A 59-year-old man was found to have liver cirrhosis with multiple nodules in the
liver during a physical examination at the Fourth People’s Hospital of
Lianyungang City. He had no other symptoms. CT examination of the liver showed a
high possibility of small liver cancer in the right lobe of the liver, cirrhosis
of the liver, and multiple cirrhotic nodules in the liver. For further
treatment, he went to the Affiliated Hospital of Tsinghua University, where he
underwent a liver transplantation and received oral anti-rejection treatment
with tacrolimus (1.5 mg every 12 hours) on the first day after surgery. His
blood concentration of tacrolimus was 1.9 ng/mL, which was within the reference
range for postoperative treatment. On the fourth day after starting the
tacrolimus, the patient suddenly shouted loudly at noon. He immediately closed
his teeth, and his upper and lower limbs bent and convulsed. EEG examination
revealed abnormalities () in the form of a bilateral explosive high-amplitude spinous wave
rhythm that quickly converted to high-amplitude three-times/second synthesis of
multi-spinous slow waves, with a total duration of 2 to 3 seconds. The patient’s
symptoms were relieved after intravenous diazepam and intramuscular
phenobarbital injections. Emergency brain CT showed posterior edematous change
as indicated by disappearance of the sulcus (), and his symptoms were relieved
after the second intravenous injection of diazepam. Meanwhile, he began oral
administration of levomethylacetanilide tablets. Arterial blood analysis
revealed no other abnormalities. On day 2, CT re-examination exhibited no
shadows of abnormal density in the brain parenchyma. Oral administration of
tacrolimus (1.5 mg every 12 hours) was continued for about 2 weeks, and the
plasma concentration was 8.81 ng/mL on re-examination. The patient had developed
no recurrence of epilepsy after 8 months of follow-up.


A 19-year-old woman presented to the emergency room (ER) with abdominal pain. Two years previously she had onset of right upper quadrant abdominal pain and fatigue, and a few months later she also began complaining of chest discomfort and intermittent dyspnea. On the day of her admission, an abdominal ultrasound (US) examination was suspicious for a large epigastric cyst and she was therefore referred to the ER.
The patient had an unremarkable personal and familial medical history. She resided in a sheepherding Bedouin settlement where stray dogs commonly foraged.
On admission, her vital signs were temperature 38.6°C, heart rate 103 beats per minute, blood pressure 114/68 mm Hg, and oxygen saturation 98% in room air. On physical examination decreased breath sounds over the right lung and right upper quadrant abdominal tenderness were found.
Laboratory studies showed leukocytosis with neutrophilic predominance and an elevated CRP level.
Double-contrast CT (computerized tomography) revealed a huge (15.0X14.5X16.0 cm) cyst in the right hemithorax encased in a multi-layered wall (). The cyst was causing almost total collapse of the right lung, significant leftward mediastinal shift, and compression of the Inferior vena cava, the right diaphragm, the right kidney and the liver. A medium-sized partially loculated right pleural effusion was also observed.
Serologic studies were positive for Echinococcus granulosus, confirming the diagnosis of a hydatid cyst.
The patient was treated for 6 days with albendazole and was then discharged in stable hemodynamic and respiratory condition, with the intention to perform surgery as soon as possible.
Following her discharge, a fulminant deterioration was noticed, and she was urgently re-admitted. On examination, she was tachycardic, tachypneic and severely dyspneic. Her oxygen saturation had decreased to 93% in room air and there was a total absence of breath sounds over her right lung.
The patient was taken urgently to the operating room. After intravenous administration of hydrocortisone, induction of anesthesia and intubation were performed. Intraoperative bronchoscopy showed findings of external compression and total occlusion of the right upper lobe bronchus, a bronchial blocker was inserted into the right main bronchus prior to withdrawal of the bronchoscope.
While induction of anesthesia and preparation for surgery was made, a US-guided drainage ( and ) of 1.5 L of straw-colored pleural fluid and 0.5 L of straw-colored intra-cystic fluid was performed, following which 20% hypertonic saline was injected into the cyst cavity and re-drained after 20 mins. Exploratory thoracoscopy revealed a large encapsulated mass protruding from the lung parenchyma with extensive adhesions. These findings dictated transition to posterolateral thoracotomy which enabled extraction of the entire internal capsule () and decapitation of the external capsule. The deep air leaks from the parenchyma were closed with sutures and biological glue. Repeat bronchoscopy performed after deflation of the bronchial blocker demonstrated normal airway patency. The right lung was almost fully expanded, and on ventilation of both lungs, no air leaks were demonstrated. However, a few moments later, the patient suddenly manifested signs of pulmonary edema including desaturation, hypotension and tachycardia. Foamy fluid was suctioned through the endotracheal tube. Arterial blood gas measurements showed respiratory acidosis with hypoxemia. Two drains were positioned in the pleural cavity, the incision was urgently closed, and the patient was immediately placed in the supine position. Respiratory stabilization was achieved by pressure-controlled mechanical ventilation and administration of IV furosemide and morphine and inhaled nitric oxide. Within 30 minutes, the patient’s condition improved and she was transferred to the Intensive care unit where she was successfully weaned from mechanical ventilation within a few hours. Her subsequent hospital course was uneventful, and she was discharged on the 5th postoperative day.


The patient, a 56-year-old male, was a known case of carcinoma of post cricoid region (hypopharynx). He had received 70 cycles of chemotherapy and 35 cycles of radiotherapy. He underwent total laryngopharyngoesophagectomy with total thyroidectomy with central compartment clearance with a tracheostomy with gastric pull-up with pectoralis major myocutaneous flap with feeding jejunostomy under general anesthesia. Tracheal rent occurred during surgical dissection, which was recognized and repaired. The patient was shifted to ICU with armored endotracheal tube (ETT) placed through a tracheostomy. On postoperative day (POD) 1, bronchoscopic-guided long-flanged tracheostomy tube (TT) was placed, and cuff was kept deflated.
On POD5, rent measuring 1 cm × 1 cm in posterior tracheal wall approximately 1.5–2 cm above carina was observed on check bronchoscopy. On POD6, the patient developed subcutaneous emphysema due to tracheopleural fistula (). He was posted for right thoracotomy with latissimus dorsi flap the next day under general anesthesia. During the procedure, the ventilation was managed with a left endobronchial tube via tracheostomy. After surgery, neuromuscular blockade was reversed, spontaneous ventilation was resumed, and the endobronchial tube was removed, and oxygen via tracheal mask was provided. The patient was shifted to surgical ICU. He developed rapid shallow breathing, desaturation, and drowsiness. Increasing oxygen flow did not improve his condition. Under bronchoscopic guidance, 5.0 mm ID, cuffed ETT was placed in the right mainstem bronchus through a tracheostomy. The cuff of the tube was placed below the rent and was intended to keep the right upper lobe ventilated. Another cuffed ETT 5.0 mm ID was placed in left mainstem bronchus under bronchoscopic guidance. Tubes were fixed at 14 cm (left) and 13 cm (right) at the level of stoma and connected via a DLT connector to a ventilator (pressure control mode) ( to ). Oxygen saturation improved thereafter.
Ventilator support was weaned off later to the spontaneous mode by the next day. Patient received intravenous morphine infusion. On POD1, right upper lobe collapse was noted on chest X-ray (). Bronchoscopy was done to recheck tube position along with pulmonary toileting.
On POD2 of thoracotomy, the ETTs were removed under bronchoscopic guidance and were replaced by 7.0 mm ID long and adjustable flange TT. The tip of the tube was kept just above the carina, and cuff was kept deflated. Patient was maintained on high-flow oxygen (AIRVO2™ Fisher and Paykel) via tracheostomy, which was weaned off after 5 days. Later, patient was managed conservatively by chest physiotherapy, antibiotics, regular bronchoscopic pulmonary toileting, nebulizations, and appropriate antimicrobial therapy. Tracheal rent was seen healing, and granulation tissue was present on a repeat bronchoscopy on POD9 following thoracotomy. Patient was discharged in stable condition from ICU on POD23 and hospital on POD30 of thoracotomy.


A 52-year-old male was admitted to Seoul Paik Hospital due to aggravated dyspnea and exertional chest pain that had developed a week ago. He was diagnosed with ES due to an unrepaired large atrial septal defect (ASD) at 8 years prior and had been taking sildenafil and macitentan (endothelin receptor antagonist) for pulmonary hypertension. Except for smoking, he had no other traditional risk factors for atherosclerosis. Electrocardiography showed right ventricular (RV) hypertrophy with strain pattern (). Chest X-ray showed cardiomegaly and an enlarged pulmonary trunk. Transthoracic echocardiography revealed RV hypertrophy and enlargement compressing the left ventricle, marginally depressed RV function, dilated pulmonary arteries, markedly elevated estimated RV systolic pressure of about 158 mmHg, which was derived from tricuspid regurgitation velocity, and a large ASD secundum (2.37 cm) (). Routine examination revealed blood pressure of 130/80 mmHg, heart rate of 92/min, and peripheral oxygen saturation of 89% at room air. Laboratory tests revealed hemoglobin of 16.7 g/dL, white blood cell of 6,220 cells/mm3, platelet of 161×103/mm3, blood urea nitrogen of 16 mg/dL, creatinine of 1.03 mg/dL, and pro-brain natriuretic peptide of 210.1 pg/mL.
Coronary angiography via the right radial artery indicated a nonocclusive left coronary artery, but found significant stenosis of the mid to distal right coronary artery (RCA) (). With use of 8,000 units of heparin, we tried to engage the 6-F Amplatz left guiding catheter into RCA, but the catheter was engaged deeply. In the cine after engagement, we found dissection of the proximal RCA with a collapsed lumen and diminished coronary flow (Thrombolysis In Myocardial Infarction [TIMI] grade 0 flow) (). Inferior ST-segments were elevated on electrocardiography and the patient complained of ongoing chest pain with agitation. His blood pressure was dropped to 72/51 mmHg at first and marginally maintained after rapid administration of 600 ml crystalloids and initiation of inotropes (dopamine 15 μg/min/kg and norepinephrine 0.1 μg/min/kg). We decided to insert a veno-arterial extracorporeal membrane oxygenation (ECMO) immediately prior to PCI. A 17-F arterial and a 21-F venous Bio-Medicus cannula (Medtronic, Minneapolis, MN, USA) were percutaneously inserted into the right femoral vessels and venous cannula was positioned at right atrium under fluoroscopic guidance. A PLS system circuit (Maquet, Rastatt, Germany) and ROTAFLOW console (Maquet) were used. After initiation of ECMO, his blood pressure was recovered to 100/60 mmHg. We changed the guiding catheter to a 6-F Judkins right 4.0 catheter and passed the guidewire successfully through the true lumen (). We performed direct stenting with two drug eluting stents (each 3.5×18 mm; Resolute Onyx, Medtronic) at the proximal section and ostium of the RCA to cover the dissection, and coronary blood flow was restored (TIMI grade 3 flow) ( and ). A significant lesion of the mid to distal RCA was treated with another drug eluting stent (3.0×38 mm; Resolute Onyx, Medtronic) (). Final angiography revealed no residual stenosis or dissection. The patient was monitored carefully in the intensive care unit after PCI. We initially started with an ECMO flow rate of 4.0 L/min, considering patient body weight. We monitored arterial blood gas analysis via both right radial artery and ECMO arterial cannula. Difference of PaO2 between upper and lower extremities (right radial artery: PaO2, 68 mmHg; saturation, 95% vs. ECMO arterial cannula: PaO2, 426.5 mmHg; saturation, 100%) was noted, which means left ventricle systolic function was sufficient to supply right carotid artery against retrograde flow from ECMO. However, he kept complaining of dizziness and his blood pressure decreased to 82/66 mmHg when a high ECMO flow rate was applied. The bedside echocardiography revealed that left ventricular systolic function was preserved, but preload seemed to be insufficient. The hemoglobin level decreased from 16.7 g/dL to 12.2 g/dL after ECMO apply. Two pints of packed red blood cells were transfused and the hemoglobin level recovered to 14.2 g/dL. Fluid resuscitation with crystalloids via both intravenous route and ECMO circuit was also effective to raise the patient’s blood pressure. With volume replacement, we reduced the ECMO flow rapidly and maintained the flow as low as possible (approximately 1.5 L/min). Intravenous heparin was infused during ECMO support with targeted activated partial thromboplastin time of 60–80 seconds and activated clotting time of 200–220 seconds. His vital signs remained stable for 7 hours despite of minimal ECMO support. ECMO was successfully removed using a percutaneous closure device (Perclose; Abbott Vascular, Abbott Park, IL, USA) without any vascular complications. The patient was discharged with improved clinical symptoms and was stable for 3 months of follow-up in the outpatient clinic.


A 60-year-old Middle Eastern male, with no significant medical history, presented to the emergency room complaining of acute nonradiating epigastric pain started about 6–7 h earlier. There was no vomiting, nausea, or anorexia. There was no jaundice, pallor, or history of loss of weight. The temperature was 37.5°C. Abdominal examination was negative for hepatomegaly, splenomegaly, abdominal tenderness, or rebounding pain. There were no signs of lymphadenopathy. A clinical diagnosis of acute gastritis was made, and the patient was given proton pump inhibitor and discharged.
Two days later, the pain started to radiate toward the back, and the patient started to suffer from severe thoracic back pain. Four days later, the pain started to radiate toward both lower limbs with the subtle beginning of the weakness of the lower limbs and progressed within a few hours later to inability to walk, with intact neurological function of the upper extremities.
He denied any history of trauma, recent history of viral illness, or illicit drug use. On neurological examination, the patient was found to have bilateral numbness and motor impairment in the lower limbs with muscle strength of 2/5. Hyperactive patellar and Achilles reflexes were observed. Pain and light touch sensation were objectively normal; there was no bowel or urinary incontinence. The straight leg raise test was negative bilaterally.
Magnetic resonance imaging revealed a space-occupying lesion in the spinal canal at the T6/T7 level without detection of any lesions elsewhere []. Because of suspected metastasized tumor, further investigations were undertaken. Computed tomography (CT) scan of the neck, thoracic, abdomen, and pelvis were performed and did not reveal any masses, enlarged visceral organs, or lymph nodes. A bone marrow biopsy presented no marrow involvement.
In view of the rapid progression of the neurological deficit, the patient emergently underwent a neurosurgical decompression through partial laminectomy with total resection of the extradural mass.
The pathohistological examination of the biopsy revealed diffuse malignant infiltration of large atypical lymphoid cells, large vesicular nuclei, prominent nucleoli, and coarse chromatin.
Numerous mitotic cells were also present []. Immune stains were positive for CD20 and leukocyte common antigen [] and negative for CD3 [] and CD30 []. These findings are consistent with diffuse large B-cell lymphoma. After the diagnosis was made, the patient underwent chemotherapy.
Day after day, the patient gradually regained the strength in all muscle groups and was able to walk 1 month after surgery. Furthermore, the patient has been followed up for over 2 years without any signs of recurrence both clinically and radiographically [].


A 40-year-old, 166-cm-tall premenopausal woman weighing 57 kg visited outpatient clinic with gradually increasing left wrist pain for the past months without a history of trauma. Seven years prior to the visit, she had been treated with lateral impingement of the ankle secondary to a calcaneal deformity for a similar episode of worsening right heel pain. At that time, plain radiography and computed tomography of the right ankle revealed significantly reduced bone density, particularly affecting the calcaneus, suggesting osteoporosis (). A whole-body bone scan revealed persistent high tracer uptake at the right calcaneus (). Calcaneal lateral wall ostectomy and bone biopsy were performed and multifocal lesions with both osteoblastic areas and osteoclastic bone resorption were observed (). No conclusive diagnosis was established, and the patient was followed-up regularly for persistent right heel pain. Three years after surgery, she presented with a trivial ankle sprain and was diagnosed with an avulsion fracture of the posterior tuberosity of the right calcaneus (). She received conservative treatment, and a short leg cast was applied until bone union and the patient was referred to the endocrinology unit for further workup.
Initial laboratory investigations revealed all biochemical, endocrine, rheumatology, and hematology evaluations were within normal, but serum osteocalcin, C-terminal telopeptide of type I collagen (CTX), and bone alkaline phosphatase (ALP) levels were elevated (). A bone mineral density (BMD) scan showed Z-scores of −1.3, −2.2, and −2.7, in her Lumbar 1 to 4 spines, left (Lt) femur and right (Rt) femur, respectively. The patient started ibandronate injections every 3 months on an impression of secondary osteoporosis without cause. After 1-year of treatment, her laboratory test values had returned to the normal range (), and BMD scans revealed that her Z-scores had improved to −0.5, −1.9, and −2.5, in her Lumbar 1 to 4 spines, Lt femur and Rt femur, respectively. Her heel pain was also subsided.
Based on previous history and a positive response to bisphosphonate therapy, PDB was sustained so bone biopsy was performed on the radial aspect of distal radius which radiograph showed osteopenia (). Histopathological findings revealed irregular broad trabeculae with disorganized cement lines and a patchy mosaic pattern in addition to a few distinct areas of osteoclastic bone resorption and prominent osteoblastic rimming (). During the further evaluation, old 2nd lumbar wedge compression fracture was noticed so follow up whole body bone scan was taken 7 years after initial scan. There was still persistent uptake in right calcaneus () and plain X-ray presented bony deformity (). She is still being treated with bisphosphonate with regular follow-up.


A 3-year-old boy, born of a non-consanguineous marriage, resident of Jaunpur presented with recurrent episodes of cough and cold with hemoptysis for 2 years. Each episode was associated with breathlessness lasting for 1 week, some requiring hospitalizations. There was no history of fever, Kochs or Koch's contact, edema, or palpitations. The child was given nebulization in each admission but showed no response. Antenatal scans were normal. He was a full-term normal delivery with a birth weight of 2.4 kg. The child did not cry immediately after birth with no other neonatal problems and had normal development. On admission, the child had tachycardia, tachypnea, pallor, and Grade 1 clubbing. Respiratory system examination revealed decreased air entry with crepitations on the left side. He had cardiomegaly with a grade 3/6 ejection systolic murmur in the left second intercostal space with a normal second heart and a tender hepatomegaly of 4 cm. Hemoglobin was 7 g/dl with normal leukocyte, platelet count, and coagulation. Tuberculosis workup was negative.
Chest radiograph revealed small left hemithorax with cardiothoracic ratio of 0.60. Transthoracic Doppler echocardiographic examination revealed an underfilled left atrium with dilated right atrium. The left pulmonary vein could not be visualized. The interatrial and interventricular septum was normal. The pulmonary artery was dilated slightly without the evidence of pulmonary hypertension. A computed tomography (CT) of the chest with pulmonary angiography showed that the left and right lower pulmonary veins were seen for a distance of 4 mm and 3 mm from the left atrial orifice, respectively. The remaining part of the veins was atretic. The right-sided pulmonary veins were dilated and drained into the left atrium. There was no anomalous venous drainage. The left pulmonary artery was hypoplastic with severe narrowing of lobar branches. The right pulmonary artery was normal. There was also a loss of lung volume on the left side with dysplastic changes. The diagnosis of left PVA was confirmed [Figures -]. Along with blood transfusion, diuretics and digoxin was started, and the patient was posted for left pneumonectomy.


A 18-year-old girl presented to the ear, nose, throat, (ENT) Head-and-neck surgery outpatient department (OPD) of our institute with complaints of bleeding from the right nostril, on and off along with swelling of the upper lip for the past 6 months. Her disease started with lateral nasal swelling which was gradual in onset for that she was treated with right lateral rhinotomy with excision of the nasal mass. On histopathology, her lesions showed chronic granulomatous pattern with the possibility of sarcoidosis. The lesion recurred again with similar complaints and provisional diagnosis of infective subcutaneous granulomatous lesion/tuberculosis/sarcoidosis/leprosy/rhinoscleroma was made. After a review of the same histopathology of slides, the impression of nonnecrotizing granulomatous inflammation with possible tuberculous etiology was made. She was put on antitubercular therapy but within 2 months, she developed adverse drug reactions. A second-time biopsy was performed from the right ala of the nose and from right middle meatus, and the histopathology findings were the same as the previous. As there was a significant regression of the lesion, the histopathology slides of the second biopsy were again reviewed and revealed the presence of eosinophil, rich mycotic granulomatous inflammation. Fungal stain revealed thick, nonseptate branching hyphal forms of zygomycosis species. She was advised intravenous amphotericin B and posaconazole which was not taken by her due to financial constraints.
After 4 months she visited the ENT, Head-and-Neck Surgery OPD of our institute. On local examination, swelling over the upper lip and bleeding from the nasal floor was observed. No abnormality detected on examination of the neck. Nasal endoscopy was done which showed septal deviation with synechiae present between the septum and left side of the nose. On the right-side crusts were present in the nasal cavity with mucoid discharge. Mass or polyp was absent. Swelling was also present on both anterior and posterior aspect extending up to the ventricular border crossing the midline. It was firm, nontender and nonfluctuant [] with the congested posterior pharyngeal wall. The mucosal biopsy was taken from the upper lip with all aseptic precautions and sent for KOH mount, fungal culture.
KOH showed ribbon-like broad hyaline sparsely septate hyphae []. Growth on sabouraud dextrose agar at 27°C: starts appearing after 48 h and after 3 days of incubation white colored, waxy to powdery with reverse was pale with satellite colonies at periphery seen []. Lacto phenol cotton blue stain of colonies revealed broad sparsely sepatate hyphae, indistinguishable sporangiophore and hyphae, sporangioles surface was smooth, covered with papilla []. It is identified as C. coronatus.[]
After culture confirmation patient was treated with itraconazole and saturated solution of potassium iodide (KI) after doing the hormonal level. As her hormonal levels disturbed, she was shifted to amphotericin B. The patient responded well to the treatment [].
Just after the interval of 2 weeks, the second case which is discussed below came across.


A 40-year-old female patient reported to the department of oral medicine and radiology with a swelling on the right side of the mandible for 2 months. The lesion grew rapidly to reach the present size with no other associated symptoms. The patient's medical and family histories were noncontributory. Extraoral examination revealed a well-defined oval-shaped swelling of size approximately 4 cm × 3 cm on the right side of the face. The skin over the swelling was stretched and surrounding tissues appeared normal. The swelling was firm in consistency, nonfluctuant, nonreducible with no evidence of paresthesia, or weakness on examination of both sensory and motor branches of mandibular nerve []. The intraoral examination showed obliteration of the right buccal vestibule with no expansion of cortical plates.
Contrast-enhanced computed tomogram showed a large heterogeneous enhancing space-occupying lesion of the soft-tissue density measuring 3.5 × 6.2 × 2.5 in relation to the right gingivobuccal sulcus. The lesion abuts right submandibular gland and displaced it inferiorly suggesting possible neoplastic etiology []. For confirmation, an incisional biopsy was done which revealed streaming fascicles of atypical spindle-shaped cells interspersed with hypocellular and myxoid regions. The atypical spindled lesional cells demonstrate wavy or comma-shaped hyperchromatic nuclei with indistinct cytoplasm. Cellular and nuclear pleomorphism is seen. Sections were of poorly differentiated malignant tumors with extensive involvement of skeletal muscle [Figures and ]. IHC was advised to rule out the possibility of fibrosarcoma, leiomyosarcoma, and synovial sarcoma using Vimentin, S-100, leukocyte common antigen, and all these markers showed strong immunopositivity for tumor cells, hence, favoring the diagnosis of MPNST [Figures and ]. Based on information obtained by clinical, histopathological, and immunohistochemical investigations, a final diagnosis of MPNST was made.
MPNST has a rare possibility of metastasis, so to rule, a chest radiograph was advised, which was reported to be normal. The patient underwent a wide local excision under general anesthesia postoperative histopathological findings and IHC confirmed the diagnosis. Subsequently, the patient was treated with radiotherapy, that is, 60 Gy in 10 fractions. She was subsequently kept under follow-up. No evidence of recurrence was noted in the 6-month follow-up period [].


A 65-year-old diabetic male presented with a 1-week history of gradual painless decrease in vision in his right eye. He has been previously diagnosed with moderate non-proliferative diabetic retinopathy (NPDR) in both eyes. His corrected distance visual acuity (CDVA) was 20/200 in the right eye and 20/20 in the left eye on Snellen’s chart; anterior segment evaluation and intraocular pressure were unremarkable in both eyes. His retinal condition was stable bilaterally with moderate NPDR. The foveal reflex was altered in the right eye with the presence of cystoid macular oedema and surrounding retinal greying; the foveal reflex was normal in the left eye. An optical coherence tomography (SD-OCT, Heidelberg, Spectralis) showed the presence of a centre involving cyst that extended till the ILM anteriorly and stopped short of the ELM posteriorly (). Inner retinal thinning was also noted and a provisional diagnosis of paracentral acute middle maculopathy (PAMM) was made. The patient was started on topical non-steroidal anti–inflammatory drugs (NSAIDs, Nepalact, Nepafenac 0.1% one drop three times daily) and a thorough systemic workup was ordered which was unremarkable. A follow-up SD-OCT 1 month later showed the cyst enlarging to an inverted flask-shaped vertical cyst involving the foveal centre that spanned the vertical extent of the retina. Importantly, the external limiting membrane (ELM) and the ellipsoid zone (EZ) seemed to be breached and there was a “plume”-shaped hyperreflective material present inside the cyst. The CDVA remained unchanged at this visit. No change was advised in management and a repeat SD-OCT 1 month later showed disappearance of the cyst with foveal flattening, inner retinal layer thinning and defective reformation of the ELM and the EZ; the CDVA had improved to 20/20.


A 50-year-old female presented with complaints of eye strain for the last 3 days. Her past medical history included dyslipidaemia and thyroid disease for the last 10 years well controlled with treatment. Her CDVA was 20/20 in both eyes and her anterior segment exam and intraocular pressures were unremarkable. The fundus examination of her right eye was normal, her left eye had superficial flame-shaped haemorrhages with macular oedema suggestive of a macular superotemporal tributary retinal vein occlusion. Her SD-OCT showed a similar balloon-shaped cyst close to the foveal centre that traversed the vertical length of the retina, breaching the ELM and EZ and filled with plume-shaped hyper-reflective material. Since her visual acuity was 20/20 she was started on topical NSAIDs (Nepalact, Nepafenac 0.1% one drop three times daily). She was followed up with serial SD-OCT scans which showed a decrease in the size of the cyst and disappearance of the plume 10 days later, disappearance of the cyst with residual parafoveal macular oedema 2 months later and complete resolution with normal foveal contour and no residual signs of the cyst or the ELM/EZ breach 8 months later.


A 58-year-old female presented with painless gradual decrease in vision in the right eye over the last 2 weeks. She is a known diabetic with a 10-year history on oral hypoglycaemic agents and insulin. Her previous visit 10 months back was remarkable for bilateral moderate NPDR with no macular oedema and an SD-OCT of her right eye showed minimal retinal thickening with early alteration of the foveal contour. On examination, her CDVA was 20/40 and 20/20 in the right and left eyes, respectively, anterior segment evaluation and intraocular pressures were unremarkable. Her fundus examination showed moderate NPDR in both eyes, the foveal reflex was altered in the right eye. On SD-OCT, a balloon-shaped cyst was noted temporal to the foveal centre that corresponded to the area of retinal thickening observed 10 months earlier, a plume-shaped hyper-reflective material was seen that almost completely filled the cyst and a fine vertical hyper-reflective line was seen that extended from the cyst through the ELM and the EZ was may be suggestive of a narrow occult defect. Similar to the previous two cases, she was also started on topical NSAIDs (Nepalact, Nepafenac 0.1% one drop three times daily); however, she did not return for a review.


A 52-year-old male presented with gradual painless blurring of vision in both eyes over the last few months. He was a known diabetic on treatment. On examination, his CDVA was 20/20 in both eyes and anterior segment exam and intraocular pressures were within normal limits. The right eye showed the presence of a retinal artery microaneurysm along the inferior arcade, mild NPDR and a normal foveal reflex; the left eye showed moderate NPDR with cystoid macular oedema. SD-OCT confirmed with findings with the presence of a solitary optically empty foveal cyst that extended vertically from the internal limiting membrane (ILM) to the outer nuclear layer. The patient was started on topical NSAIDs (Nepalact, Nepafenac 0.1% one drop three times daily). A follow-up visit 6 weeks later his CDVA remained stable at 20/20, while the SD-OCT showed vertical enlargement of the cyst with a fine vertical hyper-reflective line which extended from the cyst through the ELM and the EZ which may be suggestive of a narrow occult defect and the presence of a plume-shaped hyper-reflective material that partially filled the cyst. The patient was asked to continue the same treatment and has not yet returned for a review.


A 59-year-old male, a known diabetic for the last 15 years on insulin, hypertensive and dyslipidaemic for the last 5 years on treatment presented for a change of glasses. On examination, his CDVA was 20/20 in both eyes, his anterior examination and intraocular pressures were within normal limits. He had moderate NPDR bilaterally with an altered foveal reflex in his left eye. An SD-OCT showed the presence of retinal thickening, a balloon-shaped cyst involving the foveal centre, bounded superiorly by the ganglion cell layer and extending inferiorly through a breach in the ELM and the EZ into a small subfoveal neurosensory retinal detachment (NSD). There was the presence of a plume-shaped hyper-reflective material that partially filled the cystic cavity. He was also started on topical NSAIDs (Nepalact, Nepafenac 0.1% one drop three times daily); however, he did not return for a review.


A 32-year-old female presented to our tertiary hospital with a 24-h history of sudden onset right and left upper quadrant pain in the setting of 2 days of increasing nausea, vomiting and inability to tolerate solids. This was on a background of being 18 months post-primary RYGB and MiniMizer gastric ring insertion performed privately.
Her original surgery was uncomplicated and she had experienced excellent weight loss, dropping from 148 kg pre-operatively, to 63 kg over the space of 18 months. Unfortunately, she had developed post-prandial vomiting intermittently for the last year and was in the process of undergoing work-up at our clinics. A barium swallow had demonstrated no abnormalities and she was due for an upper endoscopy.
On presentation, she was not obstipated and did not have any per rectal bleeding. Her pain was coliky and unrelated to food. She was a non-smoker, denied any recent change in diet and was only a recreational drinker. She had no other significant past medical or surgical history. On examination, her observations were within normal limits, she had a tender epigastrium but otherwise, a benign examination. Computer tomography demonstrated an intussusception ().
The patient was positioned supine, and cut down entry periumbilically was performed with a 10-mm camera port and two 5-mm working ports inserted. On laparoscopy, a retrograde intussusception with the intussuscipien constituted the gastric pouch and the intussusceptum constituting the alimentary limb of the jejunum was identified. Just distal to the intussusceptum, there was a jejunal mesenteric defect, with demonstrable thinning of mesenteric fat and various tears. Through this defect, the remnant stomach, normally situated on the left side of the gastric pouch, was found to have herniated from the left to right through this jejunal mesenteric defect, posterior to the alimentary limb in the supracolic compartment. This internal hernia was reversed and the defect closed with non-dissolvable V-Loc™ barbed sutures. A slipped MiniMizer gastric ring was then identified 3 cm distal to the gastrojejunal anastomosis (). No fixation sutures were identified. On release of the ring, 25 cm of alimentary limb jejunum was, with gentle traction, pulled from its invagination into the gastric pouch through where the ring was originally placed (). Surprisingly, all bowel was viable and no resection was required.
The patient was commenced on clear fluids day one post-operatively, upgraded to full diet the following day and was subsequently discharged Day 3 post-operatively. The patient experienced an uneventful recovery, receiving follow up in the first month and for a second time at the 6-month mark. She continues to be asymptomatic and has not required any further significant imaging.


A 33-year-old male patient was admitted to our medical unit because of a 10-day history of fever (up to 39°C), not responsive to paracetamol. He also complained of fatigue and right frontal headache, associated with nausea and photophobia. He reported 5 bowel movements per day on average, with mucus but no previous bleeding. For the past five years had been suffering from ulcerative colitis (UC) and primary sclerosing cholangitis. His whole colon was involved and he had already taken infliximab, adalimumab and golimumab, without any clinical response. He had never been exposed to steroid therapy. At the time of admission, he had been under treatment with azathioprine (50 mg bid) and vedolizumab (300 mg every 8 weeks) for four months with a good clinical response and no signs of flares on laboratory and endoscopic evaluations. Both focal neurological and meningeal signs were negative. Chest X-ray, head CT, brain MRI and eye examinations were all unremarkable. On the neurologist’s advice, lumbar puncture was not performed as his headache was interpreted as a symptom of systemic infection. He was started on empirical antibiotic treatment with intravenous (IV) ceftriaxone and oral levofloxacin. Blood tests showed mild anaemia and thrombocytopenia without lymphocytosis.
Urine and blood cultures were negative, and echocardiogram did not show any evidence of endocarditis. Abdominal ultrasound showed splenomegaly and PET/CT imaging was compatible with an IBD flare. Faecal calprotectin was markedly increased (> 2000 mcg/g), while stool cultures were negative for intestinal infections, including Clostridium difficile. Furthermore, diagnostic tests for malaria parasites, Brucella, toxoplasma, Borrelia, Rickettsiae, leishmaniasis, beta-D-glucan and HIV were negative, as were other tests: quantiferon, monotest and antistreptolysin O titer (ASLOT).
A few days later his serology was consistent with a primary CMV infection: IgG were absent (<5 U/ml), while IgM were higher than normal (117 U/ml) and CMV DNA levels displayed an incremental trend (from 1739 to 2819 UI/m). Since the patient was also immunosuppressed, IV injections of ganciclovir (5 mg/kg) twice a day for two weeks were administered and azathioprine was stopped. His fever disappeared and his general conditions rapidly improved, so the patient was discharged. He continued with oral ganciclovir therapy (5 mg/kg bid) for 14 days. He also took mesalazine (800 mg bid) until the next colonoscopy.


We present the case of a 53-year-old woman with diabetes insipidus that has been treated with desmopressin for about 8 years, migraine with aura, and known ECD confirmed by biopsy, with possible involvement of the great cardiac vessel and infiltrative cardiomyopathy. Over the past several years, she has dealt with a multitude of intermittently exacerbated symptoms, including headaches, fatigue, lethargy, exertional dyspnea, flushing, and peripheral edema, which have been clinically attributed to mast cell activation. She was admitted twice during the previous year with symptoms of fluid retention that resolved with diuresis. She was diagnosed with ECD 2 months before this admission, with biopsy confirmation from the vertebra and the left femur. She was admitted to our institution for further management of a newly identified large pericardial effusion () and ascites, accurately to assess the extension of the cardiac involvement by this disease. In the interim, she had complained of intermittent midsternal chest discomfort associated with dry cough and palpitations that worsened while lying flat.
She was found to have an acute right frontal infarct and multifocal stenosis through the vessel imaging modalities. A magnetic resonance imaging (MRI) showed infarction in the right frontal and parietal white matter, while the magnetic resonance angiography (MRA) revealed extensive intracranial stenosis in the anterior circulation. The final cerebral angiogram showed a right internal carotid artery occlusion, likely secondary to fibromuscular dysplasia changes (). Cerebrospinal fluid tests were normal, and the neurology service concluded that these changes were consistent with vasculopathy secondary to ECD with BRAF mutation.
Transesophageal echocardiography (TEE) showed a large circumferential pericardial effusion greater than 2 cm with maximum thickness around the inferior and posterior left ventricle wall with the partial systolic collapse of the right atrium. She had an ejection fraction of 69% and large pericardial effusion without tamponade physiology. After the evacuation of the pericardial effusion via video-assisted thoracoscopic surgery (VATS), there was minimal pericardial effusion remaining. The pathology exam from pericardium showed histiocytic infiltration compatible with ECD, positive for the BRAF mutation. From these findings, it was concluded that the patient was suffering from a vasculopathy related to ECD. She received corticosteroids for the treatment of the histiocytosis caused by ECD.
She continued her desmopressin for her diabetes insipidus, and she was started on daily aspirin and prednisone, along with pantoprazole and vitamin D for stomach and bone protection. A limited echocardiogram 8 days after VATS showed significant improvement in pericardial effusion (). She was discharged on colchicine, bumetanide and bactrim for prophylaxis against Pneumocystis jiroveci pneumonia. Before going home, a cardiac MRI was done to record the extension of the disease and help with future monitoring (). It was planned to start vemurafenib due to the discovery of the BRAF mutation.


A 52-year-old woman’s medical check-up and ultrasonography revealed a 6.2-cm-sized mass in the right breast, which was diagnosed as invasive lobular carcinoma by needle biopsy. Before surgery, the patient underwent a full-body evaluation. Computed tomography revealed a mass in the right lung, which was diagnosed as pulmonary adenocarcinoma by needle biopsy (). Since the patient was suspected to have synchronous primary carcinomas in her lung and right breast with no sign of metastasis, she underwent right lower lobectomy for the lung adenocarcinoma and right mastectomy for the invasive lobular carcinoma of the right breast. The lung mass measured 2.3 cm in diameter. Microscopically, most of the lung mass exhibited typical growth patterns of lung adenocarcinoma. However, in one focus, the tumor cells showed a different arrangement and cytology; the tumor cells had minimal cytoplasm without nucleoli in a compact trabecular pattern. The typical adenocarcinoma cells were positive for thyroid transcription factor-1 (TTF-1) and E-cadherin and negative for estrogen and progesterone receptors. In contrast, tumor cells in the morphologically different focus expressed immunoreactivity for estrogen and progesterone receptors while they were negative for TTF-1 and E-cadherin (). Based on these findings, we concluded that the invasive lobular carcinoma of the patient’s right breast had metastasized to the lung adenocarcinoma. This study was approved by the Institutional Review Board of Jeonbuk National University Hospital with a waiver of informed consent (IRB No. 2019-05-027).


An 11-year-old girl complained of an aggressive reduction in activity capability and shortness of breath and presented to our cardiovascular department.
The patient started to demonstrate a reduction in activity capability 4 mo ago and progressed with accelerated worsening of her condition within the most recent 1 mo, presenting severe shortness of breath. However, the patient denied any history of past illness.
An enhanced P2 sound and a systolic murmur between sternal ribs 2 and 3 were observed. She suffered from NYHA class II heart function. Echocardiography revealed a large right ventricle and pulmonary artery trunk, with an estimated 60 mmHg pulmonary arterial pressure and normal left heart function. Computed tomography and magnetic resonance imaging scans excluded lung disease and cardiomyopathy, which could lead to PH. In addition, the results of the autoimmune antibody analysis were negative and excluded any connective tissue or rheumatological diseases.
The right ventricular catheter examination showed an elevated right ventricular pressure of 50/2 (23) mmHg, main pulmonary artery pressure of 57/21 (55) mmHg, left pulmonary artery pressure of 50/29 (36) mmHg and right pulmonary artery pressure of 60/31 (45) mmHg with a total pulmonary resistance of 8.72 woods, Qp 6.3 L/min. Therefore, the catheter evaluation failed to identify a clear cause.
Because of the rapid progression of pulmonary artery pressure with microcytic anemia and elevated homocysteine, metabolic disorders were suspected. Therefore, metabolic screening and genome sequencing was performed for this patient. Metabolic screening showed an elevation of methylmalonic acid of 51.82 µg/L, and two mutations c.80A > G/c and 609G > A were recognized at the gene for methylmalonic aciduria and homocystinuria type C protein (MMACHC), and these mutations were reported to be associated with methylmalonic acidemia, cobalamin C type.


A 39 year old man was admitted to the emergency department with paraesthesia in all limbs, diplopia, and nausea since two days. Within a few hours the patient developed weakness in both the lower and upper limbs. His neurologic examination revealed bilateral abducens nerve paralysis, facial diplegia, flaccid areflexic paralysis of the limbs (corresponding to Medical Research Council grade 3/5 in all muscles of the upper extremities and 2/5 in lower extremities) and bilateral flexor plantar responses. The bowel and bladder were not initially involved. The patient underwent electromyography examination with the possible diagnosis of GBS. The electromyogram confirmed an acute, acquired, disseminated polyneuropathy syndrome involving sensory, motor and autonomic fibers, with prolongation of the distal motor latency, prolongation of the F-wave, decreased sensory and motor nerve amplitude, disperse responses and reduced nerve conduction velocities. The cerebrospinal fluid revealed an elevated protein concentration with a normal cell count. Anti-ganglioside antibodies were negative. Intravenous immune globulin (IVIG) was then administrated at a dosage of 0.4 mg/kg/daily. The same day weakness progressed rapidly in his all extremities, rendering him unable to stand due to total loss of strength in all muscle groups. The 6th and 7th cranial nerves were involved in both sides, and respiratory muscle weakness appeared. This progressed to a respiratory failure that required mechanical ventilation. The patient remained in the intensive care unit for two months. IVIG was administrated again over 5 days for a total dose of 2 g/kg body weight, after five sessions plasmapheresis performed on every other day. Treatment with a booster IVIG (0.4 gr/kg/day) was continued every 15 days. Rehabilitation was also performed from the beginning of the disease. In addition oral prednisolone was administered at 1mg/kg/day, which was reduced by 5 mg every two weeks.
Two months after the onset of GBS, pain and decreased range of motion (ROM) emerged in both hips. NHO was diagnosed on the plain X rays of the pelvis. NHO was visible on the anteromedial and anterolateral aspects of both femurs (). The serum calcium was 10.5 mEq/L (normative range 8.6-10 mEg/L) and the serum alkaline phosphatase was 61 IU/L (40-129 IU normative range). Intravenous ibandronic acid 150 mg was given weekly until the patient began to take orally. IV ibandronic acid was discontinued and etidronate disodium was administered 10mg/kg for 10 weeks. ALP and Ca levels were measured periodically. The rehabilitative management continued, including passive and active - assistive ROM exercises for major joints, breathing exercises and electrotherapy for all upper and lower limb muscles. When the patient was discharged from the ICU after two months, the muscle strengths improved in all extremities (grade 3/5 in the upper and 2/5 in the lower extremities). The patient could stand up with support; however could not walk or sit in low position due to pain and limitation in the movements of hip joints. Passive ROM of both hips was restricted to 40º of flexion and 20º of internal and external rotation. Three-phase bone scan study showed prominent Tc99m-MDP uptake in the acetabulum, proximal femur and trochanter bilaterally (). Under computed tomography guidance, corticosteroid injection was made to the periarticular portions of both hips (). The pain decreased and a 10º degree flexion was achieved after injection. At 6 months follow up, his rehabilitation program continues. He can walk with a walker, and sit without support.


A 33-year-old man received first decitabine chemotherapy for MDS, refractory anemia with excess blast type 1. He had received itraconazole (100 mg twice a day) for prophylaxis. Two weeks later, he visited the emergency room because of fever. On examination, vital signs were blood pressure 100/60 mmHg, temperature 36.9°C, heart rate 144/min, and respiratory rate 16/min. White blood cell (WBC) count was 1,030/mm3 with 31% neutrophils, hemoglobin 7.0 g/dL, platelet count 3,000/mm3, total bilirubin 2.11 mg/dL, and C-reactive protein level 3.33 mg/dL. Serum galactomannan antigen was negative. An abdominal radiograph showed mild non-specific paralytic ileus, but there was no pain and tenderness on any abdominal point. There were no abnormal findings on chest radiograph and urinalysis. He received granulocyte colony-stimulating factor (G-CSF), piperacillin/sulbactam (4.5 g four times a day), and amikacin (7.5 mg/kg twice a day) for febrile neutropenia (). On hospital day 9 (chemotherapy day 27), he complained of whole abdominal cramping pain. There was tenderness as well as rebound tenderness on the whole abdomen. On examination, vital signs were blood pressure 120/80 mmHg, temperature 37.8°C, heart rate 90/min, and respiratory rate 20/min. WBC count was 420/mm3 with 4.7% neutrophils. Computed tomography (CT) of the abdomen revealed abnormal wall thickening and decreased density of the ileocecal valve and terminal ileum, suggesting neutropenic enterocolitis (). Supportive treatment including nasogastric tube drainage, G-CSF, ceftazidime (2 g three times a day), and ciprofloxacin (400 mg twice a day) was started due to neutropenia (WBC count 420/mm3) and thrombocytopenia (platelet count 3,000/mm3) (). Patient was broadened the spectrum of antimicrobial agents to meropenem (1 g three times a day) and metronidazole (500 mg three times a day), and an antifungal agent (caspofungin, 50 mg once daily) (). Symptoms and signs including fever, abdominal pain, and tenderness on the whole abdomen did not improve. Another abdominal CT scan was done on hospital day 18, which revealed bowel perforation at ileocecal valve and terminal ileum with partial obstruction of bowel and peritonitis (). Emergent exploratory laparotomy was performed. Gross finding showed whole small bowel dilatation, ileocecal region perforation, and ascending colon inflammation. Small bowel resection at ileocecal lesion was performed. After surgery, the patient received G-CSF, meropenem, levofloxacin (750 mg once daily), and caspofungin until the biopsy was confirmed (). Microscopic finding showed hemorrhagic infarction associated with invasive fungal infection with right angle hyphae corresponding with mucormycosis (). After the histopathologic confirmation, caspofungin was switched to liposomal amphotericin B (5 mg/kg/day) (). On hospital day 21, he still complained of whole abdominal pain, although fever and the inflammation marker were resolved and neutrophil counts had recovered. A second follow-up abdominal CT scan performed on hospital day 21 showed bowel perforation, gangrenous change at ileocecal valve, terminal ileum, and multifocal complicated fluid collection in the peritoneum due to sealed-off perforation (). Galactomanan or 1,3-beta-D glucan level was not examined that day. On hospital day 26, exploratory laparotomy was performed again, which revealed perforation of the previous anastomosis site. Segmentectomy of the small bowel perforation, mobilization of ascending colon, and ascending colon transection were performed. Microscopic histopathologic findings showed hemorrhagic infarction and fungal hyphae at resected small bowel and resected ascending colon (). He received liposomal amphotericin B for 6 weeks and was finally discharged from hospital with full recovery of general condition. Another abdominal CT scan confirmed the absence of perforation.


A 34-year-old woman (gravida 4, parity 1) was admitted because of acute swelling in both legs and paroxysmal dyspnea for 3 months. She had no history of smoking, thromboembolism, or hypercoagulopathy in her medical record. She had undergone total abdominal hysterectomy and left salpingo-oophorectomy 18 months previously for treatment of a broad ligament myoma, and the pathological examination findings were suspicious for IVL. Computed tomography (CT) showed a large multilobulated mass protruding through the inferior vena cava, occupying most of the right atrial cavity, and extending through the tricuspid valve into the right ventricle (). Abdominal magnetic resonance imaging revealed an 8-cm pelvic tumor, which was suspected to be a uterine fibroid, extending up to the inferior vena cava. The patient was diagnosed with ICL before the operation. The first-stage surgery involved abdominal tumor resection and right salpingo-oophorectomy under general anesthesia. Intraoperatively, a convoluted and vascularized tumor mass was observed growing along the left ovarian vein and extending upward along the inferior vena cava, and it was contiguous with the left pelvic wall and involved the retroperitoneum. Considering a possible hemorrhage volume of about 6000 mL, a second-stage surgery involving removal of the right atrial and ventricular neoplasms was performed 2 weeks later. The subsequent pathologic examination findings confirmed ICL. No relapse occurred for 4 years postoperatively.


A 40-year-old woman (gravida 1, parity 1) was admitted with abdominal distention and bilateral leg edema. Abdominal ultrasound revealed a huge tumor of about 30 × 25 cm with a clear boundary, and it was suspected to have originated from the uterus. A CT scan confirmed these ultrasonic findings, which were highly suggestive of IVL. The tumor invaded the left iliac crest, the common iliac vein, and even the inferior vena cava (). Thus, a combined vascular and gynecologic operation was performed. During the surgical operation, a huge myoma originating from the isthmic portion of the uterus was observed; it invaded the pelvic venous plexus and reached the hepatic portal vein. The tumor was resected completely, and bilateral salpingo-oophorectomy was performed. The intraoperative blood loss volume was about 5300 mL. The subsequent pathologic examination findings further confirmed our diagnosis (). No recurrence was found for 5 years postoperatively.


A 40-year-old woman (gravida 3, parity 2) was admitted with IVL. She had undergone myomectomy and unilateral oophorectomy at another hospital 2 months previously. During the operation, a 10-cm myoma was seen along the broad ligament, and a tumor thrombus was found in the right ovarian vessels. The postoperative pathological examination confirmed IVL. Postoperative ultrasonography revealed no thrombus in the inferior vena cava. The patient presented to our department for the second-stage surgery with no symptoms. Vascular reconstruction CT showed a tumor in the inferior vena after a 2-month interval (). We performed total abdominal hysterectomy, left salpingo-oophorectomy, and tumor thrombectomy by angiotomy (). The intraoperative blood loss volume was about 2000 mL, and the pathologic examination confirmed IVL. The patient was clinically well at 2 months postoperatively.


An 84-year-old man had a recent episode of acute severe pancreatitis because of cholelithiasis. He was also taking oral anticoagulants for paroxysmal atrial fibrillation. While investigating for pancreatitis, computed tomographic angiography incidentally revealed an isolated saccular-type, right CIA aneurysm with a maximum diameter of 35 mm (Fig. a). He was referred to us for surgery after the successful management of pancreatitis with laparoscopic cholecystectomy. His ankle–brachial index (ABI) was normal (1.26 on the right side and 1.27 on the left). He was considered as a candidate for EVAR because of his age and risk factors in his recent medical history.
His aneurysm was limited to the right CIA, and his right IIA was widely patent. However, the landing zone in the right CIA was inadequate for applying a ‘bell-bottom’ type device. In cases of conventional EVAR, sacrificing the right IIA with coil embolization is required. Therefore, we decided to preserve the right IIA using the IBE device. The patient’s terminal aorta (24 mm) was marginal to apply this device (Fig. b and c).
The device implantation was successful (procedural time, 140 min; contrast load, 120 mL; radiation dose, 275 mGy) with uneventful postoperative course. His intraoperative angiography (anteroposterior view) revealed no delay or stenosis (Video 1). However, his routine postoperative computed tomographic angiography revealed a marked compression of the contralateral leg by the bridging device in the terminal aorta, which was visible only in the left anterior oblique (LAO) view (Fig. a). Its axial image seemed similar to a subtotal occlusion (Fig. b). Although ABI on the left side was preserved at 1.06, it was lower than the preoperative value (1.27) and that on the right side (1.16). Because we considered its acute occlusion would be the worst scenario for this patient with high possibility of occurrence, we decided to perform preemptive angioplasty for preventing its occlusion. The preoperative angiography found the marked compression only in the LAO view (Video 2). A 10-mm self-expandable stent (LUMINEXX, BARD PERIPHERAL VASCULAR, INC, Tempe, USA) was implanted using balloon angioplasty with improvement (Video 3). Plain computed tomography revealed alleviation of the subtotal occlusion (Fig. c). The patient was discharged and has remained asymptomatic during the 1-year follow-up at our clinic. The computed tomographic angiography at 1 year showed successfully patent left leg without any leakage (Fig. ).


A 10-day-old male neonate with birth bodyweight of 3700 g, firstborn of non-relative parents in which the pregnancy was induced by intrauterine insemination (IUI) and the baby was born by cesarian section (C/S) with a gestational age of 40 weeks and good Apgar score was referred to the pediatric emergency department of Motahari hospital of Urmia. According to the mother’s statements, the chief complaints were shortness of breath, rapid breathing, and vomiting. Also, the patient had a history of NICU admission on 2nd day after birth because of dehydration and poor feeding for 1st day. The patient could not take enough oral feeding for the last 2 days and vomiting was progressive.
On physical examinations, the anthropometric measures were normal, the patient was non-dysmorphic and chest X-ray showed mild to moderate cardiomegaly with bilateral perihilar infiltrates. Vital signs were as follows; PR: 162/min, RR: 62/min, BT: 36.5°C, and oxygen saturation of 94% (room air). Mucous membranes were dry but skin turgor and fontanels were normal. The chest shape was normal and symmetric with subcostal retraction and tachypnea. Cardiac auscultation revealed a grade II/VI continuous murmur. Peripheral pulses were palpable. On abdominal examination, the liver span was increased on palpitation and generalized edema was obvious. Primitive neonatal reflexes such as Moro, sucking, and rooting were normal.
The urgent ABG evaluation revealed PH of 7.14, PaCO2 of 29 mmHg, HCO3 of 9 mEq/L, and PaO2 of 43 mmHg. Serum electrolytes, BUN, creatinine, serum albumin, and blood sugar were in normal ranges. Primary complete blood count (CBC) and coagulation tests were normal except for the platelet count and INR. The patient had thrombocytopenia (platelet count of 95×103/mm3) and an INR of 1.19.
Due to severe pulmonary distress, the patient was intubated and admitted to NICU. After stabilizing the vital signs and hydration, a pediatric cardiology consultation was done. Echocardiography demonstrated no visible RPA, PDA with right to left flow, mild supravalvular aortic stenosis, mild aortic insufficiency, a severe reversal flow in aortic arch, secundum type ASD, dilated right atrium, right ventricle, and main pulmonary artery, severe tricuspid regurgitation and severe pulmonary hypertension, moderate mitral regurgitation and pulmonary insufficiency, dilated inferior vena cava, mild bilateral pleural effusion, and severe ascites. Due to the findings, close cardiological follow up and CT-angiography was performed.
Multi-slice computed tomography of the heart and major vasculature with special reconstructed views (dynamic and delayed images with contrast medium) revealed; anomalous arising of RPA from ascending aorta (hemitruncus), PDA 3.5 mm in size, stretched PFO (6 mm), atelectasis and congestion in both lungs with pleural effusion ().
Serious efforts to perform cardiac surgery in addition to medical therapy such as inotropes and diuretics began. However, pediatric cardiac surgery was not available in the health care center or nearby cities. So, the patient was maintained on medical therapy and supportive care until cardiac surgery was a possibility.
Gradually, the patient’s respiratory distress increased and liver function tests were more impaired and eventually cardiopulmonary arrest occurred on the 24th day of admission in the NICU. Immediate CPR was begun by cardiac massage, intravenous epinephrine, and positive pressure ventilation. After 30 mins the patient had no vital response and CPR was stopped. The last blood tests revealed metabolic and respiratory acidosis. The last blood test values were in .


A 47-year-old woman presented with a painless mass in the right auricular helix that had been gradually enlarging over a year. Physical examination revealed a 1-cm-sized, soft, subcutaneous nodule with normal overlying skin (). She had no history of auricular surgery or trauma.
The initial clinical differential diagnosis included epidermoid cyst, adnexal tumor, such as pilomatricoma, dermatofibroma, neurofibroma, lipoma, and schwannoma. The surgical excision, which included the mass and the overlying skin, was performed under local anesthesia. Grossly, the mass was 0.8 cm in diameter, pinkish, and firm, with a well-demarcated capsule ().
A histopathologic examination showed that the dermis was infiltrated with cells containing granular cytoplasm without cytologic atypia. In the subcutaneous fat layer, the borders were irregular and infiltrating. There were irregular entrapments of adipocytes by the granular cells. These eosinophilic granules were periodic acid–Schiff (PAS)-positive. Eosinophilic ovoid bodies were surrounded by a clear halo ().
Immunohistochemical staining revealed that the tumor cells were positive for S-100 () and neuron-specific enolase (NSE), but negative for epithelial (cytokeratins), melanocytic (HMB-45), muscle markers (α-SMA) (the data was not included). This staining pattern was also suggestive of a Schwann cell origin.
Based on the immunohistochemistry results and morphological features of our case, we excluded possible diagnoses of epidermoid cyst, adnexal tumor, dermatofibroma, and melanoma. Based on the distinctive histopathological and immunohistochemical features, the patient was diagnosed as having benign cGCT. No recurrence was observed during a 24-month follow-up period post-excision.


A 74-year-old man was diagnosed with sarcoidosis uveitis at another hospital in July 2015 and was prescribed steroid eye drop therapy. Swelling of the lacrimal gland was observed in June 2018, and the patient was referred to our hospital for detailed examination (Fig. ). The best-corrected decimal visual acuity was 0.8 and 1.0 in the right and left eyes, respectively. The right eye showed opaque media, which was a nuclear cataract. Pseudophakia was observed in the left eye. Although there were no inflammatory cells in the anterior and vitreous chambers, mild retinal vasculitis and mild epi-retinal membrane were observed on fluorescein fundus angiography. Uveitis caused by sarcoidosis was thought to be relatively controlled. Lacrimal gland biopsy was performed because of the possibility that the left lacrimal gland swelling was caused by sarcoid granuloma. The biopsy revealed extensive deposition of nonstructural eosinophilic material in the secretory gland and ducts, which was direct fast scarlet positive. Immunostaining was negative for amyloid A and positive for globulin light chains (kappa and lambda) (Fig. ). Orbital magnetic resonance imaging and thoracoabdominal computed tomography revealed no other lesions. Hence, a diagnosis of primary localized lacrimal gland light chain (AL) amyloidosis was made. However, additional immunostaining revealed transthyretin positivity (Fig. ). Electrocardiography and echocardiography did not reveal any obvious heart disease. Clinically, primary localized lacrimal gland amyloidosis was suspected. Since the specimen was positive for transthyretin, the possibility of senile systemic amyloidosis was also taken into consideration, and the patient is currently being followed up.


A 43-year-old Filipino female patient, diagnosed with CML (chronic phase) was started on dasatinib as upfront therapy, and achieved complete hematologic, cytogenetic and molecular major response as per the ELN (European leukemia net) recommendations (2013). The patient got pregnant while on dasatinib, which mandated its immediate stoppage.
Alternatives were discussed with the patient: (1) to start with conventional interferon (safe and recommended); (2) to start with PEGylated interferon, but there is no data confirming its safety in pregnancy; (3) to take neither interferon nor TKIs, but this is a risky approach since the patient can progress to either an accelerated phase or blast crisis as a worst case scenario or remain in the chronic phase, which would be the best scenario, but this is not guaranteed. The patient and her husband opted for PEGylated interferon. She was referred to a high-risk pregnancy unit in the maternity hospital for close follow-up. Follow-up throughout pregnancy showed a normal fetus with no evidence of teratogenicity.


A 70-year-old woman complaining of a right breast mass was referred to our hospital. Mammography, breast composition of Breast Imaging Reporting and Data System category 2 (Fig. ), showed focal asymmetrical density in the right upper and inner quadrant. Ultrasonography showed a well-demarcated and hypervascular tumor with predominantly high-level internal echoes (Fig. ). Magnetic resonance imaging showed a heterogeneously enhanced and well-defined mass. We, therefore, performed vacuum-assisted biopsy to the tumor. Routine histological examination of the tumor revealed atypical cells with a large nucleus, scant cytoplasm, and abundant mitoses accompanied by a lot of fat cell interspersion (Fig. ). Immunohistochemical examination showed the atypical cells to be AE1 + 3, CAM5.2, CD3, CD5, CD10, CD30, CD56 negative, CD79a weak positive, CD20 positive, and MIB-1 highly positive, leading to the diagnosis of diffuse large B cell lymphoma of the breast. Cytological examination showed no lymphoma infiltration into the bone marrow. Positron emission tomography revealed no distant spread of the breast lymphoma. Under the diagnosis of stage 1A diffuse large B cell lymphoma of the breast, the patient underwent six courses of R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone) chemotherapy and radiotherapy to the breast and supraclavicular lymph nodes (maximum dose 5,270 cGy). Positron emission tomography scan taken 5 months after the completion of radiotherapy showed no tumors in the breast. The patient has been well without lymphoma recurrence for more than 6 years.


A 67 year old Caucasian female presented to the emergency department of our hospital complaining of dysphagia, pruritus and musculoskeletal pain of the arms. The patient also mentioned chronic musculoskeletal pain and acrocyanosis for which she never sought medical attention.
A careful medical history revealed that she suffers from chronic obstructive pulmonary disorder (COPD) due to long-term smoking, and was under treatment for arterial hypertension. The initial clinical examination showed normal blood pressure (115/72 mmHg), normal heart rate (77 bpm), and no pathological findings during auscultation and palpation of the abdomen. The heart and lungs auscultation did not reveal any pathological findings. Skin redness was noticed on the site of pruritus.
The blood tests did not reveal any significant findings, however the biochemical test yield findings indicative of disrupted hepatic function (). Due to these findings, further testing for HBV and HBC were requested, revealing negative results.
A cancer index test was also performed, showing no signs of neoplasia. Thyroid function test (Free T4, TSH) and thyroid ultrasound revealed no pathology as well. A quantitative immunoglobulins test indicated increased levels of IgA, IgM and IgE ().
A CT scan was performed without intravenous contrast medium due to patient-reported previous allergy. The CT scan did not demonstrate any pathological finding of the liver apart from dilation of intrahepatic bile ducts that was later confirmed sonographically.
Taking into account the pruritus, all the clinical findings and the increased ALP, AST and γGT levels as well, the possibility of PBC was suggested. So, further specialized tests (ANA and AMA tests) were requested that proved positive (), thus confirming the diagnosis of PBC.
Due to the persistent musculoskeletal pain in the arms and the sclerodactyly, Anti-centromere antibodies (ACAs) test was conducted which proved positive. During the investigation of the reported dysphagia the patient underwent gastroscopy () that revealed gastritis and esophageal veins dilation[].
Taking into consideration all the pre-mentioned positive findings, the Raynaud phenomenon and the history of acrocyanosis as well, the initial suspicion of CREST syndrome was finally confirmed.
Although, there is cure for PBC, some medications may provide relief of the associated symptoms and decelerate the progression of the disease[]. Our patient was discharged from the hospital’s clinic after improvement of her clinical and laboratory status. She was put on treatment with Ursodeoxycholic acid (250mg X 4 per os) to slow down further liver fibrosis, and Colchicine (0,5mg X 2 per os) to lower her bilirubin levels[].
For the management of musculoskeletal pain, NSAIDs in ointment form were prescribed, and as far as it concerns the dysphagia, dietary orders were given, like avoiding hot meals and eating earlier in the evening. The prescription of PPIs was not considered necessary in the specific case as the simple dietary orders proved efficient.
For the Raynaud phenomenon and the acrocyanosis the patient was advised to avoid exposure to cold, sudden temperature changes, smoking and stress and she was also prescribed nifedipine, a calcium channel blocker.


An 83-year-old male with a history of type 2 diabetes mellitus, high blood pressure and dyslipidemia was diagnosed with amyotrophic lateral sclerosis (ALS) by our neurology service, with dysarthria as the main manifestation, and treatment with riluzole was initiated. Functional tests showed a moderate restrictive pattern, although without respiratory symptoms.
Three months later, the patient was admitted to the internal medicine service due to a deterioration in his general condition in the previous week, nausea with isolated vomiting, decreased food intake and postural instability. He was taking gliclazide, metformin, irbesartan/hydrochlorothiazide, nifedipine, simvastatin, riluzole, omeprazole, escitalopram and lorazepam. There were no respiratory symptoms and baseline oxygen saturation was 93%. The physical examination showed only a widespread loss of strength, which had appeared in the previous 2 months. In the initial analysis, sodium levels of 120 mmol/l (confirmation with sodium levels of 117) and plasma osmolality of 232 mOsm/kg (normal values 280–300) were the most notable findings.
After discontinuing the thiazide and the 2 drugs associated with syndrome of inappropriate antidiuretic hormone secretion (SIADH), escitalopram and omeprazole, sodium levels were 127 mmol/l and osmolality was 263 mOsm/kg; potassium, venous blood gas, cortisol and TSH were normal, and urine sodium was 46 mmol/l with an osmolality of 560 mOsm/kg, confirming the diagnosis of SIADH. The study of other possible causes of SIADH was negative, including chest x-ray, cranial CT and chest-abdominal CT.
With the withdrawal of the drugs involved in hyponatraemia and fluid restriction, the symptoms that had appeared in the week before admission remitted. At discharge, the patient began treatment with a daily sachet of 15 g of urea and in the follow-up visit at 7 days, sodium levels had normalized to 136 mmol/l.


A 27-year-old woman was presented with a burning like lesion. The skin lesion was developed after a jellyfish stings (Rhizostomae) while she was swimming in Pattaya Sea in Thailand, five days prior to her presentation. Instantly after jellyfish stings, she was taken into the ship for rescue operations. The ship personnel applied lemon juice and baking soda to the injured area believing that this remedy is efficient in this situation.
The next day, she referred to a local hospital at Pattaya because she was suffering from a horrible pain in her arm and then she was discharged on oral Dicloxacillin and topical Hydrocortisone. The edema was extended the next days which intensified her pain. At this time, she was transferred to Loghman Hakim Hospital, Tehran, Iran. At admission, erythematous edematous patch with centrally grouped vesicles on the left arm was observed (). The patient had a high-grade fever (38.1°C) and was agitated due to her intolerable pain. Other vital signs were normal. In the sonography imaging, soft tissue edema was seen without any collection. According to the clinical and imaging findings, the diagnosis of soft tissue cellulitis due to jellyfish stings was made. The patient admitted to infections ward and intravenous antibiotics including Cefazolin at dose of 2000 mg every eight hours and Vancomycin at dose of 1000 mg every 12 hours initiated. After five days of hospitalization, the patient was recovered from left arm edema. Also, her pain decreased gradually and no obvious erythema was seen. The patient was finally discharged with a satisfactory clinical response.


A 74-year-old Japanese woman was diagnosed with T3N2cM0 hypopharyngeal cancer. We considered the disease unresectable due to retropharyngeal lymph node metastasis with radiologically proven total encasement of the internal carotid artery (). She was treated with induction chemotherapy followed by concurrent chemoradiotherapy (CCRT) as part of a clinical trial of paclitaxel, carboplatin, and cetuximab followed by chemoradiotherapy. The disease was completely resolved 11 weeks after CCRT. However, 24 weeks after CCRT, the primary tumor and neck nodes recurred.
We considered the recurrent diseases to be platinum-refractory and started nivolumab therapy. Unfortunately, despite receiving 13 courses of nivolumab therapy, the primary disease continued to progress, although the neck nodes shrank and could no longer be detected. The recurrence at the primary site soon led to narrowing of her airway, which required airway management. She could not eat adequately and depended on gastrostomy tube feeding. We could not detect any distal metastases and the retropharyngeal node had also disappeared. shows a summary of the treatment and tumor response until the patient underwent salvage surgery.
We performed total pharyngolaryngectomy (TPL) with free jejunal reconstruction. The operation time was 6 hours 54 minutes, and blood loss was 315 mL.
There were no particular difficulties encountered during the surgery apart from some adhesions in certain parts as a consequence of previous oncological treatment (). In fact, the surgery did not differ from other salvage surgeries. The recurrent primary tumor was completely resected macroscopically; however, malignant cells were present in the margins of the lymph vessels. Microvascular anastomoses were performed uneventfully using the superior thyroid artery and the internal jugular vein.
The postoperative period was uneventful and gastrostomy tube feeding was started from postoperative day 2. No leakage was detected by a barium swallow test on postoperative day 14, and the patient started oral intake of food on postoperative day 15. There were no postsurgical complications. She was discharged on postoperative day 18 with a stable and safe permanent tracheostomy and normal oral intake. We resumed nivolumab therapy on postoperative day 38.
Nivolumab is still regularly being administered and the patient continues to undergo regular follow-up at our outpatient unit. She has not reported any particular symptoms to date.


A 59-year-old, former smoker, man, presented at the Emergency Department with a sub-acute onset of increasing dyspnea and persistent dry cough. Previous medical history consisted of an occupational exposure to fine dusts and other toxic inhalants (ex pottery maker), and acute myocardial infarction previously treated with PTCA. No symptoms of collagen vascular/autoimmune disease were present.
At physical examination, digital clubbing was observed and inspiratory basal crackles were detected. Chest X-rays documented multiple bilateral pulmonary consolidations and a High-Resolution Computed Tomography (HRCT) confirmed the presence of bilateral multiple areas of consolidations with frequent air bronchograms, interlobular septal thickenings and ground-glass opacities (). Imaging was consistent with a crazy paving pattern.
Sputum examination was negative for pathogenic microbes, while pulmonary function tests revealed a moderate restrictive disease (VEMS 66,5%, FVC 61,1%, TLC 56,8%).
The post-operative course was unremarkable and the patient was discharged in 4th post-op day. At histology, the lung parenchyma showed nodular lesions characterized by an infiltration monomorphic small lymphocites and plasma-cells obliterating the alveolar spaces () associated with lymphoepithelial lesions (B). At immunohistochemistry, neoplastic lymphocytes show a B-cell clonal phenotype (CD20 immunohistochemistry, Hematoxylin counterstain, 20X (C)); moreover, stain for MIB1 shows a low proliferative index of the neoplastic infiltrate (MIB1 immunohistochemistry, Hematoxylin counterstain, 20X (D)) accounting for 3-5%. The final diagnosis of mucosa-associated lymphoid tissue-derived (MALT) lymphoma was finally made. The patient underwent chemotherapy with Rituximab and Bendamustine, with gradual and progressive improvement of the overall clinical scenario.


A 58-year-old man was admitted to our hospital because of progressive dysphagia for >1 month. By oesophagogastroscopy, a ring uplift, 36–38 cm from the upper incisors was identified. Confirmed as high-grade oesophageal intraepithelial neoplasia by pathology. Chest and upper abdominal computed tomography showed wall thickening in the lower oesophagus, range about 56 mm, showed obvious enhancement. The density shadow of strip soft tissue in the right middle lobe of the lung could be seen enhancement; the bronchi of the lateral segment of the right middle lobe were partially cut off. By fibrobronchoscopy, in the lateral part of the right lung middle lobe, the bronchial opening in the subsegment of the lung shows the vegetative new organism, and the lumen is basically closed []. Confirmed as adenocarcinoma by pathology. To exclude N2 disease by positron emission tomography preoperative preparation, no distant metastases were detected in this patient before treatment.
To achieve a good prognosis, surgical treatment of the patient was indicated. An Ivor-Lewis and lobectomy were performed through thoracoscopic surgical approach. After successful anaesthesia with two-cavity endotracheal intubation, using a standard procedure with the patient in the supine position, as previously reported.[] After that, we performed thoracoscopic lobectomy and thoracic oesophageal mobilisation in the right lateral position. Based on our prior experience, a 1 cm incision at the 7th intercostal space, along with the middle axillary line, was selected as the observation port. The principal operating port with a 5 cm access incision. Dissection of the middle lung lobe was completed first. The posterior mediastinum was exposed by adjusted operating tab. The thoracic oesophagus, along with the peri-oesophageal tissue and mediastinal lymphonodus, is circumferentially mobilised from the diaphragm to the level of arch of the azygos vein. Mediastinal lymphadenectomy is performed in the patient. After the specimen is removed from the field, an anastomosis is performed by mechanical stapled [].
The operation lasted about 350 min. The length of the postoperative hospital stay was 11d, the final pathological examination revealed a moderately differentiated squamous cell carcinoma invading the oesophageal adventitia (pT3) with no lymphatic metastasis (0/24, N0). The pulmonary nodule in the middle lobe was identified as an adenocarcinoma (pT1bN2M0). Besides, lung lesion was confirmed not a metastasis by hematoxylin and eosin staining. The patient was re-examined after half a year without any obvious recurrence or metastasis.


A 66-year-old, non-smoking woman, with a history of sub-total thyroidectomy 33 years
ago due to a thyroid nodule. Consultation with a 7-month moderate dyspnea associated
with dry cough in the last 3 months. At admission, vital signs were normal; she had
no retractions, cyanosis, or stridor. No evidence of neck masses, or jugular
engorgement, no edema or collateral circulation, normal auscultation, and normal
abdominal physical examination. Chest x-ray showed an 11 cm mass in the upper right
hemithorax with regular edges. In the chest CT scan, the heterogeneous rounded
lesion showed calcifications inside, 11 cm in diameter, which compressed the
superior vena cava without infiltration, the right subclavian vein and displaced the
trachea to the left (). Due to the size of the lesion, it was very difficult to differentiate
whether it was of mediastinal origin or one of intrapulmonary behavior. In addition,
a thyroid gamma scan was performed showing infrasternal uptake suggestive of
supernumerary thyroid glands. A CT-guided biopsy was performed from which four
fragments were obtained. H&E staining showed no pathological alteration.
Immunohistochemistry showed positive TTF-1 which confirms thyroid gland origin.
After a multidisciplinary assessment, a resection of the mass via bilateral
thoracotomy was decided, in which the mediastinum and right hemithorax were exposed.
Intraoperative findings showed a 16 cm mass of cystic content with large
desmoplastic reaction, from the mediastinum which displaced the lung and great
vessels but did not infiltrate them. The final histopathological diagnosis was of
thyroid tissue with findings of multinodular goiter, without signs of malignancy,
with cystic dilations containing colloid material, chronic inflammation and presence
of foamy histiocytes (). The patient had adequate postoperative clinical evolution.


A 70-year-old Japanese woman visited a hematologist for idiopathic thrombocytopenia. During follow-up, she had general fatigue and a persistent low fever. Her urine analysis and abdominal computed tomography (CT) revealed pyuria and a left staghorn calculus (Fig. ). Thereafter, she was referred to our department for a further examination and treatment for her urinary tract infection.
On the day of her initial visit to our outpatient department, her serum C-reactive protein level was 12.34 mg/dL, white blood cell count was 11,400/μL, and serum creatinine was 0.52 mg/dL. We performed percutaneous nephrostomy for the treatment of her pyelonephritis (Fig. ). Thereafter, 2 g/day of ceftriaxone (CTRX) was administered intravenously for 9 days. Her fever reduced, and her inflammation markers decreased. The next month, she visited our department again for fever. CT revealed that her middle and inferior calyx had not been adequately drained (Fig. ). Two additional nephrostomies were therefore performed. We washed each calyx every day and administered CTRX 2 g/day for 9 days. Inflammation slowly decreased, and thereafter, she left our hospital.
Three months after her initial visit to our department, she underwent open nephrectomy. Due to severe adhesion, the kidney was divided into two pieces and removed (Fig. ). A pathologic examination revealed that the tumor was SCC accompanied by a cancer pearl (Fig. ). Foam cells were invading the renal parenchyma, suggesting xanthogranulomatous pyelonephritis. We were able to detect the area where the urothelial epithelium and squamous epithelium were mixed. This finding suggested that the tumor was derived from the squamous metaplasia due to the staghorn calculus. Immunohistochemical staining showed that GATA3 and P40 were negative, while CK5/6 was positive (Fig. ). Based on these findings, a diagnosis of SCC rather than urothelial carcinoma or renal cell carcinoma was made. As her kidney had been broken into two pieces, it was difficult to decide whether or not the surgical margin was positive. Her disease's T stage was assumed to be at least pT3. The final diagnosis was SCC of the left kidney, and after the operation, the serum SCC marker level was 4.8 ng/mL.
One week after surgery, while her laboratory data showed improvement, general fatigue and appetite loss progressed. Two weeks after the operation, CT revealed local recurrence of the tumor (Fig. ). One month after her surgery, she was readmitted to our department because of her severe appetite loss and malnutrition. CT demonstrated enlargement of the local recurrence near the left renal hilum (Fig. ). At this point, her serum SCC marker level was 32.2 ng/mL.
Her general condition continued to worsen, and she was unable to receive chemotherapy or radiotherapy. Therefore, she received best supportive and palliative care. Two months after the operation, she died of renal SCC.


Case 1 – An 8-year-9-month-old female patient presented to our clinic with complaints of blinking, tic disorder in the form of hawking and perpetual urge for hand cleaning, which increased in intensity with a throat infection. The patient described deterioration in dexterity and involuntary hand and body movements, which became evident during the periods of exacerbation, indeed. The patient had been started on fluoxetine before, but despite using fluoxetine for six months, her symptoms did not improve, and the family discontinued the treatment voluntarily. He did not have a family history of psychiatric illness, especially OCD, Tourette syndrome, or a tic disorder. Thus, the patient stated that she had not undergone regular check-ups before. Physical examination of the patient revealed choreiform hand and body movements, vocal and motor tics. In the oropharynx examination, the tonsils were markedly hyperemic and hypertrophic. Then, the patient was asked for antistreptolysin-O (ASO), anti-nuclear antibody, rheumatoid factor, C-reactive protein, hemogram, biochemistry tests and a throat culture was sent to the laboratory. ASO level was 615 IU-ml (0-200 IU/ml), and other laboratory results were within a normal range. GAS-Streptococcus pyogenes was isolated from the throat culture. The patient was started on oral amoxicillin-clavulanic acid at a dose of 75 mg/kg/day. Echocardiography showed no cardiac involvement. Cranial magnetic resonance imaging and electroencephalography were normal. Risperidone 0.5 mg/day was started. The patient’s complaints reduced at the end of the first month. Then, monthly depot penicillin prophylaxis was started. Significant improvement was observed in the patient’s complaints one year after the initiation of the treatment.


Case 2 – A 6-year-4-month-old male patient presented with hand and body movements that increased in severity during the two years of schooling, leading to a marked decline in writing and fine motor skills during the infection periods. There were vocal tics in the form of oral throat cleaning. He also wanted his notebook and books placed in his bag in a certain order. School absenteeism increased due to increasing complaints during the periods of infection. Risperidone 0.25 mg/day had been started at the pediatric psychiatry clinic and he was referred to us because of no response. There was no psychiatric disease, especially OCD and tic disorder, in his family history. On physical examination, the patient had dancing like movements of the hands and legs and a vocal tick in the form of throat cleaning. There was a milking phenomenon in his hand and when he was asked to keep his tongue out of the mouth, he was able to achieve this for a short time. His body temperature was 37.8 oC. The oropharynx was markedly hyperemic and hypertrophic. In the laboratory tests, ASO was 927 IU-ml (0-200 IU-ml) and C-reactive protein was 38 mg/L (0-5 mg/L). White blood cell count increased (19.800). Anti-nuclear antibody, rheumatoid factor and biochemistry tests were normal. Echocardiography showed no cardiac involvement. Cranial magnetic resonance imaging and electroencephalography were normal. GAS-Streptococcus pyogenes grew in throat culture. Ampicillin-sulbactam was started intravenously. On the 3rd day of the treatment, his symptoms started to improve. Monthly Depot penicillin prophylaxis was started. Significant improvement was observed in the choreiform movements after six months.


This 44-year-old man experienced an alkali burn in the left eye 31 days before surgery and one prior treatment with amniotic membrane patching (). At presentation, there was scleral and limbal ischemia with a breadth of 6.5 mm from 3:30 to 9 o’clock and a corneal epithelial defect of 6.5 mm × 9.5 mm. His visual acuity was hand motion at 20 cm. After the combined surgery with tenonplasty and amniotic membrane transplantation, the burned ocular surface was successfully reconstructed and stabilized within 45 days. The cornea was opaque with peripheral neovascularization, and the intraocular pressure was higher than normal. Then, the patient was diagnosed as secondary glaucoma and treated with brinzolamide/timolol maleate eye drops (Alcon Laboratories, Inc., Fort Worth, Texas) and 0.2% brimonidine tartrate ophthalmic solution (Allergan Pharmaceuticals Ireland, Westport, County Mayo, Ireland). The BCVA at the final visit was counting fingers at 30 cm.


A 37-year-old man with thermal burns presented 60 days before surgery with an opaque cornea, an epithelial defect of 5 mm × 5 mm, and focal conjunctival and scleral necrosis with a breadth of 3.5 mm from 2 to 5 o’clock in the left eye (). He had received amniotic membrane patching twice as part of a prior treatment. Before this treatment, his left eye had visual acuity of hand motion. After the combined surgery, the ocular surface was gradually stabilized, with reepithelialization within 54 days. The cornea was opaque in the center and neovascularized with pseudopterygium in the peripheral. The BCVA was counting fingers at 20 cm at the final visit due to corneal opacity and a secondary cataract.


A 34-year-old woman with no diseases other than hepatitis B visited our neurosurgical outpatient department with a headache confined to the right side, along with a slowly growing mass in the right temporal area. A computed tomography (CT) scan showed signs of osteoclasia, although the mass had not invaded the brain parenchyma (). At the time, she was advised to monitor the condition over time, and a year later, she visited our plastic surgery department with a worsened headache and a larger mass. In an X-ray examination performed at the department, a lateral radiograph showed a rounded osteolytic lesion on the right anterior part of the skull (). A magnetic resonance imaging (MRI) scan showed a mass measuring approximately 2.4×2.5 cm that had not invaded the brain parenchyma. The mass was hypointense on T1 images and hyperintense on T2 images (). Based on the radiological findings above, epidermoid cyst, dermoid cyst, plexiform neurofibroma, mucocele, and eosinophilic granuloma were considered as differential diagnosis. Most probably the mass was an epidermoid or dermoid cyst [,].
Under general anesthesia, an incision was made along the centerline of the mass, and the mass was dissected from the surrounding tissues. The tumor was found to be an encapsulated cystic mass containing a typical soft butter-like semi-solid material, implying an epidermoid cyst. The tumor was completely excised, including the fibrous capsule. The mass was encapsulated and adhered to the lower dura mater, but no dural invasion was observed. After excision of the tumor, skull reconstruction was performed using Medpor Titan (Stryker, Tuttlingen, Germany) to cover the bone defect (). The patient’s neurological symptoms, such as headache, disappeared after excision of the mass, and the patient was treated without any postoperative complications. There was no evidence of recurrence at 2 years postoperatively.


A 56-year old male patient with past medical history of corrected tetralogy of Fallot (an initial palliative intervention at the age of 6 years and a second, complete repair, at the age of 12 years), percutaneous pulmonary valve implantation for severe pulmonary regurgitation at the age of 55 years, and one episode of paroxysmal atrial fibrillation who presented for palpitations and near-syncope. His medications included carvedilol 6.25 mg bid, eplerenone 25 mg od, perindopril 4 mg od, furosemide 20 mg od, Fluindione 20 mg od, and potassium supplements 600 mg tid. Clinical examination revealed a blood pressure of 107/71 mm Hg, heart rate of 260 bpm, SaO2 of 97% breathing room air, and a temperature of 37 °C. His ECG recorded at admission and 5 minutes later are presented in Figures and .
His echocardiogram revealed a mildly dilated left ventricle (ESD=55 mm) with severe LV systolic dysfunction, ejection fraction (EF) of 28% (Simpson), strain −8.6%, dilated right ventricle (48 mm at the level of the tricuspid annulus) and right atrium, sPAP of 33 mm Hg, and no pericardial effusion.
His biological workup showed no anemia, thyroid dysfunction, or electrolyte imbalance.
Considering his second ECG (), the wide QRS complex tachycardia was interpreted as atrial flutter with 1:1 A:V conduction. The patient was treated initially with IV amiodarone and oral carvedilol. An electrophysiological study was subsequently performed with the CARTO electro-anatomical mapping system, which demonstrated typical counterclockwise atrial flutter. Radiofrequency ablation of the cavotricuspid isthmus was performed, and the patient converted to sinus rhythm. The ECG in sinus rhythm at the end of the ablation procedure, recorded in the electrophysiology lab, is presented in .
This ECG showed the presence of peculiar P waves, with an initial short and flat or positive component followed by a deep negative component in leads II, III, and aVF (best visible in lead II); positive in leads I and aVL, with a P-wave duration of 159 ms, suggesting the presence of a-IAB. However, on closer inspection, despite the presence of positive P wave in leads I and aVL, one could see that the morphology and the amplitude of the QRS complexes were different from the ECG in , suggesting ECG electrode inversion.
A 12-lead ECG was recorded with the electrodes placed in the correct manner, which made the aspect of a-IAB disappear (). The patient was discharged 48 hours later.


A 48-year-old man from Northeast Nuevo Leon State presented with a history of a painless scrotal mass that progressively increased in size during the last 3 months. He denied dysuria, urethral discharge, weight loss, fever or diaphoresis. He has worked as a farmer for the last 20 years and his medical history was relevant for chronic alcohol consumption during the last 10 years (200 g/week) and a recent diagnosis of primary adrenal insufficiency treated with a daily dose of 10 mg of prednisone and fludrocortisone 0.1 mg per day in the past year. Vital signs were stable. Further examination revealed a hard and heterogeneous tumor in the posterior aspect of the right testis. Inguinal lymphadenopathy, local hyperthermia, skin lesions or color change were not present. Laboratory results showed a hemoglobin concentration of 13.1 g/dL, leukocytes 8.09 x109/L, platelets 199 x109/L, glucose 96 mg/dL, sodium 138 mmol/L, chlorine 100 mmol/L, potassium 4 mmol/L, calcium 8.4 mg/dL, urea nitrogen 16 mg/dL, creatinine 1.0 mg/dL, C-reactive protein 1 mg/L, alanine aminotransferase 36 UI/L, aspartate aminotransferase 30 UI/L, total bilirubin 0.8 mg/dL and albumin 3.8 g/dL. Color flow doppler testicular ultrasonography was performed and two nodular masses in the tail of the right epididymis with increased vascular flow were found (). An epididymectomy was performed and histologic examination revealed a granulomatous epididymitis with scattered coccidioidal spherules present within the granulomas (). The diagnosis of disseminated coccidioidomycosis was made and systemic antifungal therapy was initiated with fluconazole 400 mg once daily for 12 months. As the diagnosis of disseminated coccidioidomycosis usually relies on the histopathological identification, further serological tests or culture were not performed. Alterations in the lungs compatible with current pulmonary coccidioidomycosis were not found on the chest CT-scan. Involvement of other organs was systematically excluded in our patient as well, however, further evaluation for adrenal insufficiency will be carried out after discharge.