow-up he was completely asymptomatic and the angiogram revealed patent parent vessel without residue. The patient and his family provided their informed written consent for publication of the case and the images.


A 54-year-old female patient presented to her general practitioner with a five-month history of persistent headache, visual blurring, and mild cognitive impairment. Her past medical and surgical history included abdominal and pelvic surgery and hip replacement surgery. She took no regular medications. She gave a history of learning difficulties throughout her life.
Brain CT and MR imaging showed a compact 27 mm arteriovenous malformation located within the posterior interhemispheric fissure, adjacent to the splenium of the corpus callosum (Figures -).
There was no evidence of previous hemorrhage. Digital subtraction angiography showed that the bAVM was associated with feeding arteries arising from the posterior cerebral arteries bilaterally, as well as from the falx and tentorial arteries. There was a network of draining veins within the pineal cistern, with a large venous varix occluding the cerebral aqueduct and causing obstructive hydrocephalus. There was deep venous drainage into the Galenic venous system. None of the bAVM was located within the eloquent brain. There was one 3 mm feeding artery aneurysm arising from the left posterior cerebral artery (PCA) and two intranidal aneurysms measuring 3 mm and 4.5 mm, respectively. Preoperative TCCD demonstrated the bilateral middle cerebral arteries (MCAs), assigned red on color duplex, denoting flow towards the probe. A brief venous signal, assigned blue, was obtained from the right Sylvian fissure representing flow direction away from the probe. No venous signal was detected from the left Sylvian fissure. The venous waveform detected on the right lacked phasicity and pulsatility. The venous signals were obtained from depths ranging from 2.7 cm to 4.2 cm.
There was no papilloedema. The formal ophthalmological assessment was normal, with no restriction of the visual field, and normal ocular coherence tomography (OCT). A neurocognitive assessment revealed mild-to-moderate impairment in cognitive tasks requiring speed and mental manipulation.
The lesion was classified as a Spetzler-Martin grade 2, Lawton supplementary grade 6 bAVM. The patient was counseled about the risks, alternatives, and expectations of the various treatment options; it was recommended that she undergo ventriculoperitoneal shunting to treat her symptomatic hydrocephalus, followed by stereotactic radiosurgery to the bAVM nidus (Figures -).
Preoperative TCCD demonstrated the bilateral middle cerebral arteries (MCAs), assigned red on color duplex, denoting flow toward the probe. On the right, a brief venous signal was detected (assigned blue), with a non-pulsatile spectral trace. No venous signal was obtained from the left Sylvian fissure (Figures -).
At surgery, a right frontal burrhole was used to place an antibiotic-impregnated ventricular catheter, which was inserted using neuronavigation. This was connected to a Miethke Sprung Reservoir (Christoph Miethke GmbH & Co. KG, Potsdam, Germany) and then tunneled to a right occipital incision, where it was connected to a Miethke ProGAV 2.0 programmable hydrostatic pressure valve (opening pressure set to 10 cm H2O) and variable gravitational-assist device (0 cm to 25 cm H2O). The distal end of the shunt tubing was inserted into the peritoneal cavity via a midline mini-laparotomy.
TCCD measurements were obtained in the intensive care unit approximately two hours following shunt insertion (Figure -).
Two hours postoperatively, the right venous signal demonstrated a slight increase in pulsatility from 0.33 to 0.42 but visually, the color signal increased in prominence using TCCD. The left side demonstrated no visible venous signals preoperatively, however, a pulsatile spectral trace (PI =0.68) was obtained postoperatively and there was a distinct increase in the visibility and prominence of a signal using TCCD (Figure , ).
Middle cerebral arterial flow was lower in this patient (compared to preoperatively), with right preoperative MCA peak systolic velocity (PSV) = 55 cm/s, end-diastolic velocity (EDV) = 24 cm/s, and mean velocity = 37 cm/s. Left MCA studies showed preoperative MCA PSV = 21 cm/s, EDV = 6 cm/s, and mean velocity = 12 cm/s. There was a slight increase in postoperative velocity on the right (compared to the preoperative values) whilst the left side demonstrated little change (Figure ).
Comparatively normal individuals have velocities twice that of this patient. Unpublished data from 20 healthy volunteers derived by the primary author have demonstrated MCA PSV of 110 ± 26 cm/s, EDV of 48 ± 11 cm/s, and mean velocity of 69 ± 15 cm/s. Normal individual MCAs have a PI of 0.92 ± 0.14.
At three months postoperatively, there was a further substantial increase of color flow prominence of the left middle cerebral vein (Figure ).
Furthermore, at the three month follow-up time interval, an attempt was made to image the VP shunt on the right side of the neck using conventional color duplex imaging and a high-resolution 2-9 MHz transducer. This attempt successfully revealed CSF flow waveforms within the shunt (Figure ). The color flow was not readily discernible within the shunt, however, given that spectral Doppler is more sensitive than color, a spectral signal could be obtained (Figure ).
The patient subsequently proceeded to linear accelerator (LINAC) stereotactic radiosurgery (SRS) treatment of the bAVM, with the goal of complete occlusion. At six months post-SRS, the clinical improvements experienced after the treatment of hydrocephalus by VP shunting were maintained; however, there were not yet any radiological signs of bAVM occlusion. Further bAVM imaging will continue, as the latent interval between treatment and occlusion may be as long as three to five years.


A baby girl with bilateral porencephalic cysts was born at 40-week gestation via vaginal birth after caesarian section delivery to a 24-year-old gravida 4 para 4 mother with a history of anemia, prior caesarian section in 2015, and a body mass index of 27. The mother had no past medical history of hypertension, diabetes, deep vein thrombosis, cardiovascular disease, sickle cell disease, or sexually transmitted diseases. The mother denied the use of alcohol, tobacco, or illicit drug use during or before pregnancy, but she did admitted to the use of phentermine while she was pregnant. She reported that she was not aware of her pregnancy until she was at approximately 26-28 weeks of gestation after receiving a urine pregnancy test during a routine medical visit. At that time, she discontinued her phentermine. A urine pregnancy test was not conducted prior to starting phentermine. She was on no other medications prior to or during the pregnancy. All prenatal labs were normal with normal serology. She did not have gestational diabetes, pregnancy-related hypertension, maternal infections, or other maternal complications during the pregnancy. Brain abnormalities were first seen during prenatal ultrasound at 28 weeks of gestation, which demonstrated bilateral enlarged ventricles. Fetal magnetic resonance imaging (MRI) was performed to confirm the findings (Figure ). Delivery was uncomplicated with Apgar scores of 8 and 9 at 1 and 5 minute(s), respectively. Birth weight was 3.37 kilograms, length was 50 centimeters (cm) and head circumference was 36 cm. The patient’s physical examination was significant for increased tone and brisk reflexes in her upper and lower extremities bilaterally. Patient was admitted to the Neonatal Intensive Care Unit for observation and discharged after three days in the hospital. MRI brain at day 1 of life revealed bilateral porencephalic cysts with thinning of the right cerebral cortex and absence of a significant region of the left cerebral cortex. Occipital lobes were dysplastic bilaterally (Figure ). These findings suggest an event of bilateral middle cerebral artery (MCA) stroke in the left MCA and posterior right MCA. The infant went on to develop spastic quadriplegic cerebral palsy and global developmental delay


A 25-year-old male presented to the emergency room (ER) with complaints of rectal bleeding, described as bright red blood on formed stool, for 2 months. His medical co-morbid condition includes a 7-year history of Human Immuno Deficiency Virus (HIV) infection, nonadherence to Highly Active Retroviral Therapy (HAART) with a CD4 count <20 cells/µL and HIV viral load of 151,257 copies/mL. He is MSM (men having sex with men) and has receptive anal intercourse with men. He reported remote treatment for Genital Herpes. In the ER, his vitals were within normal limits and physical examination was unremarkable except for minimal right upper quadrant tenderness upon palpation and numerous tender perirectal ulcerations. Rectal examination revealed normal sphincter tone with empty but tender rectal vault. There was no peripheral lymphadenopathy.
Laboratory investigations showed haemoglobin of 10.4 g/dL, leukocyte count of 3.1 K cells/µL, platelet count of 339 K cells/µL, aminotransferase levels 3X ULN, total bilirubin levels of 7.0 mg/dL with conjugated bilirubin of 5.2 mg/dL, serum lactate dehydrogenase of 327 unit/L, serum gamma glutamyl transferase of 414 units/L, and alkaline phosphatase of 958 unit/L. Stool work up was negative for Cryptosporidium, Cryptococcal antigen, Clostridium difficile, Cyclospora, Microsporidium, and Isospora. No Ova and parasites isolated from stool studies. Blood cultures for regular pathogens acid-fast bacteria (AFB) were negative. Patient was admitted and colonoscopy performed to further identify the cause of rectal bleeding revealed diffusely erythematous, congested, friable rectal mucosa with two superficial ulcers (). Biopsies were taken of the rectal mucosa as well as normal appearing proximal colon and terminal ileum. Rectal swabs were negative for chlamydia and gonorrhea. Pathology for rectal biopsy revealed AFB positive organism (Figures and ) and culture revealed growth of Mycobacterium avium-intracellulare (MAI) organisms. During this clinical course, he had an unremarkable upper endoscopy and was started on clarithromycin 500 mg BID, ethambutol 1200 mg QD, and rifabutin 300 mg QD in addition to HAART therapy.
Clinical presentation was highly suspected for disseminated MAI infection. Given negative blood culture, we further pursued evaluation of elevated alkaline phosphatase and performed liver biopsy. Pathology was reported to have necrotizing granulomas composed of lymphohistiocytic collections (), highly suggestive of MAI infection. This confirmed our diagnosis of disseminated MAI. Patient was on and off the recommended regimen, flexible sigmoidoscopy after a short course of interrupted treatment again demonstrated similar initial findings in the rectum () but a follow up colonoscopy 20 months after diagnosis showed healing of rectal mucosa with negative biopsies for MAC. There was also a decreasing trend of alkaline phosphatase while on treatment, with last reported value of 218 unit/L.


An 81 year-old woman (48 kg) presented with a right femoral neck fracture after a fall from ground level. She did not sustain any loss of consciousness or head trauma. Her past medical history was significant for dementia for which she required at-home care, and longstanding goiter which she did not recall having in childhood (). No prior attempts at management of her goiter were documented. On physical exam, she was afebrile, normotensive, nontachycardic, with >95% oxygen saturation on room air. At baseline, she was alert and oriented only to self. She had no issues with phonation, but she did endorse shortness of breath while lying supine. No audible stridor was noted. The goiter was present over the entire anterior portion of the neck to below the sternal notch (8.8 × 12.0 × 10.1 cm). The goiter was firm, generally uniform, and immobile. On computed tomography (CT) scan, the goiter caused leftward tracheal deviation with a minimal tracheal diameter of 6 mm. Her Mallampati score was unable to be assessed given her dementia.
Prior to the procedure, a CT scan was obtained to elucidate tracheal diameter (6 mm at the narrowest point). Consent was obtained from patient's medical power of attorney after risks, benefits, and alternatives to anesthesia were obtained. Prior to proceeding to the operating room, the patient's vocal cords were sprayed with lidocaine 4% nebulizer, and the patient's nostrils were prepped bilaterally with oxymetazoline 0.05%. Dexmedetomidine drip was initiated for planned awake nasal fiberoptic intubation. Fiberoptic scope was passed through the nares, but the vocal cords were unable to be visualized due to the tongue, as well as debris from nasal passages. Even after readjusting the position and pulling the tongue anteriorly, there was still an inadequate view of the vocal cords. Jaw thrust maneuver yielded an improved view, however, a view of the vocal cords was unable to be maintained when attempting to pass the fiberoptic scope. Rightward pressure was then applied to the goiter in an attempt to bring the larynx into view without success. During this period, the patient became tachycardic necessitating an esmolol bolus.
Given the difficulty with the fiberoptic bronchoscopy, the decision was made to convert to videolaryngoscopy. While the patient remained sedated with a topically anesthetized airway, videolaryngoscopy revealed grade 3 Cormack-Lehane view. Eschmann stylet was advanced posterior to the epiglottis and tracheal grooves. Patient was given a bolus of 100 mg propofol to induce apnea. Armored 6.5 endotracheal tube was advanced over the Eschmann stylet and placement was confirmed with bilateral breath sounds as well as return of end tidal carbon dioxide on the ventilator. Anesthesia was maintained with sevoflurane. Over the course of the procedure, 1 mg of hydromorphone was administered for pain control. The patient required very small doses of pressors throughout the case and therefore she remained hemodynamically stable. The femoral neck fracture was repaired uneventfully. The patient remained intubated at the conclusion of the procedure due to concerns for airway edema given extensive airway instrumentation and possible tracheomalacia from her chronic goiter. She was kept sedated on propofol. On postoperative day 1, leak test was conducted which showed leak around the cuff. Dexamethasone was continued for airway edema. She was extubated successfully to BiPAP on postoperative day 3.


A 72-year-old Caucasian female with a past medical history of bilateral lobular breast carcinoma, paroxysmal atrial fibrillation, and hyperthyroidism presented with an intermittent history of nausea and vomiting leading to decreased oral intake, diarrhea, intermittent abdominal pain, and unintentional weight loss of approximately 30-40 lb. The patient complained of an average of three bowel movements per day, more diarrhea than constipation with no change in consistency and absence of blood and mucus along with generalized abdominal pain, which was cramping in nature and got aggravated after meals. She denied heartburn or consuming any particular food that caused nausea.
The patient had been diagnosed with lobular carcinoma of the right breast 24 years ago. She had been managed with partial mastectomy, radiation, and adjuvant chemotherapy including fluorouracil, methotrexate, and cyclophosphamide followed by nine years of hormonal therapy with tamoxifen. The patient had been further diagnosed with lobular carcinoma of the left breast approximately 10 years after the initial diagnosis. She had undergone lumpectomy and had completed a five-year course of hormone-based chemotherapy with anastrozole. The patient had since been in remission.
She had had similar complaints of abdominal pain six months prior to her initial presentation. Workup at that time had revealed a hemoglobin of 6.5 gm/dl (normal range: 11.7-15.1 gm/dl), hematocrit of 21% (normal range: 29.4-47.0%), mean corpuscular volume (MCV) of 88 fl (normal range: 78.9-98.6 fl), and positive fecal occult blood. Iron studies indicated iron of 10 mcg/dl (normal range: 50-212 mcg/dl), a total iron-binding capacity of 381 mcg/dl (normal range: 250-450 mcg/dl), transferrin saturation of 3% (normal range: 15-50%) and ferritin of 6.8 ng/ml (normal range: 11-307 ng/ml). Imaging studies including CT of the abdomen had been unremarkable for any acute abnormality. Esophagogastroduodenoscopy (EGD) and colonoscopy showed mild gastritis and ulcer in the sigmoid colon along with internal and external hemorrhoids. The biopsy of the gastric ulcer had revealed no malignant changes. She had been managed with proton-pump inhibitor and blood transfusion. The patient had received parenteral iron transfusions as an outpatient.
On arrival to the emergency department, she had a pulse of 58 beats/minute, blood pressure of 98/46 mm Hg, oxygen saturation of 99% on room air, and body mass index of 19.5 kg/m2. A physical examination showed pallor. Abdominal examination was significant for distension along with tenderness in the left lower quadrant. Laboratory investigations were noteworthy for hemoglobin of 8.2 gm/dl, hematocrit of 24%, MCV of 89 fl, red cell distribution width of 16.8% (normal range: 11.5-15.5%). The hepatic panel revealed aspartate aminotransferase of 190 u/l (normal range: 13-39 u/l), alanine transaminase of 157 u/l (normal range: 7-52 u/l), and alkaline phosphatase of 27 u/l (normal range: 34-104 u/l). Further workup revealed Ca-125 of 93.6 u/ml (normal range: 0-35 u/ml) and carcinoembryonic antigen (CEA) of 12.6 u/ml (normal range: 0.0-3.0 u/ml). Imaging studies of the abdomen including radiograph, MRI with contrast (Figure ), and CT scan (Figure ) confirmed the presence of distal small bowel obstruction. The patient underwent small-bowel resection along with primary anastomosis after the detection of a small bowel tumor.
Histopathology showed the presence of metastatic pleomorphic lobular carcinoma consistent with breast primary (Figure ).
Further workup showed cytoplasmic staining with p120 catenin and positive for GATA-binding protein 3 (GATA-3) (Figure ), focally positive for both estrogen receptor (ER) and progesterone receptor (PR), gross cystic disease fluid protein 15 (GCDFP-15) and mammaglobin, but negative for human epidermal growth factor receptor 2 (HER-2).
Two lymph nodes near the tumor site were identified, out of which one demonstrated the presence of metastatic carcinoma (Figure ).
The patient was subsequently started on fulvestrant and palbociclib. She has shown significant improvement in her symptoms.


The patient is a 45-year-old African male who presented with a two-year history of small lump on the right anterior chest wall. He reported that it was painless, firm and covered by hyper-pigmented skin. He sought for medical assistance after a year of noticing a gradual growth of the lesion. Subsequently, an incisional biopsy was done and histology results confirmed sebaceous adenocarcinoma. The patient absconded and presented a year later with a very large, pedunculated, mushrooming and fungating tumour. Its stalk was on the second intercostal space extending to the parasternal area ( A). The risk factors of this patient were age (45 years) and Human Immunodeficiency Virus; that is to say, he was HIV positive. On local examination, the tumour was found on the right anterior chest wall, with short and broad stalk on the second intercostal space and parasternal area. It had a mushroom configuration, which on long axis was extending from suprasternal notch to the lower third of sternal body. Transverse extend of the tumor was from right mid-clavicular line up to left parasternal area. The tumour was multi-lobulated, firm, non-tender, fixed to chest wall and had hyper-vascular, with sloughing areas. It was measuring 180 × 140 × 30 mm. There was no associated regional lymphadenopathy on examination. Examination findings of the other systems were normal.
Postero-anterior (PA) and lateral chest x-rays (CXRs) were done, with a PA showing multi-lobulated soft tissue homogeneous opacity in the upper mediastinum, which extends to the right hemithorax (A). A lateral view showed anterior chest wall homogeneous opacity extending from thoracic inlet up to the level of sterno-manubrial joint (B).
A non-contrast computed tomography (CT) scan showed anterior chest wall tumour with a calcified capsule without bones involvement (A). A CT-scan with contrast on axial cuts showed a large tumour, with a broad stalk. The tumour was infiltrating the right pectoralis muscle, as evidenced by loss of subcutaneous plane between the tumour and the muscle (B). A type 2-weighted image (T2W) axial magnetic resonance imaging (MRI) view with contrast showed a hyper-intense tumour (A). The broad and short stalk was also demonstrated, invading the right pectoralis muscle. A T2W sagittal view showed that a tumour was extending superiorly up to thoracic inlet and inferiorly up to superior third of sternal body (B).
Total excision of the tumour was undertaken with 1.5−2 cm wide surgical margins, both peripheral and deep. The invaded part of right pectoralis major muscle was also resected en bloc (A, B). A frozen section confirmed negative margins and the defect was dressed with a vacuum assisted closure (VAC) dressing to reduce wound margins and encourage uniform granulation of uneven wound surface. The skin graft was deferred pending definitive histology results for diagnosis. The histopathology results confirmed total tumour excision, and a skin graft was undertaken a week later (C). The skin graft complicated with severe over-granulation (D) for which small incisional biopsy was taken, which proved no recurrence. Conservative treatment was undertaken for a month, and the skin graft healed (E).
Grossly, a 180 × 140 × 40 mm ulcerated and multi-lobulated tumour was received. On cut section, it was white-tan and firm. The hematoxy-eosin (H/E) stained sections showed ulcerated invasive sebaceous carcinoma evidenced by ulcerated skin with an invasive basaloid tumour (A) with sebaceous differentiated (B). The tumour cells were positive for epithelial membrane antigen (EMA) and p63 (some of basaloid cells) while B-cell lymphoma 2 (Bcl-2) and carcinoembryonic antigen (CEA) were negative. Excision was completed.


The proband was a 19-year-old male with a 3 month history of exercise intolerance, myalgia and muscle weakness which was more severe in the lower limbs. Physical examination found reduced muscle tone and mild weakness of proximal muscles (MRC 4/5). Electromyography revealed myopathic motor unit changes in limb muscles without spontaneous discharges. A presumptive diagnosis of polymyositis was made and he was started on prednisone 1 mg/kg for 3 weeks, during which the serum creatine kinase (CK) level fell from 911 U/L to 190 U/L (normal range 0-174 U/L) and there was slight improvement in muscle weakness, but the myalgia and exercise intolerance persisted. Levels of other biochemical parameters also dropped but still remained above the normal range, including LDH (1624 U/L to 1066 U/L), AST (188 U/L to 76 U/L) and uric acid (738 μmol/L to 665 μmol/L) after treatment with prednisone.


A 13-year-old male, the younger brother of the proband, presented with a 1 year history of progressive muscle weakness and myalgia worse after exercise. Neurological examination found reduced muscle tone and mild proximal weakness in the lower limbs (MMT 4/5). Biochemical tests showed raised serum CK (2165 U/L), CK-MB (103 ng/ml) and uric acid levels (709 μmol/L). MRI of the lower limbs showed focal areas of increased signal intensity in the gluteal and thigh muscles on short time inversion recovery (STIR) sequences, suggesting a diagnosis of possible polymyositis. A muscle biopsy taken from the right biceps femoris showed only a small focal lymphocytic infiltrate but no other abnormalities. Echocardiography showed mildly reduced ventricular diastolic function. He was commenced on oral prednisone (1 mg/kg), following which there was a fall in the serum CK (2165 U/L to 612 U/L), CK-MB (103 ng/ml to 51 ng/ml) and uric acid level (709 μmol/L to 415 μmol/L), but there was no improvement in his muscle symptoms.
The patient then reported that he had an elder brother (the proband) who had similar symptoms, and that their parents did not have any muscle symptoms and were not consanguineous. A second muscle biopsy from the left quadriceps femoris did not provide any confirmatory evidence of an inflammatory myopathy but revealed prominent cytoplasmic vacuolation of muscle fibres strongly suggestive of a lipid storage myopathy. The patient was then started on riboflavin (30 mg, three times per day) and the prednisone was discontinued. After 3 days of riboflavin therapy there was already considerable improvement in muscle strength and exercise tolerance.
Late-onset MADD was suspected and blood was collected from all family members and sent to MyGenostics for genetic analysis. Sanger sequencing was performed for the ETFA, ETFB and ETFDH genes as well as other neuromuscular disease related genes. This identified a homozygous ETFDH gene mutation of c.250G > A (p.A84T) in exon 3, chr4–159,603,421 (Fig.a) in both brothers, and a heterozygous mutation in both parents (Fig.b). No variants were detected in ETFA, ETFB or other genes.
After 1 month of riboflavin treatment there was marked improvement in myalgia and exercise intolerance in both brothers and the MMT scores in proximal limb muscles had improved to 5/5. Both patients have since been able to walk and run freely and the younger brother is now able to participate in school sporting activities. Serum CK levels fell markedly after commencement of riboflavin and have remained normal in both patients over the past year.


A 24-year-old male admitted to the hospital with severe headache followed by altered sensorium and involuntary movements of the face and right upper limb for 2 days. He was drowsy with Glasgow Coma Scale (GCS) of 12/15 and generalized tonicity. His optic fundus showed papilledema with left hemiplegia. Further clinical examination was unremarkable with heart rate of 70 bpm, blood pressure 138/68 mmHg, respiratory rate 28 per minute, and oxygen saturation of 96% on air. He was initially treated as for meningoencephalitis with intravenous cefotaxime and acyclovir. His computed tomography (CT) scan of brain has shown hypodensities in basal ganglia and temporal lobes.
Over the next 24 hours, his condition further deteriorated with worsening respiratory distress and drop in GCS to 8/15 associated with bradycardia and hypertension (Cushing's reflex) suggestive of rapidly rising intracranial pressure. He was transferred to the intensive care unit (ICU) and started on invasive ventilation targeting brain protective measures. Magnetic resonance imaging (MRI) of the brain with arteriography and venography, revealed thrombosis of the straight, superior sagittal and right transverse sinuses associated with hemorrhagic infarcts in bi-lateral basal ganglia, thalami, and diencephalon with acute hydrocephalus and periventricular leukoencephalopathy (Figures and ). Thereafter, patient underwent urgent insertion of an external ventricular drain (EVD) followed by therapeutic anticoagulation with subcutaneous low molecular weight heparin. His cerebrospinal fluid (CSF) analysis was unremarkable except for raised proteins of 190 mg/dl. Subsequent exploration of a cause for his clinical picture, including coagulation profile, antinuclear antibodies (ANA), double stranded DNA (dS-DNA), antineutrophil cytoplasmic antibodies (ANCA), anti-beta 2 glycoprotein, anticardiolipin antibodies, NMDAR antibodies, and JACK2 mutation, was unremarkable. Blood film showed macrocytosis with raised red cell mean corpuscular volume of 107 fl/r. His serum and red cell folate and serum B12 levels, were low, leading us to suspect acquired hyperhomocystinaemia (H-Hcy). His serum homocysteine levels were more than 50 µmol/l (5.4–16.1 µmol/l). Then, he was started on vitamin supplements; B12 1,000 mcg per day for two weeks, folic acid 5 mg daily and pyridoxine 25 mg daily with a dramatic improvement of his neurology, managing to extubate on day 6 and remove EVD successfully on day 7 of ICU admission. Patient was discharged after 15 days of hospital stay without any residual neurology, on warfarin (aiming at INR 2–2.5) and vitamin supplements. On follow up at 12 weeks, his serum homocysteine level has normalized and vitB12 and folate levels were normal. Warfarin was stopped at 3 months.


A 74-year-old male came to the ED with the acute onset of worsening back pain and lower extremity weakness. His past medical history included chronic lower back pain from severe spinal stenosis, hypothyroidism, hypertension, hyperlipidemia, anxiety, benign prostatic hyperplasia, and a pulmonary nodule. Upon review of symptoms, he was found to have chills and decreased activity. Initially, there was concern for cord compression and epidural abscess. He denied any history of fever, nausea, vomiting, or abdominal pain at admission. He reported no history of recent changes in his bowel or bladder habits. He described having had dental work performed two weeks prior to presentation.
CT spine showed severe degenerative lumbar spine along with multilevel cervical spine stenosis. Neurosurgery was consulted who recommended MRI. Preliminary blood cultures grew gram positive cocci in clusters and the patient was started on vancomycin plus cefazolin. ID team was consulted and they recommended to continue with vancomycin and cefazolin until final blood culture report. Repeat blood culture grew MSSA. The source of infection was thought to be abscess in spine, however, MRI cervical/thoracic/lumbar spine showed no evidence of osteomyelitis, epidural abscess or cord compression. Yet, it showed multilevel severe spinal canal stenosis worse at L4-L5 along with multilevel bilateral neuroforaminal stenosis. Neurosurgery recommended only pain management and no acute intervention stating the surgery can be done in the outpatient setting. The patient was started on intravenous oxacillin 2 gram every four hours for total of four weeks of therapy.
A transesophageal echo was obtained which showed small 4-mm linear echo density on the ventricular side of the left coronary cusp of the aortic valve. Differentials included Lambl’s excrescence versus valvular vegetation. Cardiovascular surgery recommended against the intervention and to continue with conservative measures; antibiotics and pain management. The source of the patient’s MSSA bacteremia was deemed to be secondary to infective endocarditis.
A CT chest was performed inpatient to further elucidate the pulmonary nodule. During this exam CT chest showed bubbles of gas in the upper abdomen, rendered likely intraluminal by the radiologist’s report. The CT chest finding was followed up with a CT abdomen to rule out pneumoperitoneum. CT abdomen reported Chilaiditi sign with anterior interposition of the colon to the liver (see Figures -). No bowel perforation, small bowel obstruction, or surrounding inflammatory changes were noted on CT imaging.


A 60-year-old man with a 10 years history of T2DM was diagnosed with a metastatic cutaneous melanoma and was started on ICI treatment, with IPI (3 mg/kg every 3 weeks for four cycles) and NIVO (1 mg/kg every 3 weeks for four cycles). He had an optimal glycaemic control (HbA1C 6.2% two months before ICI initiation) under oral antidiabetic medication (metformin, sitagliptin and gliclazide) (Table ). Two weeks after the first ICI infusion, he was admitted to the hospital with vomiting, polyuria, polydipsia, hyperglycaemia (46 mmol/L), severe acidosis (pH 6.9) and presence of ketones bodies in urine (> 7.8 mmol/L) (Table ). Treatment protocol for DKA with continuous intravenous insulin was initiated with a favourable outcome within 8 hours. Further workup revealed an elevated Glutamic Acid Decarboxylase antibody (GADA) titre 19,770 IU/ml (N < 10) in line with an autoimmune T1DM. The pituitary function was in the normal range. Concurrent plasma lipase level was substantially increased (988 UI/l). According to the ongoing protocol used by our institution’s oncologists which was based on Common Terminology Criteria for Adverse Events (CTCAE) of the National Institute of Health, immune-checkpoint inhibitor-induced pancreatic injury was suspected although abdominal CT-scan was unremarkable. The oncologists decided to discontinue IPI and NIVO after this first dose and to start with prednisone 1 mg/kg/day with a tapered dosage schedule over 14 weeks. Lipase levels decreased by 60% within 48 h, and maintained close to the upper range within 14 days. The patient was discharged on multiple daily insulin injections, with a dosage of 0.6 U/Kg/day. Eighteen months later, cancer disease was in complete remission, and diabetes remained insulin-dependent (0.4 U/kg/day) and relatively well controlled (HbA1C 7.6%).


An 80-year-old woman without a history of diabetes was started on IPI-NIVO for advanced metastatic melanoma. Casual plasma glucose performed 3 months before ICI treatment was 8.3 mmol/L. Three weeks after the first infusion, she presented with transient autoimmune thyroiditis (Table ). Immunotherapy was suspended and thyroid function recovered within 2 weeks. Five weeks after this first ICI administration, she was admitted to the emergency unit for acute mental confusion developed 24 hours before admission. An infectious aetiology was excluded. In the setting of severe hyperglycaemia (48.4 mmol/L), severe acidosis (pH 7.1) and urinary ketones bodies (> 7.8 mmol/L), adequate treatment was started leading to DKA resolution within 6 hours. GADA titre was high (2000 IU/ml, N < 10) consistent with autoimmune T1DM (Table ). Multiple daily insulin injections were started. Cerebral CT-scan performed 24 hours later showed a progression of cerebral metastases and a subarachnoid haemorrhage. The patient died 3 weeks later due to disease progression.


A 19-year-old male patient presented with intermittent shoulder pain and a palpable mass in the right upper quadrant, causing abdominal discomfort. MRI-imaging revealed a hyper intense inhomogeneous mass located in the right lobe of the liver with an approximate size of 9,5 cm in diameter and five nodules in both lower lobes of the lung (). The working diagnoses was a hepatic cyst/alveolar echinococcosis.
Definitive diagnosis was made by biopsy of the pulmonary nodule of the left lower lobe, showing an undifferentiated pleomorphic embryonal sarcoma. The staging was cT2b cN0 cM1 (PUL), UICC IV, Intergroup Rhabdomyosarcoma Studies (IRS) stage 4. The multidisciplinary conference recommended neoadjuvant chemotherapy followed by a radical surgical resection.
Neoadjuvant chemotherapy was performed according to the Cooperative Soft Tissue Sarcoma Study Group (CWS-2014 guidance, treatment guidelines for soft tissue sarcoma and soft tissue tumors) for 13 weeks, with four cycles of CEVAIE. Primary tumor and pulmonary metastasis showed a good response and shrinking in restaging. The patient had normal liver laboratory values and a preoperative LiMAx of 479 μg/kg/h. A hemihepatectomy (right lobe + segment 4b) with a sFLR of 28,4 % of segments I-IVa was planned (A). Intraoperatively, the liver parenchyma was firm and macroscopically a chemotherapy-associated liver damage was suspected. Therefore, we went for an intraoperative histopathological analysis of the parenchyma in fresh-frozen sections. This revealed cholestasis, beginning portal fibrosis and single cell necrosis (). Additionally, invasion of not only the right, but also the middle hepatic vein was noted. Consequently, a FLR of 28 % combined with injured liver parenchyma seemed not to be adequate for a one-stage liver resection. To reduce the risk of a posthepatectomy liver failure, a hypertrophy concept was necessary. We decided to proceed with ALPPS for the induction of hypertrophy. A classical step I with a complete transection between segments I-IVa and IVb-VIII was done down to the vena cava. Intraoperative blood loss was 1.200 ml. No complications occurred and on POD 5 the patient was discharged with normal liver function.
Nine days after step I, re-volumetry based on a CT scan revealed a sFLR of 57,3 % (B). The following day, step-II was performed and the tumor-bearing liver part removed. shows the intraoperative situs before and after resection. The specimen is shown in . LiMAx test on POD 1 was 269 μg/kg/h, presenting a sufficient liver function of the remnant.
The patient developed bile leakage, which was treated with interventional drainage and ERC. Otherwise, the postoperative course was uneventful and the patient was discharged in good conditions on POD 14. The pathological work up demonstrates tumor free margin. Extensive sampling of the tumor bed shows broad areas of fibrosis and necrosis with haemorrhage and resorptive changes with infiltrates of foamy histiocytes. Only a few small spots of residual vital sarcoma could be detected (, ). The therapy-induced tumor regression grade was 99 %. After recovery from surgery, chemotherapy according to the CEVAIE protocol (CWS-2014 guidance) will be resumed and a complete resection of the oligometastatic pulmonary disease is planned.


A 5-year-old girl developed a sudden reduction in her vision and had deep ocular pain in her right eye. She visited a private eye clinic on the following day. She had no noteworthy medical and family histories. Her decimal visual acuity (VA) was 0.7 OD and 1.2 OS with a relative afferent pupillary defect in the right eye on day 2. She was referred to the Saitama Medical University Hospital, and ophthalmoscopy and optical coherence tomography showed a swelling of the optic disc and tortuous vessels at posterior pole of the right eye on day 3 (). Neurological and general examinations were within normal limits. MRI demonstrated hyperintensity of her right optic nerve on short TI inversion recovery (STIR) sequence and no cerebral lesions (). On the initial visit to Saitama Medical University, patient's blood was drawn for laboratory examinations and for serum antibodies against AQP4 and MOG. Laboratory examinations showed that the blood and cerebrospinal fluid tests were within the normal limits except a few items (). Spinal MRI showed no abnormalities in the cervical, thoracic, and lumbar spinal cord.
Ischemic optic neuropathy, traumatic neuropathy, toxic-nutritional optic neuropathy, rhinogenic optic neuritis, and hereditary optic neuropathy were considered as a differential diagnosis and were denied from various clinical findings. She was diagnosed with right papillitis and treated with 450 mg of intravenous methylprednisolone pulses for 3 days (days 3–5) followed by a gradual tapering of oral prednisolone (). On day 5, the best-corrected visual acuity (BCVA) decreased to no light perception, and plasmapheresis was performed for 3 days (days 6, 9, and 12) combined with high dose intravenous immunoglobulin therapy (160 mg/kg, total 2.5 g) for 1 day (day 10) (). Because the patient's vision loss was so severe, we started systemic treatment to prevent a relapse after discussion with patient's mother. The decimal visual acuity rapidly improved and reached 1.0 on day 13 [; ()]. The swelling of the optic disc and tortuosity of the retinal vessel of the right eye disappeared on days 35 and 63, respectively. From the examination of the blood drawn on the initial visit, it turned out on day 9 that she was anti-AQP4 antibody-negative and on day 19 that anti-MOG antibody-positive.
Pattern VEPs (pVEPs) were recorded several times during the course of the disease with the recording parameters conforming to the International Society of Clinical Electrophysiology of Vision (ISCEV) standards except that the checkerboard size was ~2° (). The findings represented the dynamic changes in the physiology of the visual pathways objectively (; ). The implicit times of the N75 and P100 components of the pVEPs of the right eye were prolonged compared to that of the normal fellow eye when the VEPs were elicited by stimulating the right eye throughout the follow-up period. The implicit times of the right eye became shorter on day 109 and remained prolonged on day 316.
Although the patient reported that she felt that the image of the right eye was relative dark compared to the left eye, the decimal BCVA of the right eye has remained at 1.2 for at least 1 year. The implicit time of N75 was prolonged in the right eye compared to the left eye until day 21, and thereafter it seemed to normalize and become stable for almost 1 year. On the other hand, the implicit time of the P100 component was prolonged in the right eye compared to the left eye throughout the follow-up period.
Five milligram of oral prednisolone has been used every other day, and no recurrences have been observed for at least 1 year.


A 38-year-old primiparous woman with an unremarkable medical history consulted in September 2017 for pelvic pain, menorrhagia, dysmenorrhea, and dyspareunia—suggesting the presence of adenomyosis. Magnetic resonance imaging showed the uterus with a longest dimension of 8.6 cm and an anteroposterior diameter of 7.3 cm. The endometrium had a normal aspect and thickness. A nodular lesion (measuring 6.5-5.3 cm) was present in the posterior myometrial wall. The lesion was heterogeneous (with areas of hyperintensity in a T2-weighted MRI sequence) and contained a large number of cyst-like structures. In a T1-weighted sequence, some of the cysts had a hyperintensity pattern consist with hemorrhage, whereas others appeared to be filled with fluid. Thickening of the junctional zone was suggestive of adenomyotic damage. In contrast-enhanced sequences, the lesion appeared to be well delimited and had intense but not suspicious foci of uptake. On this basis, we diagnosed interstitial myoma with focal hemorrhagic cysts (Figures and ). The patient attended a follow-up consultation in December 2017. A cervical smear was normal, and a three-month course of ulipristal acetate 5 mg/d (Esmya®, Gedeon Richter) was initiated. Due to poor tolerance (nausea and vomiting) and lack of effectiveness on bleeding, the patient stopped taking the medication. In August 2018, she consulted for a second opinion. A clinical examination showed a retroverted uterus but no vaginal prolapse. Various treatment options (myomectomy, adenomyomectomy, and hysterectomy) were discussed with the patient but none was immediately chosen. In January 2019, the woman (who worked in the catering industry) returned to our clinic complaining of abnormal bleeding for 3 weeks a month and pelvic heaviness. A pelvic examination revealed a large intravaginal mass; it measured between 6 and 7 cm, protruded from the cervix, and had the appearance of myoma. Given the recurrence of abnormal bleeding, the pain, and the aspect of the vagina, the woman agreed to have a hysterectomy without adnexectomy. Three days after this consultation, the woman was admitted to the emergency department for acute pelvic pain (rated at 9 out of 10 on a visual analogue scale) and bleeding. The very intense pain suggested torsion or necrobiosis of the myoma. The woman's pain was relieved in part by treatment with paracetamol, a nonsteroidal anti-inflammatory, and a muscle relaxant. This treatment was combined with hypnosis sessions, which increased the patient's pain tolerance. The pain level fell over the following 48 hours, and surgery was performed three days after the patient had been admitted to the emergency department.
During the (initially laparoscopic) surgical procedure, we observed an invagination of the Fallopian tubes and the ovarian and round ligaments. It was impossible to see the uterus (Figure A). We then decided to perform a laparotomy, which revealed total uterine inversion (Figure B). The round and ovarian ligaments and the Fallopian tubes (which were ligated and then severed (Figure C)) were released in a stepwise procedure. An intraoperative methylene blue test showed that the upper pole of the bladder was completely inverted. By opening the peritoneum of the bladder, we lifted up the uterus and thus released the uterine arteries. Hysterectomy was then performed. Figure shows the inverted uterus after extraction: the operator's finger (measuring about 9 cm in length) is located within the invaginated fundus. The postoperative course was uneventful, and the patient was discharged on postoperative day 3.
Histopathological assessments of the cervix (Figure A: a per-opérative view; 5B: a macroscopic view) and the uterine corpus (Figure C: a macroscopic view; 5D: a microscopic view) were performed. The resected uterus was 12.5 cm long, 8 cm wide, and 7 cm thick. The uterus was bounded by the endometrial mucosa, and the peritoneum was located in the center of the invaginated area. The very extensive adenomyotic lesions contained a number of large cysts measuring up to 35 mm in diameter. There were no signs of malignancy. On the basis of this assessment, the final diagnosis was uterine inversion caused by major adenomyotic damage.


Our patient was a 72-year-old gentleman with the past medical history of mild chronic obstructive pulmonary disease (COPD), a previous history of subsegmental pulmonary embolism, atrial fibrillation status post watchman device, peripheral vascular disease status post right femoropopliteal bypass, hypertension, and hyperlipidemia. He was an ex-smoker with a 60 pack-year smoking history and quit about six years ago. He had no recent travel. His medications included rivaroxaban, aspirin, duoneb, metoprolol, amiodarone, and amlodipine. He was recently discharged from the hospital after being treated for right fifth toe osteomyelitis. He was treated with intravenous daptomycin because he had anaphylactic reaction to vancomycin in the past. He subsequently got this toe amputation done, but the proximal bone cultures grew MRSA, so he was continued on daptomycin post-operatively. An infectious disease specialist evaluated him, and he was discharged to rehab on a six-week course of daptomycin via a peripherally inserted central venous catheter (PICC).
He was gradually recovering in rehab when his symptoms started with nausea along with non-bloody diarrhea of three days duration. He subsequently developed subacute respiratory distress and was requiring up to six liters of nasal cannula oxygen. He was sent to the hospital for further evaluation. In the emergency room (ER), he was found to be in mild respiratory distress, and his lungs exam showed bilateral crackles that were predominantly present on the right side. Heart exam was unremarkable except irregularly irregular pulse. Lower extremity exam showed well healing right lower extremity surgical scar without any edema. He was otherwise vitally stable on 4-6 liters of nasal cannula oxygen. His pertinent basic blood workup in the ER was as follows: Cr: 1.61 mg/dl, BUN: 20 mg/dl, cardiac BNP: 1432, INR: 1.7, Hb: 12.4 g/dl, WBC: 10.7 k/ul, absolute eosinophils count: 3.1 k/ul, absolute neutrophil count: 6.1 k/ul. His urinalysis was essentially unremarkable. He did not have a baseline cardiac BNP but had a normal baseline creatinine about four weeks back. In the ER, he was evaluated with a chest X-ray that showed new opacification throughout the right lung, suggestive of unilateral pulmonary edema or pneumonia (Figure ).
He was started on linezolid and metronidazole for possible aspiration/healthcare-associated pneumonia along with intravenous (IV) diuresis to treat possible pulmonary edema. He was admitted on the general medical floor.
The next day we did a CT scan of his chest to evaluate the lung parenchyma better. The coronal view of CT scan of his chest showed bilateral ground-glass opacities of the lung parenchyma with right lung predominance (Figure ). Similar findings of bilateral ground-glass opacities can also be seen on a cross-sectional view of the CT chest (Figure ).
The echocardiogram of his heart showed normal systolic function without any evidence of diastolic dysfunction. He was continued on gentle intravenous diuresis, and the plan was to do a bronchoscopy to make a diagnosis. Meanwhile, we discontinued the daptomycin on admission. We continued to support his breathing with respiratory treatments with albuterol/ipratropium, supplemental oxygen for hypoxia, antibiotics for possible bacterial pneumonia, and diuretics for possible pulmonary edema. Bronchoscopy was done after elective intubation, and gross inspection only showed clear secretions throughout the right lung. Bronchoalveolar lavage was done from the right upper/middle lobe, and fluid was sent for bacterial/fungal cultures/cytology/AFB smear and differential count. After the bronchoscopy, the patient was started on prednisone 40 mg IV twice a day. His bronchoscopic fluid analysis showed a total WBC count of 8504/UL, with 40% neutrophils and 42% eosinophils. Bacterial/fungal cultures were negative for infection, and cytology was negative for malignant cells.
At this point, based on the clinical presentation and above lab data, a diagnosis of daptomycin-induced eosinophilic pneumonia (DAP) was made. He was extubated and was transferred to the general medical floor on nasal cannula oxygen. Antibiotics and IV diuretics were stopped, and he was started on Trimethoprim-Sulfamethoxazole for pneumocystis (PCP) prophylaxis in anticipation of long-term steroid treatment. His oxygen requirement varied from six liters of nasal cannula oxygen to a 55% venturi mask for the next few days. Recovery, in his case, was relatively slow despite being on steroids, and he continued to require more than 40% FiO2 for more than a week. Given his slow recovery and ongoing relatively high oxygen requirement, he was started back on IV diuretics. He was evaluated by the cardiologist to assess his volume status better, and it was decided to do a right heart catheterization. Though the suspicion was low, we decided to stop his amiodarone as a potential contributor towards his ongoing pneumonitis. Right heart catheterization was mostly unremarkable and showed pulmonary capillary wedge pressure (PCWP) of 5. Diuretics were stopped at this point, and the patient was clinically observed on steroids. He had a prolonged recovery with possible residual lung damage as we were not able to entirely wean him off of oxygen despite two weeks of steroids. Though he was stable on 2-3 liters of nasal cannula oxygen at baseline, he was not on oxygen, and therefore it was new for the patient. He was subsequently discharged from the hospital in a stable condition and on home oxygen, with a close follow-up with the pulmonologist as an outpatient. Lung biopsy was never performed on this patient.


A 76-year-old male patient presented a rapidly growing 9-mm asymmetric, dark-brown patch on the scalp. Dermoscopic examination showed comedo-like openings, fat fingers, and irregularly distributed dots ().
The first diagnostic hypothesis was SK, but the irregularly distributed dots lead the authors to perform RCM to exclude melanoma. It showed a typical honeycomb pattern at the spinous-granular layer, epidermal projections, keratin filled invaginations, and widespread dendritic pagetoid cells. At the dermo-epidermal junction (DEJ), densely packed polymorphous dermal edged papillae were observed, in addition to an infiltration of dendritic cells between dermal papillae. At the papillary dermis level, melanophages were observed (). Based on RCM findings, it was not possible to classify this lesion as clearly benign, so it was excised.
On histopathological evaluation, hyperplastic epidermis, hyperkeratosis, acanthosis, and dendritic melanocytes were seen throughout the lesion. Melan-A and CD1a immunostain were positive and confirmed an increased density of melanocytes and Langerhans cells (). These findings were consistent with CM.


A 67-year-old woman with a past medical history of controlled hypertension and diabetes mellitus presented to the emergency department (ED) with a painful rash for approximately one week. She complained of a red rash on the left side of her lower abdomen that wrapped around to her lower left flank. The rash was also present on her right anterior chest with some other similar lesions below her left eye and the right side of her chin. She further described the rash as blisters that are exquisitely painful to touch with an “electric” type of pain. She had previously come to the same ED with the complaint of abdominal pain at which time she did not receive a definitive diagnosis, and the rash erupted two days later. Thus far, she had minimal relief with over-the-counter pain medication. She denied ever having a similar rash in the past or any known sick contacts. She did endorse having the “chicken pox” as a child. She had been in her previous state of health prior to these symptoms arising, but did note she recently had surgery on her left upper extremity. She denied any additional symptoms such as fever, chills, cough, shortness of breath, vision changes, nausea, vomiting, or diarrhea.
On physical examination, the patient’s vital signs were normal other than mild hypertension. Abdominal examination included a vesicular, erythematous rash with all lesions in the same stage of development located over the left lower abdomen (Figure ) wrapping around to the left flank (Figure ), with a few lesions crossing the midline (Figure ). She also had the same lesions on the right anterior chest and right breast (Figure ) with scattered lesions into the right anterior axillary line. A single lesion was located below the left eye (Figure ), and another single lesion on the right side of her chin. The rash was extremely tender to light palpation with associated paresthesias. Fluorescein dye was applied to both eyes to examine for potential signs of herpes zoster ophthalmicus, and no such lesions were noted. The nose and both ears including the acoustic meatus were lesion free. Neurological examination was normal. On completion of the physical examination, at least four discrete dermatomes were found to be involved. The patient was placed under contact and airborne precautions.
The patient’s complete blood count was negative for a leukocytosis or leukopenia, but was notable for the presence of atypical lymphocytes. Chemistry panel was unremarkable except for hyperglycemia. Chest radiography revealed a 3 mm calcified granuloma along the periphery of the left lower lobe (Figure ), which the patient was previously aware of. No acute findings were otherwise noted.
At this time, the patient was started on intravenous (IV) acyclovir with concern for disseminated herpes zoster and given morphine for acute pain control. Our findings and concerns were discussed with the patient who agreed with a plan for admission for further treatment, evaluation for possible immunocompromised state, and pain control. On admission, the plan for infectious disease (ID) consultation was discussed with the medicine team. Due to the unusual presentation of disseminated zoster in an immunocompetent patient, IV acyclovir was continued by ID with ongoing investigation into possible human immunodeficiency virus infection, underlying malignancy, hepatitis infection, and other autoimmune conditions. Through day 3 of her hospitalization, she continues to develop additional lesions, notably on her right upper quadrant and left ear. She also developed an acute kidney injury during her hospitalization which was likely crystal induced and resolved after IV acyclovir administration was changed from every eight hours to every 24 hours. The patient’s lesions began to crust over and resolve on day 4 of hospitalization and on day 5 she was transitioned to oral acyclovir and discharged from the hospital.


A 1-year-11-month old boy with no medical history was found to be struggling to breathe and unable to speak by his mother at home. She suspected that the patient's airway was obstructed by a foreign body and performed the Heimlich maneuver. Because the foreign body could not be removed and the patient began losing consciousness, she promptly called the emergency medical services (EMS). The EMS telecommunicator did not recognize the OHCA, and so the operator did not instruct the patient's mother to initiate bystander cardiopulmonary resuscitation (CPR); as a result, bystander CPR was not performed. Six minutes after placing the emergency call, the patient was found to have pulseless electrical activity and the EMS personnel initiated CPR. During the resuscitation, bag ventilation and chest compressions were performed. The patient was resuscitated after 20 min of cardiac arrest before arrival at our hospital.
Upon arrival at the emergency department, coma (Glasgow Coma Scale score 3/15 [E1V1M1]) and a small amount of intraoral bleeding were noted. The respiratory rate was 36 breaths/min and oxygenation was slightly impaired with a peripheral capillary oxygen saturation level of 94% on room air. The blood pressure was 129/71 mmHg, and the heart rate was 159 beats/min. Arterial blood gas analysis revealed respiratory and metabolic acidosis and the rise in lactic acid value (pH 6.89, PaCO2 58.3 mmHg, PaO2 69.3 mmHg, base excess −22.7 mEq/L, HCO3− 10.8 mEq/L, and lactate 14.4 mmol/L). Upon laboratory examination, the following results were above normal: white blood cell count (25,730/μL) and D-dimer (23.1 μg/mL) levels. A chest X-ray and computed tomography scan showed a foreign body at the lower esophagus and bilateral pulmonary consolidation ().
Because the patient presented with coma and aspiration pneumonia, tracheal intubation was performed. At that time of intubation, the foreign body was not detected in the oral and upper airways. Therapeutic hypothermia with a target temperature of 34°C was begun after one and a half hours of arrival at our hospital, for the critical management of post-cardiac arrest syndrome. After stabilizing his general condition, the patient underwent upper gastrointestinal endoscopy, which revealed a piece of pushpin in the stomach (). A pair of basket forceps was used to remove the foreign body in order not to injure the mucous membrane of the esophagus with the sharp tip of the pushpin. Follow-up endoscopy performed just after the removal of the pushpin revealed no mucosal injury.
After a hypothermic period of 36 h and rewarming period of 24 h, on post-OHCA day 4, the patient was weaned from the ventilator and tracheal extubation was performed. On post-OHCA day 6, the patient was transferred from the intensive care unit to the general ward. On post-OHCA day 11, the patient was discharged from the hospital to his home with pediatric cerebral performance category 1, defined as a condition without subsequent neurological complications.


A 59-years-old, non-smoker, male, affected by severe, late-onset asthma, in periodic follow-up at our regional referring centre for Severe Asthma.
Uncontrolled by GINA step five therapy for asthma with high dose ICS (Beclometasone 400 mcg/twice a day), LABA (Formoterol 12 mcg/twice a day), LAMA (Tiotropium 5 mcg per day), LTRA (montelukast 10 mcg per day) and Benralizumab, after two failed attempt with Omalizumab and Mepolizumab; Spirometry (07/01/2019: FEV1 1,37 L- 41% - FVC 4.05 L - 96% - FEV1/FVC 34–44%) was performed and Asthma Control Test was administered. The patient reported more than four exhacerbations/year.


A 34-year-old man was admitted to our hospital on August 2014 due to occasional discomfort in the liver area for 9 months. The patient felt fatigue occasionally and had no history of joints pain. The patient denied history of hypertension, coronary heart disease, diabetes, viral hepatitis and tuberculosis, and also denied history of surgery, trauma, blood transfusion, and food or drug allergy. He has smoking history for 7 years (about 7 cigarettes per day), and occasionally drank in recent 5 years (one time per week, equivalent alcohol intake <60 g per time). Nine months before being admitted, he had not received any additional treatment except for taking hepatoprotective drugs.
There was no abnormality in physical examination.
In the laboratory tests, liver function showed that aspartate aminotransferase was 48.5 U/L (reference range: 15–46 U/L) and alanine aminotransferase was 73.1 U/L (reference range: 0–40 U/L). The iron metabolism showed that the serum iron was 23.4 μmol/L (reference range: 10.6–36.6 μmol/L), total iron binding capacity was 47.2 μmol/L (reference range: 50–70 μmmol/L), ferritin was 12,405.0 μg/L (reference range: 20–200 μg/L), and transferrin saturation was 50% (reference range: 20–50%). No abnormal findings in the tests of blood and coagulation routine, urine and stool routine; no abnormal findings in the tests of kidney function, electrolyte, blood lipid and glycosylated hemoglobin; no abnormal findings in hepatitis B markers, hepatitis C antibody as well as alpha fetoprotein (AFP); antinuclear antibody (ANA), autoimmune liver disease-related antibodies, and immunoglobulin were normal. ECG was normal. Echocardiography showed mild tricuspid regurgitation.
Contrast-enhanced magnetic resonance imaging (MRI) of the liver and spleen showed enlarged spleen and extensive and uniform decrease of the signal in liver and spleen (Fig. ). Liver biopsy showed phagocytic Kupffer cell infiltration, expanded portal area, fibrous tissue proliferation, and a few of inflammatory cells infiltration. Iron staining was positive and copper staining was negative. The pathologic diagnosis was hereditary hemosiderosis (Figs. and ). Sequencing test was performed on the pathogenic genes in the online Mendelian Inheritance in Man (OMIM) database including HFE, HAMP, HJV, TFR2, and SLC40A1 gene. Gene mutation was not found in HFE, HAMP, HJV, and TFR2. However, it was found that the TTG at position 485 to 487 of SLC40A1 gene was deleted, resulting in the deletion of the valine 162 of encoded ferroportin1 protein. The mutation of the gene shows autosomal dominant inheritance. The patient was heterozygote for the mutation (Fig. ). Genetic test was further performed on his relatives. It was found his mother, 1 of the 2 aunts, and 1 of the 2 uncles also carried heterozygous mutation of Val162del of SLC40A1 gene (Fig. ).
The patient was given a low iron diet and phlebotomy interval 2 weeks until the ferritin 100 μg/L. At present, phlebotomy is performed every 3 to 6 months according to the results of the patient's examination. There were no significant changes in blood routine tests. With those treatments, he was asymptomatic with 5 years follow-up.
This case report was approved by the ethics committee of the First Hospital of Jilin University, Changchun, China, and the informed consent form was signed by patient.


A 22-year-old previously healthy female patient was found to have 2 giant well-circumscribed masses with rim shadowing in the region of both adrenal regions by abdominal ultrasound in a routine investigation. She was asymptomatic and then referred to our hospital for further examination and treatment. At physical examination, her arterial blood pressure was 128/77 mm Hg. Abdominal examination disclosed 2 global distention with dullness on the both sides of her upper abdomen. No other physical findings were observed. The baseline workups for this patient, including complete blood count, renal, and liver function tests, were within normal limits. Functional evaluations for adrenal hormones, including 24-hour urine cortisol, fractionated metanephrines, and plasma aldosterone to renin ratio, were also within normal limits.
During the course of investigations, an abdominal and pelvic computed tomography (CT) scan showed 10.4 × 10.1 × 13.2 cm and 12.3 × 10.5 × 12.5 cm solid and cystic masses in the left and right adrenal region containing bone and multiple soft tissue densities, respectively (Fig. ). In order to better define the condition of the tumor and its relationship with the kidney, we did a three-dimensional digital reconstruction of the kidneys, blood vessels, and masses based on CT images. Total tumor volume was 1698.6 ml, while the total volume of 2 kidneys was 347.6 ml. Both of the 2 kidneys were squeezed and deformed and the pancreas was densely adherent to left mass (Fig. ). There was no evidence of distant metastasis. In further assessment, abdominal magnetic resonance imaging (MRI) revealed a 10 × 10.8 × 12.9 cm mass and a 10.3 × 12.3 × 12.1 cm mass with heterogeneous signal intensity, heterogeneous enhancement, and predominantly fatty composition in the left and right adrenal region, respectively, suggestive of 2 adrenal teratomas. Considering that the masses are more likely to be benign tumors, that the patient is a young unmarried woman, and that we must retain normal adrenal tissue as much as possible to maintain the function, we decided to carry out laparoscopic simultaneous resection.
The patient took a supine position and the waist was blocked up. A 4 cm incision was made upon the umbilicus and a 10-mm trocar was placed. A 12-mm Trocar was inserted 3 cm under the xiphoid process, and 2 5-mm Trocars were placed 3 cm below ribs on the right anterior axillary line and 1 cm below ribs on midaxillary line, respectively. We first dealt with the lesion on the right side. Although the mass was located in the region of right adrenal gland, we found that the adrenal gland was on the external surface of the mass and distinct from the mass. We separated the tumor carefully from the adrenal gland and retained most of the normal adrenal tissue (Fig. A). Because of its large size, the tumor collapsed during careful dissection. We drained the mixed orange-light brown, fatty, and mucoid mass with black strands of hair and completely removed the tumor. On the left side, we added a 12-mm Trocar on the 3 cm below ribs on the left anterior axillary line. The tumor was densely adherent to the pancreas and aorta, which made the dissection more tedious and difficult. After careful dissection, we found the mass was densely adherent to, but still distinct from the left adrenal gland. The tumor also collapsed, and we drained the yellow-gray sebaceous substance and hair and completely removed the tumor. The left adrenal gland was retained entirely. Finally, the tumors were removed meanwhile and sent for histopathological evaluation (Fig. B). During the whole operation, we only used 5 operating holes with a 4 cm-incision (Fig. C). The operation time was about 480 minutes. The amount of bleeding was 1000 ml while the blood transfusion was 700 ml. The postoperative course was uneventful and the patient was discharged on postoperative day 11. The patient was free of recurrence after 10 months of follow-up.
The final pathological evaluation of the retroperitoneal masses confirmed the diagnosis of mature teratomas, with abundant fat deposits and different tissues such as adipose tissue, skin, and sebaceous appendages identified in the cystic wall (Fig. ). These findings confirmed the lesions to be benign mature cystic teratomas. Because these tumors usually represent metastasis from other primary sites, additional imaging with CT of the chest and abdomen, and ovarian ultrasonography was performed. No other primary tumor was identified. Therefore, we diagnosed the masses as primary retroperitoneal teratomas.


A 33-year old, previously fit and well, Caucasian woman presented to a UK acute medical unit with 2 days of gradual onset pressure-like headache, fever, neck stiffness and vomiting. There was no travel history of note or recent ill contacts. On examination, she was febrile at 38.6 °C and had photophobia with nuchal rigidity, however with a normal conscious level, and unremarkable systemic and neurological examinations. Initial tests included a normal serum white cell count and a C-reactive protein of < 1 mg/L, normal creatinine and electrolyte levels, and a normal chest radiograph. A Computed Tomography (CT) of her brain was reported as normal with no masses, haemorrhage or hydrocephalus. Cerebrospinal fluid (CSF) analysis revealed an elevated white cell count of 172 × 106/L, with complete lymphocytosis and elevated CSF pressure of 32 cm H20 (Table ). A working diagnosis of viral meningitis was made. Following a lumbar puncture, she was treated empirically with intravenous acyclovir 10 mg/kg three times a day pending her CSF virology polymerase chain reaction (PCR) screen result which came back negative after 3 days at which point the acyclovir was stopped (Table ). She also received paracetamol as an analgesic and antipyretic but did not receive steroids. Her headache improved, she was afebrile for 48 h and she was discharged home after 5 days.
A week later, the clinical phenotype had evolved considerably from that of pure meningitis, to meningoencephalitis. On representing to the Emergency Department, she had additional clinical features of confusion with impaired concentration, memory deficits and mild dysarthria. She remained febrile. She rapidly became increasingly agitated, and developed visual hallucinations and a generalised tonic-clonic seizure. Over 5 days, she developed a reduced conscious level alternating with agitation and mutism. She was intubated and ventilated, and treated empirically with intravenous ceftriaxone 2 g a day and acyclovir 10 mg/kg three times a day for presumed meningoencephalitis. She was subsequently transferred to our hospital for further investigations and care. During this period, despite ongoing antimicrobial therapy for a week, she had ongoing pyrexia and a persistently reduced Glasgow Coma Scale (GCS) requiring critical care support. She later developed marked autonomic instability, along with new orofacial and upper limb dyskinetic movements.
She underwent detailed investigations for infective and autoimmune causes of encephalitis and meningitis. Her Magnetic Resonance Imaging (MRI) brain with contrast showed a few tiny non-specific supratentorial white matter T2-weighted high signal foci which were not thought to be of clinical relevance. Otherwise the appearance of the brain parenchyma was normal with no abnormal enhancement post-contrast. A summary of her serum and CSF investigations is shown in Table . CSF repeatedly demonstrated marked lymphocytosis with elevated protein levels, along with an elevated opening pressure on one occasion and a reduced CSF/serum glucose ratio on two occasions. Investigations for a number of infections were negative. An electroencephalogram showed generalized brain wave slowing indicative of diffuse cerebral dysfunction. Serum live cell-based assay for NMDA-receptor (NMDAR) antibodies from day 14 of illness was positive. CT of the pelvis demonstrated a left ovarian lesion. Subsequent excision confirmed a stage 1A G1 immature ovarian teratoma on histopathological examination. A diagnosis of anti-NMDAR encephalitis related to ovarian teratoma was made. Due to a sampling error, a CSF NMDAR antibody result was not available.
She was treated with high-dose intravenous methylprednisolone 1 g a day for 5 days on day 28 of illness followed by five cycles of plasma exchange, and subsequently started on prednisolone at a dose of 60 mg once daily via a nasogastric tube. She was extubated after 3 weeks and she continued to demonstrate improvement in her concentration, memory and language ability. After leaving intensive care, neuropsychiatric assessment documented executive dysfunction with concrete thinking and anxiety, however these rapidly improved. She underwent a left salpingo-oophorectomy 2 months following her initial presentation. She was discharged from hospital after 3 months at which point she was independent in her mobility and self-care. Steroids were slowly reduced and stopped over 3 months as an outpatient. Further immunotherapy was not required. Because of the clear clinical diagnosis, supported by immunological assay and clinical improvement, repeat CSF tests were not clinically indicated.
Overall, she made an excellent recovery. She has remained well and returned to work as a receptionist. Further assessments revealed only subclinical executive dysfunction with no major impact on her activities of daily living. Serial gynaecological oncology reviews confirmed complete excision of teratoma and absence of contralateral disease. Oncological prognosis is considered to be good. We consider the likelihood of recurrence of encephalitis to be low.


A 65-year-old woman presented with 6-year history of subcutaneous nodule in the vulvar region and recently the mass obviously increased creating personal discomfort. She was then admitted to our hospital for treatment. Computed tomography (CT) of the pelvis showed a cystic solid mass (diameter 50 mm) in the perianal region, suggesting a benign leiomyoma. The patient had no prior history of malignancy. Then, the patient underwent wide excision. On surgery, one nodule was found to be located in the vulvar muscle space, measuring approximately 45 mm in maximum diameter. The tumor was well-defined without obvious capsule. No sign of local recurrence or metastatic disease was observed after the initial excision during an eight-months follow-up.
Grossly, the lesion was a well-circumscribed (Fig.a), solid mass, with areas of translucent quality. On cut section, there was a solid white to gray lobulated nodule, measuring 5.5 × 4.0 × 3.5 cm in size. Histologically, at low magnification, the tumor was well circumscribed, focally encapsulated, and lobulated. The tumor stroma was relatively hypervascular and comprised a mixture of myxoid and nonmyxoid components, myxoid areas accounted for 20% of the tumor volume. At high magnification, the lesion was composed of spindle-shaped to epithelioid cells with abundant amphophilic cytoplasm, consisting of vesicular nuclei and small nucleoli. In nonmyxoid area, the tumors cells arranged in storiform pattern (Fig.b, c) and some areas of tumor stroma was predominantly hyalinized or fibrous (Fig.d). In myxoid areas, tumors cells grew singly or in a loosely cohesive manner with abundant eosinophilic cytoplasm resembling rhabdomyoblasts (Fig.e, f). Rhabdomyoblasts-like cells accounted for approximately 80% of the total cells in the myxoid areas. The nuclear atypia was mild to moderate and mitotic figures were low (up to five mitosis per 50 high-power fields).
Immunohistochemically, the tumor cells were diffusely positive for Vimentin, and partially positive for epithelial membrane antigen (EMA) with at least moderate intensity, which was mainly expressed in epithelioid cells (Fig.a). Estrogen receptor (ER) was weakly expressed in some tumor cells (Fig.b). Calponin (clone: CALP and EP63) was positive for both the nucleus and the cytoplasm of the tumor cells (Fig.c). Focal expression for Bcl-2 and CD99 was observed. The tumor was negative for S100 protein (Fig.d), cytokeratin (CK), glial fibrillary acidic protein (GFAP), CK7, SOX10, CD31, CD34 (Fig.e), desmin, MyoD1, myogenin, smooth muscle actin (SMA), CD117, β-catenin and MUC4. Loss of INI1 protein expression was also confirmed (Fig.f). The Ki67 index was about 10%. The results of immunochemical staining were summarized in Table .
Using formalin-fixed, paraffin-embedded 4-mm-thick tumor samples, dual-color break-apart fluorescence in situ hybridization (FISH) was used to investigate EWSR1 and FUS1 gene rearrangements. Break-apart probes for EWSR1 (Abbott Molecular Inc., USA) and FUS1 (Abbott Molecular Inc., USA) were used, and no split signals were observed with either probe (Fig.a, b).


A 28-year-old male patient presented to our emergency department after sustaining a gunshot wound to the right forearm. The entry wound was located on the ulnar-dorsal aspect of his right mid-forearm. The exit wound was located on the volar aspect of the forearm and 3 cm distally to the elbow (Figure ). Examination revealed absent sensation on his right 5th and ulnar half of the 4th fingers. The metacarpophalangeal joints of his 4th and 5th fingers were extended and the interphalangeal joints of the same fingers were flexed, suggestive of a ‘claw’. Froment’s test was positive.
The test was performed by asking the patient to hold a piece of paper in his hands, pinched between his thumbs and index fingers. The examiner then attempted to pull the paper away from the patient. It was noted that in order for the patient to maintain his hold on the affected side, his thumb flexed at the interphalangeal joint. The left, uninjured side demonstrated normal thumb adduction (Video ).
Peripheral pulses were noted to be equal bilaterally, and there were no other injuries. X-rays of the forearm revealed no fractures. Neurotmesis of the right ulnar nerve was suspected and he was referred urgently to the orthopedic surgery department for further care. The patient was taken to theatre for repair the same day.


A 58-year-old male with comorbidities of hypertension, chronic obstructive pulmonary disease, benign prostatic hyperplasia, and gastro-esophageal reflux disease was initially referred to the gastroenterology clinic by primary care physician for the surveillance colonoscopy, and evaluation of dark colored stools.
Patient noted dark colored stools during the Christmas weekend associated with nausea, vomiting, and abdominal pain. The abdominal pain was epigastric, gradual onset, severe in intensity, nonradiating, aggravated with emesis with no specific relieving factors. Denies any hematemesis. He had colonoscopy 10 years ago and had history of colonic polyps. At the time of assessment in the clinic he denied any nausea, vomiting, and worsening abdominal pain or other symptoms.
Physical examination of cardiovascular, respiratory, gastrointestinal, and neurological system was unremarkable. Vitals were within normal limits. Recent laboratory workup showed hemoglobin of 14.7 g/dl with normal renal and hepatic parameters including normal albumin levels. He has past medical history of anemia and esophagogastroduodenoscopy (EGD) and Colonoscopy which revealed arteriovenous malformations (AVMs). He also has history of Alcoholic pancreatitis, alcohol dependence, smoking but no history of any drug abuse. Family history was negative for the colon cancer and stomach cancer. For gastroesophageal reflux disease, he was prescribed PPIs as needed but he was not taking it regularly due to little or no relief from them.
For the evaluation of GI bleed, he underwent colonoscopy in January 2016 which showed normal ileum, few 2–4 mm hyperplastic polyps in the sigmoid colon, and few 3 mm polyps in the rectum which had reactive lymphoid aggregate and focal epithelial hyperplasia on biopsy. Other findings included diverticulosis in the sigmoid colon and in the descending colon and nonbleeding internal hemorrhoids. In January 2016 an EGD showed normal esophagus but enlarged gastric folds and erythematous mucosa in the antrum and stomach biopsy showed gastric antral mucosa with focal mild increase of eosinophils and congestion. Three nonbleeding angioectasias were seen in the duodenum which were treated with argon plasma coagulation (APC). Mucosa biopsies were negative for H. pylori infection. For evaluation of the AVMs, Small bowel enteroscopy was done on February 2016 which showed mucosal changes in the jejunum and biopsy revealed small bowel mucosa with dilated lymphatics. EGD was repeated for polypectomy in February 2016 which showed enlarged gastric folds and were ligated. There was normal duodenal bulb and mucosal resection was successfully performed. The biopsy was consistent with fundic gland polyps.
He was referred to the gastroenterology clinic after eighteen months for abnormal abdominal computerized tomography findings of gastric wall thickening which he had for abdominal pain. This epigastric pain was gradual in onset, severe in intensity, radiating to right upper quadrant, and progressively worsened. To rule out gastric malignancy, EGD and EUS were done. EGD in September 2017 showed giant gastric folds, much larger than seen on the prior endoscopies. This is shown in . The biopsied enlarged gastric folds revealed gastric fundic mucosa with foveolar hyperplasia, dilated fundic glands and chronic gastritis which is shown in . Another gastric fundus nodule biopsy revealed gastric fundic mucosa with foveolar hyperplasia and mild chronic inflammation.
With the background of thickened gastric folds and episodes of recurrent pancreatitis, the patient underwent upper endoscopy ultrasound for the evaluation of the pancreas as well as the stomach on March 2018 which showed wall thickening in the body of the stomach. The thickening appeared to be primarily within the submucosa (Layer 3) of the stomach (). There was no sign of significant pathology in the common bile duct, gall bladder, and the main pancreatic duct.
Stomach biopsy results were consistent with Menetrier disease. Patient was followed in the clinic and explained the risks of adenocarcinoma associated with this disease. Our patient has worsening of the disease in one and half years.


A 53-year-old Chinese female with no history of Kawasaki disease or chest trauma was admitted to our hospital because of decreased exercise tolerance and lower extremity edema for 3 months. Physical examination found mild pretibial edema and no heart murmur was heard. Laboratory test revealed elevated eosinophil cell number (0.99 × 109/L) and percentage (14.4%). Tumor markers were negative for malignancy. The transthoracic echocardiography (TTE) showed a cystic mass (3.5 × 2.7 cm) with hyperechoic external capsule locating below the tricuspid annulus (Fig. a). Moreover, moderate tricuspid regurgitation was detected, which was mainly attributed to the malcoaptation of leaflets. No ventricular wall akinesia or ST segment change was found on TTE or electrocardiogram. Further assessment with coronary computed tomographic angiography (CTA) showed an irregular mass with internal contrast enhancement locating below the tricuspid annulus (Fig. b&c). A feeding vessel originating from RCA was found to supply the mass. No significant coronary stenosis of left anterior descending artery or left circumflex artery was found (Fig. d). Considering the mass featuring right-sided location, presence of feeding vessel, irregular shape and internal enhancement, diagnosis of right ventricular malignant tumor was initially made.
Tumor resection was performed via median sternotomy and the cardiopulmonary bypass was established with aortic and bicaval cannulation. After right atriotomy, a mass with about 3.5 × 2.5 cm in size was detected locating at the right ventricle below the tricuspid annulus (Fig. e). In addition, the posterior leaflet of the tricuspid valve was extensively affected by the mass. The mass was fully exposed until the posterior leaflet was detached from annulus and was confirmed as a thrombosed right coronary artery aneurysm with collagen-like degenerative fibrous coating. The aneurysm was resected as much as possible and the stump of the feeding vessel was closed by continuous suture with 5–0 prolene. The posterior leaflet was reattached to the annulus by continuous suture with 5–0 prolene (Fig. f). Due to absence of evidence of myocardial ischemia either on TTE or electrocardiogram, no bypass surgery was performed.
The histologic and immunohistochemistry study confirmed the diagnosis of coronary artery aneurysm with CD34, CD31 positive and D2–40 negative. The patient had an uneventful recovery and was discharged home on the 7th postoperative day. However, on follow-up three months later, TTE showed a dilated RCA with the proximal diameter of 6.1 mm and a fistula between RCA and right ventricle with a diameter of 2.3 mm. No evidence of remaining or recurrence of aneurysm was detected. The patient had no complaint and no evidence of myocardial ischemia was detected, so we decided to follow up the patient closely. Further treatment will be taken if evidences of myocardial ischemia occur in the future.


A 64-year-old man was hospitalized for “recurrent fever and cough with brown sputum for 7 days.” No tuberculosis or asthma was reported in the family history. The patient had a 20-year smoking history with 30 cigarettes per day. Oxygen saturation was 93% under a 5 L/min oxygen flow rate. Auscultation showed a widespread audible expiratory wheeze on both upper lungs and moist rales on both lower lungs. Breathing sound was low and the patient had the symptom of expiratory dyspnea. Initial laboratory findings showed serum IgE levels of 128.0 U/mL (ULN 100.0) and positive A. fumigatus IgG. Sputum culture was performed and A. fumigatus grew. Multiple plaque shadows were detected on both lungs by a chest radiograph. Lung inflammation with central bronchiectasis was identified by high-resolution computed tomography (HRCT) (Fig. A).
The patient was diagnosed with ABPA and had received regular treatment comprising intravenous methylprednisolone (40 mg/d) and voriconazole (200 mg every 12 hours). After 5 days of treatment, symptoms were significantly improved and the methylprednisolone was administered orally. The HRCT was reviewed and showed that infiltration had been significantly improved (Fig. B). Eventually, the patient was discharged with oral steroids and voriconazole (200 mg every 12 hours) treatment.
The patient was re-hospitalized for hemoptysis and dyspnea 2 months after discharge. Auscultation found a little moist rale at the base of both lungs. The rest of the physical examination was normal. The result of the D-dimer test was 5630 μg/L (ULN 500 μg/L). HRCT of the chest showed pulmonary inflammation, which indicated an improvement compared with the previous examination (Fig. A). However, chest angiography revealed filling defects in the main pulmonary artery and both branches of the pulmonary artery, indicating the occurrence of PTE (Fig. B).
The patient was then diagnosed with PTE and received treatment of low molecular heparin (0.4 mL every 12 hours for 7 days) and rivaroxaban (15 mg twice a day). Symptoms gradually improved and the patient was discharged with continued anticoagulant treatment. Currently (over 1 years after diagnosis) the patient is in remission of the disease, and no further thromboembolic complications have been observed.


A 68-year-old man presented with a mass in his back. He had a history of atrial fibrillation, diabetes mellitus, and a brain tumor which was completely resected. He was admitted to hospital in May 2015 because of the increasing size of the back mass. On radiograph, the mass was suspected to be STS, and he was referred to our hospital in August 2015. We palpated the elastic soft tumor in his back. X-ray showed no abnormality in the bone. Magnetic resonance imaging (MRI) showed a tumorous mass of 54 × 43 × 30 mm in the latissimus muscle of the back which exhibited low intensity on T1-weighted and high intensity on T2-weighted images (Fig. ). On computed tomography (CT) of the thorax and abdomen and a head MRI, there was no distant metastasis. After an open biopsy, we diagnosed the mass as DDLPS. Because of the patient's history of heart disease, we avoided the use of doxorubicin, a standard chemotherapeutic agent for DDLPS. Instead, gemcitabine+docetaxel (GD) combination therapy was administered as the first-line treatment. After 2 cycles of GD treatment, however, MRI showed that the mass was increasing in size. Furthermore, abdominal CT and head MRI revealed lymph node and brain metastases, respectively (Fig. ). To reduce the symptoms caused by the local disease, we performed a wide resection of the back mass. Pathologic diagnosis of the resected specimen was also DDLPS with positive immunohistochemical staining for murine double minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) (Fig. ).
After the surgery, the chemotherapy regimen was changed to pazopanib at a dose of 600 mg/d, and Gamma Knife treatment was performed for the brain metastasis. Pazopanib induced an elevation in serum transaminases (Grade 2); thus, we reduced the dose of pazopanib to 200 mg/d. However, bone metastases were found in the left tibia and right femur (Fig. ). Subsequently, we performed wide resection of the metastatic tumors in the left tibia and right femur and reconstruction with liquid nitrogen-treated bone. We switched the chemotherapy regimen to eribulin; however, we found a further recurrence of brain metastasis and disease progression after 6 cycles of eribulin treatment (Fig. ). We performed CyberKnife treatment for the brain metastasis. Finally, we used trabectedin (1.2 mg/m2) as the fourth-line of chemotherapy with central venous catheter. Firstly, we administered trabectedin (0.89 mg/m2) because we concerned about adverse events. Overall, 11 cycles of trabectedin were performed with decreasing dosages due to adverse events, including hypophagia and elevation of transaminases. The frequency of trabectedin, dosage alteration, and changes in serum transaminases were shown in Fig. . Stable disease (SD) was achieved for 18 months during trabectedin treatment until termination of the treatment as a result of a new metastasis in the lung (Fig. ). The patient was then provided with best supportive care. The patient has provided informed consent for publication of the case.


Case 1: An 88-year-old female with multiple medical problems, including recurrent GI bleeding, COPD, glaucoma, cachexia, and osteoarthritis, was abruptly left without a caregiver when her son unexpectedly died. She experienced worsening mental status and mood in the last year of her life, including an episode of delirium. Prior to establishing the GRT, this type of patient would have received a referral to the geriatrics clinic for comprehensive geriatric assessment. Instead, the primary care practice was able to manage the patient’s complex needs without a geriatrics referral. The GRT’s enhanced expertise in evaluation of her cognitive disturbance, enhanced teamwork processes, and awareness of community resources led to a timely referral to adult protective services and establishment of a new healthcare power of attorney (HCPOA) to replace her son who had recently died. Soon after, the patient experienced a serious health crisis. Her HCPOA was able to advocate effectively due to the work that had been done, and the patient was transferred to an inpatient hospice, where she died while receiving comfort care, in accordance with her wishes.


A 43-year-old female patient suffered from head trauma resulting from a car accident. She was seated in the passenger seat of a minivan was stopped at a signal light behind a truck, and another truck collided with her car from behind. The minivan then repeatedly collided with the trucks in front and behind. During that collision, her head repeatedly struck the minivan seat resulting in whiplash injuries. The patient reported that she did not experience loss of consciousness or post-traumatic amnesia. The patient’s initial Glasgow Coma Scale score was 15. After the head trauma, she felt pain in both legs, mild motor weakness in all four extremities, and memory impairment. Although she visited several hospitals to determine the cause of her pain, she was unable to obtain a precise diagnosis for her pain because the conventional brain and whole spine MRIs did not show any abnormality. At eight years from onset, she was diagnosed as CRPS type 1 with the clinical features of hyperalgesia and mild edema and motor weakness of both legs; moreover, abnormality was not detected on plain radiography for hand and leg, three-phase bone scan, and thermography. Nine years after the head trauma, she visited the rehabilitation department of our university hospital complaining of pain in the right arm and both legs. The characteristics and severity of the pain were as follows: Constant tingling and burning sensation with allodynia and hyperalgesia (visual analog scale score: Right arm and leg, 9; left leg, 7) []. She also exhibited mild weakness of the four extremities (4−/4), mild edema of both legs, and memory impairment. However, trophic changes of the arm and legs, including skin and nails, were not observed. In addition, she mentioned that she did not experience the distal edemas on the right arm and both legs, which could observe during the acute stage of CRPS []. Electromyography for all extremities and trunk failed to detect any abnormality (A). The patient provided signed, informed consent, and the study protocol was approved by the institutional review board of our university hospital (YUMC 2019-06-032, approved on 21 June 2019).


An 82-year-old man (height, 167 cm; weight, 58 kg) was scheduled for mitral valve repair due to exacerbated heart failure symptoms. His past medical history included aortic valvular stenosis with post-aortic mechanical valve replacement and atrial fibrillation treated with oral warfarin. The patient had no upper gastrointestinal symptoms or related medical history. Written informed consent was obtained from the patient.
Four days before mitral repair surgery, warfarin was replaced with intravenous heparin (10,000 units/day), which was administered until 6 h preoperatively. Under general anesthesia, a TEE probe was inserted, without resistance, to evaluate perioperative cardiac function and residual regurgitation using mid-esophageal and trans-gastric views, as appropriate. The inferior vena cava (IVC) could not be visualized in trans-gastric view, despite several manipulations of the probe with the venous cannula placed into the IVC. After cardiopulmonary bypass (CPB), an unexpectedly large echo-free space in the stomach was detected on TEE. The mitral valve repair was successfully undergone, the TEE probe was removed, and a nasogastric tube was inserted. Surprisingly, 700 mL of dark brown blood was aspirated from the nasogastric tube. We suspected upper gastrointestinal injury, but the patient was transferred to the intensive care unit, as the blood was dark brown and the bleeding appeared to have stopped.
Approximately 6 h postoperatively, another 100 mL of fresh blood was aspirated from the nasogastric tube. Thus, we immediately performed an esophagogastroduodenoscopy (EGD) examination and computed tomography (CT) scan. The EGD examination showed a mucosal laceration spanning from the esophagogastric junction to the gastric body, with a perforation in the center of the laceration (Fig. ). On this occasion, a clip was placed to stop the hemorrhage from the injured area, but the perforation was too large to close with the clip under EGD. The CT scan showed localized free air in the abdominal cavity, which helped establish a definite diagnosis of gastric perforation (Fig. ). Initially, surgical treatment was considered for the repair of the perforation. However, we selected conservative management to avoid increasing operative stress, to minimize the need for total gastrectomy (including the lower esophagus), and to reduce the risk of infection spread. Fortunately, spontaneous closure was expected, as there were no abdominal symptoms and the perforation site was covered with the lesser omentum and inferior surface of the liver.
Nasogastric tube aspiration was continued postoperatively, and the patient was administered total parenteral nutrition. On the day of surgery, the patient received hemostatic therapy with carbazochrome sodium sulfonate hydrate (100 mg) and tranexamic acid (0.5 g), as well as routine antibiotics including sulbactam sodium (0.5 g) and ampicillin sodium (1.0 g). Tazobactam sodium (0.5 g) and piperacillin sodium (4.0 g) were initiated after diagnosis of gastric perforation and continued until the perforation site had healed. Intravenous omeprazole sodium (20 mg) was used as a proton pump inhibitor until oral intake. On postoperative day 1, hemostatic therapy included carbazochrome sodium sulfonate hydrate (200 mg), tranexamic acid (1.5 g), and menatetrenone (20 mg). As there were no persistent abdominal symptoms and the nasogastric aspirate contained no blood, the tracheal tube was removed on postoperative day 3. A follow-up CT scan revealed a reduction in the echo-free space in the abdominal cavity, and upper gastrointestinal examination revealed no leaking of the contrast medium into the abdominal cavity. Thus, the patient began enteral nutrition on postoperative day 10 and oral intake on postoperative day 12. To prevent rebleeding, anticoagulation was restarted only after enteral nutrition was established (warfarin, 3 mg). To protect the gastric mucosa, oral esomeprazole (20 mg) and ecabet sodium (2000 mg) were started upon establishment of oral intake and continued until after discharge. A follow-up EGD examination on postoperative day 23 revealed that the perforation site had healed, with scarring (Fig. ). The patient recovered without any complications and was discharged from the hospital on postoperative day 24.