A 14-year-old female patient reported to the Department of Oral Medicine and Radiology with a chief complaint of discoloration of the tooth since birth. Patient gave a positive history of discoloration with deciduous and permanent dentition, there were no complaints of unusual sensitivity to foods or thermal changes, no systemic symptoms, but because of psychological impact of her appearance and difficulty in mastication, she came to our dental outpatient department for treatment. Past dental history and medical history was not significant. Patient was residing in a fluoridated area since her birth. The subject was the first child of nonconsanguineous and normally developed parents.
She has a sister with no congenital abnormalities. Pregnancy of her mother and birth were uneventful. The clinical pictures of the patient are given [Figures and ]. On general examination, the patient had a mild degree of hypertelorism, postaxial polydactyly with the right hand on the fifth finger, and bilateral postaxial polydactyly on both right and left foot. Her psychomotor and mental developments were within normal limits [].
On intra-oral examination, no soft tissue abnormalities were noted. Salivary ductal orifices were patent and saliva was of normal consistency and flow. On hard tissue examination, she had a normal complement of teeth with clinically missing 42, generalized brownish discoloration of teeth, which was exceeding more than 75% of the tooth structure with pitting and no morphological disfigurement seen. Generalized attrition was also present [Figures and ].
The hand-wrist radiograph of the right hand showed postaxial polydactyly, carpometacarpal fusion in the fifth finger, and partial fusion between fifth and sixth meta-carpels []. The lateral skull view of the patient showed frontal bossing and increased posterior-anterior dimension of the skull []. The posterior-anterior view of the skull [] shows expanded mid-facial structure, one of the common midline facial disorders seen in craniofacial ciliopathies.
In the present report, patient presents with two ciliary ectodermal alterations, one is a craniofacial abnormality, that is, hypertelorism, frontal bossing, microdontia, and other polydactyly. Based on the features mentioned above, with a thorough review of the literature, we classified the patient under Type 1 class of craniofacial ciliopathies [].


A 45-year-old male reported to the outpatient department of VSPM's DCRC, Nagpur, with the chief complaint of pain and swelling in the floor of mouth on the left side since 1 month. Detailed history revealed that it has started as a small swelling which used to increase before meals 1 month back. It gradually increased within a month.
Intraoral examination revealed a well-defined elongated swelling of approximately 2.5 cm × 1 cm in size in the floor of the mouth in relation to lower left incisors to first molar region. Overlying mucosa was inflamed and the swelling was hard in consistency and tender on palpation []. Radiographic evaluation included cross-sectional mandibular occlusal view which revealed a large well-defined elongated homogenous radio-opacity in the floor of the mouth on the left side in relation to lower left canine to first molar region. It was approximately 2.5 cm × 1.5 cm in size [].
On the basis of clinical and radiological findings, a diagnosis of left submandibular duct sialolith was made. As it was a large sialolith, we elected to remove the sialolith surgically under local anesthesia []. The sialolith removed measured 25 mm (i.e. 2.5 cm) in length [].


A seven years old girl was sitting in the rear seat of a car with her abdominal seatbelt fastened when the vehicle was involved in a high speed head-on collision. The grandmother, rear sitting too, had an ankle fracture.
When arriving at the emergency department, physical examination of the patient revealed lumbar pain, abdominal pain and paraplegia. The abdomen was diffusely tender and neurologic examination showed a caudal equina syndrome with a sensory deficit corresponding to level L3, a decreased rectal tone and paraplegia.
Radiologic evaluation included X-rays, computed tomographic (CT)-scan and magnetic resonance imaging (MRI). These examinations showed a Chance type fracture with dislocation of vertebra L3 (Figure ) with entrapment of a small bowel loop in the spinal canal (Figure ), a splenic subhilar fracture with perisplenic free fluid, difficulty to follow the left ureter at the lumbar level and a fracture of the right ischiopubic branch. MRI confirmed the aspect of small bowel loop into the spinal canal.
She underwent a two-stage emergency surgery. Firstly, the patient was placed in a prone position. A prophylactic antibiotic treatment was performed, using a 3rd generation cephalosporin. The Chance fracture was treated by open reduction and osteosynthesis by pedicular fixation from L2 to L4. Secondary she underwent left transverse susombilical laparotomy. It revealed the intracanalar incarceration of a jejunum loop (Figure ), an injury of the ascending colon, a large section of the retroperitoneum at L3 level, a section of the left psoas muscle, a complete section of the left lumbar ureter and a left lumbar arterial wound. No obvious sign of hepatic or pancreatic injury were observed. The L3-L4 discectomy was performed to release the incarcerated bowel loop. A 10 cm-small bowel resection was performed because of the suffering aspect of the incarcerated bowel fragment. A primary end-to-end anastomosis was performed and the perivertebral space was irrigated. The injured left ureter was then sutured end-to-end on a JJ probe (18 cm, 4.7 Charriere). The right colonic serosa wound was closed and the retroperitoneum was drained.
Antibiotics were continued postoperatively to prevent meningitis and peritonitis. A removable thoracolumbar brace was prescribed. She didn’t show any neurologic recovery after surgery.
This young girl was discharged from the hospital on day 12. The JJ probe was removed one month after the traumatism and intermittent bladder catheterization were initiated.
She started to complain of severe headaches in sitting position that completely resolved with recumbent position. An MRI was performed for a suspicion of dural tear. It showed a leakage of cerebrospinal fluid (CSF) from the medullary canal to the abdominal cavity, through the L3-L4 intervertebral disc and the left L3-L4 intervertebral foramen (Figure ). The abdominal meningocele was developed along the left psoas muscle. It was decided to follow up this collection by ultrasonography. After one week, the patient could tolerate the seated position without any complain. Ultrasonography controls didn’t show any increase of the meningocele. The patient was discharged from the hospital and was asked to wear a full time brace. Three and a half months after the traumatism, an MRI showed a clear decrease of the meningocele. Three months after, CSF signal was only visible at the level of the L3-L4 intervertebral disc space, and disappeared 6 mo later.
One year after the traumatism, the patient finally underwent an anterior L2-L4 anterior interbody fusion through a standard left lobotomy. No LCS leakage was identified during this surgical procedure. The L2-L3 intervertebral disc and the remnants of the L3-L4 intervertebral disc were removed and replaced by cancellous bone grafts, without osteosynthesis.
At last follow up (9 mo after the second surgical procedure), the patient still did not show any neurologic recovery. The evoked somatosensory potentials didn’t show any neurologic activity in both legs. The AP and lateral full spine x-rays performed in the seated position showed satisfactory spine morphology (Figure ).


A 51-year-old male with chronic alcohol abuse was admitted to hospital with intermittent abdominal pain that increased in intensity after meals and jaundice of the mucosae and skin. He had a two-year history of steatohepatitis, chronic pancreatitis, as well as secondary insulin-dependent diabetes mellitus.
The ultrasonographic examination revealed enlargement of the pancreatic tail. Blood analysis showed slight anemia (hemoglobin 9.8 g/dL; hematocrit 29.2%), normal glycemia, and slightly increased serum levels of amylase (141 u/L).
Due to magnetic resonance imaging (MRI)-based suspecting a pancreatic tumor and mechanical jaundice, it was decided to perform a laparotomy. A splenectomy and pancreatic tail resection was performed. No gallstones were identified. The intraoperative exploration of the abdominal cavity also revealed a well-defined jejunal cystic swelling. The resection of the jejunum (135 mm in lenght) and end-to-end anastomosis was performed.
Gross examination of the surgical specimens showed a fibrotic pancreatic tail. The round-shaped cystic lesion of the jejunum had a diameter of 20 mm and was covered by intact jejunal mucosa. The serosal surface was intact. On the cut section, the monolocular cyst was filled with serous fluid and had a smooth inner surface. The cystic wall thickness was roughly 2-3 mm (Figure ).
Histopathological examination revealed large fibrotic areas in the pancreatic tail and pancreatic ducts with focal dysplasia (panIN-1a). The jejunal cyst was covered by Keratin 20 positive intact jejunal mucosa. It was located in the submucosa and muscularis propria. A cystic denuded inner surface with fragmented flat epithelium, marked by both Keratin 20 and Keratin 7 was noted and the capillary rich granulation tissue dissociated the underlying smooth muscle fibers. The muscularis layer was shared by the cyst and adjacent jejunum. The communication area was also lined by granulation tissue. In the deep muscularis propria, proliferation of pancreatic-type Keratin 7 positive/Keratin 20 negative ducts and acini, without cytological atypia, as well as rare Langerhans islands were also observed. No positivity for c-KIT or DOG-1 was noted.
Based on the macroscopic and microscopic findings, the final diagnosis was “jejunal duplication cysts, with synchronous Heinrich’s type I ectopic pancreas[] and low grade intraepithelial neoplasia of the pancreatic tail”.
Postoperative evolution was uneventful. The patient refused to stop alcohol consumption. At 27 mo after surgery he was admitted to hospital with scleral jaundice and a 7 mm large gallstone was found in the common bile duct. Laparoscopic choledochoduodenostomy was performed without postoperative complications.


An 83-year-old male presented to the Emergency Department with a 2-day history of diarrhea without blood. Past medical history included Alzheimer disease, arterial hypertension and diabetes mellitus (well controlled with insulin, but with known microvascular complications - retinopathy and nephropathy). Clinical information was provided by the caregivers because the patient was not communicative. They denied any history of trauma or topical medications. The patient was dehydrated and hypotensive. Abdominal exam was normal. Anal inspection revealed a darkened color of the anoderm (). Digital rectal examination was painful, with apparent hypotony of the anal sphincter, no palpable masses and no blood. Lymphadenopathy was absent.
Laboratory results revealed the following: leukocytes (16,000/µL; 4,000–10,000/µL), C-reactive protein (101.6 mg/L; <5 mg/L); creatinine kinase (346 U/L; <250 U/L); lactate dehydrogenase (196 U/L; 50–115 U/L). Flexible sigmoidoscopy showed a circumferential darkening of the anal canal (total distance of 25 mm), with a proximal margin 2 mm below the dentate line and a normal rectal mucosa with brown molded feces inside the rectum (). Histopathology showed a polymorphic inflammatory infiltrate, with extensive necrosis and ulceration up to the hypodermis (). Microbiology was negative. No imaging study, such as computed tomography, magnetic resonance, endoanal ultrasound, or angiography, was performed due to the patient's performance status. Anal sphincter function was not evaluated because the patient was not cooperative.
After admission, diarrhea was no longer observed. Hypotension quickly improved after fluid therapy with normalization in the first 2 hours after admission. Anal lesions were not improved after 5 days of medical therapy (broad-spectrum parenteral antibiotics and intravenous fluids). On day 6 after admission, surgical debridement with superficial necrotic tissue removal and hemostasis with an absorbable haemostatic gelatin sponge was performed. During surgery, gangrenous superficial tissues were noticed, but muscular tissue and the anal sphincter were spared. The patient's perianal findings were clearly improved 5 days after surgical treatment (). After 10 months of follow-up, neither cutaneous lesions nor fecal incontinence had occurred.


A 23-year-old female presented with a cystic lesion in the distal body of the pancreas. No mass was palpable through abdomen examination. Her serum carcinoembryonic antigen (CEA) was 2.5 ng/dL and serum carbohydrate antigen (CA) 19.9 was 6.7 IU/dL. Triphasic computed tomography (CT) abdomen showed a 4.5 cm × 3.2 cm cystic lesion in the body of the pancreas with calcifications. The diagnosis of a mucinous cystic neoplasm was made and the patient was planned for laparoscopic distal pancreatectomy.
She was placed in supine position with a 30° right lateral tilt. Pneumoperitoneum was created using a Veress needle and four ports were placed (supraumbilical for camera, epigastric, left mid-clavicular and left midaxillary as working ports). After assessing for metastatic disease, gastrocolic ligament was divided and access was gained to a lesser sac. Resectability of the lesion was assessed. Splenic flexure was mobilised to expose the inferior edge of the tail of the pancreas. Peritoneal incision was made along the inferior edge of the pancreas from body to tail. The pancreas was then dissected off from the retroperitoneum. After completing inferior and retroperitoneal dissection, dissection was started in the superior aspect of the pancreas. During the mobilisation, the common hepatic artery was accidentally clipped and divided, assuming it as the splenic artery. The common hepatic artery was densely adherent to the pancreatic body and had an anomalous course (i.e., after arising from the coeliac axis, it was running to the left side just adjacent to the superior border of the body of the pancreas for a few centimetres and hence mistakenly interpreted as splenic artery). Abnormal course of the common hepatic artery was confirmed and primary reconstruction was planned using the left gastric artery as conduit. Distal pancreatectomy was completed, after which the distal part of the hepatic artery was mobilised. Bolus dose of heparin was given. Left gastric artery was skeletonised and mobilised. Its proximal part was occluded using endo bulldog clamp while the distal part was doubly clipped and divided, keeping adequate stump for reconstruction. Distal stump of the hepatic artery that was clipped, was exchanged to endo bulldog clamp and the edges were refashioned []. An end-to-end anastomosis was done using a 7-0 prolene suture in an interrupted fashion using a 3 mm endo needle holder []. Hepatic artery blood flow was ascertained by intraoperative laparoscopic Doppler study. The total duration of reconstruction was 45 min and the estimated blood loss was 75 mL. Postoperatively, the patient was observed in the intensive care unit (ICU) for 2 days. Liver enzymes were transiently elevated on the 1st postoperative day; they became normal by the 4th postoperative day. She received heparin infusion for 5 days and then was switched to aspirin. She was discharged on the 7th postoperative day with an advice to continue aspirin for 90 days.


A 42-year-old man, previously healthy, was living with his step father and other members of an extended family in a small farm. He presented with a first episode of a motor seizure that started on the left side of his body before becoming generalized. Shortly afterwards, he noticed left hemiparesis and dysarthria; he was admitted to our institution through the emergency department.
After a physical examination, magnetic resonance imaging (MRI) of the patient revealed a well-defined, spherical lesion, located in the superior aspect of the anterior limb of the internal capsule and right striatum, with surrounding edema []. Laboratory studies found no systemic compromise and no underlying immunocompromise. We decided to excise and analyze the aforementioned lesion. Performing an image-guided frontal craniotomy, using the Leksell Stereotactic G-Frame (Elekta Instruments AB, Stockholm, Sweden), we planned the trajectory to avoid the head of the caudate nucleus, the genu of the internal capsule, the putamen, and other critical structures. The mass was completely excised and the thalamostriate vein, which was adhered to the mass, was preserved. Craniotomy was performed, instead of a stereotactic biopsy, because we suspected the lesion to be a high-grade glioma that was accessible to surgical resection.
In the pathological analysis, there was an evident atypical T and B infiltrate; morphological and phenotypical characteristics of Grade 1 lymphomatoid granulomatosis. The patient was subjected to thoracic and abdominal screening, which revealed paratracheal, jugular, and inguinal adenopathies, but no other masses.
After consulting with the hematology group, the patient received a four-cycle medical treatment with rituximab and prednisone. Clinically, he recovered almost completely with strength of 4/5 and complete reintegration to his daily activities, which involved bimanual work. Six months after his diagnosis, a new MRI showed the absence of new or residual lesions.
Two years after the surgery the patient continued to be free of seizures, and his MRI showed no evidence of new lesions, areas of restriction of diffusion, or anomalous enhancements that could indicate residual or recurrent tumor [].


A 34-year-old woman presented with bilateral itching, redness, and mucinous discharge. Several bilateral woody hyalinized masses were identified on the tarsal conjunctiva []. She also had mucinous saliva; besides, a cyst partially limiting the vocal cord movement was identified over the right posterior cricoids. Neither did the patient have any contributory history of ocular trauma nor did any of her family members. Initially, unusual, varied-size giant papillae were found over the tarsal conjunctiva, and received mass excision 3 times, cryotherapy, triamcinolone injection (40 mg/ml), antiproliferative soaking with mitomycin C, and amniotic membrane transplantation because of medically refractory symptoms. Although symptoms were dramatically relieved after each surgery, the patient was always followed-up for tumor regrowth with associated discomfort for up to 1–2 years after each surgery []. Recurrent conjunctival lesions were assessed by immunohistochemical microscopy for serial samples of conjunctival biopsies. Pathological analysis confirmed the diagnosis of LC [] and revealed the characteristics of IgG4-RD []. Combined with clinical presentation and elevated serum IgG4 level (274 mg/dl), IgG4-RD was further diagnosed.
There was no obvious lymphadenopathy in physical examination and chest X-ray. Laboratory examination revealed that eosinophil (0.7%), rheumatoid factor (nonreactive), anti-nuclear antibody (negative), erythrocyte sedimentation rate (3 mm/h), C3 (98.4 mg/dl), C4 (15.3 mg/dl), IgG (1500 mg/dl), and IgM (230 mg/dl) were all within normal limit, but IgE (133 IU/ml) and IgA (531 mg/dl) were elevated.


In 2009, a 49-year-old premenopausal woman was diagnosed to have right breast cancer and synchronous bone metastases. She underwent modified radical mastectomy (MRM). Pathological evaluation revealed axillary nodal metastases and estrogen receptor (ER), progesterone receptor (PR), and HER2 receptor (3+) were all positive by immunohistochemistry (IHC). She received six cycles of docetaxel, carboplatin, and trastuzumab, followed by maintenance adjuvant trastuzumab and tamoxifen. One year after completion of adjuvant trastuzumab, she developed liver metastases. She was started on capecitabine and lapatinib, on which she had a progression-free interval of 6 months. At this time, she was given six cycles of epirubicin and cyclophosphamide. At the end of this regimen, she presented with headache and vomiting. Magnetic resonance imaging (MRI) of the brain and spinal cord showed patchy leptomeningeal enhancement suggestive of LC. Her cerebrospinal fluid (CSF) examination revealed the presence of adenocarcinoma cells. She was treated with a combination of lapatinib, letrozole, and IT trastuzumab (50 mg 2 times/week for 4 weeks followed by 50 mg once per week for 4 weeks, followed by 150 mg once every 3 weeks) along with IT hydrocortisone (50 mg) on every occasion. She had rapid symptomatic improvement within 4 weeks of this treatment. The CSF cytology was negative for malignant cells after the 16 IT injections of trastuzumab. This treatment was well tolerated except for mild headache post-IT administration. She had symptomatic (increasing headache and vomiting) and radiological progression (new parenchymal brain lesion and diffuse increase in the leptomeningeal enhancement along both cerebral and cerebellar hemispheres) after 7 months of IT therapy. At this time, CSF cytology was also positive for malignant cells. On this occasion, she received whole-brain radiation therapy (WBRT) while IT trastuzumab was continued at 3 weekly intervals. The patient again had symptomatic improvement within 4 weeks and radiological response (decrease in parenchymal lesions and leptomeningeal enhancement) at 16 weeks.


In 2011, a 62-year-old woman was diagnosed to have left locally advanced breast cancer (T3N1M0) which was ER and PR negative and HER2 positive (3+ by IHC). She was treated with neoadjuvant chemotherapy (12 cycles of weekly paclitaxel and trastuzumab) followed by MRM. This was followed by four more cycles of chemotherapy (epirubicin and cyclophosphamide) and locoregional radiation therapy. She received maintenance trastuzumab to complete a total of 1 year of this drug. Two months after completion of treatment, she presented with headache and vomiting. MRI of the brain revealed parenchymal metastases. She received WBRT followed by single agent lapatinib at the dose of 500 mg 2 times/day. After 4 weeks, she had good symptomatic improvement with resolution of headache and MRI revealed reduction in cerebral lesions. After 8 months of treatment with lapatinib, she developed headache, backache, and ataxia. A repeat MRI showed increasing brain metastases along with diffuse leptomeningeal enhancement. CSF cytology revealed adenocarcinoma cells consistent with LC. She was started on IT trastuzumab (150 mg every 3 weeks) combined every time with IT hydrocortisone (50 mg). She was simultaneously started on intravenous trastuzumab in usual doses, and lapatinib was escalated to 1250 mg/day. After the 2nd dose of IT trastuzumab, there was a significant clinical improvement, and the CSF cytology became negative for malignant cells. At the time of this report, she has completed 7 months of this treatment without evidence of disease progression and continues to be symptomatically well controlled.


A 19-year-old primigravida woman at 37-weeks gestation with lower abdominal pain, dyspnea, and pitting edema presented to the emergency room at Dankook University Hospital. She had received a prenatal management at a local clinic, and there were no abnormal findings including the past medical history and family history. A headache or the right upper abdominal pain was not observed at her admission to the hospital.
At the admission to the emergency room, laboratory findings were identified as follows: blood pressure of 160/90 mmHg, pulse rate of 79 beat/min, respiratory rate of 20 breaths/min, protein in urine (+++), hemoglobin concentration of 8.6 g/dL indicating anemia, platelet count of 181×103/µL, blood urea nitrogen/creatinine of 10.5/0/73 mg/dL, and aspartate transaminase/alanine transaminase of 20/7 U/L. An electrocardiogram showed normal sinus rhythm, but a large amount of pleural effusion was observed on chest radiogram(). Oxygen saturation was normal, and the estimated fetal weight and the amniotic fluid volume on ultrasound examination were also within the normal range.
The patient complained of labor pains at regular intervals of 5 minutes, and a fetal heart rate deceleration was shown on cardiotocogram. Her cervical dilation was 1 cm and effacement was 75% on a pelvic examination. She was diagnosed with preeclampsia and underwent an emergency cesarean section under the spinal anesthesia.
The baby was healthy with the weight of 2,900 g, and the Apgar score of 8 to 9 at 1 and 5 minutes; and there was no problem with the cesarean section. In 40 minutes after the surgery, the patient complained of severe dyspnea and chest pain with the blood pressure of 160/85 mmHg, pulse rate of 146 beat/min, respiratory rate of 30 breaths/min, and oxygen saturation of 90%. On auscultation, the right lung sound was absent. Chest X-ray () and chest CT scan were done, and the chest tube was inserted.
Aortic dissection with rupture was diagnosed with the finding of descending aortic dissection at the level of T3 to T7 and massive hemothorax on the chest computed tomography scan () and it was classified DeBakey type III dissection. The patient was transferred to intensive care unit and was monitored. The patient did not receive intravenous antihypertensive medication because her blood pressure was maintained at 120/70 mmHg. However, cardiopulmonary arrest occurred 5 hours after the cesarean section, and the patient died without reaction to cardio-pulmonary resuscitation.


A 60-year-old P4L4 postmenopausal woman presented with chief complaints of foul smelling discharge per vaginum with low grade fever on and off since 1 year after that she had high grade fever since 1.5 months. She had menopause at 50 years and had never taken hormone replacement therapy. There was strong family history of tuberculosis in her close relatives. Her general physical examination was unremarkable. Her per-speculum examination showed unhealthy cervix, ulcerative lesion on the posterior lip of cervix from 3 to 5 o'clock position and pus was coming freely from that area (). On probing it, that area was 1 cm in depth. On pelvic examination uterus was bulky in size, bilateral fornices free and non-tender. In her complete blood picture, total leucocytes count was 10,000 cells/µL, with neutrophils 63% and lymphocytes 32%. Her erythrocyte sedimentation rate was raised (65 mm) and montoux test was positive. Her Pap smear was unsatisfactory due to low cellularity.
Ultrasound revealed a fluid collection in uterine cavity around 70 ml. In contrast-enhanced computed tomography cervix was bulky, heterogeneously enhancing thickened both lips of cervix, and endometrial cavity was distended with fluid suggestive of pyometra, obliteration of fat planes between uterus and bladder; uterus and rectum (). Also there were multiple enlarged lymph nodes like portocaval, retrocaval, precaval, aortocaval, preaortic and para-aortic in contrast-enhanced computed tomography of abdoman and pelvis. All these findings can occur in malignancy as well as inflammation.
Patient has undergone pyometra drainage and cervical biopsy from posterior lip of cervix was done. Her peroperative finding was 50 mL of pus drained which was thick and cheesy. Her symptoms persists inspite of empirical antibiotic therapy given for 2 weeks. Final diagnosis was made only after histopathological examination of cervical biopsy which showed XGI of cervix likely of tubercular etiology. Numerous granulomas are seen the cervical stroma. The surface epithelium is ulcerated at most places (H&E, ×40) (). Epithelioid cell granuloma with Langhan's giant cell is seen beneath the ulcerated epithelium (H&E, ×100) ().
Although her smear for acid fast bacilli was negative and BACTEC with radiometric culture (the radiometric method; Becton Dickinson, Towson, MD, USA) was negative, patient was started on antituberculosis treatment (ATT) category 3 regimen (2 months of isoniazid, rifampicin, and pyrazinamide; 4 months of isoniazid and rifampcin) because of raised erythrocyte sedimentation rate, Mantoux positive, strong family history of tuberculosis and histopathology report. Although endometrial sampling was not taken and planned at second sitting but she improved gradually on ATT and hence endometrial sampling deferred later on also. She has been followed for 6 month and became asymptomatic.


A 6-month-old male infant was brought to the emergency department with complaints of fever for 1 month, passage of dark stools for 1 week, and discoloration of upper extremity for 2 days. Parents also volunteered the history of fleeting maculo-papular rash. The child was referred for Doppler imaging (Philips HD 11 XE ultrasound machine, PHILIPS USA) of the upper extremity and sonography of the abdomen on emergent basis. Doppler USG showed partially thrombosed fusiform aneurysm of right subclavian artery, axillary artery, brachial artery, and non-thrombosed aneurysm of the right subclavian artery [ and ].
Subsequently, ultrafast low-dose CT angiography (Philips Brilliance 40 Slice CT scanner; PHILIPS USA) was done in emergency settings which confirmed the color Doppler findings and additionally showed aneurysms of bilateral common carotid, extracranial internal and external carotid, and bilateral vertebral arteries []. There were aneurysms involving multiple coronary arteries, with giant fusiform aneurysm of left anterior descending artery measuring 13 mm [Figures and ]. CT angiography of thoraco-abdominal aorta revealed small saccular aneurysm at the origin of celiac artery measuring 29 × 25 mm, with fusiform dilatation of superior mesenteric artery [ and ]. There were non-thrombosed fusiform aneurysms of bilateral common iliac artery and common femoral artery without the involvement of the main aortic trunk []. The caliber of thoraco-abdominal aortic trunk was normal with distal smooth tapering.
Based on the imaging features, a possibility of infantile panvasculitis was considered. Review of the clinical parameters revealed normochromic-normocytic anemia, leukocytosis, and elevated erythrocyte sedimentation rate (ESR; 43 mm in first hour) and C-reactive protein (34 mg/l). The blood cultures and peripheral smears were negative for microbes and had no malarial parasites []. Based on the clinical history of conjunctivitis, fleeting rash, swelling of extremities, and multiple aneurysms of medium to small-sized vessels, a diagnosis of infantile atypical Kawasaki disease was made. Apart from the supportive management, the infant was started on intravenous immunoglobulin, but succumbed to death on the third day of admission.


A 55-year-old man, a bus conductor by occupation, presented with progressive skin darkening, thickening, and tightening over his left lower limb since six months. There was difficulty in extending the left knee with pruritus over the darkened skin. The patient consumed a strict vegetarian diet. Dermatological examination revealed a poorly demarcated area of brownish hyperpigmentation and induration over the anterolateral aspect of the left thigh, crossing the knee joint and extending to the anterior aspect of the left leg, sparing the foot [Figure –]. The overlying skin was puckered and Groove sign was positive. In addition, a well-defined 13 × 4 mm, hypopigmented, and indurated plaque was present over the left iliac fossa []. Dermoscopy of the left iliac fossa lesion revealed an ivory white hue with follicular plugging and comedo-like openings []. Laboratory evaluation revealed a differential eosinophil count of 31% with absolute eosinophil count of 2080/µL, an elevated erythrocyte sedimentation rate (ESR) of 26 mm/h, and a positive C-reactive protein. Serum IgE levels were mildly elevated (516 IU/L). Rheumatoid factor, antinuclear, and antithyroid peroxidase antibodies were negative. Peripheral blood smear showed no atypical cells. Serum calcium, lactate dehydrogenase levels, and liver and renal function tests were normal. Hematological consultation was sought for the peripheral eosinophilia and possible malignant potential, which revealed no abnormality. Ultrasound study of the left lower limb revealed diffuse fascial thickening with subcutaneous edema. Deep biopsy done from the indurated lesion on the thigh revealed fascial thickening with homogenous collagen bundles present in the deep dermis and also replacing a large portion of the adipose tissue. A lymphoplasmacytic infiltrate with few eosinophils were observed in dermis, subcutaneous tissue, fascia, and muscle, consistent with the diagnosis of EF []. Magnetic resonance imaging showed fascial thickening without any myositis, with enhancement and increased signal intensity on fluid-sensitive sequences, corroborating EF. Biopsy from the plaque on the left iliac fossa revealed features consistent with LS [Figure –]. IgG and IgM titers to Borrelia burgdorferi were negative. The patient was treated with oral prednisone 60 mg daily and 1% topical tacrolimus ointment for the indurated lesion and clobetasol propionate ointment for the LS lesion. Within six weeks, there was significant improvement in edema, induration, and pliability of the EF lesion and decrease in ESR and peripheral eosinophilia. Prednisone was gradually tapered over three months. The patient is presently under periodic follow up. The LS lesion remained nonresponsive to therapy.


Twenty-five years before being diagnosed with lymphoma, a 73-year-old man developed hematochezia and was diagnosed with ulcerative pancolitis. The patient was previously treated with a brief trial of prednisone, but was subsequently maintained on balsalazide at a dose of 6,750 mg per day with good control of his symptoms, without diarrhea or rectal bleeding. Colonic biopsies 2 years ago showed histologic evidence of chronic colitis in his left colon and rectum. The patient was never treated with immune modulators or biologic agents for his ulcerative colitis. The patient also had longstanding polymyositis for the past 6 years, initially diagnosed on the basis of symptoms including proximal muscle weakness, a creatine kinase (CK) level greater than 10,000 U/L (reference range: 44 - 196 U/L), and a positive electromyography (EMG) study suggestive of an inflammatory myopathy. He had responded to high doses of steroids, and methotrexate was added several years ago with a gradual dose titration up to 20 mg weekly. The patient’s inflammatory muscle disease had never been completely controlled; serial CK levels remained in the range of 1,500 - 2,000 U/L.
A few months prior to the current presentation, the patient developed dysphagia that was clinically felt to be a manifestation of polymyositis. The patient’s blood pressure was 151/85 mm Hg, his heart rate was 70 beats per minute, and his body weight was 203 pounds with a BMI of 32.3 kg/m2. On physical examination, the patient had weakness in his lower extremities and some difficulty getting up out of a chair without the use of his hands. No lymphadenopathy was noted. Due to the suboptimal control of his polymyositis symptoms, mycophenolate mofetil (Cellcept) at a dose of 1,000 mg per day was initiated and methotrexate was stopped on April 27, 2016, approximately 70 days prior to his surveillance colonoscopy in an outside hospital.
Esophagogastroduodenoscopy (EGD) performed for his dysphagia revealed abnormal motility of the esophagus, compatible with presbyesophagus, and a small hiatal hernia. A 17-mm Savary-type dilator was introduced for empirical dilatation. No definitive stricture was noted. A surveillance colonoscopy revealed normal mucosa in the cecum, ascending colon, and transverse colon. The descending colon and sigmoid colon, however, were characterized by erythema, friability, and congestion. In addition, a polypoid, nodular area with a slight stricture was noted in the sigmoid colon at 50 cm. The rectum was endoscopically normal. Biopsies were taken from the descending colon and from the polypoid, nodular, strictured area in the sigmoid colon.
The sigmoid colon biopsy revealed a neoplasm comprised of a solid proliferation of large mononuclear cells with a high nucleus to cytoplasm (N/C) ratio, irregular nuclei, and distinct nucleoli (). Tumor cell nuclei approximated the size of normal macrophage nuclei and were more than twice the size of normal lymphocytes. The tumor cells expanded the lamina propria and pushed the crypts apart, focally infiltrating the crypt epithelium (not shown). There was focal necrosis within the tumor (). By immunohistochemistry, the tumor cells were diffusely positive for CD20 (), PAX5 (), and CD30 (), strongly positive for BCL2 and MUM1 (not shown), and variably reactive for BCL6 (). There was a high Ki67 labeling index (about 80-90%) (). Tumor cells were negative for CD3, CD5, CD10, cyclin D1, and SOX-11. The tumor cells were positive for EBV by in situ hybridization (). A similar atypical lymphoid infiltrate was noted in the lamina propria () and crypt epithelium () in the descending colon biopsy. In addition, the descending colon biopsy showed mildly active chronic colitis (not shown). The overall histology and immunophenotype supported a diagnosis of an EBV-positive diffuse large B-cell lymphoma (DLBCL), non-germinal center type, involving the descending and sigmoid colon and arising in a background of ulcerative colitis.
Staging positron emission tomography with computed tomography (PET-CT) revealed intense focal pathologic uptake in the colon corresponding to the patient’s biopsied lymphoma in a background of generalized physiologic uptake. There were no additional sites of increased uptake. The patient is currently scheduled to receive six cycles of dose-adjusted rituximab, etoposide, prednisolone, oncovin, cyclophosphamide, and hydroxydaunorubicin (DA-R-EPOCH) chemotherapy.


A16-year-old boy sustained injury to left knee while playing. He had severe pain and swelling in the knee. He was not able to bear weight on affected limb. X-rays revealed displaced tibial spine fracture [, ]. He was treated in cast by orthopedic surgeon. Patient was not comfortable in the cast and came to us. It was a displaced fracture. We did MRI to rule out other injuries []. Patient was treated arthroscopically under spinal anesthesia in supine position.
Anterolateral and anteromedial portals were used. Both the portals should be standard or slightly low as higher portal may put the scope above the fractured fragment and one may not be able to see the fracture clearly. Fractured fragment was clearly defined with the help of a shaver. It also involved significant portion of medial tibial platue [, ]. Fracture was reduced with the help of ACL zig and under C- Arm guidance, provisionally fixed with guide wire from superolateral portal taking care not to cross physis. As it was a large fragment, it did not sit completely from medial side so another wire was used from superomedial portal to fix medial side of fragment perpendicular to lateral wire, again without crossing the physis. Two 3.5 mm partially threaded cannulated screws (Zimmer) were directly used; 45mm from lateral side and 35mm from medial side []. Stability of the fragment was assessed with a probe and was found to be very stable []. Post-operatively, patient was given a knee brace []. Post-op X-rays showed proper position of both screws [, ]. He was allowed to walk full weight bearing with the knee brace and with support. Knee ROM from 0-90 was allowed for 2 weeks then 0-120 for next 2 weeks. After one month, he was allowed to walk without knee brace. At 6 months, patients was allowed to run. Now we have 8 months follow-up.


A 55-year-old man with no significant medical history was transferred to our hospital with a 14-day history of fever and lethargy. Over the previous 5 weeks, he had been treated for traumatic subdural hemorrhage after a car accident. Two weeks prior to transfer, a high fever suddenly developed and persisted despite administration of broad-spectrum antibiotics including ceftriaxone, piperacillin-tazobactam, or meropenem in combination with moxifloxacin. There was a healing abrasion on his hand after the car accident, but there were no stigmata of endocarditis. He had no indwelling medical device and no medical history of allergy.
On admission to our hospital, his blood pressure was 150/100 mmHg, pulse rate was regular at 100 beats per minute, and temperature was 38.0°C. He was slightly drowsy and confused but exhibited no neurologic deformity. Heart sounds were regular without murmur, and breathing sounds were normal.
His white blood cell count was 7,400/mm3 with 79.4% neutrophils. Laboratory data were as follows: hemoglobin, 11.2 g/dL; platelets, 251,000/mm3; C-reactive protein, 79.7 mg/dL; procalcitonin 0.18 ng/mL; and erythrocyte sedimentation rate, 52 mm/h. Electrolyte levels and kidney and liver function tests were normal.
Electrocardiogram showed normal sinus rhythm. Computed tomography (CT) of the chest showed pulmonary emphysema, and abdominal CT was normal. Brain CT revealed a small, chronic subdural hematoma on the left frontal convexity.
The patient initially received 2 g ceftazidime intravenously every 8 hours in combination with oral metronidazole (500 mg every 8 hours). On the third day of admission, 2 separate sets of blood cultures were positive for Gram-positive cocci. After the second set of blood cultures, intravenous vancomycin (1 g every 12 h) was added empirically. On the sixth day, Kocuria kristinae was identified in both blood cultures using the Vitek 2 system (bioMérieux, Marcy-l’Etoile, France) in the absence of definitive laboratory guidelines for determining the antibiotic susceptibility. While the initial blood culture was regarded as contaminated, when the same pathogen was isolated from the consecutive blood culture, we determined that K. kristinae might be the true pathogen. At this time, initial empiric ceftazidime and metronidazole were discontinued and intravenous vancomycin was maintained. Because K. kristinae is not a common infective endocarditis pathogen, we also performed 16S rRNA sequencing analysis to confirm the identification of the pathogen in the blood isolates. The universal eubacterial primers fU1 (5’-TTGGAGAGTTTGATCCTGGCTC-3’) and rU2 (GGACTACCAGGGTATCTAA-3’) were used. The 16S rRNA gene sequence (766 bp) was blasted with NCBI Blast website . The sequence was 99.00% identical to that of C. striatum was identified (GenBank accession number JF342700.1). Transthoracic echocardiography showed 2 mobile oscillating masses at the tip of the mitral valve leaflet. Transesophageal echocardiography revealed two hypermobile echogenic masses on the anterior and posterior mitral valve leaflets, 10 mm and 8 mm, respectively (). There was no involvement of either the subvalvular structure or paravalvular structure. There was no evidence of any metastatic lesions at other sites.
Thus, the patient was diagnosed with C. striatum infective endocarditis not K. kristinae infective endocarditis. After vancomycin was administrated, the fever subsided dramatically. Because of the high embolic risk, mitral valve replacement was performed. Interestingly, K. kristinae, which was found in the blood cultures, was also cultured from mitral valve vegetation despite administration of vancomycin for 14 days. The patient completed 6 weeks of intravenous vancomycin for prosthetic valve endocarditis. At a follow-up over one year later, the patient remained free of infection


A 36-year-old healthy man with an unremarkable medical history was referred from a local hospital for an abdominopelvic CT finding that was compatible with splenic infarction due to splenic artery dissection.
The patient complained of abdominal pain in the left upper quadrant and the epigastric region as well as back pain. Upon physical examination, he had mild tenderness to palpation in the upper left quadrant and the epigastric region without abdominal guarding. Vital signs were stable, with a blood pressure of 130/80 mmHg, heart rate of 80 beats/min, and body temperature of 36.8°C. His visceral arteries were evaluated in detail by mesenteric CT angiography, which showed dissections in the celiac trunk, common hepatic artery, and splenic artery with mural thrombi, and partial infarction of the spleen (). The patient was treated with intravenous heparin and meperidine to control pain. Abdominal pain subsided after three days. A follow-up CT angiogram performed three days after admission showed no changes in the celiac trunk, common hepatic artery, splenic artery dissection, or partial infarction in the spleen. After 10 days in the hospital, the patient was discharged with aspirin, with a follow-up after 12 days.


A 42-year-old male patient presented to the ED with a complaint of abdominal pain persisting for one hour on January 21, 2015. He had undergone a small bowel resection and open reduction internal fixation with a screw in the right femur due to a traffic accident in 2004. Since then, he had been taking acetaminophen and tramadol to relieve lower back pain, with no other medications taken regularly. He had sharp pain in the left upper quadrant and the epigastric region. A physical examination revealed tenderness in the same area without signs of peritoneal irritation. His blood pressure was 130/80 mmHg, with a heart rate of 68 beats per minute and a body temperature of 36.8°C. A contrast-enhanced CT scan of the abdomen and pelvis revealed celiac artery dissection with an intimal flap and dissection of the splenic artery with mural thrombus, accompanied by splenic infarction (). He was hospitalized in the vascular surgery department for conservative treatment with intravenous heparin for anticoagulation. The next day, his blood pressure increased to 170/110 mmHg and he received intravenous and oral calcium channel blockers to lower his blood pressure. Afterwards, his antihypertensive drug regimen was changed to an oral angiotensin II receptor blocker based on consultation by a cardiologist. Intravenous meperidine and oral acetaminophen were used to control pain. The patient was discharged 2 days after admission without any complications.


The patient is a 34-year-old man with eight class education. He was referred to a psychiatrist on behalf of his company because of decreased job performance due to obsessive behavior.
The patient was diagnosed with obsessive-compulsive disorder (OCD) in Axis I and border intelligence in Axis II accordance with Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition diagnostic criteria. Fluoxetine began with a 10 mg daily dose and had reached 60 mg/day 5 months later. Symptoms of OCD showed an appropriate response to treatment, but the patient complained of decreased libido, so bupropion was added to the treatment at a dose of 75 mg daily.
The next morning, about 11 h after reception of the first dose of bupropion, the patient referred to the emergency room while complaining of muscle contractions in the right side of the face, neck, and shoulder. Biperiden was intramuscularly injected to the amount of 5 mg, and the patient was referred to a psychiatrist due to lack of response. The patient's head was in a state of extension. During the spasms episode, his eyes turned upward and the patient was unable to make eye contact. There were no other signs such as fever, and neurological tests were normal. There was no family history of head trauma, seizure or substance abuse, or family history of psychiatric disorders and movement disorders. With respect to previous psychiatric history and the diagnostic check, neither conversion nor malingering disorder was raised for the patient. Secondary acute dystonia diagnosis was connected to bupropion, and an abnormal involuntary movement scale (AIMS) was done. The AIMS score was 22. Thus, biperiden was intramuscularly injected to the patient to the amount of 7.5 mg and the patient's symptoms were resolved after about 30 min. Bupropion treatment was halted.
Complete blood count, Vitamin B12 level, folic acid level, ferritin, biochemistry, and screening for Wilson's disease were performed. Cranial magnetic resonance imaging was performed to rule out secondary causes of dystonia and showed no pathologic point. At follow-ups 24 h, 2 weeks, 4 weeks, 8 weeks, and 12 weeks later, no recurrence of symptoms of dystonia occurred. Treatment and follow-up of the patient in the outpatient clinic still continue, and the patient is under treatment with fluoxetine up to 50 mg/day.


A 30-year-old Korean man was admitted to our hospital due to left flank pain associated with ureter stone. Initial complete blood count (CBC) showed a hemoglobin (Hb) level of 11.9 g/dL (reference range, 13–17 g/dL) and a platelet count of 127,000/µL (reference range, 150,000–350,000/µL) with a white blood cell (WBC) count of 46,030/µL (reference range, 4,000–10,000/µL): 30.6% neutrophils, 7% lymphocytes, 3.9% monocytes, 55.7% eosinophils, and 1% basophils. Peripheral blood (PB) smear also showed increased number of eosinophils (). Blood urine nitrogen (BUN) and creatinine levels were within reference ranges. Causes of eosinophilia due to allergy and parasite infections were excluded from further studies. Bone marrow examination was performed, showing hypercellular marrow with eosinophilic precursors, including mature eosinophils, being counting up to 36.2% (). The chromosome study showed a normal karyotype; however, the FIP1L1-PDGFRA rearrangement was detected by fluorescence in situ hybridization (FISH) analysis (). Diagnosis of CEL with FIP1L1-PDGFRA rearrangement was made. Our patient was treated with daily administration of imatinib mesylate (100 mg) and followed up with CBC. After 1 year, the follow up CBC showed normalized leukocyte count of 8,170/µL with 3% eosinophils. Furthermore, eosinophil precursors were within reference range of bone marrow aspiration. FISH analysis using a FIP1L1-PDGFRA probe showed normal signal patterns (without deletion signals).


A 10-year-old Asian Indian male child was brought to the emergency services of our tertiary care hospital with progressive bulging of the left eye for the past 7 days. It was associated with deep constant periorbital pain, inability to close the eye, fever with no rigors or chills, discharge, and decreased vision. The patient gave a history of scratching of boil over the left upper eyelid 3 days before start of symptoms. There was no history of upper respiratory tract infection, toothache, ear discharge, insect bite, or trauma. The patient had received oral penicillins before presentation at our center, but there was no relief.
Examination of the affected eye revealed visual acuity of 6/24, hypoglobus, mechanical ptosis, restriction of ocular movements in all directions; swelling of lids with overlying redness of skin with raised temperature and tenderness on palpation. Anterior segment examination showed conjunctival chemosis and diffuse descemet folds while posterior segment examination was unremarkable. Investigation showed hemoglobin of 13.6 g/dL, total leukocyte count of 12,500/mm3, erythrocyte sedimentation rate of 45 mm in 1st h, and no pus cells in urine. Blood and urine cultures were negative. Noncontrast computerized tomography (NCCT) scan showed ill-defined hypodense collection in the extraconal department of the left orbit, extending up to orbital septum and causing proptosis suggestive of orbital abscess. There was associated with thickening of superior rectus-levator palpebrae superioris (SR-LPS) complex but no evidence of sinusitis [].
A straightforward diagnosis of orbital cellulitis with abscess formation was made; and intravenous amoxicillin (25 mg/kg/day in three divided doses) and clavulanic acid and metronidazole (7.5 mg/kg/day in three divided doses) and amikacin (15 mg/kg/day in three divided doses) were started empirically. Abscess was drained and pus samples were sent for microbiological examination. We were left in a fix when there was no improvement even after 3 days of treatment. Microbiology report unveiled the mystery behind this odd behavior and guided us toward the correct path for further management. Culture on blood agar showed greenish discoloration suggestive of alpha (complete) hemolysis while MacConkey agar showed magenta-colored colonies. Microscopy shows Gram-positive spectacle-shaped cocci arranged at an angle to each other and in lines. The organism was catalase negative, grew on 6.5% sodium chloride, and produced black colonies on blood tellurite agar. This confirmed the presence of Enterococcus faecalis []. Sensitivity as per the Clinical and Laboratory Standards Institute revealed resistance to ciprofloxacin, penicillins, and cotrimoxazole and sensitivity to clindamycin, teicoplanin, linezolid, gentamicin, and doxycycline. After pediatric consultation, intravenous clindamycin (20 mg/kg/day in three divided doses) and gentamicin (1.5 mg/kg/day in three divided doses) were started. Swelling and proptosis started reducing by day 2 of revised therapy. On day 10, best-corrected visual acuity improved to 6/6, swelling and proptosis disappeared, and extraocular movements became full and free. Some amount of ptosis persisted while extraocular movements were full and free. Repeat NCCT showed thickened LPS-SR complex, with involvement of tendons suggestive of myositis [Fig. and ]. The patient was then put on oral steroids 1 mg/kg/day. After 1 month of steroid treatment, there was improvement in the amount of ptosis [Fig. and ].


A 57-year-old man was transferred to emergency department of Ajou University Hospital for treatment of Stanford type A aortic dissection. The patient had chronic kidney disease (serum creatinine, 1.8 mg/dL) and mild cognitive impairment (Mini-Mental State Examination score, 16) due to previous tuberculous meningitis. The initial electrocardiogram showed inferior myocardial infarction, complete atrioventricular block, and a temporary pacemaker that was inserted before transfer to our hospital. Emergency surgery was performed, and the operative findings showed intimal tearing at the proximal ascending aorta with occlusion of both coronary ostia by the flap. We performed hemiarch graft interposition with a Hemashield 22-mm graft and coronary artery bypass grafting (CABG) to the left anterior descending (LAD) artery and right coronary artery with saphenous vein grafts (SVGs). The patient was treated with hemodialysis during the early postoperative period, but was discharged on the 100th postoperative day without renal replacement.
Three months after discharge, the patient was admitted to the nephrology division to manage acute renal failure on preexisting chronic renal failure (serum creatinine, 4.3 mg/dL). A permanent hemodialysis catheter was inserted and hemodialysis was performed for 3 days. The patient’s renal function improved gradually, and he was therefore discharged on the 11th day of hospitalization with a permanent hemodialysis catheter (serum creatinine, 2.1 mg/dL). Two months after discharge (8 months after first operation), the patient was admitted to the infectious diseases division to treat hemodialysis catheter-related sepsis. The patient’s white blood cell (WBC) count, percentage of neutrophils, and C-reactive protein (CRP) level were 7,900/μL, 93.2%, and 26.02 mg/dL. The causative organism was Streptococcus pyogenes. The permanent hemodialysis catheter was removed and the patient was treated successfully with systemic antibiotics for 3 weeks.
Twelve months after that admission (21 months after first operation), the patient revisited our emergency department complaining of a 3-day history of general weakness and fatigue. The mediastinum was wider than had been observed in a previous study (), and contrast-enhanced chest computed tomography (CT) was performed (). This revealed a 5×4×5-cm hematoma alongside the ascending aorta graft. Contrast enhancement of the hematoma suggested rupture of the artificial graft, and we performed aortography to obtain a precise diagnosis. This showed the extravasation of contrast medium from the proximal portion of the ascending aorta and rapid flow of contrast medium in the pseudo-lumen alongside the ascending aorta ( ). We performed an emergency explorative sternotomy under the impression of a rupture of the artificial graft.
The upper portion of the pericardium was bulging and resembled a pseudoaneurysm sac. Before opening the bulging pericardial sac, we started cardiopulmonary bypass and lowered the patient’s temperature to 28°C. After opening the pericardial sac under total circulatory arrest, two oval defects were seen in the proximal portion of the artificial graft. The inner surface of the sac was smooth and clean, with no evidence of pus or current infection. The defects in the artificial graft were the sites of the proximal anastomoses of the SVGs from the previous CABG, but no visible SVGs were present in the operative field ( ). The flow of the native coronary arteries was intact in preoperative aortography, and no newly developed regional wall motion abnormality was found on intraoperative transesophageal echocardiography. We resected the original artificial graft and interposed a new artificial graft. No evidence of coronary artery malperfusion was found while weaning the patient from cardiopulmonary bypass or during the postoperative period. Coronary artery multidetector CT (MDCT) performed postoperatively revealed obstructed SVGs and ectatic changes of the distal portion of the SVG to the LAD artery (). The systolic function of the left ventricle and the flow in the native coronary artery were intact. The patient was discharged on the 42nd postoperative day.


A 68-year-old man with past medical history of hypertension, hyperlipidemia, and colonic diverticulosis presented with progressive right lower quadrant abdominal pain and constipation during the prior 9 mo. Colonoscopy with good cecal visualization, performed 2 years earlier for routine colon cancer screening, had revealed a normal colon and normal appendiceal orifice. Physical examination revealed normal vital signs, soft abdomen, minimal right lower quadrant tenderness, no rebound tenderness, and no palpable abdominal mass. Laboratory analysis revealed hemoglobin = 13.1 gm/dL, leukocyte count = 12000/mL, and serum bicarbonate = 28 mmol/L. Serum electrolytes, serum parameters of liver function, serum parameters of renal function, and serum lactate level were within normal limits. Abdomino-pelvic computed tomography (CT) revealed a dilated, heterogeneous, appendix with an 8-cm-long, ovoid, periappendiceal mass containing a fistula to sigmoid colon (Figure ), and revealed no findings suggestive of pseudomyxoma peritonei or peritoneal implants, including intraperitoneal fluid, peritoneal calcifications, or scalloping of the liver. Abdomino-pelvic magnetic resonant imaging (MRI) showed on coronal view a dilated, 8-cm-long, appendix fistulizing to the sigmoid (Figure and C); and showed on axial view an abnormally thick, enhancing, appendiceal wall without significant peri-appendiceal inflammation (Figure ). Open laparotomy revealed an extensive mass involving appendix, cecum, sigmoid colon, anterior abdominal wall, and urinary bladder (Figure ); no peritoneal implants, and no pseudomyxoma peritonei. The abdominal mass was removed en-bloc, including resection of sigmoid colon, cecum (with preservation of ileocecal valve), appendix, right vas deferens, testicular vessels, and minimal amounts of anterior abdominal wall; and shaving off of small parts of the walls of the urinary bladder and small bowel. Gross pathological examination of the resected mass revealed an appendix-to-sigmoid fistula, as confirmed by a probe, from prior perforation of a promontoric (preileal/postileal appendix traveling from cecal base towards the sigmoid in the pelvis) appendix (Figure ). Microscopic pathology showed well-differentiated, invasive, mucinous, adenocarcinoma diffusely involving the appendix, sigmoid, and cecum through the serosa (Figure ). Histopathology showed no invasion of adjacent organs, such as the bladder wall or anterior abdominal wall. Lymphovascular invasion and satellite peritumoral nodules were not present. All surgical margins and all 13 resected lymph nodes were devoid of cancer (Stage pT4b N0). The patient developed postoperative ileus from which he recovered, and was discharged 11 d postoperatively. No postoperative chemotherapy or radiotherapy was administered. The patient was doing well 1 year later, with no ascites, pseudomyxoma peritonei, or cancer recurrence, as observed clinically by an oncologist and by repeat abdomino-pelvic CT examination.


A 64-year-old woman with upper abdominal pain and back pain was referred to our hospital for examination of a pancreatic mass that had been identified by the previous doctor on abdominal ultrasonography (US). Laboratory examinations revealed anemia (hemoglobin level of 10.3 g/dL) and a slightly elevated carbohydrate antigen (CA) 19-9 level (65.1 U/mL). Liver, biliary, and pancreatic enzyme activity levels were normal. Abdominal US revealed a heterogeneous hypo-echoic mass with a cystic component in the pancreatic body. Contrast-enhanced abdominal computed tomography (CT) revealed a well-demarcated low-density mass measuring 35 mm in diameter, with an enhancement of the peripheral portion of the lesion in the pancreatic body during the delayed phase (Figure ). Magnetic resonance imaging (MRI) revealed a tumor showing mixed signal intensity and a fluid-fluid level on T2-weighted images, suggestive of intratumoral hemorrhage (Figure ). Endoscopic retrograde pancreatography (ERP) revealed compression of the main pancreatic duct (MPD) in the pancreatic body without any strictures or upstream dilatation of the MPD. Although the appearance on these images was not typical of conventional pancreatic cancer, cytology of the pancreatic juice samples collected during ERP was suggestive of adenocarcinoma. Based on a preoperative diagnosis of PDAC, surgery was performed. On laparotomy, the tumor had rapidly increased in size and involved the common hepatic artery. Therefore, distal pancreatectomy and splenectomy with en bloc celiac axis resection were conducted. On gross examination, a poorly demarcated, whitish tumor measuring 60 mm in diameter was observed (Figure ). In the central portion of the tumor, a large cystic lesion filled with hemorrhagic material was present. Histologically, the major part of the tumor exhibited a sarcomatous appearance and was composed of non-cohesive, pleomorphic tumor cells (Figure ). In the minor part of the tumor, a moderately differentiated tubular adenocarcinoma component was detected (Figure ). The central part of the tumor formed a cystic lesion, which contained hemorrhagic and/or fluidic material and was surrounded by necrotic area of the sarcomatous tumor (Figure ). Immunohistochemical analyses revealed that the sarcomatous component was positive for both pancytokeratin (AE1/AE3) (Figure ), an epithelial marker, and vimentin, a mesenchymal marker (Figure ). In contrast, the adenocarcinoma component was positive for only pancytokeratin (Figure ) but was negative for vimentin (Figure ). Based on these pathological findings, the tumor was diagnosed as pleomorphic ACP according to the JPS classification and was classified as T3N1M0, Stage IIB, according to the UICC TNM staging system. Despite receiving gemcitabine-based postoperative chemotherapy, the patient died of local recurrence and multiple liver and lung metastases five months after surgery.
We conducted a comprehensive search of Ichushi-Web (a domestic medical literature database service provided by the NPO Japan Medical Abstracts Society) from 1995 to 2014 using the term “anaplastic carcinoma of the pancreas”, and we retrieved 59 previously published case reports of patients with ACP who underwent surgical resection and for whom clearly presented data were available in the Japanese language literature (Supplementary Table ). The clinicopathological data and radiological findings described for these 60 cases, including the present case, were evaluated. The tumor stages of each case were assigned according to the UICC classification system based on the surgical and pathological findings reported in the literature unless the descriptions of the extent of the tumor were insufficient. The prognostic outcome of each case was also obtained from the published data. The clinicopathological, radiological, and morphological characteristics of the 60 cases are summarized in Table . The average age at diagnosis was 61.5 years (age range, 32 to 85 years), and 38 of the 60 cases (63%) were men. Most patients presented with symptoms such as abdominal pain (n = 29, 48%), back pain (n = 10, 17%), fatigue (n = 8, 13%), fever (n = 6, 10%), jaundice (n = 6, 10%), body weight loss (n = 6, 10%), and abdominal discomfort (n = 6, 10%). In four cases (7%), pancreatic tumors were diagnosed incidentally. The tumors were located in the pancreatic head in 32 cases (53%), the body and/or tail in 25 (42%), and the entire pancreas in three (5%). The tumor size ranged from 1.5 to 24.0 cm, with a median of 6.0 cm. Laboratory studies revealed severe anemia (hemoglobin level of < 10.0 g/dL) in 12 cases (20%) and a markedly elevated leucocyte count (> 12000/mm3) in 12 cases (20%). Elevation of serum CA19-9 levels (> 37 U/mL) was observed in 33 cases (55%). Tumor or rim enhancement on abdominal CT was observed in 49 cases (82%). On imaging studies including abdominal CT, US, and MRI, a cyst-like appearance of the lesion was observed in 28 cases (47%). Regarding surgical procedures, pancreaticoduodenectomy (PD), including pylorus-preserving PD and subtotal stomach-preserving PD, was performed in 32 cases (53%), distal pancreatectomy in 24 (40%), total pancreatectomy in two (3%), and duodenum-preserving pancreatic head resection and tumorectomy in one (2%) each. Combined resection of adjacent organs, including the stomach, colon, jejunum, or left adrenal gland, was required in 14 cases (23%). Vascular involvement, including involvement of the common hepatic artery, celiac artery, or portal vein, were observed in 7 cases (12%). In the resected specimens, macroscopically visible hemorrhagic necrosis was observed in 46 cases (77%). Cyst formation was observed macroscopically in 20 cases (33%). According to the sixth edition of the General Rules for the Study of Pancreatic Cancer by the JPS[], the 60 cases were histopathologically classified as giant cell type (n = 11), pleomorphic type (n = 18), spindle cell type (n = 10), or GCCO type (n = 21). Lymph node metastases were found in 18 of 45 cases for which relevant data were available (40%). Recurrence was reported in 34 cases at one or more sites. The representative sites of recurrence were the liver (n = 23, 68%), a local site (n = 10, 29%), the peritoneum (n = 8, 23%), or lymph nodes (n = 7, 21%). Only two cases (6%) presented with lung metastasis. Regarding clinical outcome, 29 of 57 patients (51%) for whom relevant data were available died of their disease within 12 mo (< 1-year survivors). In contrast, seven patients (12%) were reported to be 5-year survivors. According to histological subtype, the group of < 1-year survivors comprised seven of 11 patients with giant cell type (64%), 13 of 18 with pleomorphic type (72%), seven of 10 with spindle cell type (70%), and two of 18 with GCCO type ACP (11%). Furthermore, the group of 5-year survivors comprised 0 (0%), 4 (22%), 1 (10%), and 2 patients (11%) with giant cell type, pleomorphic type, spindle cell type, and GCCO type ACP, respectively.


Patient is a 6-year-old girl who presented with headache and vomiting, both of four weeks duration. Headache was insidious, dull, predominantly left sided, worse in the morning associated with vomiting which relieves it. She also had associated gait disturbance. Neurological examination showed a fully conscious female child with intact high cerebral function. She has truncal ataxia, otherwise the cerebellar function was preserved. Other systems were unremarkable.
Cranial computed tomography and magnetic resonance imaging revealed left cerebellar tumor with obstructive hydrocephalus ( and ). She had an initial right frontal Venticulo-peritoneal shunt for cerebrospinal fluid diversion, and late left retrosigmoid suboccipital craniotomy with microscope assisted gross total resection of left hemispheric tumor one week later under general anesthesia. The excision was confirmed by radioimaging after surgery ().
Intraoperative finding was soft greyish to yellowish mass with partly cystic consistency and a fairly well defined brain-tumor boundary. There was a leash of tumor vessels that bled briskly. She had fever and mild anemia post-surgery and these were managed. She was discharged from the hospital two weeks later with a Karnofsky score of 90% and is still being followed up in the out-patient, while awaiting possible radiotherapy if her condition does not remain stable. Histology showed an extensively lipidized tumor composed of sheets of mature adipose tissue with some neurocytic cells in the background minority with hyalinized blood vessesls. No mitosis was seen. Immunohistochemistry was positive for S-100 and desmin consistent with lipocystic medullocytoma.


A 19-year-old man presented to the orthopedic surgery department with pain in the left buttock after military training which including running with hyper-flexion of hip for 3 hours, twice a day for 5 days. When he hyper-flexed his left hip joint, he felt and heard a crack. Initially about one week later, he referred to the local hospital because of persisting hip pain. On radiographic evaluation, he diagnosed as pelvic bone fracture and recommended for further evaluation. However, he refused to get more examination due to the alleviation of the hip pain. Although he got rest without any military training for the next six weeks, there was no improvement and tingling sense developed over the posterior aspect of left thigh. During the first consultation with the patient, he complained difficulty on sustained standing and sitting on a locked position.
His body weight, height, hip and lumbar T-score for dual-energy x-ray absorptiometry (DXA) scan were 76 kg, 183 cm, –0.7 and –0.1 respectively. On physical examinations, there was no limitation on range of motion of the left hip joint. Also he could walk without any pain except mild tenderness on the area distal from the ischial tuberosity. On the radiographs of the pelvis anteroposterior and frog leg view (), there was a 35×40×20 mm sized bony fragment, 15 mm distance from the left ischial tuberosity. After the patient admitted to our hospital, he had an examination of computed tomography () and magnetic resonance imaging for rule out pathologic conditions. There was a bony fragment at left ischial tuberosity, which was mainly from conjoined origin of biceps femoris and semitendinosus tendons without any bone marrow edema (). Overall, he diagnosed as old avulsion fracture at ischial tuberosity. He determined to get conservative treatment on condition of a gradual alleviation of the symptom. During the admitted 2 months, he just got rest and high intensity laser therapy. Thanks to the improvement except discomfort on running, he discharged and returned to his military unit.


A 41-year old pregnant woman wanted to receive a first-trimester combined screening test at 15 gestational weeks. This screening was indicated because of parental anxiety due to advanced maternal age. Couples have an age-dependent risk for fetal aneuploidy, of which trisomy 21 is the most common form. There was no fetal pathological ultrasound finding or history of a fetus or child with a major congenital anomaly. The couple has two children that are phenotypically normal females ().
From the amniotic fluid material, a karyotype analysis was performed at 16 gestational weeks. The samples underwent rapid aneuploidy testing for trisomies 21, 13, and 18 or for a monosomy X before conventional karyotyping, and the results were normal. There was no large structural chromosome anomaly present. A more detailed conventional karyotyping analysis reported an abnormal karyotype: 46, XY, add(9)(p24).
An abnormal karyotype can originate from parents that carry a balanced translocation or de novo; therefore, parental blood samples were collected. The parental standard chromosomal analysis showed a normal karyotype.
To further analyze the additional material of unknown origin, a CMA from the amniotic fluid was performed. A single nucleotide polymorphism (SNP) microarray revealed a male fetus with balanced chromosomal translocations on 9p and balanced chromosomal rearrangements, but another chromosomal abnormality was detected: arrXp22p22.13 (16,992,941-17,729,022)x2; Xp21.3(28,791,782-28,903,443)x2. The fetus had a de novo duplication that was 736 kb in size at p22.13 and a duplication that was 112 kb in size at p21.3. The 22.2 and Xp21.3 duplications have not been previously reported, and it is unknown whether they are considered pathogenic, benign, or of unknown significance compared to those in public National Institute of Child Health and Human Development (NICHD) databases. Translocations or deletions of Xp22.2 may cause Nance-Horan syndrome (NHS). NHS is an X-linked genetic disorder. Comparative mapping has identified the NHS gene to be localized in the Xp22.31-p22.13 region to a 15-18 cM interval between the loci DXS85 and DXS1226. Translocations or deletions of Xp21.3 are recognized as a possible cause of mental retardation. Microduplication may be more likely to identify differences that have uncertain significant clinical variations and incidental findings.
Unlike conventional karyotyping, CMA cannot detect balanced translocations. Therefore, we recommended the use of fluorescence in situ hybridization levels of resolution on microduplication. On the basis of these experiments, the parents decided to conduct no further management. The child was born a phenotypically normal male weighing 2,750 g at 37 gestational weeks. Routine karyotyping of child blood samples was not performed.


A 32-year-old male underwent LAGB for obesity 12 years previously. He presented at the outpatient clinic with a productive cough, small-volume hemoptysis, and fever that had persisted for 7 days. He reported two recent months of frequent choking while asleep at night. Upper gastrointestinal endoscopy performed 2 weeks ahead of the presentation showed a dilated esophagus containing fluid and food. The patient had a 10-pack year history of smoking and had poor compliance with proton pump inhibitors. At presentation, his temperature was 39.3°C; blood pressure, 100/70 mmHg; pulse, 107 beats/min; respiratory rate, 22 breaths/min; oxygen saturation while breathing room air, 96%; weight, 118 kg; and height, 179 cm (body mass index = 36.9). Chest auscultation indicated diminished breath sounds and few crackles in the right lung. Laboratory tests showed a white blood cell count of 12.8 × 103/µL; hemoglobin level, 13 g/dL; platelet count, 324 × 103/µL; and C-reactive protein level, 169 mg/L. The renal profile was normal. Chest X-ray showed relatively well-defined right paramediastinal apical lung opacity [arrow in ]. A same-date high-resolution chest computed tomography (CT) confirmed a 5.5 cm right upper lobe apical segmental mass that contacted the mediastinum [arrow in ], and esophageal dilation with internal fluid [arrowhead in ].
The patient was hospitalized with a presumptive diagnosis of community-acquired pneumonia. He was administered 2 g intravenous ceftriaxone once daily and 600 mg of clindamycin every 8 h. Sputum staining for bacterial or mycobacterial tuberculosis yielded negative results. Since the fever persisted for 3 days, diagnostic bronchoscopy was performed, which revealed signs of gastroesophageal reflux disease (GERD) and a narrowed apical segment of the right upper lobe. Bronchoalveolar lavage of the apical segment of the right upper lobe tested negative for malignancy or infection. Multiple transbronchial lung biopsies from the apical segment of the right upper lobe were pathologically examined and showed an exudate of fibrin and neutrophils and reactive respiratory type of epithelium []. No fibromyxoid masses, histiocytes, or evidence of malignancy was identified. The pathological diagnosis was consistent with organizing pneumonia.
The patient was administered 40 mg of daily prednisone for 1 month, which was afterward tapered over 3 months. He completely recovered 1 month after hospital discharge. A follow-up chest CT, 1 month after hospital discharge, revealed near-complete resolution of the right upper lobe lesion, with minimal residual scarring [arrow in ]. The gastric band was adjusted to prevent future aspiration incidents, and the patient was counseled to quit smoking.


A 52-year-old, previously healthy male who suffered from sudden convulsion for 3 hours was admitted in intensive care unit (ICU). The patient was suddenly unconscious around 8:30 am after breakfast. He was found foaming, limb stiffness, and urinary incontinence. The seizure lasted 5 to 7 minutes each episodes along with the interval of 20 to 30 minutes. Physical examination: the patient was in a coma, T:37.0°C, HR: 98 bpm, BP: 123/87 mm Hg. Neurological examination: Babinski sign (+) on his right foot. Computer tomography (CT) scan and electroencephalogram (EEG) were unremarkable and electrocardiograph (ECG) showed sinus tachycardia. Serology assay on the day of admission revealed ALT:64 IU/L, AST:39 IU/L, CK: 230 U/L, LDH: 55U/L, HBDH: 321U/L, CK-MB: 44 U/L, cnT ∼0.1 μg/L. The cerebrospinal fluid (CSF) routine test and culture were assessed as negative. Supportive treatments were done and the patient was discharged with a better health condition without a definite diagnosis.
One month later, the patient died suddenly and unexpectedly. The cause of death was determined as TETS poisoning through the toxicological test. According to the police investigation, the suspect poisoned the victim with TETS a month ago. Therefore, the patient's blood samples during his hospitalization were sent for the toxicological test immediately, and the results showed 0.42 μg/mL and 0.35 μg/mL by gas chromatography-mass spectrography (GC-MS), demonstrating TETS poisoning (see Fig. ).


A 48-year-old man was witnessed generalized convulsions and aphasia and brought to emergency room. His past medical history was unremarkable and free of injuries. Physical examination: The patient was in a coma, T: 36.7°C, HR 86 bpm, BP 164/100 mmHg, and his myodynamia decreased. Dysphagia and dysphonia observed. The patient's brain CT showed hypoxic–ischemic changes lightly at hippocampal regions and cerebral cortex. The results of blood routine test, plasmic electrolytes, chest CT scan, and ECG were unremarkable. The biochemical test showed CK 413.0U/L, ALT 58 U/L, AST 47 U/L, and hematuria observed, indicating myocardial injuries and dysfunction of liver and renal. The CSF routine test and culture were assessed as negative. Through responsive treatment, the patient's clinical symptoms disappeared 8 days later without a clear diagnosis.
The patient presented similar symptoms twice in next 4 months. The etiology was not clear until his wife was arrested for poisoning her child to death 6 months later. The suspect confessed that she intended to poison her husband and put FAA in the daily dishes for several times in last few months. However, her husband recovered after treatment every time. The blood sample taken from the patient was sent for GC-MS analysis, and the result shown FAA poisoning with positive of FCH2COO− derivative (1.13 μg/mL) (see Fig. ).


A 13-month-old female was admitted to the Pediatric Emergency Department due to a generalized tonic-clonic seizure lasting for a few minutes. She was born in the 39th week of gestation without perinatal problems. She could control her head 4 months after birth and rolled over at 5 months. At 7 months, she could sit alone. However, she could not stand or walk until 14 months of age.
Biochemical findings showed a low serum calcium of 4.8 mg/dL (normal range, 8.3–10 mg/dL); phosphorus of 4.3 mg/dL (normal range, 2.5–4.5 mg/dL), and elevated alkaline phosphatase of 1,665 U/L (normal range, <269 U/L). PTH was 178 pg/mL (normal range, 10–65 pg/mL) and 25(OH) D3 was 75.1 ng/mL. Initial 1,25(OH)2D3 level was 5.26 pg/mL (normal range, 15–90 pg/mL). Echocardiography showed QT prolongation. An evaluation for renal tubular acidosis was normal, with normal urine pH. Tubular reabsorption of phosphate was 88%. Radiographic images of the wrist showed cupping and fraying of the ulna and distal radius, as well as metaphyseal widening, indicating rickets (). Harrison sulcus and rachitic rosary were also observed (). There was no family history of rickets.
A mutation analysis of the CYP27B1 gene was performed at the age of 10 years. All coding exons and intron in flanking regions of CYP27B1 were amplified by polymerase chain reaction and directly sequenced using an ABI3130x1 Genetic Analyzer (Applied Biosystems, Foster City, CA, USA). She was homozygous for c.589+1G>A in the splice donor site in intron 3 (), which was reported to be pathogenic.
The patient was immediately treated with intravenous calcium gluconate (20 mg/kg/day) for the hypocalcemic seizure. After her symptoms were relieved, she was discharged with oral calcium carbonate (30 mg/kg/day of elemental calcium) and calcitriol (0.5 µg/day). Compliance with the treatment was good, resulting in rapid amelioration of the clinical, biochemical, and radiological abnormalities. The serum calcium normalized within a month, and there were no recurrent seizures. The cupping and fraying of the ulna and distal radius was improved after 5 months of therapy. After 2 years of treatment with calcitriol and calcium supplement, follow-up radiological images demonstrated fully healing of the long-bone bowing and flaring (). The patient's biochemical parameters and anthropometry during the follow-up period are shown in .
The patient had neither genu valgum/varum nor scoliosis. Nephrocalcinosis did not develop during the follow-up period. But she had severe dental caries. At her last visit at the age of 17.8 years, her height and weight were 166 cm (1.1 standard deviation score [SDS]) and 68 kg (1.6 SDS), respectively, and she had no symptoms with the calcitriol (0.5 µg/day) and calcium (400 mg/day of elemental calcium) treatment ()


A 74-year-old female was admitted to the Emergency Department of Sekghoma Memorial Hospital, Serowe village, Botswana, past midnight with progressive swelling of her face, tongue and breathing difficulty for about 8 h. The family gave a history of change of antihypertensive medication recently. She has been started on tablet enalapril, 20 mg, once daily, orally, 2 days back. On examination, she was conscious, coherent but very anxious. Her heart rate was 123/min, regular. Blood pressures were high and reading was 180/96 mmHg. Her room air oxygen saturation was 91% (SpO2). There was no stridor. On auscultation of chest, air entry was good bilaterally and there were some conducted sounds from pharynx due to excessive secretions. The tongue was grossly swollen, hard in consistency, it was wedged between the teeth and she was not able to close her mouth []. Lot of secretions were pooling in and dribbling from the mouth. There was no space in oral cavity for oral intubation. Only possible airway managements were a blind nasal intubation or surgical airway. It was not possible to transfer this case to a tertiary care hospital by helicopter as there were landing issues in darkness. Transfer by road was risky for patient as it would have taken 5 hours. It was decided to go ahead with management locally. The patient was started on oxygen support at the rate of 5 l/min by facemask for building up her oxygen reserves in case of airway emergency. Lateral view X-ray of neck was done which showed airway patency as fine []. Elective blind nasal intubation as a proactive airway management was risky due to less chances of success and more risk of further airway compromise. There were no ear, nose, and throat (ENT) specialists available for either evaluation or surgical airway management. She was injected with 100 mg of hydrocortisone, intravenously and 0.5 ml of injection adrenaline (1:1000) subcutaneously. Injection pheniramine maleate, 45.5 mg, was injected intravenously. She was positioned in sitting position. Intermittent atraumatic oral suction was advised. She was observed for about 30 min in which she gradually became worse and swelling of face and tongue increased. Blood pressures were shooting up. Provisional diagnosis was enalapril-induced angioedema. We decided to go ahead with fresh-frozen plasma infusion under intravenous beta-blocker antihypertensive coverage. Her blood group was O-positive and hospital being a peripheral center; we had only blood storage facility where O-positive fresh-frozen plasma was not available. Blood bank was 3 h away. We decided to go ahead with transfusion of the only pint of O-negative fresh-frozen plasma available at our storage facility. Injection metoprolol, 5 mg, intravenously was instituted and then we started with O-negative fresh-frozen plasma infusion intravenously, 220 ml over next 30 min. On post-fresh-frozen plasma infusion, patient's condition started improving []. Blood pressures remained stable around 150/90 mmHg after metoprolol injection. Over next 2½ hours, she recovered grossly and after 6 h she was able to close her mouth completely. We shifted her to wards after counseling about ACEI and ARB. We explained in detail the need to avoid enalapril.[]


This is the case of a 43-year-old female with past medical history of bronchial asthma, migraine, and crack smoking, who came to San Juan City Hospital because she started to present blood in her urine 5 days prior. Also, she reported fever, chill, and fatigue. The patient also presented with right knee erythema, warmth, swelling, and pain. The knee pain was 8/10 in intensity that increased when moving. The patient denied surgeries or traumas to the area. She also denied allergies and used no medications. Her history was remarkable for smoking crack daily for 30 years. Her last use was the day prior to admission. The physical exam showed bilateral purpuric macules in the ear helix; eroded plaques and elevated borders over the left foot; a 0.4 mm lesion at the finger dorsum; as well as vascular lesions in the knee and forearm ( and ). Toxicology was positive for cocaine. HIV test and hepatitis profile were nonreactive. Other laboratories showed increased levels of cytoplasmic ANCA (c-ANCA; 1.7 IU/mL), perinuclear ANCA (P-ANCA; 1.7 IU/mL), and antinuclear antibody (ANA; 3.1 units). Immunoglobulin G levels were 2,354 mg/dL and immunoglobin M was 490 mg/dL, which are both elevated. The ANA test showed homogenous pattern with titers >1:320 Dils. ANCA specificities to neutrophil lactoferrin were negative, as well as to cathepsin G and lysozyme. However, an enzyme-linked immunosorbent assay for elastase was positive. Also, cryoglobulins were positive. Complement levels were decreased; C3 = 81.9 mg/dL and C4 = 13 mg/dL. Anticardiolipin antibodies were negative (immunoglobin G: 9 U/mL; immunoglobin A: 7.6 U/mL; immunoglobin M: 8.2 U/mL), as well as lupus anticoagulant. The patient had an elevated erythrocyte sedimentation rate of 107 mm/hour and an elevated C-reactive protein level of 72.3 mg/L. The cell blood count showed leucopenia (white blood cell count = 4.5 × 103/Ul), a microcytic anemia (hemoglobin = 9.2 g/dL, hematocrit = 28.2%, mean cell volume = 71.39 fl, and red blood cell distribution width = 19%), and platelets of 183 × 103 uL. Her d ifferential showed 75% neutrophils, 20% lymphocytes, 2% monocytes, and bands of 2% with an absolute neutrophil count of 3,465 cells/mm3. Urinalysis came with proteinuria (150 mg/dL), occult blood (250 uL), red blood cells (loaded – not countable/high power field), and many amorphous crystals. The punch biopsy of her ear showed superficial thrombosis of superficial vascular plexus with perivascular lymphocytic infiltrates and deeper sections showed epidermal necrosis and necrotizing vasculitis (). Cultures of the ulcers came back with no organism growth. Also, blood cultures and urine culture were negative for an infective organism. This patient was started on a high dose of steroids, but didn’t finish treatment since she escaped from the hospital.


A 47-year-old man had a history of metastatic lung carcinoma and a pathological fracture involving the 10th thoracic vertebral body. The patient presented complaining of back pain in the lower thoracic area for 3 months that progressively became severe; at presentation the patient rated the pain as 9 on the 10-point VAS scale. MRI of the vertebral column showed a lytic metastasis within the 10th vertebral body that was well confined to the body of vertebra (). Through a left costopedicular approach, a 9-gauge needle was used to aspirate the contents of the lytic lesion, followed by a saline wash. PSV was done to fully fill, but not overfill, he empty lytic lesion. Complete filling of the lytic focus within the left aspect of the vertebral body with 2.5 mL of cement was achieved. The cement demonstrated a nearly focal appearance within the lytic lesion (). Following the procedure, pain was rated 1 week, 1 month, 3 months, and 6 months postoperatively; the VAS value was 5, 4, 3, and 2, respectively.


A 29-year-old caucasian male was referred for management of 1 week vision loss. Visual acuity was 20/50 in the affected right eye (OD) and 20/25 in the left eye (OS). Intraocular pressure and anterior segment examination of both eyes were normal. Posterior segment examination was normal OS, whereas OD revealed an ill-defined reddish-yellow intraretinal mass with overlying exudation, hemorrhage, and subretinal fluid (SRF) along the inferotemporal periphery [–]. Ultrasonography showed a dense intraocular mass measuring 1.7 mm in thickness with SRF and attached posterior hyaloid, whereas fluorescein angiography showed peripheral nonperfusion with hyperfluorescence of the lesion and late leakage, consistent with a retinal VPT. Macular optical coherence tomography (OCT) revealed an ERM, with vitreomacular traction, mild macular edema, and distortion of the normal foveal contour [ and ]. Treatment options at that time included laser photocoagulation, photodynamic therapy, or cryotherapy, and considering small size, peripheral location, and presence of an ERM, trans-scleral cryotherapy was provided.
Two months following cryotherapy, the tumor showed regression, but vision worsened to 20/400 secondary to progressive vitreomacular traction and CME [ and ]. At this time, options included intravitreal steroid injections, anti-VEGF injections, or PPV with membrane peel. After discussion of the risks and benefits, the patient elected PPV with combined ERM and internal limiting membrane (ILM) peel. Six months postoperatively, vision returned to 20/50, and OCT showed resolution of vitreomacular traction with mild residual extrafoveal CME, slight flattening of the foveal contour, and mild ellipsoid irregularity on OCT [ and ].


A 31-year-old female with a history of travel to Malaysia 6 weeks prior presented with a 3-week history of high-grade fever with chills. She reported daily 38.3°C fever without diurnal variation. She had a history of watery, nonbloody diarrhea 3 days prior and occasional cough. She denied any history of breathlessness, chest pain, diabetes mellitus, or hypertension.
On examination, her general condition and vital signs were normal. The patient had prominent bilateral parotid swelling measuring roughly 5 by 4 cm which was soft, tender and nonfluctuating [], and mild right inguinal lymphadenopathy. Rest of the systemic examination was normal.
The patient was investigated as shown in . Viral markers and multiple blood cultures were negative. In view of clinical features of persistent fever with leukopenia, serological tests for Cytomegalovirus IgM antibody and Epstein–Barr virus IgM were done, which were also negative.
The patient was started on injection amoxicillin-clavulanate (dose 1.2 g twice a day) and symptomatic treatment. A computerized tomogram (CT) scan (chest and abdomen) showed multiple mildly enlarged nonenhancing, nonnecrotic discrete lymph nodes in the sub-mental and bilateral deep cervical regions and upper abdominal lymphadenopathy, suggestive of a granulomatous etiology. A biopsy of the cervical lymph node showed brownish fragment of 2 cm on gross examination and
histiocytic necrotizing lymphadenitis with no evidence of acid-fast Bacillus or malignancy on microscopy. Tuberculosis (TB) mycobacterial growth indicator tube and gene expert Mycobacterium tuberculosis RIF done from the lymph node were negative.


A 32-year-old woman was transferred to our hospital with posterior neck pain and difficulty walking. She was very slender and pale. On neurologic examination, she had grade IV spastic quadriparesis and hyperreflexia of all limbs. There was neither past medical history nor any positive neuromuscular marker. Magnetic resonance imaging (MRI) was obtained immediately and showed a huge mass extending from the foramen magnum to the entire cervical spinal canal (). MRI also revealed a small meningioma in the left cerebellopontine angle. The giant tumor was highly enhanced on T1-weighted MRI with contrast and extended from the lower clivus to the C7 level. T2-weighted MRI again showed a huge tumor with hyperintense signal that was severely compressing the spinal cord and displacing it posteriorly. T1 axial MRI images showed widening of the dural sleeve of the C2 ganglion and tumor extending into the intradural space (). This suggested a possible tumor origin.
Initially, we planned to operate in conjunction with a spinal surgeon. After general anesthesia, intraoperative monitoring was applied. Closed observation with Somatosensory evoked potential (SSEP) was done while setting the position. The patient was placed in the lateral decubitus position for a left farlateral approach. However the patient's neck was kept straight to protect from compression of the spinal cord and brainstem by the tumor. Skin preparation was performed from behind the ear to the cervico-thoracic junction. At first, a hockey stick skin incision extended from the retroauricular area to the level of the C2 vertebral body. The posterior neck muscles were dissected layer by layer to identify and protect the vertebral artery. The tumor extended posteriorly into the myofascial planes. We determined that the tumor originated in the C2 dorsal root ganglion (). The vertebral artery was displaced laterally. The vertebral artery was mobilized laterally after lateral suboccipital craniectomy and C1 laminectomy had been performed. We removed the tumor in the anterior foramen magnum and posterior fossa after cutting the C2 root of the tumor origin through the left far-lateral corridor. The tumor was soft and the margin was clear and non-adhesive. After removal of the tumor in the posterior fossa with an ultrasonic aspirator, we carefully and gently pulled out the tumor from the spinal canal with tumor forceps. The tumor slipped out of the spinal canal without any resistance. Finally we could remove the rest of the tumor without extension of the laminectomy (). The patient fully recovered during the postoperative period without surgery-related complications.


We report the case of a 74-year-old female patient who regularly consumed multiple
drugs, with no previous adverse reactions. She was observed in the emergency room
for a violaceous plaque of 5cm in diameter with central blistering on the medial
side of the fifth right-hand finger, as well as multiple, smaller, round
erythematous lesions on the upper back and lateral side of the arms.
These skin lesions appeared less than 12 hours after taking etoricoxib
(Exxiv®) for joint pain. The patient described a similar
episode two months beforehand, involving only the right-hand finger. She was treated
with betamethasone cream and fusidic acid, leading to clinical improvement.
Six weeks later, with the cutaneous reaction in remission and some residual macular
pigmented lesions, patch testing was performed, applying the allergens from the
European baseline series and a series of 12 NSAIDs on non-lesional skin, involving
occlusion for 48 hours in Finn Chambers. Additionally, in ill-defined residual
hyperpigmented areas (two on the upper back, and one on the right-hand finger), we
applied etoricoxib 10% in vaseline using Finn chambers with occlusion periods of 24
hours. As we did not have pure etoricoxib, an Exxiv® 90mg pill was
grinded into fine powder and manipulated in vaseline to obtain a 10% concentration
of the active drug. Readings performed after 24 hours revealed a positive reaction
to etoricoxib only in previously involved areas ( and ). No positive
reaction to celecoxib (Celebrex®) 10% was observed in lesional or
normal skin.
A cutaneous biopsy in a positive test area located on the upper back at 24 hours
revealed spongiosis, lymphocyte exocytosis and marked vacuolization of the basal
cell layer, with some apoptotic keratinocytes, associated with a lymphocytic dermal
infiltrate, with some intravascular neutrophils, consistent with the diagnosis of
FDE ().