Our patient is a 60-year-old female who had a history of proliferative diabetic retinopathy with chronic DME. She had previously been treated with multiple intravitreal injections of anti-vascular endothelial growth factor (VEGF) including both bevacizumab and ranibizumab for a total of 33 injections over a 5-year period. Other treatments included 1 intravitreal triamcinolone injection in year 2 and 1 sub-Tenon's triamcinolone injection in year 5. She underwent multiple sessions of retina lasers over 5 years including macular focal and pan retinal photocoagulation with persistent DME (Fig. ). She was concurrently diagnosed with ocular hypertension 5 months after initial presentation with an intraocular pressure (IOP) of 24 mm Hg. She was started on topical therapy and has remained with good IOP control to date. The patient was treated with an injection of Ozurdex via the pars plana with topical anesthetic 59 months after her initial presentation with a best corrected visual acuity (BCVA) of 20/40+2. The patient was without complaints following the procedure.
Six weeks later, an intralenticular foreign body was incidentally noted and determined to be the recently placed Ozurdex implant (Fig. ). The patient's visual acuity had improved to 20/30, and her macular edema had resolved on exam and confirmed on optical coherence tomography (Fig. ). The patient was then referred to the retina service for further management given this rare complication. She remained asymptomatic for 8 months with 20/30 vision and stable IOP on topical therapy.
Eight months following Ozurdex injection, her BCVA declined slightly to 20/50 with a mild myopic shift of 0.625D spherical equivalent. This shift was attributed to increased nuclear sclerosis despite no visible lens changes on exam including around the implant. The patient was offered cataract surgery, which was performed 12 months following the injection of Ozurdex into her lens. Her DME remained resolved at the time of cataract extraction.
Cataract extraction was performed via phacoemulsification by standard technique through a clear corneal incision. The Ozurdex implant was noted to be brittle but could be easily removed by phacoemulsification. No capsular defect was noted intraoperatively, and no vitreous was encountered. A single-piece intraocular lens (IOL) was placed into the capsular bag. One day postoperatively, her vision without correction was 20/40. Two months following phacoemulsification and the removal of the Ozurdex implant, her BCVA was 20/30+, J1+ at near, and the macular edema remained resolved without any further treatment (Fig. ).


A 36-year-old African male was admitted in August, 2010 with high-grade fever and bloodstained urine of 7 days duration. He had a history of intermittent passage of dark-colored urine, which led to a diagnosis of PNH in 1999 in Senegal, Africa; pulmonary and mediastinal tuberculosis in 2001; and episodes of malaria. His symptoms included hyperpyrexia, vomiting and hematuria. He was febrile (38°C), with heart rate 110 bpm, BP 180/90 mmHg, oxygen saturation of 100%. Bilateral costovertebral angle tenderness was detected. Cardiac, pulmonary and abdominal examination was normal. As there were no details available of his original diagnosis of PNH, the patient was retested to rule out other causes of hemolysis.
Laboratory tests revealed estimated glomerular filtration rate <15 mL/min/1.73 m2, microcytic anemia with thalassemia trait (hemoglobin [Hb] 6.7 g/dL, mean corpuscular volume 73.7 femtolitre, ferritin 459 ng/mL, total iron binding capacity 222 ug/dL, serum iron 16 ug/dL), advanced azotemia (serum creatinine 16.2 g/dL; urea 210 mg/dL), hyperphosphatemia (6.8 mg/dL) and hyperuricemia (11 mg/dL). Although total bilirubin was normal, hemolysis was confirmed by high lactate dehydrogenase (LDH) levels (4233 U/L), high reticulocyte count (absolute number 99 290/mm3) and decreased haptoglobin value (17.9 mg/dL). White blood cell and platelet levels were 8 150 and 252 000/mm3, respectively. Urinalysis showed pH 7, specific gravity 1008, trace proteinuria (21.2 mg/dL), microscopic hematuria (> 10 red blood cells [RBCs]/high-power field), without any casts and the presence of trace hemoglobinuria (0.60 mg/dL). Urine culture failed to show bacterial growth, despite leukocyte esterase of 125 C/uL and 50 white blood cells/high-power field.
Urinary tract ultrasound showed bilateral enlarged kidneys, with a pervasively hyperechogenic and thickened cortex; colored Doppler echography of the renal arteries and veins was normal. A peripheral blood smear showed fragmented RBCs, however, a diagnosis of thrombotic microangiopathy was ruled out by elimination. Indirect Coombs test was negative, while direct was slightly positive. Complement fractions C3–C4 were normal and agglutinins were absent. G6PD level was normal. Malaria was ruled out as a cause of hemoglobinuria, given three negative blood samples; BK virus in urine was not detected. An in vitro indirect test for Mycobacterium tuberculosis based on measurement of a cell-mediated immune response to a peptide cocktail simulating mycobacterial proteins ESAT-6 and CFP-10 (Quantiferon-TB Gold Plus [QFT-Plus] test) suggested a probable diagnosis of tuberculosis. Chest X-rays and a high-resolution computed tomography scan were negative at the time of hospitalization and a repeat quantiferon test was negative. Kidney biopsy was not performed.
A diagnosis of PNH was confirmed by FLAER-based flow cytometry: the patient’s granulocytes were deficient in CD59, CD55 and CD16 with a clonality of 86% in the monocytic compartment and 89% in the myeloid compartment.
The patient was adequately hydrated with intravenous (IV) fluids and NaHCO3, and received a packed RBC transfusion. Levofloxacin and later prednisone were started empirically, aimed at controlling hemolysis. During hospitalization, renal function normalized without further intervention, serum creatinine level decreasing from 16.2 to 1.7 mg/dL. Hb gradually rose to 8.6 g/dL without further transfusions. LDH progressively decreased, to 1361 U/L, while haptoglobin level normalized. At discharge, after 15 days of hospitalization, the patient was asymptomatic, afebrile for 5 days, had a heart rate of 80 bpm, BP of 130/70, saturation of 100% while breathing ambient air. He continued regular follow-up and maintained normal renal parameters.
A low transfusional need was maintained from 2011 to 2012. However, from September 2013 due to recurrent episodes of hemolysis and hematuria (LDH progressively increasing to up to 2000 U/L; haptoglobin remained under normal value), Hb level decreased and required monthly RBC support. Given the high risk of thromboembolic events, a standard-dose eculizumab therapy was started in March 2015, consisting of eculizumab 600 mg via 25–45 min IV infusion every week for weeks 1–4, followed by 900 mg IV for the fifth dose 7 days later, then 900 mg IV every 14 days thereafter. The patient has been closely monitored during eculizumab therapy, and no thrombosis or other complications have so far occurred. LDH and Hb levels stabilized (>500 U/L and > 15 g/dL, respectively) as well as the creatinine levels.


A 9-year-old previously healthy girl was admitted following 5 seizures accompanied by vomiting and headache in 1 day. There was no history of fever, trauma, infection, vaccination, or drug usage. The neurological examination was normal. Laboratory testing revealed normal erythrocyte sedimentation rate, complete blood count, and biochemistry (blood gas analysis, blood electrolyte, blood ammonia, and blood lactic acid). Chest radiography and ophthalmological examination results were normal. Brain computerized tomography (CT) and magnetic resonance imaging (MRI) were normal. Virus serologic test results for hepatitis A, B, and C; herpes simplex, Epstein–Barr virus, cytomegalovirus, varicella, measles, rubella, mumps, and human immunodeficiency virus (HIV) were negative. Blood culture and tuberculin skin test results were negative. Blood cellular and humoral immunity were normal. Cerebrospinal fluid (CSF) analysis and bacterial culture were negative. Electroencephalography (EEG) was normal. She had complete recovery and was discharged 3 days later.
One week after being discharged home, the girl developed unexplained personality and behavior changes including agitation, amnesia, periods of hyperexcitability, and intolerable paroxysmal skin itching. The patient was readmitted to the pediatric neurological unit, and her medical history was updated with information that the girl had played with neighbor's dog in the previous 6 months. The neurological examination was normal and the skin was free of rash. CSF analysis revealed a lymphocytic pleocytosis (18 × 106/L) with 87% lymphocytes and normal glucose and protein levels. CSF polymerase chain reaction test for HSV was negative. Toxicological screening was negative. Testing for antibodies to cell surface or synaptic proteins and oligoclonal bands of immunoglobulin (Ig)G was conducted by Peking Union Medical College Hospital. A repeat brain MRI was normal. The EEG demonstrated 14 Hz sporadic positive spikes without diffuse slow activity, and epileptiform discharges. Serum serologic tests for adenovirus, Epstein–Barr virus, Coxsackie virus, rubella virus, cytomegalovirus, and enterovirus were negative. Mycoplasma antibody was negative. A T gondii serologic test result was positive (Table ). Humoral and cellular immunity tests were normal. After the third day of her readmittance, the patient developed high fever and experienced generalized convulsions for 1 minutes. The patient then began a 10-day course of oral azithromycin. Two weeks after admission, the patient had substantially recovered with residual mild agitation remaining. Subsequently, a positive result for NMDA receptor antibodies was received (positive antibody reactivity in CSF (Fig. ), weak positive antibody reactivity in serum, other paraneoplastic antibodies, and oligoclonal bands of IgG were negative). Because of her substantial recovery from clinical symptoms, the patient's parents refused immunotherapy including gamma globulin and methyl prednisone. After completing tumor screening via chest and abdomen contrast-enhanced CT and pelvic ultrasound, for which the results were normal, the patient was discharged. She received follow-up 2 months later, and presented with complete resolution of symptoms. Toxoplasma gondii serologic test results were rechecked and the levels of serum IgM and IgG had declined notably at this 2-month follow-up (Table ).


A 37-year-old, 9-month pregnant woman presented with a dark red colored, bean-sized, pedunculated mass on her left palm (). It was first noticed 10 years previously as a tiny papule, which remained unchanged without any discomfort for a long time, but it suddenly increased in size after she conceived. As it enlarged, she began to feel pruritus and tenderness in the lesion. There was no history of trauma or infection, and palpable regional lymph nodes were not detected. A shave biopsy with complete removal was performed, and the specimen revealed that a large aggregation of neoplastic cells replacing epidermis extended into the dermis in broad anastomosing bands (). The tumor was predominantly composed of uniform, dark basophilic, cuboidal poroid cells which were united by intercellular bridges (). There was some mitosis, but no cellular atypia was found. Peripheral palisading, which is a characteristic feature of basal cell carcinoma, was not seen. The tumor cells were focally positive for epithelial membrane antigen (). The structures staining with periodic acid-Schiff stain were also observed (). Based on the above findings, the tumor was finally diagnosed as eccrine poroma. She has been under observation with no evidence of recurrence for 5 months.


A 6-year-old girl, of Turkish descent, presented with a 6-week history of right posterior cervical lymphadenopathy. She had no recent history of fever, night sweats or weight loss. Past medical history was significant for FMF with a known M694V mutation in the MEFV gene (and an r40w variant of unknown significance in the MVK gene), diagnosed at age one year and well managed with colchicine 0.3 mg orally twice/day. Complete blood count (CBC) showed white blood cell count 8.3 × 103/μl, hemoglobin 130 g/L, and platelet count 298,000/μl. Lactate dehydrogenase was 850 IU/L, uric acid 174 umol/L and ESR 2 mm/h. An excisional biopsy of a cervical lymph node showed effacement of the normal nodal architecture with a smaller population of large atypical cells with large prominent eosinophilic nucleoli and occasional classical diagnostic Reed-Sternberg cells. Immunohistochemical stains revealed positivity for CD30, CD15 and PAX5. The tumour cells were focally positive for CD3 and negative for ALK-1, CD45 and CD20. In situ hybridization for EBV-EBER (where EBER is Epstein–Barr encoding region) was positive in the majority of large atypical cells. Staging included PET computerized tomography which demonstrated localized hypermetabolic right cervical lymph nodes with the absence of bulk disease. Therefore, the final diagnosis was stage IIA classical Hodgkin lymphoma, nodular sclerosis subtype. The patient () was treated according to the local standard of care for low risk HL using 2 cycles of OEPA (vincristine 1.5 mg/m2/dose IV days 1, 8 and 15, etoposide 125 mg/m2/dose IV days 1–5, prednisone 60 mg/m2/day PO days 1–15, and doxorubicin 40 mg/m2 IV days 1 and 15) chemotherapy only.
Her first treatment course was complicated by prolonged myelosuppression including CTCAE grade 4 neutropenia, fever and severe abdominal pain. She needed to be admitted on day 8 for clinical sepsis, requiring three intravenous fluid boluses and antibiotics. Furthermore the second infusion of Vincristine and Doxorubicin was postponed to Day 16. No other signs of colchicine toxicity, such as bloody stool, renal failure or disseminated intravascular coagulation were noted. Blood cultures remained negative. She was discharged home after twelve days but needed readmission on day 22 due to severe abdominal pain and loose stools, treated with intravenous hydration and morphine for nine days. After consultation with the Rheumatology service, colchicine was discontinued from day 27 of cycle 1 onwards, to prevent possible interactions with chemotherapy. Cycle 2 was started 36 days after start of cycle 1. She tolerated this much better, without any complications and count recovery within the expected timeframe. Clinical and radiographic evaluations showed no evidence of disease at the end of therapy. The patient currently remains in remission 17 months after completion of therapy. Subsequently, she developed similar fever attacks and colchicine was resumed at the same dose 3 months after completion of chemotherapy. Her FMF has been well controlled since, without any signs of colchicine toxicity.


A 53-year-old female patient who was a known case of Type II diabetes mellitus and hypertension for 8 years and on regular medication presented with breathlessness, pain in the left lower hemithorax and in the left lumbar region associated with fever and vomiting for 4 days. Ultrasonography of thorax, abdomen, and pelvis revealed mild bilateral basal pleural effusion, nonvisualization of the left kidney with normal morphology, and location of the right kidney and associated cholelithiasis.
Plain CT of the thorax revealed bilateral mild pleural effusion (right > left) with the extension of fluid along the right horizontal fissure. Plate-like atelectasis was noted in basal segments of the left lower lobe. Air foci were also noted in subcutaneous and intermuscular fat planes of anterior, left lateral, and posterior chest wall in the left mid and lower hemithorax extending into the left axilla [].
Plain CT of the abdomen and pelvis revealed bulky left kidney measuring approximately 11.2 cm × 4.7 cm showing a well defined hypodense abscess of size 11.2 (length) cm × 3.5 (width) cm along anterolateral aspect of the left kidney with extension into perinephric space showing multiple air foci. Inflammatory thickening of left anterior, posterior, and lateroconal fascia was seen. Extension of the abscess was noted in the left anterior pararenal space, posterior to pancreatic body and tail. Inferiorly, the abscess was extending anterolateral to left psoas, anterior to left iliacus, posterior to left colon (which was compressed and displaced anteriorly) up to the left inguinal region. No radiopaque calculus or hydronephrosis or hydroureter was noted on either side. Multiple air foci were noted in retroperitoneum on the right side, anterolateral to right psoas muscle. Air foci were seen just beneath anterior abdominal wall on either side extending into intermuscular fat planes of abdominal wall muscles, in subcutaneous fat in anterior abdominal wall on the left side, extraperitoneally beneath anterior abdominal wall on either side, in perivesical fat, medial to bilateral obturator internus, between urinary bladder and uterus, and in subcutaneous fat in left posterior upper abdominal wall. Mild ascites were noted in the pelvis. A lamellated radiopaque gallstone of size 11 mm × 10 mm was noted [Figures –].
A diagnosis of emphysematous pyelonephritis of the left kidney with left renal and perinephric abscess with extension in retroperitoneum up to left inguinal region and necrotizing fasciitis of abdominal and thoracic wall was made.
Renal function tests were deranged (blood urea: 77 mg/dL and serum creatinine: 3.1 mg/dL). Urine routine and microscopy revealed abundant pus cells and sugar ++.
Drainage of the abscess with pigtail catheter revealed Klebsiella pneumoniae on culture studies []. The patient was managed conservatively with antibiotics and antidiabetics.


A 22-year-old woman with no significant medical background presented with approximately one year history of focal sensorimotor right upper limb seizures, characterised by occasional twitching and pins and needles in the right hand and forearm. Over the previous two months she also developed a slowly progressive weakness in her right hand. Seizure activity was well controlled on monotherapy with levetiracetam. An MRI of the brain demonstrated an avidly enhancing lesion centered in the left perirolandic region (involving precentral and postcentral gyri) measuring 38 × 43 × 39 mm [Figure and ] Multiple serpiginous signal voids were observed in the lesion, but no definite dural involvement, raising the suspicion of an intra-axial tumor. Given the location of the tumor, surgery was performed with the aid of preoperative transcranial magnetic stimulation and intraoperative motor mapping (monopolar stimulation, “train of 5 technique”).[] Both methods demonstrated that the primary motor cortex was located in front of the tumor, therefore confirming the location of the tumor within the central sulcus [Figure and ]. Gross total resection of the tumor was achieved with preservation of the motor mapping throughout the procedure [Figure and ].
The toluidine blue stained intraoperative smear preparation confirmed neoplastic tissue with a loose matrix and monomorphic tumor cells together with associated mast cells but no definite diagnosis could be given []. Histology showed a tumor composed of cells both epithelioid and spindle-shaped with oval, relatively monotonous, sometimes eccentric, nuclei and prominent nucleoli [Figure –]. The cytoplasm was frequently vacuolated and also there were many eosinophilic cytoplasmic inclusions in keeping with rhabdoid cells []. The background matrix was a combination of mucoid/myxoid areas and more hyalinised elements. Although there was some infiltration into surrounding cerebral tissue noted, for the most part the neoplasm was well demarcated from surrounding brain. Focal mitotic activity was evident in addition to a few necrotic foci.
Immunohistochemistry showed strong, diffuse positivity with vimentin, synaptophysin, CD34 [], calretinin and in almost all cells with smooth muscle actin (SMA). EMA was focally positive and with a paranuclear dot-like immunoreactivity in a few cells. There was focal positivity with pan-neurofilament and neurofilament 200 KD. Pan-cytokeratin, MNF116, desmin, CD31, L1CAM, AFP, STAT6, S100, IDH1, CD99, histone 3 (H3K27M) and GFAP were negative. ATRX showed preserved nuclear staining. The INI1 (BAF47) was negative in the tumor cells []. The proliferation index was estimated between 12–15% by Ki67.
There was no evidence of mutations of BRAF V600E, IDH1, and IDH2 by pyrosequencing methods from genomic DNA extracted from formalin fixed paraffin embedded tissue. There was also no evidence of a KIAA1549-BRAF gene fusion by RT-PCR analysis. The MGMT promoter was unmethylated.


A 62-year-old Korean male patient presented with jaundice and dark urine in December 2016. He had a history of hypertension and right-sided renal cell carcinoma diagnosed in September 2004, for which he underwent right radical nephrectomy in October 2004. Pathological analysis reported a conventional type, Fuhrman nuclear grade 2 tumor with hemorrhage and sclerosis (T1N0M0). He had no known family history of renal malignancy. He did not smoke. He did not excessively drink alcohol either.
Physical examination did not yield any significant findings. Laboratory results showed the following: aspartate transaminase (AST), 169 U/L; alanine transaminase (ALT), 213 U/L; total bilirubin, 2.4 mg/dl; gamma glutamyl transferase (γ-GT), 992 U/L; lipase, 221 U/L; amylase, 155 U/L; alkaline phosphatase (ALP), 650 U/L; carcinoembryonic antigen (CEA), 4.21 ng/ml; and cancer antigen 19-9 (CA19-9), 10.9 U/ml. Magnetic resonance imaging (MRI) pancreaticobiliary, positron emission tomography-computed tomography (PET-CT), and endoscopic retrograde cholangiopancreatography (ERCP) showed a firm whitish mass from the duodenum superior duodenal angle to the ampulla of Vater (). Endoscopic ultrasound-fine needle aspiration (EUS-FNA) cytology analysis revealed clusters of atypical pancreaticobiliary epithelial cells suspicious for malignancy. The initial impression was ampulla of Vater cancer with duodenal and pancreatic extension. Differential diagnoses of duodenal cancer or pancreatic cancer were also considered.
The patient underwent laparoscopic pylorus preserving total pancreaticoduodenectomy in January 2017. As a result of the previous operation, there were severe adhesions at retroperitoneum and previous renal vein ligation sites. The colon and duodenum were rotated due to the absence of the right kidney. It was difficult to dissect the superior mesenteric artery (SMA) lateral margin due to adhesion after the previous retroperitoneal space dissection. After the resection phase, pancreaticojejunostomy (duct-to-mucosa) and hepaticojejunosotmy were done via laparoscopic approach. Duodenojejunostomy was done through a small mini laparotomy around the umbilicus where surgical specimen needed to be removed. Operation time was 510 min and estimated blood loss was 400 ml. Gross findings for the specimen included a protruding mass on the ampulla of Vater (). Pathological examination revealed metastatic clear cell renal carcinoma involving the duodenum, ampulla of Vater, and pancreas (). The tumor size was 5.4 cm in maximum diameter. Lymphovascular invasion was noted without perineural invasion. All resection margins including the common bile duct, pancreatic duct, duodenum, and retroperitoneum were negative for carcinoma cells. Total retrieved lymph nodes were 13 without any positive nodes.
The length of hospital stay was 12 days postoperatively. During hospital stay, there were no significant postoperative complications. The patient was regularly followed up in Urology and Surgery Outpatient Departments to consider adjuvant chemotherapy.
Based on literature review, a total of 11 patients with renal cell carcinoma metastasized to the ampulla of Vater have been reported (). Nine patients were males and three were females. Their mean age was 62.6±11.6 years. These patients had relatively early stage renal cell carcinoma when initial nephrectomy was performed. Mean time interval from initial nephrectomy to diagnosis of metastatic renal cell carcinoma to the ampulla of Vater was 82.5±64.9 months. The most common symptom was obstructive jaundice. Palliative procedures such as ERCP with sphincterotomy, endoscopic ampullectomy, percutaneous transhepatic biliary drainage with stent insertion, and choledochoduodenostomy were performed in five patients. Pancreaticoduodenectomy was performed in seven patients, including the present case (). Mean overall survival time for patients who had metastatic renal cell carcinoma to the ampulla of Vater from initial nephrectomy was 106.3±57.9 months (95% CI: 72.6–137.8 months), excluding two cases without mentioning the survival time. Mean survival after a resection for metastatic ampulla of Vater cancer was found to be superior to that with palliative management (137±40.1 months vs. 59.5±50.1 months; p=0.00134). However, there was no statistically significant difference in overall survival between the two groups according to age, gender, or interval time from initial nephrectomy to diagnosis of metastasis to the ampulla of Vater ().


A 58-year-old female came to the emergency department with complaints of fever and cough with yellowish expectoration for the past 1 week.
General physical examination was normal. The patient was fairly built and adequately nourished, with no significant history of any infection, trauma, or malignancy in the past. The patient had no history of tobacco chewing, smoking, alcohol intake, or drug use. Nothing significant was noted in the family history.
The patient had a respiratory rate of 26/min, pulse rate was 82/min and regular, temperature was 99 degrees F, blood pressure was 130/90 mmHg, and peripheral oxygen saturation was 100%.
On examination of the chest, decreased movements were noted in the right side in the inframammary, infra-axillary, and infrascapular areas. Tactile vocal fremitus was decreased on the right side, and on percussion, dull note was heard over the same area. Breath sounds were decreased in the right inframammary, infra-axillary, and infrascapular areas.
Laboratory investigations were found to be normal. Echocardiography (ECG) showed a normal sinus rhythm, and two-dimensional ECG revealed left ventricular ejection fraction of 55% with no signs of hypertensive or valvular heart disease. A chest radiograph showed homogeneous soft tissue opacity at right mid and lower zone obscuring the underlying lung field and left hemidiaphragm, suggestive of a loculated moderate pleural effusion or elevated hemidiaphragm due to subdiaphragmatic pathology []. A high-resolution computed tomography (CT) scan of the chest was performed for further investigation. Elevation of the right hemidiaphragm was noted, with mild right pleural effusion and basal atelectasis. The rest of the lung parenchyma was normal, with no evidence of altered attenuation, mediastinal lymph node enlargement, or interstitial lung disease. A whole-body positron emission tomography (PET)/CT scan with plain and postcontrast CT was performed as a further diagnostic modality to rule out neoplastic causes. The PET/CT scan showed pulling up of the right hemidiaphragm with atelectasis of the adjacent lung parenchyma without any discrete uptake of fludeoxyglucose contrast medium, providing conclusive evidence of complete eventration of the right hemidiaphragm with shift to the left. Minimal right-sided pleural thickening was also noted []. No other significant abnormality was seen anywhere else in the body. With all of these radiological findings, a diagnosis of complete eventration of the right hemidiaphragm was made.


A 9-month-old boy, 2nd progeny of a nonconsanguineous marriage presented with generalized skin rash and fever for 1 week. Three days before the onset of symptoms, he had running nose and loose stools which settled spontaneously over the next 2 days. Routine blood investigations done before presentation revealed anemia (haemoglobin 6.8 g/dl) with borderline normal total leukocyte count (4000/cu mm). Other blood counts were not available. He received antibiotics (intravenous injection ceftriaxone and injection amikacin and also oral amoxicillin) and packed red blood cells (approximately 40 ml/kg of body weight). The indication for blood transfusion was not clear, and child was transfused with group matched whole blood (one unit) donated by his uncle. High-grade fever and rash developed 3 days following the packed cell transfusion. The rash was initially noticed on the trunk which spread over face and limbs, eyes, and involved mucosa of oral cavity. There was no history of abortions or sibling deaths in the family.
Examination revealed a sick looking child, with generalized plethoric skin with papules and vesicles filled with clear fluid along with peeling of the skin involving entire body and oral mucosa. Conjunctival membrane was involved with eyelid edema and mucopurulent discharge. He was febrile and irritable but hemodynamically stable. Abdominal examination was remarkable for hepatosplenomegaly while examination of other systems was within normal limits. Investigations of the child at presentation to our center are summarized in .
He was started on broad spectrum antibiotics. Work up done for infectious etiology including blood culture, urine culture, malarial antigen test, serology for dengue, chikungunya, leptospirosis, and rickettsia was negative. Cytomegalovirus (CMV) polymerase chain reaction in urine was positive with 12000 copies/ml which may explain the pretransfusion anemia and low normal total counts. However, possibility of transfusion associated CMV transmission cannot be ruled out. Screening for autoimmunity (Direct Coomb's test and antinuclear antibody) was noncontributory. Incidentally, serum vitamin B 12 was found to be 91 pg/mL (normal range 200–835 pg/mL), probably contributing to pancytopenia. General condition of the child deteriorated further by day 3 of hospitalization, with persistent fever and bleeding per rectum. Blood counts further decreased on day 3 with total leukocyte count 700/cu mm, and platelet count of 25,000/cu mm; however, haemoglobin remained at 16.2 g%. S. Ferritin was 2529 ng/ml. In view of fever, pancytopenia, organomegaly, raised serum ferritin a possibility of hemophagocytic syndrome was considered, and a bone marrow aspirate was performed but it was inconclusive.
On day 4, C-reactive protein was increased to 185 mg/L. However, erythrocyte sedimentation rate (ESR) was only 5 mm/h, probably reflecting hypofibrinogenemia in the present clinical context. He was treated with granulocyte colony-stimulating factor and steroids as per hemophagocytic lymphohistiocytosis (HLH) protocol. Total counts decreased to 370/cu mm at day 5 with worsening of liver functions. He succumbed to death on day 8 of hospitalization due to refractory shock. Postmortem bone marrow biopsy [] showed extensive hemophagocytosis; skin biopsy [] showed acanthosis; and presence of necrotic keratinocytes in the epidermis suggestive of toxic epidermal necrolysis (TEN) and liver biopsy revealed micro and macro steatosis. Multiple triggers, namely, CMV infection, massive blood transfusion, and Stevens–Johnson syndrome (SJS), all of which may predispose to hemophagocytic syndrome were identified in the patient.


A 55-year-old female (height, 120 cm) with OI type III (Sillence classification) was referred to us 9 months after failed treatment of a spontaneous right femoral neck fracture (). Diagnosis was based on clinical history, radiological and genetic findings. Clinical diagnosis included short stature, multiple fractures due to minimal or no trauma, delayed healing and nonunion of fractures and progressive deformities. Radiological key findings included deformities of the long bones with multiple fractures, coxa vara, protrusio acetabuli and very thin, deformed and osteopenic bones. Genetic testing showed mutation COL1A1 c.2461G>A p.(Gly821Ser) (heterozygous).
Initial treatment had consisted of two cancellous screws. In addition a small bone biopsy was done at the request of her treating internist (with consent of the patient). Postoperative radiographs noted an iatrogenic fracture at the site of the bone biopsy (). Initially this was treated non-operatively with bedrest. However, pain was difficult to control and there were no early signs of healing. At 2.5 months she could not tolerate bedrest any longer. She was taken back to the operating room and plate fixation with cerclage wires was done (). She was followed regularly for 9 months. She then requested a referral to our department where she had been treated many years prior for a left femoral shaft nonunion.
When we saw her she was taking 70 mg morphine orally, with continuous pain in her hip and hardly the ability to stand for transfer. Prior to her femoral neck fracture she was able to perform a short transfer from wheelchair to bed. Since the age of 7 years she was wheelchair bound. The last years taking morphine for progressive and severe pain and neurologic spasms related to spinal cord injury due to many vertebral compression fractures and severe scoliosis. Plain radiographs and a computed tomography (CT) showed a femoral neck nonunion as well as a subtrochanteric nonunion at the previous biopsy site, with additional deformities of pelvis and distal femur. There was very close contact between the posterior wall and the femoral neck nonunion site (). We planned a Pauwels type osteotomy to address both the femoral neck nonunion as the ipsilateral subtrochanteric iatrogenic nonunion. In addition we planned a distal femoral osteotomy to obtain better alignment of the lower aspect of the femur. She was placed supine on a radiolucent table. A lateral approach was done. All hardware was removed. We did not do a formal debridement of both nonunion sites. We first addressed the varus deformity of the distal femur. A lateral closing wedge osteotomy was done using an oscillating saw under cooling. A pelvic reconstruction plate was used with standard screws. The distal screws were drilled eccentrically to provide compression across the osteotomy site.
Next we addressed the proximal femur with the femoral neck and subtrochanteric nonunion. We anticipated a Pauwels osteotomy of 40°. At the subtrochanteric nonunion site a closing wedge was removed from the lateral cortex. Using K-wires as joy-sticks the osteotomy was aligned. The best available fit for this deformed femur was a titanium Philos plate (DePuy Synthes, Amersfoort, the Netherlands). The plate was customized to fit on the proximal femur. Screws were placed after drilling in the femoral neck using an oscillating to prevent a fausse route. A fibular allograft was wedged in between plate and bone to increase bone stock and stability. The plate was then secured to the shaft (). Post-operatively there were no complications. At 5 months a CT showed healing of the femoral neck nonunion. She was so pleased with the increased range of motion of her right hip that the Pauwels osteotomy had given her that she requested a similar procedure on the left side, which was done 9 months after the right side. This osteotomy also healed with good results for the patient (). Seven months after the left Pauwels osteotomy (and 16 months after the right osteotomy) she sustained a left distal femur fracture for which she was treated with a medial sided Philos plate and allograft bone chips.
At 2.5 years after the last surgery on her right hip a CT was taken because of increasing right hip pain when standing. The CT showed fully healed osteotomies and consolidation of the femoral neck nonunion. The most posterior screw in the right femoral neck was partly out of the neck (before re-entry into the femoral head) and was impinging on the ischium because of increasing protrusion of the femoral head into the acetabulum. This screw was removed in addition to removal of part of the greater trochanter to limit impingement. At four and a halve year she had new onset pain in her left groin during sitting. A CT showed a fresh fracture of the left ramus inferior os pubis. The nonunion of her right femoral neck was noticed to be solidly headed (). There was full incorporation of the allograft and healing of the subtrochanteric iatrogenic/pathologic fracture ().


A 43-year-old male (brother of case 1; height, 135 cm) with an OI type III phenotype (Sillence classification) was treated for a femoral neck fracture 5 months prior (). The genetic testing showed mutation COL1A1 c.2461G>A p.(Gly821Ser) (heterozygous). The CT of the fracture suggested a stress-type fracture with abutment of the posterior wall of the acetabulum against the femoral neck. There was bowing of the femoral shaft, varus deformity of the proximal femur with protrusio acetabuli and subsequent femoroacetabular impingement. Initial treatment was a Pauwels valgus subtrochanteric osteotomy of 30° and fixation of the femoral neck with a locking screw in a long spanning 4.5 LCP titanium pelvic reconstruction plate (DePuy Synthes). The osteotomy improved alignment with subsequently a better biomechanical environment for the femoral neck fracture to heal. Although a second more distal femoral shaft osteotomy had been considered to better align the remainder of the shaft, it was felt too risky at that time. Five months later there was no healing of the femoral neck fracture. The Pauwels osteotomy had healed (). There was ongoing hip pain and he could not ambulate as well as before. As there was no good alternative it was felt that further valgization might provide a more beneficial loading of the nonunion according to Pauwels' philosophy. Under general anesthesia with the patient supine on a radiolucent table we removed the previous hardware. Next, a 40° closing osteotomy at the subtrochanteric level was done. Distal to the osteotomy there was a sagittal deformity (apex anterior). To correct this, a 10° anterolateral closing wedge was done at the apex of the deformity. We now had 4 components: the proximal femoral neck and head, the rest of the femoral neck to the subtrochanteric osteotomy, the proximal femoral shaft and the distal femoral shaft. Despite numerous attempts it was not feasible to use one device to fix all 4 components. We used 2 separate plates to fix the 4 components. We were able to place two screws in the femoral neck for improved fixation and stability. He recovered without complications. At 2 months after surgery plain radiographs and a CT showed healing of the femoral neck and of both osteotomies (). At latest follow up 6.5 years after the last hip surgery he had no or limited pain and was able to walk approximately 10 meters without assistive devices or help. At work as a systems administrator he uses a walker.


A 75-year-old man was transferred to our hospital by ambulance early in the morning because of a severely impaired consciousness level with a Glasgow Coma Scale of 7. For nearly 5 years at a clinic close to his home, he had uneventfully received twice weekly HD due to diabetic nephropathy. He had been free of serious hypoglycemic episodes for several years. Approximately 40 days before the emergency transportation, he began receiving teneligliptin at a dose of 20 mg/d because of worsening glycemic control with a glycoalbumin (GA) level of 27.1%. Despite having eaten a comfortable meal on the previous night, his blood test results on his arrival at the hospital revealed a severe hypoglycemic state: 23 and 45 mg/dL in the venous and capillary blood, respectively. Teneligliptin, a DPP-4 inhibitor, was the only ongoing antidiabetic medication, which he had last taken the previous evening. His other medications and their daily dose were as follows: 75 mg of clopidogrel, 60 mg of nifedipine, 2 mg of doxazosin, 240 mg of furosemide, 20 mg of olmesartan, 5 mg of zolpidem, 3 g of calcium carbonate, and 300 mg of ursodeoxycholic acid. At the time of dialysis, he also received intravenously 6000 units of epoetin alfa, 5 µg of maxacalcitol, and 1 g of levocarnitine chloride. The patient informed us that he had taken these medications the previous evening and taken them exactly as prescribed. Thus, an ordinary cause of severe hypoglycemia was not determined, includng the prescription. He became fully conscious shortly after an intravenous injection of glucose. However, the decision to hospitalize him was determined primarily because of significant fluid retention, which was coincidentally revealed by chest computed tomography imaging (). At the time of admission, the GA level was reduced to 22.6%.
The problem of anasarca was settled by near-daily HD with little difficulty. His dry weight reduced from 55.0 to 51.0 kg. Thereafter, HD frequency was increased to 3 times weekly. In marked contrast, severe hypoglycemia reemerged immediately following recovery from hypoglycemia and required frequent glucose injections (), despite his consuming full meals. Teneligliptin was immediately discontinued, although its causal association with his hypoglycemia was not determined at the time of hospitalization. Doxazosin and nifedipine were subsequently stopped on the following day and 5 days later, respectively, to avoid a blood pressure drop due to the removal of excessive fluid by frequent HD. Zolpidem was disused throughout the hospitalization so that the patient’s conscious level could be precisely evaluated and because the patient did not call for a hypnotic agent. Incidentally, calcitriol was used as a substitute for maxacalcitol during the hospitalization because of the availability at our facility. After the following afternoon, his overt hypoglycemia eventually disappeared. After 9 days of admission, he was discharged with preprandial blood glucose levels of approximately 120 mg/dL, and no significant hyper- or hypoglycemia.


A 7-year-old girl presented with complaints of abdominal pain and vomiting that had begun 2 days earlier. Anamnesis revealed that she had persistent pain on the right side of the abdomen, had experienced bilious vomiting 3 to 4 times a day, and there was no weight loss, fever, or history of trauma. Her physical examination findings were a body temperature of 37.2ºC, heart rate of 96 bpm, and arterial blood pressure of 110/80 mmHg. There was diffuse abdominal tenderness and a mass 4x5 cm in size was palpated in the right renal bed (). Other systemic examination results were within normal limits. The hematological parameters were reported as hemoglobin: 11.6g/dL, hematocrit: 34%, mean corpuscular volume: 83.3 fL, mean corpuscular hemoglobin: 27.7 pg, mean corpuscular hemoglobin concentration: 33.2 mg/dL, red cell distribution width: %14.3, white blood cell count: 12800/mm³, platelet count: 347.000/mm³, and C-reactive protein level: 0.314 mg/dL. Results of hemochemistry and complete urinalysis were within normal limits. On abdominal ultrasound, an 8x6.5-cm mass lesion with predominant vascularization localized on the lower right kidney was detected. Doppler ultrasound did not reveal any thrombus in the renal vein or vena cava inferior. Her peak heart rate ranged between 110 and 120 bpm. In the sixth hour of hospitalization, her hemoglobin level dropped to 6 g/dL. Emergency abdominal computed-tomography was performed for suspected intratumoral bleeding and an erythrocyte suspension was infused. Abdominal tomography revealed a mass arising from the inferolateral end of the right kidney, not crossing the midline, and almost completely filling the perirenal fat capsule and the retroperitoneal region, with a predominant hemorrhagic component with indistinct borders (). The initial diagnosis was Wilms tumor. Emergency surgery was performed. The hematoma was drained and a right nephroureterectomy was performed (). Positron emission tomography and brain magnetic resonance imaging did not detect any evidence suggesting metastasis. The histopathological examination of a bone marrow biopsy specimen was unremarkable. The diagnosis of CCSK was made based on the histopathological result of nephrectomy specimen testing. Radiotherapy was performed on the tumor bed, and a combination chemotherapy consisting of doxorubicin, cyclophosphamide, etoposide, and vincristine was initiated. The chemotherapy treatment was continued for 6 months, and follow-up at 8 months revealed no further problem.


A 70-year-old woman experienced chest pain at rest. She was admitted to our institution to undergo coronary angiography (CAG) because her cardiac CT scan indicated coronary artery stenosis. On admission, her vitals were stable. Blood testing revealed hyperlipidemia. ECG, echocardiography, and chest radiography showed no specific findings. CAG showed no significant coronary stenosis, and subsequent SPT using acetylcholine (ACh) revealed bilateral spasm at the distal segment of the right coronary artery (RCA) at a dose of 50 µg of ACh, and a focal spasm at the mid-segment of the left anterior descending (LAD) coronary artery at a dose of 30 µg of ACh. Intracoronary infusions of nitroglycerin relieved the bilateral coronary spasms (Figure ). She was diagnosed with VSA and was prescribed diltiazem hydrochloride (100 mg capsule, BID) and pitavastatin calcium hydrate (2 mg tablet, QD). After 10 mo, isosorbide dinitrate patches (40 mg patch, QD) were added because her chest pain recurred four times a month. After that, however, she felt discomfort due to dermal isosorbide dinitrate, so the patches were discontinued and she was switched from diltiazem hydrochloride to benidipine hydrochloride (4 mg tablet, BID), which improved her anginal symptoms. After 4 mo, she switched from benidipine hydrochloride to a generic CCB. At that time, she began to have angina attacks four to five times a month. Therefore, we switched her back to the brand-name CCB, and she has not experienced chest pain since (Table ).


A 50-year-old man, who had previously undergone percutaneous coronary artery stenting for unstable angina and was also diagnosed as having VSA, was treated with many kinds of vasodilators. He was admitted to our institution to undergo CAG due to increased frequency of chest pain at night and during early mornings, with an overall frequency of three or four times per week over several months. His coronary risk factors included smoking (40/d × 30 years) and hypercholesterolemia. On admission, his vitals were stable. ECG, chest radiography, and echocardiography showed no specific findings. CAG showed no significant coronary stenosis, including stented segments. SPT, performed when taking vasodilators, revealed diffuse spasms in the three major coronary arteries (at doses of 30 µg of ACh for the RCA and 50 µg of ACh for the left coronary artery). Intracoronary infusions of nitroglycerin relieved the bilateral coronary spasms (Figure ). We questioned him in detail about his past medications to determine treatment options. He had been on medication [isosorbide dinitrate (20 mg tablet, BID and 40 mg patch, QD), diltiazem hydrochloride (100 mg tablet, BID), verapamil hydrochloride (80 mg tablet, at bedtime), sarpogrelate hydrochloride (100 mg tablet, BID), ezetimibe (10 mg tablet, QD), aspirin (100 mg tablet, QD), rosuvastatin calcium (20 mg tablet, QD), ethyl icosapentate (900 mg capsule, BID), and nitroglycerin (0.3 mg tablet, prn)] before admission, but the frequency of angina attacks had been increasing ever since he switched from a brand-name CCB to a generic CCB several months earlier. After we switched back from the generic CCB to the brand-name CCB, the frequency of angina attacks decreased to once per week (Table ).


A 63-year-old man with a history of a conventional cholecystectomy was referred to our department for an incisional subcostal hernia and chronic back pain. Physical exam also showed an umbilical hernia without complications and a bulging of the midline above and below the umbilicus that caused the patient an aesthetic discomfort. Computed tomography (CT) confirmed the diagnosis and showed diastasis recti measuring 6 cm [Figure –].
We decided to perform a subcutaneous video-endoscopic repair of umbilical hernia and subcostal incisional hernia with plication of both aponeurosis of the recti muscles [Figure and ]. Finally, a polypropylene mesh measuring 15 cm × 10 cm was placed in the supra-aponeurotic space (onlay) and fixed using an absorbable strap fixation device []. Therefore, we corrected all pathologies simultaneously.
The patient underwent an uneventful recovery and was discharged at 24 h post-procedure. Drainage was placed in the supra-aponeurotic space to prevent post-operative seroma formation and was removed at 7th post-operative day. His long-term follow-up was asymptomatic with no complications.


We report a case of a 75-year-old man who had a near-fatal kinking of his mammary artery graft upon sternal closure due to distended, emphysematous lungs impinging on the mammary graft.
The patient had a past medical history of severe chronic obstructive pulmonary disease (COPD) with bullous emphysema. He was dependent on home nasal cannula oxygen. Pre-operative Pulmonary Function Tests revealed an FEV1/FVC of 50%. Patient had a 60 pack-year history of smoking and had quit smoking 3 months previously. He also had coronary artery disease (CAD), previous percutaneous transluminal coronary angioplasty (PCTA), hyperlipidemia, atrial fibrillation, and peripheral vascular disease. Body mass index (BMI) was 20.95. Pre-op labs showed hematocrit of 34.6, normal coagulation parameters, a blood urea nitrogen (BUN) of 14, a creatinine of 0.9, and a serum bicarbonate of 22.9.
Patient arrived for elective CABG, but transesophageal echocardiography (TEE) also revealed the unanticipated finding of severe mitral regurgitation.
During the surgical exposure of the mammary artery, the surgeon noted that the emphysematous lung bullae were interfering with his surgical exposure. Thus, he requested that the tidal volume be decreased to facilitate his surgical exposure. This was done in a step-wise fashion down to a 300 mL tidal volume, with a compensatory increase in respiratory rate up to 22.
The patient underwent an elective CABG times three and a mitral ring placement.
The patient was able to be weaned from cardiopulmonary bypass utilizing norepinephrine infusion at 7 µg/min, epinephrine 5 µg/min, and milrinone 5 µg/min.
Subsequently, after the sternum was wired closed, the patient suffered a severe hemodynamic deterioration requiring emergent re-opening of the chest. No surgical bleeding was found in the chest, nor was pericardial fluid observed. Careful surgical examination revealed kinking of the mammary artery to LAD graft upon sternal closure due to distended, emphysematous lungs impinging on the mammary graft.
The patient was re-heparinized. The mammary artery was transected close to its origin at the subclavian artery and clipped. To verify that the artery and graft were otherwise patent, the mammary artery was back-bled, indeed verifying sufficient flow. The combination of regional wall abnormality on transesophageal echocardiography, visible tension on the mammary artery, and otherwise free flow in the grafted vessel confirmed the etiology of emphysematous lung kinking of the artery. With an off-bypass technique, the surgeon then fashioned a proximal anastomosis of the free LIMA onto the vein graft of another vessel. Flow was confirmed with Doppler ultrasonography, and perfusion was confirmed by verifying regional wall motion on transesophageal echocardiography.
In this position, the graft no longer was impinged upon by the distended emphysematous lungs. Subsequently, the patient’s sternum was able to be closed without hemodynamic impingement. The patient was transferred uneventfully to the post-op cardiac care intensive care unit.


A 59-year-old man with hypertension presented with choking easily, muffled voice, and foreign body sensation of the throat for 2 months. He had a history of stroke attack twice in 2010 and 2013. Aspirin was used for the prevention of recurrent ischemic strokes and he abstained from alcohol and cigarettes. The patient visited our institution for professional assistance. Flexible nasopharyngoscopy showed multiple exophytic tumors in the larynx and pharynx, especially involving the supraglottis. The tumors’ growth pattern presented as a multifocal mass disseminating on the aryepiglottic folds, glottis, pyriform sinus, vallecula, and oropharynx. The tumor nearly obstructed the airway and the glottis was not visible (Fig. ). Postgadolinium T1-weighted magnetic resonance imaging (MRI) revealed multifocal heterogenous enhancing tumors primarily involving the supraglottis with invasion through the thyroid cartilage. The left tonsil, bilateral aryepiglottic folds, bilateral pyriform sinus, and glottis were also involved further leading to airway narrowing. MRI of the neck confirmed several enlarged bilateral level II lymph nodes with central necrosis suggesting cervical metastasis (Fig. ). Computed tomography of the chest revealed multiple nodules, up to 1.4 cm in size, in both lungs, which is compatible with lung metastasis.
Since the airway was obstructed by the tumor, CO2 laser debulking was performed to remove the laryngeal and hypopharyngeal tumors to establish a secured airway.[ Pathology revealed that the tumor was mainly located in the submucosal layer with invasion of the squamous mucosa. It was composed of nests of moderately differentiated cuboidal to low columnar epithelial cells in solid, cribriform, and tubular arrangements (Fig. A and B). The tumor cells had a pale eosinophilic to cloudy cytoplasm and enlarged nuclei with frequent mitoses and a high nuclear proliferative index ratio (20%). An infiltrative growth pattern of the tumor cells with lymphovascular invasion (LVI) and tumor necrosis was also found. Immunohistochemically, the tumor cells were diffusely positive for cytokeratin 7 (CK7) and androgen receptor (AR) stains, and some tumor cells were also positive for GATA binding protein 3 (GATA-3) stain (Fig. C–E). After CO2 laser debulking, the respiratory and swallowing functions improved significantly and tracheostomy was avoided. Furthermore, the patient was treated with cisplatin-based chemotherapy that was administered concurrently with radiotherapy for the treatment of the primary lesion and the enlarged bilateral neck lymph nodes. The patient has survived for >1 year after the initial diagnosis.
Written informed consent for publication of clinical details and accompanying images were obtained from the patient. Because it was a case report without any research involving human beings or experimental subjects, the ethical approval was not required in our institute.


A 29-year-old healthy female presented to the musculoskeletal oncology department with increasing right-hip pain for the past 8 years. The patient was working as a bar manager and described increasing pain that was interfering with activities of daily living and sports. Physical examination showed a normal gait pattern. The patient exhibited full range of hip motion with pain on flexion adduction internal rotation (FADDIR), and tenderness on palpation of the anterior aspect of the groin. AP pelvis radiographs demonstrated a radiolucent lesion at the inferior margin of the right femoral head confirmed by computed tomography (A and B). Magnetic Resonance Imaging (MRI) showed a subcortical lesion within the inferior margins of the right femoral head, sized at 14 × 20 × 14 mm with a lobular appearance without cortical breaching or edema.
Due to continued pain and limitations of activity, it was recommended that the patient proceed with arthroscopic resection. Surgery was performed without traction. The proximal anterolateral portal was first established for the visualization of the peripheral compartment. A large lesion in the medial aspect of the femoral head was demonstrated (C). A distal anterolateral portal was then established under direct visualization penetrating the capsule medial to the viewing portal. Capsulotomy between the two entry points was performed with a hooked radiofrequency probe (VAPR; DePuy Mitek, Raynham, MA, USA) to facilitate instrument maneuverability. Curette, shaver and burr were used to facilitate complete tumor resection (D). The capsule was repaired using absorbable sutures. The pathology report confirmed benign enchondroma. At 15 months post-operatively the patients Modified Harris Hip Score (MHHS) and VAS scores had improved from 84 and 5 pre-operatively to 100 and 0 post-operatively. The patient was also able to return to jogging and Pilates post-operatively without pain.


A 39-year-old female presented with 3 years of progressive left hip and groin pain with limitations to her activities of daily living. The pain was exacerbated by positional changes (standing from a seated position), walking and accompanied by a clicking sensation around the hip joint. On physical examination, the patient walked with a mild-antalgic gate. Tenderness was elicited upon palpation of the left groin. The patient exhibited full range of motion both passively and actively. AP pelvis radiographs and a CT scan revealed a triangular bony protuberance emanating from the medial aspect of the femoral neck proximal to the left lesser trochanter (A).
The patient underwent arthroscopic excision of the lesion. Due to the peripheral location of the tumor traction was not applied and no distal fixation of the limb was used during the procedure. The entire limb was prepped and draped to allow unrestrained motion of the hip during the surgery. A proximal anterolateral portal was established for the direct visualization of the lesion. The leg was then mobilized into the figure-of-4-position, in order to bring the medially located lesion into an anterior position and to relieve tension of the Iliopsoas tendon (B). The distal anterolateral working portal was established under direct visualization. The acetabular labrum and the femoral head–neck junction appeared to be intact. A bony prominence proximal to the lesser trochanter was detected with associated signs of irritation and impingement on the iliopsoas tendon. The tumor was excised using curved osteotome, burr and shaver (C–E postop radiograph). The pathology report confirmed benign osteochondroma. Post-operatively, the patient complained of anterolateral numbness in the distribution of the lateral femoral cutaneous nerve, which resolved completely by 3 months. The patient was able to return to full activity without limitation in motion or pain on activity. The pre-operative MHHS improved from 85 to 96 at 24 months post-operatively.


A healthy 20-year-old male presented to the musculoskeletal oncology department after incidental diagnosis of a cystic lesion located in the left femoral head (A). The lesion was found incidentally after the patient underwent an MRI of the lower abdomen for suspected appendicitis. Six months after the incidental finding the patient began to complain of left groin pain.
The patient had hip pain with flexion abduction and external rotation and a negative FADDIR test. CT scan demonstrated a lobular lesion in the epiphyseal region of the femoral head sparing the articular surface (B). The lesion measured 2.3 × 15 × 1.6 cm. Due to continued symptoms and the subchondral location at the weight bearing zone of the femoral head, hip arthroscopy including biopsy samples, debridement and curettage was suggested. Due to the size and location of the lesion bone substitute was used to fill the cavity.
Hip arthroscopy was first performed with traction for evaluation of possible articular penetration of the lesion. A lateral portal was established under fluoroscopy and then a mid-anterior portal [] was created under arthroscopic visualization with the aid of fluoroscopic guidance. Capsulotomy between the two entry points was performed with a hooked radiofrequency probe (VAPR; DePuy Mitek, Raynham, MA, USA) to facilitate instrument maneuverability. The femoral head was probed and appeared to be intact (C). Traction was then released and the peripheral compartment was inspected. The location of the lesion was identified using fluoroscopy (D). The capsule was further divided in a T shape. A small window on the lateral cortex of the femoral head was created. The lesion was excised using a curette and a shaver to the underlying bleeding bed of bone (E). The cavity was than filled with an osteo-conductive (HA) Bone Paste (BoneSource BVF, Stryker, Mahwah, NJ, USA) (F). The capsule was closed at the end of the procedure and the pathology report confirmed benign enchondroma. Post-operative radiographs at 18 months after surgery showed marked increased density of the femoral head (A and B). The patients MHHS and Visual Analogue Scale score were improved from 74 and 6 pre-operatively to 95 and 0 post-operatively with 30 months follow up.


A 26-year-old male was referred to the nuclear medicine department by the rheumatologist with a 3-year history of polyarthralgia. The pain was initially involving knees, followed by the wrist and subsequently the ankles and small joints of hands and feet. The pain was progressive in nature and associated with swelling in joints. No history of early morning stiffness. He also noted some change in the shape of his nails since his childhood. Other symptoms to suggest chest or abdominal disease were absent. His father also had a history of nail changes, otherwise family history was unremarkable. No history of any dermatological problem.
On examination, Grade III clubbing was present in the fingers and toes [Figures and ]. The distal end of both forearms was swollen. No swelling was present elsewhere in the other joints. No clinical evidence of skin lesions noted. Examination of other systems was unremarkable.
On further investigations, his erythrocyte sedimentation rate was 34 mm in 1st h, and hemoglobin, leukocyte, and other cell counts were found to be normal. Liver function test was within normal limits. Human leukocyte antigen B-27, rheumatoid factor, and anti-cyclic citrullinated peptides were negative. X-ray chest and knee joint findings were normal. Echocardiogram study showed no evidence of pulmonary artery hypertension.
Skeletal scintigraphy was performed 3 h after the administration of 740 MBq/20 mCi of Tc-99m methylene diphosphonate (MDP). Planar images showed diffusely increased tracer concentration in the distal third of bilateral radius and ulna with expansion of epiphyseal region, symmetrical increased tracer concentration along the cortical margins of bilateral distal femorae, entire shaft of tibiae and fibulae []. Increased tracer concentration is also seen in the distal phalanges, wrists, MCP joints of both hands, metatarsal and intertarsal regions of both feet []. Single-photon emission computed tomography (CT) with low-dose CT of the wrist was done which showed increased tracer concentration along the entire width of distal third of bilateral radius and ulna. Corresponding CT shows evidence of periosteal thickening bilaterally with areas of minor lucency in the left distal radius suggesting possible acrolysis []. Increased tracer concentration is also seen in distal phalanges with soft-tissue swelling (consistent with clubbing).


A 19-year-old primigravida, vaginally delivered at home, presented after 9 days with complaints of abdominal pain and distension for 4 days and breathlessness for 2 days. On examination, her general condition was poor. Patient was dehydrated with pulse rate of 130/min, blood pressure of 80/40 mmHg and respiratory rate of 40/min. Her chest auscultation did not reveal any abnormality. On abdominal examination, diffuse tenderness was present. She was resuscitated initially with fluids and then noradrenaline infusion was started at 5 μg/min. Ultrasound abdomen revealed hypoechoic collection of approximately 800–900 ml. Exploratory laparotomy was done under general anaesthesia and 1 L of pus was drained. Patient was shifted to (ICU) for mechanical ventilation in view of sepsis and hemodynamic instability. During her course of stay in ICU, sepsis was controlled and she became haemodynamically stable. Ionotropes were gradually tapered and stopped. However all the efforts of weaning her off the ventilator proved futile. Patient continued to be lethargic, stuporous and was not obeying verbal commands. On 7th postoperative day, she developed hypertension (153/90 mmHg) for which amlodipine 5 mg once a day was started. On the 8th day, she passed a tapeworm in stools which were followed by two episodes of generalised tonic clonic seizures lasting about 30 s each. Loading dose of phenytoin 1000 mg was administered intravenously which was followed by 100 mg thrice daily. Computed tomography (CT) scan was planned keeping the diagnosis of neurocysticercosis in mind. However CT head revealed hypodensities in bilateral occipital lobe which the radiologist diagnosed as PRES. After the diagnosis was made, dose of amlodipine was increased to 10 mg OD to keep blood pressure <130/90 mmHg. Intravenous Dexamethsaone 8 mg thrice daily was added to the treatment. Gradually her level of consciousness improved. Trachea was extubated on day 11 and she was shifted to ward on day 15 without any neurological sequelae.


An 18-year-old primigravida term pregnancy with pregnancy induced hypertension on irregular treatment, presented to emergency with a history of six episodes of seizures. On examination, patient was drowsy but arousable. Her pulse rate was 120/min and blood pressure was 156/100 mmHg. Her chest auscultation revealed no abnormality. She was initially managed conservatively on magnesium sulphate (MgSO4) administered intravenously and tab labetalol (100 mg thrice a day). Labour was induced on admission. However, patient developed another episode of seizure 4 h later. Immediate vaginal delivery, with birth of a healthy female baby, was carried out. Patient developed a large episiotomy hematoma, which was drained under general anaesthesia as the patient was drowsy and not maintaining her airway reflexes. Patient was shifted to ICU postoperatively for mechanical ventilation and observation. The patient however remained agitated with opisthotonic posture despite midazolam infusion. Hence, midazolam was substituted by diazepam and intermittent boluses of sodium thiopentone were given for control of convulsions. For control of persistent high blood pressure, infusion of sodium nitroprusside (0.5 μg/kg/min) along with tab amlodipine through nasogastric tube was started. As the patient remained agitated despite above treatment, CT head was planned to rule out intracranial bleed or oedema. CT head revealed hypodensities in bilateral parietal and frontal lobes suggestive of PRES. Hypertension was aggressively controlled by titrating the dose of sodium nitroprusside. Injection dexamethasone was added. Patient was mechanically ventilated for 3 days and observed in ICU for a further 4 days. She was shifted to ward without any neurological sequelae.


A 20-year-old male presented with a painful gait and slow mass growing in his right calf for 3 years. The mass was 6 × 4× 3 cm in size and was firm and nontender. X-ray of the right tibia and ipsilateral femur showed similar lesions with characteristic ground glass appearance suggestive of fibrous dysplasia. Further, X-ray of the skull showed an osteoma.
Fine needle aspiration of the calf mass yielded clear, gel-like material and the tibial lesion yielded blood. Smears prepared from the gelatinous material showed few spindle cells in a myxoid background. These spindle cells had oval-to-spindled nuclei with bland chromatin along with long cytoplasmic processes that were intertwined at areas. Few thin-walled capillaries were also noted []. Cytological diagnosis of an intramuscular myxoma was made.
Smears from the tibial lesion yielded only blood along with a few scattered bland appearing spindle cells. Definite opinion was not possible and biopsy was advised.
Histology of biopsies from the tibial and femoral lesions showed curvilinear trabeculae of metaplastic woven bone in hypocellular and fibroblastic stroma without osteoblastic rimming; a diagnosis of fibrous dysplasia was made.
The combined radiological, cytological, and histopathological findings combined were consistent with the diagnosis of Mazabraud's syndrome.


A 27-year-old male patient had a history of road traffic accident (RTA) in 2010, leading to right BPI. Postinjury he was managed conservatively and had been on physiotherapy since then with no sign of motor and sensory recovery. His electromyography (EMG)-nerve conduction study findings confirmed the right BPI with right median and ulnar-radial sensory nerve action potential reduced and compound muscle action potentials absent.
After 15 months of the RTA, on examination, he was hypotonic and hyporeflexia with grade 0 muscle power in the right adductors, abductors external rotators, and biceps. Voluntary control was affected in the right hand. Hand functions were poor on the right side. Sensations had returned partly in the median part but were absent in the lateral side of the right arm. For assessing the degree of independence in activities of daily living (ADL), functional independence measure (FIM) scale was used and the score was 125. Functionally, he was independent in most ADL and mobility but needed assistance in tasks like tying a shoe lace, etc., where the bilateral hand and finger movements were required. The magnetic resonance imaging of cervical spine revealed C7-D1 pseudomeningocele in the right side representing traumatic nerve root avulsion with posterior central protrusion of C4-C5 disc with mild bulge indenting thecal sacs and mild degenerative changes in the cervical spine. Muscle strength was assessed using modified Medical Research Council's Manual Muscle Testing scale (mMRC-MMT). The details of the muscle strength charting of all the muscles are given in . EMG reports showed no response in the musculocutaneous biceps and axillary deltoid muscles suggesting complete denervation with no sign of reinnervation.
The selection of the patient was based on the World Medical Associations Helsinki Declaration.[] The protocol was reviewed and ethical approval was taken from the Institutional Committee for Stem Cell Research and Therapy. The procedure of cellular therapy was explained in detail, and a duly filled informed consent was obtained from the patient prior to the therapy.
On the day of transplantation, 100 ml bone marrow was aspirated from the left anterior superior iliac spine under local anesthesia using bone marrow aspiration needle and was collected in heparinized tubes. The BMMNCs were separated from the aspirate using density gradient method. Manually, the cell viability was calculated using trypan blue dye which was confirmed by TALI machine using propidium iodide. Fluorescence-activated cell sorting analysis showed CD34+ count to be 0.86%. The separated cells were diluted with cerebrospinal fluid as it has factors that are known to promote cell growth.[]
The cells were injected intrathecally at the level between L4 and L5 and intramuscularly at total 19 specific motor points on the right upper limb at the following muscles: supraspinatus (1), infraspinatus (1), deltoid (3), pectoralis major (1), biceps (1), triceps (1), brachioradialis (1), flexor carpi radialis (1), flexor carpi ulnaris (1), thenar, lumbricals (3), interossei (3), adductor pollicis (1), and extensor digitorium (1). Simultaneous intravenous administration of 1 g methyl prednisolone in 500 ml of Ringer's lactate solution was carried out to decrease immediate inflammation and to enhance the survival of the injected cells. A total number of cells injected were 219 × 106 with 94% viability.
Following transplantation, he underwent multidisciplinary neurorehabilitation. Physiotherapy consisted of suspension exercises while giving resistance to supraspinatus muscle. Occupational therapy included scapular strengthening exercises (for trapezius, rhomboids, and serratus) and theraband exercises with the help of unaffected hand. He was advised to continue all the above exercises twice in a day. Counseling was provided by a psychologist to cope better with the disease. Pre and post the intervention, routine blood tests were performed to monitor the adverse events.
The patient was discharged at 1 week posttransplantation and was advised to continue the rehabilitation at home. The follow-up assessment was conducted after 3 and 7 months after the therapy. In view of the improvements observed after the treatment, the patient underwent second cell transplantation 8 months after the first transplantation. The transplantation procedure was replicated.


The authors report a case of a 64-year-old female, with no relevant family history and a past medical history of recently diagnosed supraventricular tachycardia, arterial hypertension, type 2 diabetes mellitus, euthyroid multinodular goiter and chronic venous insufficiency. She was referred to our Institution from other Hospital where she went due to paroxysms of palpitations, headache and diaphoresis, that led to biochemical investigation that was remarkable for: total plasmatic cathecolamines: 73 747 ng/L (normal range (NR) <598 ng/L), plasmatic noradrenaline: 73 589 ng/L (NR <420 ng/L), adrenaline: 130 ng/L (NR <84 ng/L), plasmatic dopamine: 28 ng/L (NR <94ng/L), aldosterone: 87,5 ng/dL (NR 4–31 ng/dL), plasmatic renin activity: 33 ng/mL/h (NR 0,5–4 ng/mL/h), Vanylmandelic acid: 39,2 mg/24 h (NR <13,6 mg/24 h). Abdominal contrast enhanced CT scan revealed a large (70 × 35×78 mm3), hyperdense (20 HU) right adrenal mass. Abdominal magnetic resonance (MRI) also described an adrenal mass with 66×33 mm2, suggestive of pheocromocitoma (slightly hypointense on T1 and markedly hyperintense on T2 weighted imaging). No vascular nor locoreginal lymph node involvement were found and the left adrenal was radiologically normal. The patient underwent genetic testing with next generation sequencing, that excluded mutations of the succinate dehydrogenase (SDHB, C, D and A), MEN2, VHL, Neurofibromatosis type 1 genes. Genetic testing for MAX and TMEM127 were not performed due to institutional unavailability. Pre-operative pharmacological therapy was initiated with phenoxybenzamine (10 mg twice a day), followed by bisoprolol (20 mg/day) and amlodipine (5 mg/day), which rendered the patient fit for surgery. The patient underwent transperitoneal laparoscopic right adrenalectomy. Intraoperatively, a hipervascularized right adrenal gland with adhesions to the upper pole of the kidney was identified and excised, with no complications recorded. In the immediate post-operative period, antihypertensive drugs were stopped and blood pressure remained normal until the second post-operative day, when hypertension and tachycardia recurred, leading to urinary metanephrine reevaluation on the 10th post-operative day. The results were highly suggestive of disease persistence (urinary normetanephrines >10 500 ng/L (NR <600 ng/L)). Histology revealed no signs of neoplasia. Abdominal CT scan revealed the persistence of a solid heterogeneous nodule, with 70 × 49 × 87 mm3 (AP × T × L) adjacent to upper right renal pole, with peripheral contrast enhancement and central necrosis (Figs –). This findings suggested abdominal paraganglioma (of the organ of Zuckerkandl). Due to the higher malignant potential of paragangliomas, an 123I-MIBG scintigraphy was performed, excluding metastatic disease.
One month later the patient was reoperated via an open approach, which revealed a tumoral lesion in the inter-aorto-cava location, from the right diaphragmatic pillar to the right renal artery. No intraoperative complications were reported and the post-operative period was uneventful. Histological result revealed an 84 g capsulated, solid tumor, with 80 × 60 × 25 mm3. Immunohistochemistry revealed positivity for chromogranin and synaptofisin, compatible with paraganglioma (Figs –). No vascular, lymphatic or capsular invasion was ocumented.


A 16-month-old male infant was admitted with severe developmental delay and a history of infantile spasms since 3 months of age. He was markedly hypotonic with abnormally coarse hypopigmented curly hair. Serum copper and ceruloplasmin values were 2.5 μmol/L and 6.05 μ/L, respectively. Electron microscopy of the hair showed typical changes of pili torti. MRI and MRA at 14 months of age showed excessive vessel tortuosity with cerebellar atrophy and some periventricular deep white matter lesions [Figures and ]. He remained stable on two anticonvulsants. Developmental progress was limited. He developed bladder diverticula at 2 years of age. He is currently 4 years old and has recurrent admissions for lung aspiration and seizures.


A 3-year-old boy (from Torbat-e Jam, Khorasan, Iran) with a chief complaint of abdominal pain and pain in both hips, much on the right side, was presented to the Pediatric Emergency Department of Imam Reza Hospital in Mashhad, Iran. The patient was examined in the Pediatric Emergency Unit. He had mild fever and severe tremor, and on examination, abdominal distention and severe sweating were overtly observed. His vital signs were respiratory rate = 32/min, pulse rate = 89/min, blood pressure = 139/93 mmHg, and temperature = 36.8°C. The history was negative for trauma, use of drugs, previous diseases, or fever. The initial examination of the hip and abdomen had no important clinical symptoms except for abdominal distention. Generalized tremor and abdominal pain continued. Imaging was performed, and the chest and abdominal radiography showed generalized ileus. Sonography of the abdomen and hip was normal. Laboratory analysis was also performed, and the urine analysis was normal. Further laboratory data are shown in .
Given the high prevalence of brucellosis in Iran, an evaluation utilizing Wright and 2ME standard tests was performed which was negative. The abdominal pain continued, so abdominal sonography was performed and the result was normal. The imaging for kidney, urine, and bladder was normal too. Therefore, according to the laboratory data and imaging, several nondigestive causes of abdominal pain in children such as pneumonia, hydronephrosis, psoas abscess, pelvic osteomyelitis, and pyelonephritis were ruled out. Pediatric surgical consult was requested for the patient and the surgical problems were also ruled out.
Due to the ongoing symptoms such as severe abdominal distention and tremor, a toxicology counseling was requested. On a second history taking from the parents, we found that the patient's father was a farmer and the boy was in the farm with his father prior to the onset of the symptoms. There was also a hesitant history of sudden burning sensation on the right malleoli, hence the diagnosis was made based on the history and symptoms as a spider bite. After the use of acetaminophen and midazolam with appropriate dose, pain was recovered, and the patient discharged without any complaint after 4 days.


A 46-year-old man visited our emergency room because of a 7-cm continuous laceration from the left temple to the lower eyelid caused by a sharp metal plate during work. The laceration margin was irregular, and partial tearing of the orbicularis oculi muscle was observed. Primary closure was performed under a local anesthetic in the emergency room, and sutures were removed on postoperative day 7. Some partial necrosis was observed at the suture site margin, and dressing changes were performed until postoperative day 13. In the first year after the wound closure, the patient returned because he was facing difficulties and aesthetic problems when opening the eye due to the scar fold at the suture site (). There was a severe scar fold in the lateral canthus of the patient’s eye such that the width of the horizontal orbital fissure of the right eye was much narrower (26 mm) than that of the left eye, and the discrepancy in the width of the horizontal orbital fissure of the left and right eyes was 0.4 mm. We designed a Z-plasty technique to release the scar fold. However, there are several drawbacks to the classic Z-plasty: when a smaller “Z” is designed, the smaller transposition flap makes it impossible to sufficiently widen the length of the horizontal orbital fissure, and when a larger “Z” is designed, the incision becomes longer, which results in a long scar forming near the lateral canthus. Moreover, baggy skin was observed in the lower eyelid due to the post-traumatic scar (); thus, designing a classic Z-plasty would have resulted in a dog-ear in the lower eyelid. Therefore, we used a modified Z-plasty design to ensure a smaller postoperative scar and simultaneously resolve the issue of the baggy skin in the lower eyelid.
First, we marked a dot on the point “C” where the previous scar line and newly formed fold met. Second, along the previous scar line, we marked a dot on the point “B” located 0.5 mm lateral to point C. Lastly, according to the standard “BC” line, we drew isometric “AB” and “CD” lines in a 60º direction. For the classic Z-shape design, to simultaneously excise the baggy skin in the inferior part of the lower eyelid, the CE line was drawn as an extension of the BC line, and the DE curve line was drawn to include the baggy skin for excision. Here, the DE and BE were designed to be similar in length (). A local anesthetic was infiltrated into the incision site, the lateral canthal region, and the lateral portion of the upper and lower eyelid. Skin incisions were made from point A to point D and from point C to point E. The skin flap was elevated with intramuscular dissection by cutting the dense connective tissue between the skin and the orbicularis muscle of the lower and upper lateral canthal areas.
After excision of the fan-shaped CDE area, the ABE skin flap was turned down, and the BCD skin flap was turned up (i.e., point C was moved toward point A, and point B was moved toward point D). Then, the skin flap was sutured with 6-0 silk (). Sutures were removed on the fifth postoperative day. There was no significant functional problem in opening the eyes during the 8-month follow-up, and the width of the right horizontal orbital fissure had improved to 29 mm ().


A 50-year-old woman referred to our hospital with decrease of consciousness after traffic accident (pedestrian hitted by motorbike) 17 h before admission. Her initial GCS score was E3V2M5 (10/15), pupils bilaterally were in normal size, and there was no hemiparesis. The patient had left occipital swelling. The brain CT scan examination showed the presence of infra- and supra-tentorial EDH [], linear fracture on left temporal bone, diastasis fracture on left lambdoidal suture, intracerebral hematoma (ICH) burst lobe on frontal and temporal base regions, and brain edema. The EDH was evacuated immediately. After surgery, the patient showed an increasing recovery without any neurological deficit in the postoperative period. On postoperative CT, there was no residual supra- or infra-tentorial hematoma []. She was discharged on the 17th postoperative day.


A 38-year-old woman referred to emergency room with a history of fallen down 4 days before the admission from a tree of three meters height. On examination, her GCS score was E1V1M4 (6/15), pupils bilaterally were in normal size, and sluggishly reacted with no hemiparesis. The patient had left occipital swelling. The brain CT scan of brain was done and revealed infra- and supra-tentorial EDH [], linear fracture on left temporal bone, diastasis fracture on left lambdoidal suture, and brain edema. In view of poor GCS score and CT scan finding, the patient was taken up for surgery for evacuation of EDH. After surgery, she was consulted to head-and-neck surgeon for tracheostomy. Her postoperative GCS was E2VtM4. Unfortunately, the patient died on the 11th postoperative day.


A 31-year-old man was transferred from local hospital to emergency room in our hospital for further assessment after traffic accident in unconscious condition 20 h before admission. On initial assessment, his GCS score was E3V4M5 (12/15), pupils bilaterally were in normal size, and there was no hemiparesis. He had left occipital swelling and was very agitated. The brain CT scan was done and showed infra- and supra-tentorial EDH [] and linear fracture on left parietal and occipital bone. The patient then was transferred urgently to operating theater and underwent evacuation of the hematoma. Postoperatively, the patient made a good recovery. He was discharged on the 5th postoperative day.


A 35-year-old male patient with no known medical illness presented with a history of bilateral lower limb ascending weakness for the past 2 months associated with numbness of the lower limbs and instability during walking. The onset of the symptoms was insidious and gradual progressive in nature. There were no history of trauma, spinal surgery, infection, or spinal anesthesia. There was no urinary or bowel involvement. On examination, the power of both lower limbs was 4/5. Deep tendon reflex was bilaterally brisk, Babinski's down going. There was reduced sensation from T6 below. Magnetic resonance imaging (MRI) of the spine revealed well-defined, nonenhancing extradural cystic lesion within the spinal canal measuring 1.8 cm × 2.7 cm × 5.2 cm extending from mid T5 to T6 vertebrae segment. It appeared hypointense on T1 and hyperintense on T2, compressing spinal canal anteriorly []. Patient underwent T5, T6 laminoplasty, T4 partial laminectomy and excision of cyst on June 6, 2016. The cystic membrane was exposed and excised []. After excision of the cyst the dorsal portion of the cord gradually regained its normal diameter and adequate cord pulsation returned. The postoperative period was uneventful. The patient had a complete neurological recovery with the power of both lower limbs 5/5 on the 3rd postoperative day. Histopathological examination of the resected specimen confirmed the diagnosis of an arachnoid cyst []. On regular follow up, he could walk without difficulty with no limb weakness.


A 6-year-old boy presented to the emergency room in the evening hours with a complaint of an acute painful left inguinal swelling noticed since morning. There was no history of vomiting, distension of the abdomen, or fever. There was no history of similar episodes. On examination, he had tachycardia; cardiovascular system, respiratory system, and abdominal examination were normal. Groin examination revealed a tender, irreducible swelling in the left inguinal region with normal bilateral descended testes, leading to a working diagnosis of strangulated inguinal hernia. However, a quick ultrasonography showed a soft-tissue swelling as the content of hernia with reduced vascularity adding the possibility of torsion of a supernumerary testis. With these two diagnoses in mind, the child was taken up for an emergency inguinal exploration.
Left inguinal exploration revealed an indirect left inguinal hernia with no bowel or omentum in it, but an elongated, oval, firm, purplish-blue structure connected distally to the upper pole of the left testis by a fibrous cord and proximally entering the peritoneal cavity by a fibrous cord. The fibrous cords on either side had undergone torsion by 360*, leading to early gangrenous changes []. The firm swelling was excised by dividing the fibrous cords from the upper pole of the testis (taking care to avoid injury to the testis) distally and as high as possible proximally. Herniotomy was done. Oral feeds resumed on the same day, and the child was discharged on the 1st postoperative day. Histopathology revealed a fibroelastic capsule covering a tissue comprised of red pulp, white pulp, and dilated sinusoids, consistent with an ectopic spleen. There was no testicular tissue noted in the excised specimen, clinching the diagnosis of SGF, the continuous type []. Ultrasonography abdomen showed normal orthotopic spleen. Subsequent follow-ups were uneventful.


We present a case of 71-year-old male with the histories of untreated arterial hypertension, tobacco consumption and a large incarcerated umbilical hernia that requires surgical treatment. The patient came to the emergency room with a two hour history of sudden swelling, cyanosis and coldness of his left lower limb (LLL). He also complained of severe pain and functional impotence of the limb. Upon examination, he presented a blood pressure of 154/88 mmHg, pulse rate of 93 beats per minute and an oxygen saturation of 97%. Abdominal examination revealed no guarding or rebound tenderness. He also presented with cyanosis and cold skin in the LLL (). The distal pulses were not palpable but there were no signs of neuronal damage.
Blood tests performed at the emergency department showed hemoglobin 15.4 g/dL, platelets 234,000/µL, leukocytes 7,700/µL. The coagulation tests were normal. Arterial blood gases showed pH 7.44 with pCO2 29 mmHg and pO2 48 mmHg. Chemistry test, including creatinine 0.78 mg/dL, glucose 106 mg/dL, alanine transaminase (ALT) 35 IU/L, and aspartate transaminase (AST) 30 IU/L, were normal. Cardiac markers were not tested. A chest X-ray showed right fibrothorax that was already present in a previous X-ray performed 7 years ago.
Upon admission a computerized tomography (CT) angiography of the LLL was performed (), which showed a hyperacute and extensive proximal DVT affecting the iliac vein, common femoral vein, femoral vein, and popliteal vein. CT also showed a partial external compression of the external iliac vein by an adenopathy. Arterial blood flow within the LLL was diminished, but there was residual flow. These findings were compatible with the clinical diagnosis of PCD. A CT of the thorax and abdomen was also performed, and revealed a segmental PE in the lower lobe of the right lung. It also confirmed the presence of retroperitoneal adenopathies and a prostatic node. A large umbilical hernia was also observed, with no signs of complication. The prostate-specific antigen (PSA) level was 124 ng/mL and antiphospholipid antibody tests yielded negative results. A transthoracic echocardiogram showed normal right ventricle function with a pulmonary artery systolic pressure of 22 mmHg.
The patient was treated with unfractionated heparin during the first 48 hours, along with Trendelenburg position and compression stockings. He was admitted to the Venous Thromboembolism Unit of the Internal Medicine Ward. Treatment was then switched to bemiparin, the low molecular weight heparin (LMWH), with a dose of 10,000 IU per day (115 IU/kg/day). The patient showed general improvement, including less pain, edema reduction, and progressive disappearance of cyanosis in the LLL.
Given the proximal extension and severity of the DVT and the patient's stability, mechanical removal of the thrombus was considered. However, the patient presented a large umbilical hernia that rendered him unable to lie in the prone position for the duration of the procedure, which was therefore dismissed.
In the fourth day of bemiparin treatment, the patient developed a drug induced rash. Allergy to LMWH was confirmed and treatment was then switched to apixaban. Nine days after admission, he was discharged on apixaban and compression stockings. Considering the local compression of the iliac vein by local adenopathy, the patient was referred with priority to the urology outpatient clinic for diagnosis and treatment of prostate cancer.
A week later, the patient underwent a prostatic biopsy, which confirmed the presence of a prostatic acinar adenocarcinoma with no bone infiltration on scintigraphy. Hormonal therapy was initiated.
After 6 months of treatment with apixaban and hormonal therapy, the patient's condition slowly improved, with a marked reduction of LLL edema and normal skin color . Levels of PSA decreased to 2.7 ng/mL six months after the initiation of hormonal therapy and reached values of 0.2 ng/mL a year later. The rapid response to hormonal therapy added to anticoagulant therapy probably contributed to the improvement of PCD. Upon examination during follow-up, he presented with incipient post-thrombotic syndrome.


A 22-year-old man, previously healthy, was admitted to the emergency room with 1 day history of fever and right upper quadrant (RUQ) pain. His vital signs were as follows: blood pressure 110/79 mmHg, pulse rate 68 beats per minute, and body temperature 38.9°C. On physical examination, there was tenderness in the RUQ area and a positive Murphy's sign.
Laboratory examination revealed the following: white blood cell (WBC) count 9610 mm/3; blood urea nitrogen 7.6 mg/dL; creatinine 0.8 mg/dL; total bilirubin 2.27 mg/dL; direct bilirubin 1.96 mg/dL; aspartate aminotransferase 160 IU/L; alanine aminotransferase 287 IU/L; alkaline phosphatase 238 IU/L; gamma-glutamyl transferase 919 IU/L; C-reactive protein 1.6 mg/dL. Abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) revealed diffuse gallbladder wall thickening and distension as well as a small filling defect in the common bile duct (CBD) []. After a blood culture sample was obtained, empirical intravenous (IV) ceftriaxone was administered on hospital day (HD) 1. On HD 2, therapeutic ERCP was performed. Written informed consent for ERCP was obtained from the patient. After endoscopic sphincterotomy (EST), a CBD stone was removed by retrieval balloon and basket, and a 7Fr endoscopic nasobiliary catheter was inserted into the CBD []. The ERCP procedure was performed without difficulty, and no adverse event occurred during ERCP manipulation. Following ERCP, the patient developed a fever with a return of RUQ pain, and his antibiotics were changed to IV piperacillin/tazobactam; then, a second blood culture sample was obtained on HD 2. There was no growth on the first blood culture performed on HD 1. On HD 5, his condition worsened, with the development of severe chills, high fever, and RUQ pain. Repeat bloodwork revealed an elevated WBC count of 15460 mm/3 with 82.4% neutrophils and a creatinine of 2.4 mg/dL. On the second blood culture performed after ERCP on HD 2, colonies of yeast were noted, and IV fluconazole (400 mg) was administered. To evaluate GB function, cholescintigraphy was performed, and an absence of GB filling was noted; therefore, laparoscopic cholecystectomy was performed on HD 7. After cholecystectomy, the patient clinically deteriorated into candidal septicemia, and subsequently, into acute respiratory distress syndrome (ARDS) and acute kidney injury (AKI). Therefore, the patient was intubated and placed on a mechanical ventilator and administered continuous renal replacement therapy until HD 14 (postoperative day, POD 7). On HD 18 (POD 11), the patient complained of decreased visual acuity. On ophthalmologic examination, chorioretinal infiltrative spots of inflammatory cells due to hematogenous spread of Candida were noted []. The second blood culture performed after ERCP on HD 2 revealed growth of C. albicans sensitive to fluconazole; therefore, a 28-day course of IV fluconazole was prescribed. Bile culture was negative for bacterial and fungal growth. After 2 weeks of IV fluconazole and intravitreal voriconazole therapy, the chorioretinal infiltrative spots were improved on ophthalmologic examination, and the visual acuity also improved []. Resolution of the candidemia was confirmed by no growth of C. albicans on follow-up blood culture. On HD 44 (POD 37), the patient was discharged without additional sequelae.


A 71-year-old male patient who received right nephroureterectomy developed bladder tumor recurrence on routine cystoscopy follow-up. We planned TURBO using fluorescent light-guided cystoscopy with 5-ALA. The patient was administered 5-ALA (ALAGLIO; Chugai Pharmaceutical Co., Tokyo, Japan) according to protocol 4 h prior to TURBO. A laser device was used (Aladuck LS-DLED; SBI Pharmaceuticals, Tokyo, Japan). Before setting the margins for TURBO, we checked the fluorescent lighting of the tumor. The tumor margin was easily detected by fluorescence (Fig. ). TURBO was then performed without any complications. The final pathological diagnosis was pathological Ta, and the tumor was resected without a positive surgical margin.


A 34-year-old Bakhtiari woman who was a known case of LP based on clinical and pathological diagnosis, referred to our hospital with hoarseness and dysphagia accompanied by cutaneous lesions. She presented with hoarseness and typical skin lesions since adolescence. About 7 years ago she developed a gradually progressive dysphagia to solid food as a predominant symptom and complained of intermittent post-prandial chest pain without regurgitation or weight loss. She had no history of gastroesophageal reflux disease (GERD), seizures, visual disturbances, or respiratory obstruction. None of the other family members were affected. Examination revealed an otherwise healthy individual with typical clinical findings of LP (). Systemic examination and laboratory tests did not reveal any abnormalities. Upper gastrointestinal endoscopy showed widespread mucosal thickening and irregularity in the pharynx and larynx. Upper and middle parts of the esophagus were normal, but an esophageal ring with normal overlying mucosa was observed in distal esophagus (). High-resolution manometry showed absent peristalsis ().