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A 60-year-old Japanese woman was admitted to our hospital because of visual field loss and unsteady gait. The patient spent time gardening as a hobby and was on infliximab and methotrexate therapy for rheumatoid arthritis. Two months before admission, her left visual field loss had steadily progressed, and she felt low-grade fever and general malaise. A high signal in the right occipital lobe and the left parietal lobe was detected in the MRI fluid-attenuated inversion recovery (FLAIR), and the lesions were enhanced by gadolinium (Fig. A–D). Brain biopsy of the right occipital lobe lesion demonstrated granulomatous inflammation, but no pathogenic micro-organisms were detected using either microscopic examination or culture. A diagnosis of neurosarcoidosis was made by the neurologists based on the brain biopsy findings.\nThe symptom of unsteady gait was temporarily relieved by intravenous methylprednisolone (IVMP); although, it worsened soon after discharge, followed by readmission to our department. At the time of readmission, there were no notable findings in the chest and abdomen and no uveitis was noted. A brown spot with a scab, about 10 millimeters in diameter, was observed inside the left upper arm. Neurologically, left lower visual field and mild weakness in the distal muscles of the upper limbs were observed. Deep tendon reflexes and muscle tonus in the upper and lower limbs were normal, and there was no ataxia or sensory impairment. Analysis of blood samples showed no abnormal findings in the blood cell count or the coagulation system. Serum angiotensin-converting enzyme II activity was 12.9 U/L and lysozyme level was 6.2 μg/mL. Antinuclear and antineutrophil cytoplasmic antibodies were negative. Specific antibodies for aquaporin 4 and myelin oligodendroglia were also negative. Tumor markers were not elevated. Antiparasitic antibodies and blood cultures were also negative. The CSF collected 1 week after brain biopsy was colorless and transparent, and the initial cerebrospinal pressure was 12 cmH2O. The CSF cell count was measured to be elevated at 43/μL (neutrophil 0/μL, lymphocyte 38 /μL, and monocyte 5/μL), in addition to the increased protein levels at 77.8 mg/dL. Cerebrospinal fluid sugar level was 86 mg/dL, while the blood glucose level was detected at 192 mg/dL. The CSF/serum ratio of albumin (Q-albumin) was elevated at 14.2 (CSF albumin: 57 mg/dL, serum albumin: 4010 mg/dL). Gram staining did not detect any bacteria, and common bacteria and acid-fast bacteria were not cultured from the CSF. Cryptococcus neoformans antigen, Aspergillus antigen, Toxoplasma antibody, and Human Polyomavirus 2 DNA were all negative. Transthoracic echocardiography showed no obvious thrombi or warts. Skin biopsy performed on the brown spot on the left upper arm showed dense lymphoplasmacytic inflammation distributing from the upper dermis to the subcutaneous adipose tissue with a few epithelioid cell granulomas. Contrast-enhanced computed tomography (CT) of the region from the cervix through the pelvis revealed no neoplastic lesions or bilateral hilar lymphadenopathy, and gallium scintigraphy did not reveal any abnormal accumulation.\nBrain MRI after IVMP showed enlarged lesions with internal necrosis (Fig. E–H). At 16 days after admission, ceftriaxone (2 g/day), acyclovir (1.2 g/day), fosfluconazole (0.4 g/day) sulfamethoxazole (1.2 g/day), and trimethoprim (0.24 g/day) were initiated along with the second IVMP to cover both autoimmune encephalitis and infectious meningitis caused by bacteria, herpes virus, Cryptococcus neoformans, and parasites. However, the treatment response was poor, and new lesions appeared in the medulla oblongata (data not shown). Because the patient suddenly fell into a coma on day 21 after admission, intratracheal intubation was performed and ventilator management was started. Brain MRI revealed progressed necrosis and edema (Fig. I–L). A second brain biopsy with needle aspiration from reddish-brown tissue revealed necrotic tissue which was considered to be reflecting brain abscess, but no pathogenic micro-organisms were detected using either microscopic examination or culture. Furthermore, neither tumor nor granulomas were observed in the specimen.\nIn order to identify the causative pathogenic micro-organism, the initial brain biopsy specimens were subjected to shotgun metagenomic sequencing using a next-generation sequencer (NGS). The DNA extracted from the brain specimens was comprehensively analyzed using Illumina sequencer (MiSeq®) and compared with the sequences registered in the database through the BLASTN analysis. As a result, 129 DNA fragments homologous to B. mandrillaris were identified from the 9 million DNA fragments derived from the brain specimens (Fig. A). The PCR analysis for the mitochondrial rRNA of B. mandrillaris [] revealed bands of the PCR product in the patient’s brain and skin biopsy specimens (Fig. B, Additional file : Fig. S1). The pathogen detected from the present case was presumed to be a strain with a high degree of similarity to B. mandrillaris V039 based on phylogenetic analysis using MEGA X [] (Fig. C). Based on these findings, histopathological specimens of the brain tissue (Fig. AD) and skin biopsy (Fig. E) were re-evaluated microscopically. Brain biopsy of the right occipital lobe lesion demonstrated granulomatous inflammation, containing epithelioid granuloma with palisading necrosis (Fig. A) and multiple epithelioid granulomas without necrosis (Fig. B). Moreover, multinucleated giant cells containing amoebic trophozoites were seen on hematoxylin and eosin (H&E) stain (Fig. C) and these trophozoites were confirmed immunohistochemically with anti-B. mandrillaris rabbit antibody (Fig. D). Skin biopsy also showed a few amoebic trophozoites (Fig. E) which were confirmed by immunohistochemical staining. Thus, the patient was diagnosed with GAE caused by B. mandrillaris. The treatments with azithromycin (0.5 g/day), sulfamethoxazole (1.2 g/day), trimethoprim (0.24 g/day), and fosfluconazole (0.2 g/day) was initiated; however, the patient died 44 days after admission. |
An 81-year-old Caucasian woman presented with a five-day history of severe generalised abdominal pain, which was colicky in nature with non-bile-stained vomiting, distention, and two days of not having opened her bowels. The abdomen was soft, with general tenderness and guarding in the right upper quadrant. There was no relevant family and social history.\nShe was diagnosed three years previously with squamous cell carcinoma of the cervix with bladder involvement (Stage IVa on the International Federation of Gynaecology and Obstetrics staging - FIGO), in which she underwent external radiotherapy and intrauterine balloon therapy. She also had a common bile duct stent inserted for obstruction secondary to a pancreatic lesion in the uncinate process of unknown aetiology.\nPast medical history further included a left nephrectomy for renal cell carcinoma five years previously, a ureteric stent after an episode of hydronephrosis secondary to a renal stone, chronic kidney disease and hypertension.\nThe patient was alert although in some discomfort. Respiratory examination elicited good air entry bilaterally with no added sounds. Cardiovascular examination sounds S1 and S2 present with no additional sounds. The abdomen was soft but distended with generalised tenderness and guarding in the right upper quadrant. No masses or herniae were elicited and there was no sign of peritonism or rebound tenderness. There was no evidence of pitting oedema, and calves were soft. Digital rectal examination showed dark stool but no blood visible, with no palpable masses. Laboratory-based investigations showed a raised alkaline phosphatase (1035IU/L) but were otherwise within normal limits.\nThe abdominal radiograph (Figure ) showed that the stomach was markedly distended and the fundus was fluid-filled. The small bowel pattern was normal. Acute colonic abnormality was absent. There was a metal biliary stent in situ with the presence of gas in the biliary tree. Appearances raised the possibility of gastric outlet obstruction.\nComputed tomography (CT) of the abdomen and pelvis (Figure ) showed the following: “There is increased soft tissue seen around the bile duct stent. Non-specific mild distension of small bowel loops proximally with no lead point seen.”\nThe patient was diagnosed with gastric outflow obstruction of an unknown cause. It was suggested that this could be due to a known pancreatic lesion of unknown aetiology or new malignancy. The CT scan and normal bilirubin level also confirmed the biliary system's patency; the raised alkaline phosphatase (ALP) may indicate liver or bone metastases.\nA Ryles tube was inserted for decompression, and the patient was discussed at the radiology and upper gastrointestinal multi-disciplinary team meeting. The patient underwent oesophago-gastroduodenoscopy (OGD), which showed a stricture at D1-D2. Biopsies of the lesion were taken and a 12-cm stent was inserted with the most proximal aspect at the duodenal bulb.\nBiopsies showed lymphovascular permeation of the mucosa and submucosa by nests of tumour cells resembling squamous cells. The cells were P16 and P63 positive, in keeping with squamous cell carcinoma. A fluorescence in situ hybridization (FISH) analysis of the sample detected human papillomavirus 16.\nPost-enteric stent insertion, the oral intake was slowly re-introduced after a confirmatory radiograph of the stent position. The patient had episodes of vomiting and a barium meal was performed, which showed the stent in situ with contrast passing freely. It was noted that the stent lumen was severely narrowed at the level of the second and third part of the duodenum; however, the bolus could pass well.\nPost-procedure, there was a noted increase in the patients' ALP (3245IU/L). Ultrasound of the abdomen showed a dilated intrahepatic and common bile duct. The patient was offered a percutaneous transhepatic cholangiogram to relieve the blockage for symptom relief, however, the patient refused further interventions. It is believed that the enteric stent may have interfered with the patency of the previously placed biliary stent. The patient was discharged home to continue with palliative care in the community. The patient subsequently passed away at home due to her illness. |
A 21-year-old female patient presented to endodontic clinic at the University Dental Hospital at King Saud Dental University with the chief complaint of intermittent spontaneous pain related to upper left side.\nPatient has recently noticed a small localized swelling between teeth in the maxillary left posterior region. The pain started at the same area a month ago. Patient reported no sensitivity to cold or heat stimulation.\nThe patient reported no history of trauma. However, she had received orthodontic treatment 2 years ago. The patient had multiple restorations mostly carried out by a pedodontist one year prior to her admittance at this hospital.\nPatient medical history was noted to be noncontributory.\nClinical examination revealed the presence of localized erythematous swelling at the buccal interdental papilla between the left maxillary first molar and second premolars (Figure and B).\nIt was noted that the left maxillary second premolar and first molar had MOD tooth-colored restorations. Meanwhile, the left maxillary second molar had MO amalgam restorations. The teeth were tested electrically and thermally. It was found that left maxillary first molar was not responsive to the thermal or electric pulp tests; whereas, the other tested teeth responded normally. Moreover, it was noted that this tooth was sensitive to percussion and tender to palpation. On probing, a respective pocket depth of 12 mm and 10 mm was detected in the mesiobuccal and mesiolingual regions of left maxillary first molar (Figure and D).\nA two-dimensional radiographic examination was conducted that revealed the presence of a breakdown of the crest of alveolar bone mesial to left maxillary first molar and an irregular molted radiolucency mesial to the pulp space of left maxillary first molar but separated by radiopaque line, and extending into the radicular dentin (Figure and B). Cone-beam computed tomography examination confirmed the size and the external perforation of the existing resorption defect (Figure ). |
A 28-year-old Asian woman (G3P1) who had undergone emergency cesarean delivery owing to a compound presentation at full term was referred to our institution with a suspicion of abnormally located gestational sac. She had undergone laparoscopic cholecystectomy and open appendectomy previously. She did not have any medical, family, or psychosocial history. She had missed her menstrual period without any other symptom and visited a private obstetrical clinic to confirm the pregnancy. However, she was diagnosed as having an abnormal pregnancy such as cervical or CSP by USG.\nAt our institution, she reported that her last menstrual period was just 5 to 6 weeks prior. However, USG revealed a gestational sac in the anterior lower uterine segment with a fetus measuring 4.83 cm crown-rump length (CRL) with positive cardiac activity, corresponding to 11 weeks and 6 days of gestation. Color/power Doppler images depicted a hyperechoic rim of a choriodecidual reaction with excessive vascularity (Fig. ). Although we could observe a definitive abnormally located gestational sac, our patient did not have any pain during the physical examination. She admitted that her last menstrual period was different from her usual menstrual periods. Because CSP or cervical pregnancy was suspected, we performed computed tomography (CT) for a definitive diagnosis. The CT scan showed an intrauterine gestational sac in the lower uterine segment bulging through the anterior uterine wall at the site of the cesarean scar. No invasion of the urinary bladder was observed (Fig. ). On presentation, her β-human chorionic gonadotropin (β-hCG) level was 66,536.8 IU/L (Day 1). Initially, we injected 50 mg of methotrexate (MTX) mixed with 9 mL of normal saline in the amniotic sac through a 22-G needle transabdominally under USG guidance. Simultaneously, 2 ml of amniotic fluid was aspirated for termination of the pregnancy. However, fetal cardiac activity was still observed 2 days later (Day 3), without significant changes in the serum β-hCG levels (65,342.5 IU/L). We decided on laparotomy instead of laparoscopy because of the large CRL (Day 4). The intraoperative finding showed bloody amniotic fluid, blood clot, placenta, and a fetus at the lower segment of the uterus. A transverse uterine incision was made at the lower segment of the uterus (Fig. ). The gestational sac was removed, as well as most of the trophoblastic tissues that were adherent and invading the wall of the lower uterine segment. The fetus and placenta showed no definitive abnormalities (Fig. ). The estimated blood loss was 1.2 L at intra-operation, without immediate complication. The uterine defect was repaired into two layers by using 2–0 Vicryl sutures. Our patient received 3 units of packed red blood cells (PRBC) at the ward postoperatively. The serial β-hCG level was 1958 IU/L at 4 days after the surgery (Day 8). She was discharged in good condition 5 days after the operation (Day 9). After 1 month (Day 39), her β-hCG levels returned to normal (2.8 IU/L). She was very satisfied with the fact that she had recovered well without the need for intensive care or further treatment without the need for hysterectomy. |
The patient was a 75-year-old male with a medical history of diabetes mellitus, hypertension, and benign prostatic hyperplasia. He presented to the Emergency Department (ED) complaining of recurrent, non-radiating paraumbilical colicky pain for six months. The pain was exacerbated by lying and relieved by sitting and bowel movements. It was associated with vomiting, abdominal distension, and constipation with alternating diarrhea. The patient had a history of hemorrhoidectomy 15 years ago. The abdominal examination was unremarkable, except for the presence of significant abdominal distention and mild umbilical tenderness.\nAn abdominal CT scan with IV contrast was done, which showed a dilatation in the small bowel reaching up to 4.8 centimeters with multiple air-fluid levels and transition zone at the distal ileal loops. There was circumferential mural thickening of the ileal loops in the right lower quadrant with surrounding fat strandings and prominent mesenteric lymph nodes. The rest of the ileal loops and the large bowel was collapsed. The constellation of findings was assumed to represent a neoplastic or inflammatory process. In addition, a plain abdominal radiograph was done, showing dilated small bowel loops, suggestive of small bowel obstruction (Figure ). The CT scan images showed high-grade small bowel obstruction and transition zone with ileal wall thickening, fat stranding, and prominent lymph nodes (Figures , ).\nThe patient underwent laparoscopic exploration, which revealed two mid ileal masses. The proximal part related to the masses was dilated, where the distal part was collapsed. The mid ileal masses were attached to the small bowel mesentery, and hence the procedure was converted to the midline laparotomy. The affected bowel was found 2.5 meters distal to the duodenojejunal (DJ) junction. Oncological resection of the masses was excluded to avoid superior mesenteric artery injury. Therefore, a small bowel resection accompanied by multiple intermediate size lymph node resection was made. At that time, side-to-side stapled anastomosis was also done.\nRegarding histopathology, the measurement of the obstruction site was 11 x 5 cm. There were nine well-circumscribed, firm submucosal nodules, and the largest nodule measured 2.2 cm in its maximum dimension. The mesenteric lymph node specimens consisted of multiple fragments of fibrofatty tissue measuring 8 x 4 x 3 cm in aggregate (Figure ). The histopathological findings showed grade 1 well-differentiated NETs of the mid-ileum with one metastatic lymph node (mpT2, N1). Furthermore, the muscularis propria was invaded by the tumors. Among the immune histochemistry markers, the tumors were positive for chromogranin A, synaptophysin, CD56, CDX2, and CKpan, while negative for CK7, TTF1, PAX8, CK20.\nThe patient was discharged six days after the surgery in a stable and good condition. No adjuvant therapy was recommended since the tumors were well differentiated. He was discharged on enoxaparin, ciprofloxacin, and paracetamol. A follow-up after three months for physical examination, blood tests, and CT scan was recommended. After that, follow-ups should be done every 6-12 months for five years, and then every 12-24 months for 10 years. |
The male patient was 66 years old. He suffered from hypertension for 40 years and the blood pressure was up to 155/105 mmHg when untreated. He had been taking 75 mg captopril and 3.6 g salvia tablet daily to maintain the blood pressure of about 130/75 mmHg in daily life. The body mass index (BMI) was 21.5 kg/m2 before the operation. He was diagnosed with rectal carcinoma and colon multiple polyps. The main diagnostic methods are computed tomography and electronic colonoscopy examination. Computed tomography demonstrated a tumor in the colon. Electronic colonoscopy examination revealed a 0.5 × 0.5 cm polyp on transverse colon and sigmoid separately. A nodular bulge could be seen, 4 cm from the anus at 6 o'clock position when the patient was on left lateral position, and the surface eroded. Hemorrhoids and anal fissure were excluded through the differential diagnoses. The patient received Miles surgery for removal of rectum and distal sigmoid colon. The histopathological results displayed adenocarcinoma. The blood pressure was 125/75 mmHg the next day after the operation, with no anti-hypertensive therapy, and the BMI was 20.5 kg/m2. The patient recovered smoothly and was discharged 10 days postoperatively and began to receive the postoperative chemotherapy program with oxaliplatin, 5-fluorouracil and calcium folinate 3 weeks after the operation. In one course, oxaliplatin (85 mg/m2) and calcium folinate (400 mg/m2) was taken on the first day, while 5-fluorouracil was taken for 3 days (400 mg/m2 on the first day and 1,200 mg/m2 for the next 2 days), repeating every 2 weeks. The patient took 6 courses of postoperative chemotherapy totally for 3 months. The patient tolerated the postoperative chemotherapy during the 3 months courses with mild adverse reactions such as nausea and vomiting but without severe adverse reactions such as serious renal or hepatic impairments. A several-month telephone follow-up was conducted after he was discharged and no recurrence and metastasis of the rectal carcinoma was found based on computed tomography, electronic colonoscopy examination, and tumor markers tests including normal levels of carbohydrate antigen 199, carbohydrate antigen 125, carbohydrate antigen 153, carcinoembryonic antigen, and α-fetoprotein through the whole course.\nMore attention was paid to the recurrence and metastasis of the rectal carcinoma at the beginning. Subsequently, we found the blood pressure of the patient had been maintained in the normal range without anti-hypertensive drug treatment. Blood pressure was measured at 3–5 pm and the BMI was calculated according to the body weights, recorded respectively at 2 weeks, 3, 6, and 8 months after the operation. Blood pressure of the patient decreased to normal level since 2 weeks postoperatively and has been normal until now without any anti-hypertensive treatment. The relevant data collected during the hospitalization and follow-up have been showcased as a timeline in , as well as the variation trends of the blood pressure and BMI of the patient during the follow-up. Family history revealed no history of cardiac or other congenital abnormalities. Past history recorded no alcoholic intake or smoking. |
An 82-year-old male patient presented with worsening symptoms of chest discomfort and exertional dyspnea {New York Heart Association (NYHA) III} for 2 months. He had past medical history of hypertension and dyslipidemia. Four years ago, the patient had been treated with percutaneous coronary intervention with implantation of stents at the left main artery and the distal left circumflex artery. At that time he also underwent percutaneous transluminal angioplasty with implantation of self-expandable nitinol stents at the bilateral iliac arteries. On admission, transthoracic and transesophageal echocardiography showed severe AS and mild aortic regurgitation (AR) due to degenerative change with calcification (). The aortic valve area was 0.44 cm2. The peak and mean pressure gradients across the aortic valve were estimated to be 97 and 59 mm Hg, respectively, while the diameter of the aortic annulus by echocardiography was 25 mm. The left ventricle (LV) showed a dilated enddiastolic dimension (64 mm) with LV hypertrophy. The LV systolic function was globally reduced with an LV ejection fraction (EF) of 39%. Based on the coronary angiography, the stent at the left main artery was patent. However, the stent implanted at the distal left circumflex artery showed in-stent restenosis, which was subsequently treated with a 3×30 mm paclitaxel-eluting balloon (SeQuent® Please, B. Braun Melsungen AG, Berlin, Germany). The previously inserted stents at the right and left iliac arteries were patent, however computed tomography (CT) angiography revealed that the minimal lumen diameter of the stented right iliac artery was 5.5 mm and that of the left iliac artery was 4.5 mm. Both common femoral arteries also showed significant stenosis with a minimum lumen diameter of 4.0 mm (). The diameter of the left subclavian artery was measured to be 7 mm on CT angiography. The calculated EuroSCORE was 44.08%.\nIn this patient we decided to perform TAVI by transsubclavian approach. The procedure was carried out in a hybrid operating room under general anesthesia. After surgical exposure of the left subclavian artery, a 0.035 inch Amplatz Super Stiff wire (Boston Scientific, Natick, MA, USA) was inserted into the LV through an 18 Fr introducer sheath (). Balloon dilation of the stenotic aortic valve was performed with a 23 mm balloon (Z-med, NuMED Inc., Hopkinton, NY, USA) under rapid pacing using a temporary pacemaker. Under angiographic guidance, a 29 mm CoreValve was slowly deployed at the aortic annulus. An immediate post-procedural aortogram showed good position of the CoreValve with mild AR (). The vascular access site at the left subclavian artery was closed surgically without any complications. The post-procedural echocardiography demonstrated well functioning bioprosthetic aortic valve with mild paravalvular AR. The peak and mean pressure gradients across the aortic valve decreased from 97 and 59 mm Hg to 42 and 23 mm Hg, respectively. The aortic valve area increased from 0.44 cm2 to 2.86 cm2. The LVEF improved from 39% to 52%. Four days post procedure, the patient was discharged with improved symptoms (NYHA I). There was no sign of stroke or any conduction abnormality as indicated by the electrocardiogram. The patient has been free from any major cardiovascular events or symptom aggravation for a total follow-up duration of 6 months. |
A 38-year-old male patient was referred to Asan Medical Center due to severe shock attributable to contained aortic rupture around the aortic root. The patient had been treated with systemic corticosteroids after being diagnosed with Behcet’s disease 14 years previously. The patient had previously undergone aortic valve surgery three times in another hospital for the management of aortitis and consequent aortic regurgitation associated with underlying Behcet’s disease. The first two mechanical aortic valve replacement (AVR) procedures had been performed 14 years previously, followed by a Ross procedure two years after the second operation.\nWhen the patient presented to our emergency department, he was in severe shock (systolic blood pressure, 65 mmHg) despite maximal intravenous inotropic support (epinephrine and norepinephrine), and exhibited a drowsy mental state. On enhanced computed tomography, a contained aortic rupture was found around the aortic root and the ascending aorta, abutting the sternum. The contained hematoma was measured up to 72 mm in diameter (). An emergency operation was planned. A transesophageal echocardiography performed in the operating room showed mild aortic regurgitation (AR) and severely depressed cardiac function with a left ventricular (LV) ejection fraction of 17% despite full inotropic support.\nMassive hemorrhage during the reopening of the sternum and consequent catastrophic events were anticipated; therefore, deep hypothermic circulatory arrest (DHCA) was implemented before opening the sternum, using femoral cardiopulmonary bypass (CPB) with direct trans-apical LV venting through a left-side mini-thoracotomy (). Once the nasopharyngeal temperature cooled to 18°C, sternal opening was attempted using an oscillating saw. As expected, a massive hemorrhage occurred from the ruptured aorta while the hematoma was being removed after sternal opening, at which point the CPB was stopped. Distal ascending aorta clamping was achieved securely within one minute of DHCA, and CPB was restarted. Two discrete rupture sites were found in the aorta: one at the distal anastomosis site of the previous Ross procedure and the other at the right coronary button site (). After complete adhesiolysis of the mediastinum and the removal of the previously implanted pulmonic autograft, several tiny pseudoaneurysms were identified at the level of the aorto-ventricular junction, and further resection of this area was therefore conducted. DHCA was then reimplemented (15 minutes) in order to conduct hemiarch replacement using a 28-mm Hemashield vascular graft (Boston Scientific, Boston, MA, USA), which was followed by the reimplementation of CPB and systemic rewarming. During this period, complete root replacement was carried out, using a 27-mm composite mechanical valved conduit (St. Jude Medical Inc., St Paul, MN, USA). The circulatory arrest and cardiac ischemic times were 16 minutes and 188 minutes, respectively. The patient failed to be weaned from CPB due to profound LV dysfunction, therefore, CPB was substituted with venous-arterial extracorporeal membrane oxygenation (ECMO). Massive hemorrhage from the surgical sites continued despite meticulous hemostatic maneuvers and massive blood product transfusions. The patient was transferred to the intensive care unit (ICU) with an open sternum. The transfusion of large quantities of blood products for two days resulted in adequate hemostasis. Follow-up echocardiography showed a significant recovery of LV function at this point, and the patient was successfully weaned off ECMO after four days of ECMO support, after which a delayed sternal closure was performed. The follow-up computed tomography images and echocardiogram were unremarkable in that neither paravalvular dehiscence nor leakage on the anastomosis site was observed. LV function was found to be normal, with an ejection fraction of 56% without any inotropic support. The patient was discharged on postoperative day 31 without any neurologic sequelae or residual complications. On the echocardiographic follow-up performed six months after the surgery, cardiac function was completely normal, with adequate function of the mechanical valve and no pathologic findings around the aortic root and ascending aorta. The patient has undergone 14 months of follow-up while prescribed an oral corticosteroid medication, and has exhibited no cardiovascular complications. |
In May 2015, a 31-year-old male was presented to the emergency room with pain of the foot after eversion trauma while jumping on a trampoline. Initial trauma screening revealed a hemodynamically stable patient with a complicated (Gustilo type 3) subtalar joint dislocation of the right leg (Figure ). During trauma screening no other injuries were detected. The patient was transported to the OR within 4 h of presentation. The dislocated talus was immediately reduced using a surgical spoon and a femoral head impactor. We performed meticulous wound debridement, after which the wound was closed transcutaneously over a subcutaneous low vacuum drain. A joint bridging external fixator was constructed with additional kirschner-wires for reduction and temporary stabilisation (Figure ). A postoperative computed tomography (CT) scan showed several small fracture fragments of the talar bone with congruent ankle and subtalar joints. There were no fractures of the adjacent tarsal and metatarsal bones.\nThe medial wound healed uneventfully. Eight weeks postoperatively the external fixator and kirschner wires were removed during a visit at the outpatient clinic. At this time the patient engaged in physical therapy and commenced bearing full weight on the affected leg. One year after injury the patient is walking without pain, only using a cane for long distances. Range of motion of the ankle is not restricted (Figure ). Conventional radiographs show early signs of talar avascular necrosis (Figure ), without associated clinical symptoms.\nMethods: The search was limited to humans aged over eighteen years with a subtalar dislocation. PubMed/MEDLINE, EMBASE, and the Cochrane Library were searched from up to April 1st 2016. Exclusion criteria were minor age and subtalar dislocation in medial, anterior or posterior direction. Case reports were excluded to limit report bias. The lists of references of retrieved publications were manually checked for additional studies potentially meeting the inclusion criteria and not found by the electronic search. Studies reporting various types of subtalar dislocation were identified and only included if the results of patients with lateral subtalar dislocation could be extracted separately.\nWe collected all information regarding the level of evidence, baseline patient characteristics, baseline clinical findings and mean period of follow-up. Data regarding type and timing of surgery, postoperative regimen, complications, functional outcome, radiological outcome and patient satisfaction were extracted.\nResults: The literature search displayed 367 studies, of which eight studies including fifty patients could be included[,-]. General characteristics can be found in Table . All reported numbers are sample-size weighted. Eighty-seven percent of patients were male. Mean age was thirty-four years. Subtalar dislocations were evenly distributed over de left and right extremity. Thirty out of 50 patients suffered a complicated injury. Unfortunately Gustilo grading of complicated injury was not reported in all studies and therefore not available for analysis in this study. Associated fractures of the tarsal or metatarsal bones were present in seventeen patients, posterior tibial tendon rupture was present in six patients.\nClosed lateral subtalar dislocations were treated with reduction and below the knee casting. Closed reduction was successful in all of these cases. All complicated lateral subtalar dislocations were treated with wound debridement. Additional fixation with k-wires was performed in three patients. An external fixator was placed in eleven patients, the other nineteen patients were treated with a below the knee cast. The patients were non-weight bearing for a mean period of seven weeks (range 4-12), after which the cast was removed and patients were allowed to bear weight. Mean follow-up was fifty-five months. Four studies[,,,] provide patient reported outcome with the use of the American Orthopaedic Foot and Ankle Society score (AOFAS)[], which has not been validated for this purpose. Mean AOFAS score at follow-up was 82. Ankle function was reported as good in all patients with closed lateral subtalar dislocation. Severe pain was reported in nine patients with avascular necrosis and in two additional patients with severe osteoarthritis.\nRadiologic assessment revealed subtalar osteoarthritis (Altman et al[] type 2 or 3) in eight out of fifty patients. Avascular necrosis was present in nine additional patients. Patients with avascular necrosis were eventually treated with arthrodesis of the ankle in most cases. Four patients with complicated lateral subtalar dislocation suffered deep infection requiring treatment with antibiotics. |
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. |
A 21-year-old primigravida at 37-week gestation presented to the outpatient clinic for a routine obstetrical examination. Her pregnancy course had been uneventful. Her blood pressure at this visit was 184/108. She was asymptomatic except for occasional mild headaches. A urine dipstick showed 3+ proteinuria. Patient was diagnosed with preeclampsia and was admitted to the hospital for emergency induction of labor. She was started on IV magnesium for preeclampsia and labor was induced with oxytocin. She underwent an emergency cesarean section due to fetal decelerations during induction. Subsequently, she delivered a healthy male infant by low transverse C section. Four days after partum, she started spiking high fevers up to 39.5 degrees Celsius and developed tachycardia with heart rate of 140 to 150/min. She was otherwise completely asymptomatic and clinical exam was negative for any localizing signs. Her cesarean section incision looked clean; she had no breast tenderness. Infectious work-up including blood and urine cultures was negative. Her chest X-ray was normal; EKG showed sinus tachycardia. She was empirically started on broad spectrum antibiotics. After 48 hours of IV antibiotics, patient continued to spike high fevers and was still tachycardic. The differential diagnosis of endometritis and septic thrombophlebitis was high on the list even though her abdominal exam was benign. Computerized tomography (CT) scan of the abdomen and pelvis with contrast () was ordered to rule out septic thrombophlebitis, which showed a large right retroperitoneal 9.2 cm anteroposterior × 14 cm transverse × 12.5 cm craniocaudal mass with severe right renal hydronephrosis. Due to absence of any fat layer between the kidney and the mass, it was felt to be coming from the lower pole of the right kidney. The mass extended superiorly to the inferior vena cava (IVC) with a possible tumor thrombus in the IVC. The liver was mildly enlarged measuring 19.6 cm with fatty infiltration. The first segment of the sacrum showed a 3 cm × 2.1 cm × 2.6 cm lytic lesion. CT scan of the chest showed 2 pulmonary nodules measuring 7 mm and 11 mm in the left and right lower lobes of the lung, respectively. Due to the above imaging findings, suspicion was high for a stage 4 metastatic cancer arising from the kidney. Renal ultrasound confirmed a large retroperitoneal mass with hydronephrosis. MRI lumbar spine reported an abnormal marrow signal in the S1–S3 segments and an exophytic bony metastatic lesion in the dorsal aspect of S1 causing severe narrowing of the thecal sac. MRI abdomen/pelvis confirmed extension of the retroperitoneal mass into the inferior vena cava (IVC). MRI brain and CT brain were obtained showing calvarial lesions consistent with hemangiomas. A percutaneous CT guided right renal mass biopsy () revealed a cellular epithelioid proliferation arranged in nests and composed of round to ovoid cells with hyperchromatic nuclei, inconspicuous nucleoli, and granular amphophilic cytoplasm. Immunohistochemical staining was negative for HMB-45, S100, PAX-8, cytokeratin AE1/AE3, CD117, and DOG-1 but was positive for synaptophysin and chromogranin (). The overall morphological and immunohistochemical findings were consistent with a diagnosis of paraganglioma. 24-hour urine testing revealed high levels of urinary nor epinephrine which was 862 mcg/24 hours (normal range 15 to 100 mcg/24 hours) and dopamine which was 902 mcg/24 hours (normal range 52 to 480 mcg/24 hours). Plasma-free metanephrine levels were also elevated at 1690 pg/mL (normal range 18 to 101 pg/mL). The patient continued to remain severely hypertensive after delivery and was started on combined alpha and beta blockade with doxazosin 8 mg daily and propranolol 40 mg twice daily. The patient underwent right sided nephrectomy, removal of the retroperitoneal mass, and resection of the perirenal IVC. Her postoperative course was complicated by severe hypotension and extensive bilateral lower extremity deep venous thromboses in the external iliac and common femoral veins. She required vasopressors for two days and was started on therapeutic anticoagulation with enoxaparin for the DVTs. She was discharged home 2 weeks after her surgery. A metaiodobenzylguanidine (MIBG) whole body scan done later as an outpatient did not demonstrate any focal abnormal uptake. Whole body PET scan showed increased FDG uptake in 3 pulmonary nodules, a large lytic sacral lesion, and a T9 vertebral body lesion all consistent with metastases. The patient underwent gamma knife palliative radiation of the S1 lesion and was started on Zometa for her bony metastases. The tyrosine kinase receptor inhibitor Sunitinib was started to treat her systemic disease.\nThe patient underwent genetic counseling and pursued genetic testing for multiple hereditary paraganglioma syndromes. She had no known family history of paraganglioma, pheochromocytoma, kidney cancer, or thyroid cancer. Next generation sequencing of MAX, NF1, RET, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127, and VHL identified a likely pathogenic variant in SDHB, c.418G>T (p.Val140Phe). This result confirmed a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome, an autosomal dominant condition with reduced penetrance. |
A 23-year-old woman, who had been a known case of XP since her childhood, was admitted to our hospital for excision of face mass and skin graft surgery. The pathology test of the lesion showed squamous cell carcinoma. Her airway examination revealed some anatomical and pathological abnormalities. The stiffness of her neck and jaw caused decreased motion and limited head extension and jaw protrusion. Her mouth opening was limited to < 20 mm; through this opening two relatively large upper incisor teeth were observed. Pharyngeal examination revealed score 4 mallampati. Also, we found Pectuscarinatum in her chest, a multiple erotic keratotic lesion and a deformity in her nose, and keratoconjunctivitis and corneal opacity in her eyes. All these findings predicted difficult intubation ().\nShe had a short stature and weight of 28 Kg (BMI = 19), cachectic appearance with some deformities in the extremities. A neurological examination showed a minor decline in intelligence quotient (IQ). She was calm and cooperative but not completely oriented to time and space, with some decreased muscle strength and deep tendon reflexes. Her cardiovascular examination and paraclinical evaluation results were within the normal limit. General anesthesia was planned for the surgery. The patient was admitted to the operating room without any premedication to avoid unwanted sedation. All of the patient’s body was covered to protect it from UV and artificial lights in the operating room. We established standard monitoring including: pulse oximetry (SpO2), capnography (end tidal CO2), precordial cardioscopy and non-invasive blood pressure (NIBP). A peripheral venous on the arm was cannulated with a 22 G catheter. Once the preparations of anesthesia and surgery teams and the availability of difficult intubation equipment were ensured, the patient was cautiously pre-medicated intravenously with 25 µg of titrated fentanyl and 0.5 mg midazolam, to maintain spontaneous breathing. Then, general anesthesia was induced with inhalation of sevoflurane (manufactured by Aesica Queen borough, UK) 2.5% to 5% without using a neuromuscular agent while maintaining spontaneous breathing. After elimination of reflexes and reaching enough depth of anesthesia by bispectral index (BIS) count of about 55, an appropriate laryngeal mask (number 2.5) was inserted and floated; fixed when good lung ventilation was ensured. The surgeon infiltrated 5 ml of 2% lidocaine around the surgery site before starting the surgery. Anesthesia was maintained by 2% sevoflurane with a 50:50 mixture of oxygen and air. The procedure lasted 110 minutes without any complications. The patient’s non-invasive blood pressure (NIBP) reading was between 125 and 98 as maxima, and 81 and 6 mmHg as minima; her heart rate was between 75 and 105 beats/minute, her SpO2 ranged from 97% to 100%, and her ETCO2 was between 33 and 37 mmHg. The surgery was completed uneventfully. After 10 minutes, when the patient was awake and obeyed to open her eyes, she was extubated. Postoperative analgesia was assured by bupivacaine infiltration around the wound at the end of the surgery and prescription of acetaminophen (as 325 mg-suppository) every four hours. The patient was discharged from the hospital one day after the surgery without any new complaints. |
A 12-year-old boy was admitted with history of fever of three days duration, vomiting and pain on the right side of neck, radiating to the right upper limb. He also complained of throat pain and pain on swallowing solids. He had excessive salivation and wanted to spit out frequently but was able to drink, and there was no hydrophobia or aerophobia at presentation. He was bitten by his pet puppy on his right thumb with oozing of blood about 2 months back. The puppy died after a few days, which was not taken seriously. Two weeks back another child in his neighborhood who was also bitten by the same puppy died of classical symptoms of rabies. After this he was administered 4 doses of purified chick embryo vaccine by intramuscular route. Rabies immunoglobulin was not given.\nOn examination, the boy was febrile, with a pulse rate of 92/min and BP of 120/84 mm of Hg. He was conscious and oriented, but was apprehensive and agitated and feared imminent death. There was pain in the neck radiating to the right upper limb, but there was no pain or paresthesia at the site of bite. The right upper limb was weak (Grade 3) and hypotonic with depressed biceps, triceps and supinator jerks. There was no weakness of the other limbs initially. Deep tendon reflexes were normally elicited in the other limbs; he had extensor plantar response bilaterally and meningeal signs were absent. At this point, a provisional diagnosis of rabies vs. ADEM was entertained as there was a definite history of bite followed by a very late and inadequate immunization. A third possibility of other viral encephalitis was also considered while planning treatment. Investigations revealed Hb 11.9, TC 10,700 with P60 and L40, and an ESR of 30. RFT (urea 23, creatinine 1, Na 135, K 3.2) and LFT (S.Br 0.7, SGOT 117, SGPT 29, ALP 222) were normal. He was empirically started on Acyclovir to cover for treatable encephalitis, postponing LP till imaging. Methyl prednisolone was started at a dose of 30 mg/kg/day considering the possibility of ADEM as the illness developed two weeks after anti rabies vaccination. Within 12 hours, the weakness progressed to involve all four limbs, he developed retention of urine and the respiration became shallow. An MRI of the brain and cervical spine performed on a 1.5 Tesla scanner was taken, which showed bilaterally symmetrical hyperintensities in T2 and FLAIR images in basal ganglia [], thalamus, dorsal medulla and central gray matter of the cervical spinal cord [] extending to the dorsal segments. The next day, he developed the classical symptoms of hydrophobia and aerophobia, and developed respiratory failure. The child was ventilated. But he developed hypotension and shock in spite of vasopressors and succumbed on the third day due to cardiac arrest. A postmortem impression smear of the cornea was positive for rabies antigen by fluorescent antibody test. Further evaluation including brain biopsy could not be done as the parents were not willing for the same. |
In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.\nThe right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().\nUpon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.\nAfter the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.\nAfter three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.\nMetallic pieces left the digestive tract spontaneously after a couple of days. |
To our knowledge, this is the first reported case of an index finger MCP joint dislocation surgically treated by a lateral approach.\nThe authors describe a case of a 16-year-old male who suffered a fall onto his outstretched right hand during a soccer game. The patient presented to the ER with pain and deformity of the index finger MCP joint. Volarly, the prominence of the second metacarpal head was evident ().\nRadiographs confirmed a dorsal index finger MCP joint dislocation and showed a small dorsal osteochondral fragment (Figures and ).\nAfter multiple unsuccessful reduction attempts under ring block by different physicians, the patient was referred to surgery.\nUnder general anesthesia, a lateral surgical approach () was performed on the MCP joint. A straight longitudinal incision was made over the lateral aspect of the MCP joint; the volar neurovascular bundle and the dorsal branch of the digital nerve were identified and retracted with Farabeufs.\nInterposition of the volar plate () preventing the reduction was observed. Applying gentle traction and flexion, the MCP joint was reduced, and proximal volar plate reinsertion with a 4-0 Vicryl suture was performed.\nThe posterior joint capsule was identified and split longitudinally, above the collateral ligament. Once adequately exposed, a small osteochondral fragment was found (). Reduction and retrograde fixation of the osteochondral fragment with a 1.7 mm screw were performed, burying the screw head in the cartilage.\nThe joint capsule, subcutaneous layer, and skin were closed using appropriate sutures. Reduction was confirmed by intraoperative fluoroscopy.\nThe patient was placed in a volar splint with approximately 45° of flexion and discharged on postoperative day zero without any complications.\nImmobilization was removed by week 3. Radiographic control revealed joint congruence, and the patient was encouraged to actively mobilize the finger.\nAt week 6, the fracture was consolidated (Figures and ). The joint was painless and presented slight stiffness (ROM 0-70°). The patient could return to competition with protective syndactyly.\nOne year postoperative, there was no pain, growth disturbance, or joint stiffness, with full ROM of the index finger. |
A 57-year-old Latina woman with a past medical history of HIV on anti-retroviral therapy (ART) presented with complaints of a 2 month history of cough productive of yellow sputum with associated streaks of blood, 12 pound weight loss, bilateral hand swelling, finger clubbing and knee pain. Chest CT revealed a prominent, slightly rounded consolidation of the inferior right upper lobe with adjacent ground glass and interstitial opacities likely representing pneumonia (). The patient was admitted and initially treated for community acquired pneumonia. However, an interval chest radiograph done one month later revealed a persistent and enlarging right upper lobe infiltrate ( &). The patient subsequently underwent bronchoscopy, which revealed a protruding endobronchial lesion almost completely obstructing the right upper lobe bronchus limiting any further visualization of the RUL segments. Initial pathologic analysis of specimens biopsied during bronchoscopy revealed non-small cell lung carcinoma (NSCLC). Subsequently, a positron emission tomography (PET) scan was performed and showed a hypermetabolic RUL pulmonary mass with obstruction of RUL bronchus compatible with malignancy in addition to low level uptakes in the adrenal and thyroid glands. A MRI of the abdomen was done to better examine the adrenal gland, however, no masses were observed, only mild thickening. It was determined by the tumor board that the patient would best benefit from tumor excision and adjuvant chemotherapy. Subsequently, a definitive diagnosis was established by way of a RUL lobectomy. The resected specimen consisted of multiple fragments of tan/black soft tissue measuring 0.3 × 0.3 × 0.2 cm in aggregate. Pathologic analysis of the resected specimen showed sarcomatoid giant cell carcinoma, tumor size 9.5 cm with invasion of the visceral pleura and 1/13 hilar lymph node involvement. The pathological stage was pT3N1Mx based on the tumor node metastasis (TNM) staging system. By immunohistochemistry, the tumor cells were diffusely positive for CK7 while negative for CK20, TTF-1 and p40. The patient was started on adjuvant chemotherapy (do you know the chemo meds?) and followed as an outpatient.\nThis case reveals a possible paraneoplastic syndrome associated with pulmonary SC, the impact of HIV HAART on carcinogenesis and the prognosis of HIV patients with SC. Paraneoplastic syndromes are a group of clinical disorders that are associated with malignant diseases and are not directly related to the physical effects of the primary or metastatic tumors. [] Hypertrophic osteoarthropathy is a paraneoplastic syndrome consisting of a triad of symptoms including clubbed fingers, symmetric polyarthritis, and periostitis of the long tubular bones. [,] The above patient presented with signs and symptoms suggestive of this paraneoplastic syndrome which has not yet been reported to be associated with this type of cancer in the literature.\nSC is a rare malignancy accounting for less than 1% of all lung cancers. [,] It is characterized by components of sarcoma or sarcoma like structures (spindle cell or giant cells) and it includes five histological variants.9 Immunohistochemical studies play an important role in determining the histological type of non-small lung carcinoma. In general, CK5/6 and p63 are markers of squamous cell carcinoma. SP-A and TTF-1 are markers of adenocarcinoma. CK7 and CK20 are important in discriminating primary lung carcinoma and metastatic lung carcinoma []. In our patient, the immunohistochemistry was positive for CK7, but negative for TTF-1, p40 and CK20. The CD7/CK20 pattern suggested a primary lung carcinoma.\nDue to the heterogeneity in histopathology, surgical biopsy is required for definitive diagnosis. Treatment principle is the same as NSCLC, however, compared to NSCLC, patients with SC have a worse prognosis, higher recurrence after tumor resection and a lower survival rate. [,] The prognosis of these patients depend on tumor size and presence or absence of metastases. [] Fishback et al. in 1994 concluded that tumor size > 5 cm, a clinical stage > stage I, and lymph node involvement was associated with a significantly shorter patient survival. The median survival of patients with sarcomatoid carcinoma is 10 months, which is significantly lower when compared to that of more common lung carcinomas (20 months for adenocarcinoma, 18.5 months for squamous cell carcinoma, and 12.6 months for large cell carcinoma). []\nAlthough uncertain, the effects of factors such as a history of HIV may alter the prognostic variables of SC in the present case. A substantive number of patients have died despite non-detectable HIV viral loads and well preserved immunity measured by CD4 cell counts. It is also unclear if HIV directly increases the risk of this malignancy in this patient population. However, HIV/AIDS is well known to increase the risk of certain malignancies including Kaposi sarcoma and non-Hodgkin’s lymphoma, both of which are classified as AIDS-defining cancers (ADC). In the post - HAART era, the non-ADCs including anal cancer, lung cancer, liver cancer and head/neck cancer constitute an increasing proportion of documented malignancies in HIV patients. Therefore the role of HAART therapy and its carcinogenic properties in relation to the increased incidence of non - ADC needs to be explored as there has been only one reported case of giant cell carcinoma of the lung not associated with HAART therapy. For example, azidothymidine approved for HAART therapy of HIV-1 integrates into DNA, resulting in mutations of the hypoxanthine-guanine phosphoribosyl-transferase and thymidine kinase genes and other genotoxic effects in cultured cells. [] Such mutations may contribute to cellular carcinogenesis.\nThere are currently no standard effective chemotherapeuthic regimens for pulmonary sarcomatoid giant cell carcinoma. However, traditionally platinum-based chemotherapy is used if the patient is not a surgical candidate or if adjuvant chemotherapy is indicated. Adjuvant chemotherapy is indicated for nodal or chest wall involvement because they are associated with worse prognosis. [,] SC are more resistant to first line platinum based chemotherapy in comparison of other subtypes of NSCLC. [] The role of targeted treatment like EGFR inhibitors is still not completely understood in SC as compared to NSCLC. Our patient continues to follow up at the oncology clinic and completed 4 cycles of combination cisplatin and docetaxel therapy with supplemental G-CSF four months after surgery. |
A 23-year-old female with no significant past medical history presented with an acute onset of altered mental status and seizures. After stabilization, she began exhibiting hallucinations and bizarre thoughts which led to her eventual discharge to a mental health facility. She returned 72 h later with fever, tachycardia, worsening psychosis, unintelligible speech, and decreased responsiveness to external stimuli. The etiology of the fever was unclear at the time of admission. However, given her fever, tachycardia, and change in mental status, an infectious etiology was of highest concern and so she was started on broad spectrum antibiotics and acyclovir. On admission, her Glasgow Coma Scale (GCS) score was 9 and she was not alert or oriented to person, place, or time. The remainder of the neurologic examination was notable for fast unintelligible speech, inability to follow commands, and decreased responsiveness to external stimuli except pain. She initially was spontaneously moving all four extremities, had no significant cranial nerve abnormality, normal muscle tone, and 2+ symmetric reflexes bilaterally in upper and lower extremities. Imaging including magnetic resonance imaging (MRI) of her head and computed tomographic (CT) scans of her chest, abdomen, and pelvis were negative. An extensive infectious work-up was negative, however; her lumbar puncture did show a lymphocytic pleocytosis (). Continuous electroencephalogram monitoring was obtained and notable for severe generalized slowing and 2–3 Hz rhythmic delta activity with 15–18 Hz sharply contoured beta activity overlying delta activity consistent with extreme delta brushing. Additional evaluation was notable for positive serum NMDA antibodies (1:320) and CSF NMDA antibodies (1:80). Given these results, further imaging studies were completed to evaluate for teratoma, and an MRI abdomen and pelvis revealed a 6-mm right ovarian teratoma. At that time, high-dose methylprednisolone 1 g/day and plasma exchange (PLEX) were initiated. The patient was taken for definitive surgical therapy the next morning. Post-operatively, she was continued on methylprednisolone for a total of 5 days with five treatments of PLEX. She continued to decline with worsening mental status, loss of response to pain, and respiratory failure requiring intubation. GCS score at this time had now decreased to 3. She then began treatment with intravenous immunoglobulin (IVIG) for five doses. There was no response to IVIG, and both her respiratory and neurologic status remained unchanged. No testing was completed looking for genetic abnormalities or variations in her Cytochrome P450 system. Her final pathology was consistent with a mature teratoma.\nDespite initial interventions, her condition failed to improve and her serum NMDA antibodies increased to 1:1280. With the lack of clinical improvement and increasing antibody titer, there was concern for treatment failure or a potential residual teratoma. Repeat imaging including a positron emission tomography (PET)/CT was completed with negative results. Given this, she was started simultaneously on a combination of rituximab (375 mg/m2) and cyclophosphamide (750 mg/m2) 2 weeks after PLEX and IVIG were completed. Two months after rituximab and cyclophosphamide, significant neurologic recovery occurred and she became alert, oriented, and able to follow commands. In addition, she was weaned from the ventilator, regained her ability for fluent speech, and had resolution of her seizures. No relapses were noted and 4 months post-treatment, the only notable neurologic sequelae was short-term memory loss. In total, she received four cycles of rituximab and two cycles of cyclophosphamide with plans for further rituximab 6 months after the initial dose. Eventually, her NMDA antibodies decreased to a titer of 1:80. |
In June 2009, a 68-year old man presented to you hospital with a 1 month history of worsening axial back pain at the thoracolumbar junction. Past medical history of the patient was positive for high blood pressure and benign prostatic hyperplasia. At first the patient was assessed by his general practictioner and was prescribed oral antibiotic therapy with ciprofloxacin for a suspect urinary tract infection. Therapy was unsuccessful and patient developed fever, night sweats and fatigue over the course of the following 15 days. The patient was admitted to our hospital with the suspect diagnosis of pyogenic spondylodiscitis. Spinal X-ray, CT and MRI scans were obtained (A,B). Imaging showed destructive lesion at T11-T12 with disc and endplate involvement, suggestive of pyogenic spondylodiscitis type B3.1 []. A CT guided biopsy of the lesion was positive for S. hominis pyogenic infection. The patient was started on intravenous teicoplanin and ceftriaxone therapy which was continued for 4 weeks and followed by other 4 weeks of iv therapy with teicoplanin and co-trimoxazole. At the end of iv antibiotic therapy the patient was prescribed oral therapy with co-trimoxazole and rifampicin for 4 more weeks. Before starting antibiotic therapy the patient was prescribed a rigid TLSO brace to relieve pain and prevent development of spinal deformity. The brace was poorly tolerated by the patient and for this reason operative treatment with percutaneous posterior screw-rod instrumentation was offered. Posterior fixation from T10 to L1 was performed uneventfully (C,E). The patient was allowed free mobilization soon after surgery. Clinical and laboratory parameters improved significantly over the course of the follow-up and complete healing of the infection was determined at 3 months after the diagnosis. The patient is currently 80-years old and completed the 10-years follow-up after the procedure. He lives indepedently at home, does not complain of any back pain. The imaging obtained at 10 years follow-up shows complete healing of the lesion with fusion of the vertebral bodies. No mobilization of the instrumentation is noted (D,F). |
A 6-year-old girl presented to the Emergency Department with a suspected mandibular dislocation after falling off her scooter. She was previously fit and well with no significant medical history. She had complete restriction of all mandibular movements and trismus. The dental occlusion demonstrated a cross-bite with all the posterior teeth in contact and a mandibular asymmetry with the chin significantly deviated to the left side. Radiographic investigations (orthopantomogram (OPG) and postero-anterior (PA) mandible images) were performed but confirmation of the dislocation could not be determined on these views (Fig. ). Consequently, a computerized tomography (CT) scan was performed that confirmed significant displacement of the condyle anteriorly and superiorly into the infratemporal fossa (Fig. ). In addition calcification of the lateral and medial pterygoid muscles was noted on the affected side indicating chronic nature of the condition. Discussion with the patient’s general dental practitioner (GDP) revealed that the patient had a pre-existing dental malocclusion comprising of a cross-bite and mandibular asymmetry and this was also corroborated by a photograph taken well before the scooter accident. On this evidence it was therefore presumed that the condyle had been dislocated for many months or years and the scooter accident had only highlighted this. There were no other traumatic events of note in the history. The TMJ could not be convincingly reduced via a Hippocratic manoeuvre under a short general anaesthetic despite several attempts. In order to re-image the TMJ a magnetic resonance imaging scan was performed to prevent additional irradiation. This scan confirmed that the TMJ was still dislocated and had not been reduced. Due to the unsuccessful closed reduction, it was decided to perform an open reduction via a Risdon incision. When reduction with traction wires at the mandibular angle failed a bone hook was placed into the sigmoid notch and the dislocation was reduced using a fair degree of force. |
We present a case of a 79-year-old African American male with a past medical history of diabetes mellitus type II, essential hypertension, and atrial fibrillation with symptomatic normocytic anemia resulting from three distinct GI cancers. The patient was treated with gastroduodenectomy with Billroth II anastomosis, gastric fundus resection, and distal pancreatectomy. The patient initially presented to the emergency department (ED) with syncope and tachycardia. He reported experiencing symptoms of fatigue, abdominal pain, and lightheadedness for 2 months. Complete blood count revealed a hemoglobin level of 4.9 g/dL requiring three packed red blood cell transfusions.\nOn the following day, due to his symptoms and concurrent severe anemia, the GI mass was suspected, and a CT of the abdomen and pelvis with contrast was promptly ordered. The findings of the CT report demonstrated a 1.3-cm ovoid filling defect in duodenal bulb () and a 1.5-cm cystic lesion in the pancreatic tail (). Furthermore, the patient completed an esophagogastroduodenoscopy (EGD) which exhibited a mass in the duodenal bulb that was partially resected for biopsy. The biopsy confirmed invasive adenocarcinoma of gastroduodenal origin with a histological grade of III/III under World Health Organization (WHO) classification.\nThe patient underwent exploratory laparoscopic resection of the duodenal bulb mass and pancreatic tail mass. During the surgical removal of the duodenal bulb tumor, the examination of the stomach showcased a pedunculated mass near the fundus on the posterior wall of the stomach. The posterior wall mass was excised with duodenal and pancreatic masses and all samples were sent to pathology.\nThe pathological report for proximal duodenum mass showed an in situ and invasive well to moderately differentiated adenocarcinoma measuring 4.5 × 2.3 × 2.3 cm with infiltration into the submucosa (). The posterior gastric wall mass was reported to be a GIST, 1.3 × 1.1 × 0.8 cm in size. This tumor was of low grade, spindle cell type, with dystrophic microcalcifications (). The cells stained positive with Dog1, CD34, and c-KIT. The mass on the tail of the pancreas was shown as a well differentiated neuroendocrine tumor measuring 0.8 × 0.5 × 0.5 cm. The tumor stained positive for AE1/AE3, CAM5.2, and diffusely strong staining with neuroendocrine markers (synaptophysin, chromogranin (CGA), neuron-specific enolase (NSE), and CD56) (). The oncology report demonstrated that all of the cancers were excised completely with clean margins and therefore, no chemotherapy treatment was planned.\nThe patient’s postoperative course revolved around management of any complications related to surgery and maintaining the hemoglobin levels. The patient’s postoperation day (POD) 1 hemoglobin/hematocrit (H&H) levels were 10.2/35.1. The H&H levels on POD 7 were 8.9/29.8 and POD 14 were 9.1/31.7. The patient did not report further abdominal pain and was no longer having episodes of syncope. The patient is currently recovering well. |
A 69-year-old female with a past medical history of metastatic breast cancer status posttreatment with abemaciclib/fulvestrant and radiation therapy, iron deficiency anemia, diabetes mellitus type 2, hyperlipidemia and a recent left leg deep vein thrombosis status post thrombectomy and stent placement presented from an outpatient rehabilitation unit due to three days of worsening confusion as reported by her family. Approximately 5 weeks ago, the patient began experiencing acute intermittent confusion for a few days as reported by her family followed by subjective fevers. She subsequently was admitted to an outside hospital and was diagnosed with an ESBL E. coli urinary tract infection. After one week of receiving IV antibiotics, the patient was discharged with an additional one-week course of oral ciprofloxacin for treatment. During that prior hospitalization, her course was complicated by the onset of progressively worsening bilateral lower extremity weakness and paresthesias which severely limited her ability to ambulate. At that time, this complication was believed to be secondary to being in a state of sepsis due to the patient’s urinary tract infection. Consequently, she was started on ciprofloxacin and was discharged to an acute rehabilitation facility; however, she experienced little to no improvement in her strength despite being compliant with her prescribed antibiotics.\nOn physical examination, the patient had normal vital signs and was noted to be alert and oriented with no signs of apparent respiratory distress. No suprapubic or costovertebral angle tenderness was elicited. Her neurologic exam demonstrated significantly decreased strength of her bilateral lower extremities with preserved sensation to light touch grossly. The remainder of her exam was unremarkable. Pertinent admission laboratory data included a leukocytosis of 18,300/uL with neutrophilic predominance, normocytic anemia with a hemoglobin of 8.9 mg/dL and mean corpuscular volume of 97.1 fL, hypercalcemia of 12.9 mg/dL, mild hypoglycemia of 69 mg/dL, and decreased thyroid-stimulating hormone at 0.16 uIU/mL but normal T3 and T4 levels, normal vitamin B12, vitamin B1 and folate levels, negative blood cultures and a fairly normal urinalysis. Chest x-ray and computed tomography scan without contrast of the head showed no acute abnormalities. Of note, the patient had a brain magnetic resonance imaging (MRI) study approximately one-month prior that showed a small right frontal meningioma that appeared stable on her CT this admission. CT of the lumbar spine without contrast showed mixed lytic/sclerotic appearance of the bones suggesting metastatic disease and multilevel spondylosis without high-grade spinal canal stenosis.\nGiven the patient's presentation of acute encephalopathy in the setting of leukocytosis and history of recent ESBL E. coli UTI, there was concern that the patient was inadequately treated with ciprofloxacin. Thus, she was given a one-time dose of Fosfomycin, along with IV fluids for her hypercalcemia, which improved her mental status and laboratory abnormalities. Work-up for the hypercalcemia was otherwise unremarkable and ultimately attributed to dehydration. Inpatient neurology and neurosurgery services were consulted for additional assistance with the management of acute paraparesis. No neurosurgical interventions were warranted. Additional laboratory work-up, including serum and urine protein electrophoresis, neuronal antibody (amphiphysin), CV2.1 IgG antibody and Purkinje cell/neuronal nuclear IgG antibody levels, was unremarkable. The patient underwent further imaging with MRI of the cervical, thoracic, and lumbar spine, which showed diffuse osseous metastatic lesions but no evidence of spinal cord compression or severe stenosis. A subsequent electroencephalogram demonstrated no signs of any epileptiform activity. An electromyography (EMG) study was obtained revealing complex polyphasic motor unit potentials in the right anterior tibialis, medial gastrocnemius, vastus lateralis, and tensor fascia lata muscles. Paraspinal muscles were not examined due to the patient being on anticoagulation for her history of deep vein thrombosis. Sensory nerve conduction studies were obtained and shown below (Figure , Table ). Motor nerve conduction studies were obtained which showed no response in the lower extremity muscles or in the right radial study (Figure , Table ). The patient also had poor F-wave responses on motor testing in her lower extremity muscles (Figure ). The patient was treated with a five-day course of intravenous immunoglobulin (IVIG) for suspected GBS. This resulted insignificant improvement in her lower extremity strength bilaterally. At the end of her hospital course, she was discharged to a skilled nursing facility for further care. |
A 48-year-old Sri Lankan woman was referred to our general surgical clinic for the management of a bladder stone following successful treatment for a urinary tract infection complicated with upper tract involvement. She complained of intermittent nonspecific lower abdominal pain, dysuria, and hematuria over the previous 6 months. During the same period of time she had three uncomplicated urinary tract infections that were managed by her general practitioner. The urine culture grew a pure growth of Proteus each time. She was managed with orally administered co-amoxiclav, according to the antibacterial sensitivity report, for 1 week during each episode. She was put on nitrofurantoin as a urinary antiseptic after the third episode of urinary tract infection. On presentation for the complicated, fourth urinary tract infection, this case underwent further investigation. She was found to be septic with a heart rate of 110 beats/minute, blood pressure of 130/90 mmHg, temperature of 38.9 °C (102 °F), and respiratory rate of 20/minute. She had neutrophil leukocytosis (18 × 109/ml), but her liver and renal function tests were normal. The radiographs of her kidney, ureter, and bladder showed a large bladder stone with three limbs and an imprint of a typical Copper T380A IUCD (Pregna International Ltd., Mumbai, India) in the middle of the stone (Fig. ). An ultrasound scan of her kidney, ureter, and bladder confirmed the intravesical location of the stone and left-side pyelonephritis. Urine culture yielded a mixed growth of coliform and Proteus. She was managed with intravenously administered cefotaxime according to the antibacterial sensitivity report for 1 week and was continued on the nitrofurantoin until she underwent surgery.\nOn further inquiry, our patient indicated that she had an IUCD inserted 15 years previously, after the delivery of her third child. Two years later, the threads of the IUCD could not be found during a routine visit to a Well Woman Clinic, and it was documented as a missing IUCD. A further workup was not conducted since she did not return for a follow-up. She had forgotten about the missing IUCD and only mentioned it after being questioned. She denied having any urinary or lower abdominal symptoms until the last 6 months. She did not have any previous medical conditions. She was a housewife and had no other risk factor for urolithiasis. She had no family history of urolithiasis. Her general and abdominal examinations were otherwise unremarkable.\nThe diagnosis of a bladder stone formed around a migrated IUCD was made and an open vesicolithotomy was scheduled for 4 weeks later due to the large size of the stone. The vesicolithotomy was uncomplicated, and the interior of her bladder was normal. A large bladder stone with three limbs measuring 6 × 5 cm was removed, the stone was broken, and the IUCD was found inside. The three limbs of the stone were shaped to cover the three limbs of the IUCD, with the threads of the device also completely covered by the stone material (Fig. ). Her postoperative period was uncomplicated and she was asymptomatic after the removal of the stone. At 6 months there were no further attacks of urinary tract infections. |
A 41-year-old male resorted for an abdominal distention and intermittent abdominal pain lasting for one month. It was learned that the patient who had no complaints of defecation problems or haemotochesia, lost about 5 - 6 kg of weight after the onset of disturbances. In his family history, it was seen that his fathet died because of colon cancer at the age of 64 and that his older sister (at age 45), little sister (age 35) and two children of his mother’s brother received treatment for colon cancers. In genetic research of the patient covering Revised Amsterdam Criteria, MLH-1 germ-line mutation was determined. In physical examinatiom, a hard, fixed, palpable mass filling up the epigastric region was present. Rectal examination showed a normal sphincter tonus, a rectum full of formal faeces and no haemotochesia.\nIn contrasted abdominal CT scan; a mass of 12 cm that shows signicant wall thickening and filling defect at transverse colon level (), a polypoid mass causing filling defect bulking from mucosa fitting rectosigmoidal colon in pelvis entrance () were detected. No aoter intraabdominal pathological view were obtained. Colonoscopy was planned fort he patient. A polypoid mass 19 cm from anal vestibule and an invased tumor in the middle of transverse colon that does not allow passage were determined. Multiple biopsies were obtained from both of the masses. The pathology reported the distal located polypoid lesion to be low differanciated mucinous adenocarcinoma and proximally located invased lesion to be an adenocarcinoma developed on the basis of a tubulovilleous adenoma.\nAn operation was decided to be made. During operation, an invased tumoral mass of 12 × 15 cm on abdominal wall in the middle of transverse colon that has exceeded serosa was seen. Lymph nodes some of which over 1 cm of width were present in omentum and intestinal meso. Other intraabdominal organs looked normal macroscopically. Total proctocolectomy, ilioanal anastomosis and omentectomy were performed. In pathological report, it was declared that invasive tumor exceeded serosa and metastasis were present in seven of the lymph nodes. K-ras gene mutation was determined in genetic analysis. In PET-Scan performed before adjutant chemotherapy; multiple metastatic lymph nodes in sol inferior jugular region, anterior mediastinum and abdominopelvis were established (). The phase of the tumor was detected to be T4N2M1 according to TNM classification. |
An 18-year-old female presented to the Department of Plastic Surgery in surgical super specialty block. History revealed repeated administration of intramuscular injections at bilateral gluteal regions. Clinical examination showed hard mass in bilateral gluteal area. Ultrasound scan of the gluteal areas showed hard lesions with pockets of necrosis within. Biopsy revealed calcified tissue. Patient was further evaluated by dermatologists for connective tissue disorders and muscular dystrophies. Laboratory investigations for rheumatoid arthritis, systemic lupus erythematousus (SLE) were done. Patient's clinical examination, blood investigations for connective tissue disorders and muscular dystrophies were negative.\nThe lady was posted for bilateral gluteal mass excision and flap cover procedure under anesthesia. Patient's preanesthesia check-up was done a day prior to surgery. Lady gave a history of getting intramuscular injections for fever and body ache from adolescence. Lady weighed 40 kilos and height of 150 cm. General physical examination showed thickened skin and small discrete bony masses beneath the skin in areas like face, elbows, knees and surgical site. Airway examination-mouth opening vertically allowed three fingers. Angles of the mouth were inelastic and allowed three fingers with difficulty. Jaw and neck were normal with a normal range of neck movements. Systemic examination was normal. There was restriction in the movement of the hip joint due to hard mass over gluteals. Fungal infection was present on the back till lumbar region. Anesthesia technique planned was general anesthesia with the endotracheal tube as position needed for surgery was the prone position.\nNil per oral guidelines were advised, and a written informed consent was taken from the patient Tablet Ranitidine 150 mg and Tablet Alpralozam 0.5 mg was advised for the night. On the day of surgery intravenous line was secured in preoperative holdup area and intravenous premedication of injection midazolam 1 mg was administered. Patient was shifted to operation theater and preinduction monitors for pulse oximetry, electrocardiography and noninvasive blood pressure were placed. Premedicated with the injection fentanyl 2 mcg/kg; preoxygenated for 3 min and induced with injection propofol 2.5 mg/kg. After confirming the ease of mask ventilation, injection rocuronium bromide 0.9 mg/kg was administered. Ventilated with air-oxygen mixture and sevoflurane. Laryngoscopy was tried with Macintosh blade 3 at a neuromuscular score of 0 and MAC of 1. Cormack–Lehane view was grade 4. BURP maneuver did not improve the view. Second dose of injection propofol 1 mg/kg was administered, and laryngoscopy was reattempted with MacCoy blade 3. Bougie was used and no. 7.5 Endotracheal tube was placed and secured with tape. Positioning of the tube was confirmed with capnography tracing and auscultation. Patient was positioned in a prone position with adequate padding at pressure points. Intraoperatively patient was maintained on IPPV with isoflurane, air-oxygen and intermitted doses of rocuronium and injection fentanyl.\nSurgical duration lasted 3 h. Patient was turned supine and extubated when fully awake. Patient was discharged on 7th post-operative day. Postprocedure follow-up was done and histopathology report showed calcification in the dermis layer. This confirmed the diagnosis of calcinosis cutis. |
We report the case of a 76-year-old Caucasian man who presented to our hospital for acute onset epistaxis associated with headache and dizziness. He had a history of arterial hypertension, left bundle branch block, dyslipidemia and heart failure, for which he was taking antihypertensive and antiplatelet drugs. He also reported a traumatic head injury in the context of a road traffic accident seventeen years before, after which he had otorhinolaryngology (ENT) surgery twice: the first time for traumatic fracture of the nasal pyramid and second time to correct a nasal septal defect.\nBrain computed tomography (CT) showed small, subtle areas of subarachnoid hemorrhage located in both frontal and left temporal lobes and bilateral 5mm hygromas (). A subsequent CT angiography ruled out an aneurysm, and the patient was admitted to the Department of Neurology.\nGeneral clinical examination was unremarkable. Blood pressure was 155/80 mmHg and his heart rate was regular at 80 beats per minute (bpm). Neurological examination showed a left-sided peripheral facial palsy (sequela from his car accident) without any other pathological findings. Headache and dizziness occurred within minutes of adopting an orthostatic position and significantly improved when lying supine. His epistaxis resolved within hours from the onset and the otorhinolaryngology exam showed evidence of hemotympanum, considered to be a consequence of epistaxis. A thorough anamnesis revealed that he had a few episodes of isolated headache attacks sometimes associated with dizziness over the previous years, all presenting a strictly orthostatic character. These episodes were sometimes associated with symptomatic episodes consistent with post-nasal drip.\nThe brain magnetic resonance imaging (MRI) was consistent with the diagnosis of SIH, showing bilateral subdural hygromas, diffuse contrast enhancement of the pachymeninges, superior sagittal sinus engorgement, and small areas of cortical and subarachnoid hemorrhage in the left temporal and right frontal regions. Susceptibility weighted imaging showed evidence of superficial siderosis at the level of the frontal lobes bilaterally, which could be due to past episodes of subarachnoid hemorrhage or a consequence of his past head trauma ().\nThe patient was confined to bed rest and received an increased hydration regime, generous caffeine intake, and low dose dexamethasone (8 mg/day). Two spinal cord MRI scans aiming to identify a potential CSF leak were performed on separate days, but no signs of dural leakage were identified. His headache and dizziness progressively improved, but two days after admission, he complained of left ear fullness. A high-resolution CT scan of the temporal bone showed an oblique fracture involving the left temporal bone and communicating with the posterior superior marginal mastoid cell. Liquid density was visualized in the left mastoid cells, and therefore, a new brain MRI with fine T2-weighted sequences was performed, confirming this finding ().\nRepeated and more elaborate ENT examination showed left hemotympanum with intermittent pulsatile discharge from the pharyngeal opening of the Eustachian tube, and nasal endoscopy revealed oto-liquorrhea. However, repeated nasal endoscopy performed two days later, intending to obtain a sample for b2-transferrin testing, was unsuccessful because the discharge had stopped. Besides ear fullness, the patient remained symptom-free and was discharged home. He was further referred for ENT and neurosurgical follow–up in an experienced center for further assessment of the opportunity of surgical exploration aimed to clearly localize and seal the cranial dural defect and the temporal bone fracture. He did not report any other symptoms in the first month after discharge and refused surgery proposed by a joint neurosurgical-otorhinolaryngology team. |
An 18-year-old female patient reported with pain in lower left side of the jaw to the Department of Endodontics. Her medical history was noncontributory. On intraoral examination, there was deep carious lesion in mandibular left first and second molar. First molar tooth showed delayed response to pulp vitality testing (electric and cold pulp testing) and second molar showed no response at all. Teeth were tender to percussion. Intraoral periapical radiograph of first molar revealed that the presence of large pulp chamber with small thinned-out roots and a coronal radiolucency involving pulp chamber without any periapical changes. There was an isolated radioopaque density with respect to the distal root periapical region. The second molar also showed large pulp chamber with coronal radiolucency involving it with widening of apical periodontal ligament space []. Based on the clinical and radiographic findings, a diagnosis of mesotaurodontism with advanced stage of irreversible pulpitis was given to mandibular left first molar and hypotaurodontism with symptomatic apical periodontitis was given to mandibular left second molar. The radio-opaque mass seen with respect to periapical region of mandibular left first molar was diagnosed as idiopathic osteosclerosis. Upon approval from the patient, root canal treatment was advised for both the molars.\nMandibular left second molar (37) was anesthetized with inferior alveolar block using 2% lignocaine with epinephrine 1:100000 and pulp chamber of 37 was accessed under rubber dam isolation. Working length were determined with # 15 K files in mesial canals and # 20 K file in distal canal by ingles method and confirmed with electronic apex locator and radiovisiography []. Biomechanical preparation of pulp chamber was achieved by circumferential filing with ISO K files of size # 25 in all canals followed by further shaping using ProTaper files (F4 for distal canal and F3 for mesial canals) []. Canals were irrigated with 2.5% sodium hypochlorite, 17% aqueous solution of EDTA and 2%W/V chlorhexidine gluconate and saline as the final irrigant using 26-G needles. After thorough drying of canals with paper points, calcium hydroxide intracanal medicament was placed. Patient recalled after 4 weeks. In next visit tooth was asymptomatic and canals were dry. Obturation of the canals were done using GP cones and AH plus sealer with conventional lateral compaction technique. Access cavity was cleaned with dampened cotton and restored with varnish and amalgam.\nIn mandibular left first molar (36), after access opening an extra canal was located lingual to the distal root []. Root canal therapy of 36 was done similar to 37 along with the extra-distolingual canal. Canals were enlarged using ProTaper files upto F3 in all four canals. Obturation of the canals was done using lateral compaction in root canals and vertical compaction using softened gutta percha in pulp chamber. Access cavity was cleaned with dampened cotton and restored with amalgam []. |
A 21 year old female patient, newly diagnosed with Graves’ disease was referred from a peripheral hospital with a two week history of progressive yellowing of the eyes associated with generalised abdominal distension, marked right hypochondrial pain and mild generalised pruritis. Ten days prior to her admission, she developed worsening exertional dyspnea, easy fatigability, bilateral lower limb swelling , palpitations, orthopnea , profound weight loss and generalised muscular weakness and heat intolerance. She had been amenorrheic for 4 months and was not taking any medications. She had no history of smoking or alcohol consumption.\nClinical examination on admission revealed a thin, diaphoretic and mildly agitated lady. Her axillary temperature was 39.2°C. She had jaundice with bilateral pitting lower limb oedema, a fine resting tremor, exophthalmos and eyelid lag. The thyroid gland was palpable, diffusely enlarged and non tender with a bruit. Cardiovascular examination revealed a rapid, large volume and regular pulse of 140 beats/min with an isolated systolic hypertension of 150/70 mmHg. The jugular venous pressure was raised and neck veins engorged. She had a heaving apex beat displaced to the 6th intercostal space anterior axillary line with a gallop and a soft apical systolic murmur. Abdominal and respiratory examinations revealed a tender hepatomegaly with ascites and reduced breathe sounds on the right infra axillary and scapular areas respectively.\nA chest radiograph done revealed a cardiomegaly with a right sided pleural effusion. An abdominal Ultra sound scan done showed an enlarged liver with a normal echo texture, ascites, normal biliary ducts and gall bladder. A thyroid ultra sound scan was not done. Electrocardiography showed sinus tachycardia, with a rate of 142 beats/min and left ventricular hypertrophy by voltage criteria. The Echocardiography showed globally dilated cardiac chambers with moderate mitral and tricuspid regurgitation, an elevated ejection fraction of 82% (40-70%) and moderate pulmonary hypertension.\nThe laboratory investigations done are summarised in Table below.\nThyroid function tests revealed a suppressed thyroid stimulating hormone level (TSH) of <0.005 uIUml (0.27–4.2), raised free thyroxine levels of 267.5 nmol/L (normal range: 66–181) and free triiodothyronine of 10 nmol/L (normal range: 1.3-3.1).\nA thyroid nuclear scan showed a diffuse homogenous increased uptake of radioactive iodine with no background uptake. Thyroid stimulating hormone receptor antibody, Antithyroglobulin and antithyroid peroxidase antibodies were not done due to financial constraints.\nAn impression of thyroid storm due to untreated Graves’ disease with cholestatic liver injury, high output congestive heart failure due to thyrotoxic heart disease and acute kidney injury was made. The patient’s Burch-Wartofsky score for thyroid storm was 90 [].\nShe was started on oral carbimazole, Prednisolone, Propranolol, Lugol’s iodine, intra venous fluid replacement, oral anti histamines for the pruritis, oral frusemide and Captopril for the heart failure.\nThe patient was discharged 10 days later with great improvement and on a follow-up assessment 10 weeks later; she was feeling generally well with resolution of the jaundice and clinically euthyroid. A repeat electrocardiography, echocardiography, renal and liver function tests were all normal. |
This is a case of a 10-month-old female infant who presented to the emergency department (ED) for vomiting and decreased per os (p.o.) intake secondary to rhinovirus. After attempts to feed the infant p.o. failed in the ED, she was admitted overnight. Vital signs and labs were normal, prompting treatment with supportive care. The infant received a 20 ml/kg bolus of normal saline in the ED via peripheral IV in the dorsum of her right hand, and once admitted was started on 1.5× maintenance IVF consisting of D5W 1/2 Normal Saline and 20 KCl at 55 ml/hr. Overnight, she tolerated the IV fluids well and was able to take a bottle. Urine output measured 62 ml/kg. IV fluids were to be discontinued that morning. At morning rounds, the mother reported that the patient did well overnight, but mentioned her shirt became wet after holding the infant. The IV line was examined and no leaks or abnormalities were noted. The IV was flushed and removed by the nurse at 0730 without incident. 30 minutes after removal of the IV, the patient began to have an acute reaction at the IV insertion site extending up the right arm. The patient's right hand and forearm appeared erythematous and tense, with eruption of bullae observed on dorsum and palm of the hand. The bullae and erythema extended up the forearm and upper extremity to the chest wall. The arm also had diffuse edema from mid-humerus to hand. Furthermore, a large white lesion in the center of the dorsum of the right hand with surrounding deep purple bruising was noted concerning for necrosis (). Bullae were cultured, and the infant was immediately started on diphenhydramine, pediatric acetaminophen, and ibuprofen p.o. 4% topical lidocaine cream was applied directly to the arm. Both the orthopedic as well as plastic surgery teams were consulted at this time.\nThe patient had no recorded allergies, but the mother of the infant had a severe latex allergy and siblings in the family had documented sulfa allergies. Based on family history, infant was placed on latex allergy alert. At 1-hour post IV removal, physical exam showed a tender swollen right arm. Radial pulse was palpable, and capillary refill was under 3 seconds. Additionally, the white central lesion did not blanche, but was compressible. After an additional 30 minutes, the erythema and induration had regressed from the chest wall and was only seen from mid-humerus to the hand. Compartment pressures were stable. After consulting with plastics and orthopedics the decision was made not to perform fasciotomy but to continue with conservative management. In addition to the aforementioned medical therapy, the patient's mother was instructed to keep the patient's arm elevated. The patient's arm was cleaned, treated with Bacitracin ointment, and covered with Xeroform and a loose Kerlix overlay. The care team followed the patient with serial visits throughout the remainder of the day and night, noting improvement throughout. By the following morning, the non-blanching white area had resolved, bullae were still visible, and the patient appeared to be improving (). The patient was able to be discharged to home on day 3 of admission, and followed at the outpatient clinic by the primary team as well as plastic surgery.\nDifferential diagnoses for this encounter included atypical hypersensitivity reaction to latex, acute reaction to IV infiltration complicated by acute compartment syndrome (ACS), and compartment syndrome due to infection or necrotizing fasciitis. Gram cultures grew no organisms, including up to 3 days later. 5 days after discharge, mother reported significant improvement, and that the infant was tolerating dressing changes well. She was also moving her right arm more. Edema was still noted, but bullae and erythema were improving and epithelization was progressing well. The plastics team recommended Vaseline with Xeroform changes (). 11 days after discharge, the parents reported continued improvement and increasing ROM in patient's right arm (). Follow-up at 5 weeks showed continual improvement, but not complete return to baseline (). Referral was made for physical therapy for continued help with range of motion and use of right hand and arm. |
A 38-year-old woman, gravida 5 para 4 presented at 32 weeks pregnancy. She first presented to her primary general practitioner with a small vulvar lesion that she had first noticed one year prior. The lesion was suspected to be benign Bartholin’s cyst and treated with broad-spectrum antibiotics. The patient noticed no improvement and had many other consultations with different clinics when she was advised to the same diagnosis and treatment. Initially, the swelling was constant in size, however, it started to enlarge rapidly. A significant increase in size was noticed during pregnancy and causing a restless feeling. The patient was thus referred to our hospital. She had no significant gynecological past history, prior gynecological malignancies, or condyloma acuminatum. Her pregnancy course was uneventful. The medical, surgical, and family histories were noncontributory. No medications had been taken before or during the pregnancy.\nPhysical examination revealed a healthy woman with normal vital signs. Abdominal examination reveals a gravid abdomen with a fetus of appropriate size for the gestational age in cephalic presentation. Pelvic examination revealed a vulvar mass at the left side involving left labia majora and minora. The mass was painless, rounded, firm in consistency, freely mobile with an intact overlying skin measuring 15.0 x 10.0 cm diameter (Figure ).\nNo palpable lymph node was detected. Pelvic Magnetic Resonance Imaging (MRI) with heterogeneous signal intensity on T2-weighted images showed a solid mass with no cystic signal intensity measuring 4.8 x 7.2 x 10 cm. The radiological impression was labial leiomyoma.\nAt 34 weeks of pregnancy, the patient was admitted for tumor resection. A baseline fetal cardiotocography and obstetrics ultrasound were normal. Under spinal analgesia, the tumor was completely resected with minimal blood loss (Figures , ). No frozen section samples were taken. The patient and her fetus tolerated the procedure well. The histopathology report revealed leiomyosarcoma. The cease was discussed with the gynae-oncology team and the patient as well and a plan was agreed for induction of labor for further radiological assessment and treatment. Therefore, the patient was induced at 34 weeks and had a vaginal delivery; the antepartum and postpartum course were unremarkable.\nThe patient was referred after delivery to the Gynaeoncology department for follow-up and further assessment and management. She had pelvic MRI, abdominopelvic CT with contrast, PET-CT head, neck, and lower extremity and show no tumor deposit, neither local nor distal. On post-surgical follow-up, the patient was asymptomatic and had no radiotherapy or adjuvant chemotherapy. She had no evidence of recurrence or distant metastasis on 12 months follow-up after surgery.\nHistopathology\nGross evaluation revealed a well-defined mass measuring 10.0 x 8.0 x 5.0 cm and showing tan whorly cut surfaces with no obvious areas of hemorrhage or necrosis. Histological examination showed smooth muscle neoplasm arranged in fascicles. The tumor cells showed mild to moderate nuclear atypia and increased cellularity. The mitotic index was nine mitotic figures per 10 high power fields (HPF) when the high method is used. Small foci of tumor necrosis were identified (Figure ). The tumor cells were positive for smooth muscle markers (smooth muscle actin [SMA], Desmin, and Caldesmon) (Figure ). Based on the above-mentioned findings and applying the current WHO classification the case was diagnosed as leiomyosarcoma. |
We present the case of a 13-year-old female patient, known with severe visual field loss, who referred for another opinion regarding the ophthalmological diagnosis.\nAnamnesis at presentation revealed that at the age of 9 years and 3 months, on a routine ophthalmological examination, papillary calcification and retinal hemorrhage were discovered in the left eye. At that moment, the suspicion of intracranial calcifications was raised. The patient underwent clinical neurological examination, EEG, and cerebral MRI, all of them revealing a normal aspect. The patient was also recommended fluorescein angiography, which showed papillary autofluorescence. The diagnosis established then was papillary drusen in both eyes, buried in the right eye and mixed in the left eye and the patient was recommended to keep it under observation, together with a periodical examination of the visual field.\nThe patient had had multiple examinations of the visual field over the time.\nThe first visual field examination of the right eye showed an arcuate nasal defect, in the superior nasal quadrant, sketching an aspect of nasal step, structure of the sensitivity defect that in 3 years time evolved into a quadranopsia.\nIn the left eye, the first visual field examination showed inferior nasal quadranopsia, extended superiorly with a nasal arcuate defect respecting 20° centrally, which after three years evolved into a paracentral diffuse defect with an island of central vision of 5°.\nHowever, over the time, the examination of the visual field was made with different types of machines, and no correlation of the modifications could be made objectively.\nThe patient received several different diagnoses from several different ophthalmologists, among which optic nerve drusen; papillary oedema and hamartoma have to be mentioned.\nAt presentation, the patient’s visual acuity was 20/ 20 with correction for the RE and 20/ 20 without correction for the LE, with a refraction ROD: -1 DSf<> -0.75 DCyl, 179* and ROS: +0.50 DSf<>-0.75 DCyl, 167* and a cycloplegic refraction: OD: -0.75 DSf<> -1 DCyl, 168*, OS: +0.75 DSf<> -1 DCyl, 170*.\nThe intraocular pressure was 19 mmHg GAT in the right eye and 13 mmHg GAT in the left eye.\nSlit lamp examination of the anterior segment revealed no pathological changes for both eyes, and the red-discrimination test was also normal.\nFundoscopy presented only with papillary pathological modifications.\nThe optic disc in the right eye was elevated, with relatively clear margins, pink color, and the absence of cupping. At 5 o’clock meridian, a nodular yellow mass, with irregular outline, could be noticed ().\nIn the left eye, the optic disc was also elevated, pale, of irregular outline, and the absence of cupping was noticed. Nodular, yellow, reflective protrusions, with irregular contour and brambleberry shape could be noticed ().\nThe retinal vessels, the macula, and the retinal periphery presented no pathological changes in either of the eyes.\nThe clinical examination suggested the diagnosis of optic nerve drusen in both eyes. B-scan ultrasonography and optical coherence tomography (OCT) examinations were used for the confirmation of the diagnosis.\nB-scan ultrasonography is considered the gold standard method for the detection of optic disc drusen. In this patient’s case, it showed round, hyperechoic structures, observed at the optic nerves of both eyes. The A-scan mode, which was overlapped on the structure only for the left eye, showed hyperreflectivity at the anterior side of the optic nerve, of supraretinal intensity.\nOptical coherence tomography is a useful examination in the assessment of the structure and the anatomical shape of the drusen, and in the analysis of retinal nerve fiber layer (RNFL) and GCL-IPL complex.\nFor patients under 18 years old, however, there is no normative database regarding the normal values of the analyzed parameters, therefore these analyses are useful only for patient’s follow-ups.\nThe OCT scan of the optic nerve showed a prominent aspect of the optic disc, with a lower value of average RNFL thickness in the left eye compared to the right eye ().\nMacula was structurally normal, with an asymmetry of macular thickness, thinner in the left eye, compared to the right eye ().\nThere was also an asymmetry of thickness regarding the GCL-IPL complex, which was thinner in the left eye compared to the right eye ().\nThe investigations confirmed the diagnosis of optic nerve drusen in both eyes.\nThe differential diagnosis in the case of this patient took into consideration the following pathologies:\n• Papilloedema – excluded by B-scan ultrasound;\n• The existence of an intracranial expansive process – excluded by clinical and imagistic examinations;\n• Optic nerve tumors\no Astrocytic hamartoma – the proliferation of astrocytic cells occurs above the optic disc, whereas optic disc drusen is located in the substance of the optic nerve.\no Optic nerve sheath meningioma – excluded by clinical and imagistic examinations.\n• Leber optic neuropathy – it typically presents with severe loss of central vision.\n• Infiltration of the optic nerve (leukemia, lymphoma) – excluded by normal laboratory tests.\nThe patient’s visual field examination at presentation revealed a superior nasal altitudinal scotoma at the right eye (), and at the left eye an important constriction of the visual field, with the preservation of a small 15* island of temporal paracentral vision ().\nThe patient was not recommended any treatment, but only periodical follow-up with visual field examination at every 4-6 months, and annual OCT.\nThe patient came back a year later for follow-up. At examination, there was no progression of the visual field alterations (,), but the intraocular pressure was at the superior level of the normal range, 21 mmHg GAT for the right eye and 20 mmHg GAT for the left eye.\nTherefore, the patient was recommended the treatment with a prostaglandin analogue to prevent the exacerbation of the visual field loss in order to attenuate the mechanical compression on the ganglion cells axons and to improve the blood flow to the optic nerve head. |
A 30-year-old female patient with noncontributory medical history reported to the department of periodontics with the complaint of GS, and she was not satisfied with her smile []. This was the patient's first consultation regarding her concern, and clinical examination revealed that she had a healthy periodontium. The examination also showed that the patient had incompetent lips and that her gingival display was excessive on smiling. It could also be noted that the maxillary central incisors had a lower gingival zenith when compared to the lateral incisors, and it was not esthetically appealing to the patient [].\nLocal anesthesia was obtained in the maxillary right premolar to left premolar region by injecting 2% lignocaine with 1:100,000 epinephrine. The recontouring of the gingival zenith of the maxillary central incisors was carried out using a soft-tissue diode laser (Zolar Technology) with a power setting of 0.5 W and an energy setting of 120 mJ. The laser-assisted surgery resulted in esthetic reshaping of the gingival margin of the maxillary central incisors []. Following this, a partial-thickness flap was elevated between right and left maxillary premolar regions, and the incision for lip repositioning was placed about 10 mm from the first incision in the labial mucosa. Both the incisions were connected, and the resultant strip of connective tissue from the partial-thickness flap was removed []. The flap margins were sutured with resorbable sutures in a simple interrupted manner []. A periodontal pack was applied to the surgical site.\nThe patient was prescribed with antibiotics (amoxicillin 500 mg tid for 5 days) and nonsteroidal anti-inflammatory drugs (paracetamol 650 mg tid for 4 days) to aid in postoperative healing. The patient was instructed to refrain from active lip movements such as talking, smiling, and opening the mouth wide. The patient was also advised to adhere to postoperative instructions such as application of ice pack, avoiding hot and spicy food, and having a soft diet. The patient was recalled after 1 week for the reapplication of the periodontal pack. The surgical area was irrigated with saline, and it showed satisfactory healing for the 1st week. The periodontal pack was reapplied. The 2-year follow-up revealed esthetically enhanced smile, and the patient was satisfied []. |
A 22-year-old female reported to the department of prosthodontics with the chief complaint of pain in the right ear region while chewing all kinds of food for 2.5 years. The patient had undergone extraction of all four third molars due to the same before 1.5 years, but the pain did not resolve. She had no significant medical history and family history but reported that she had been delivered through forceps delivery method. The preextraction record in the form of an orthopantamogram (OPG) was available []. The nature of pain was dull, continuous, located in the right TMJ region, and aggravated on chewing both soft and hard food.\nOn extraoral cranial nerve examination, there was a pain while clenching the teeth (testing motor component of the trigeminal nerve). The interincisal distance was 32 mm. Muscle examination was done[] []. TMJ examination revealed deviation of the mandible toward the left side while opening. There was a pain during left lateral movement and protrusive movement. On palpation, both the TMJs were tender. Clicking was present in the right TMJ when the patient tried to open the mouth wide. The load test revealed pain on the initial gentle loading.\nIntraoral examination revealed crowding of 31, 32, 33, 41, 42, and 43 [Figures and ]. Enamel wear facet was present in 47. The occlusion was anterior guided Angle's Class I malocclusion with the interferences at distobuccal cusp of 17 and 32.\nThe investigations were carried out to exclude any systemic disease affecting the TMJ. OPG and computed tomography scan of the right and left TMJ confirmed for condylar hypoplasia and early arthritic changes [Figures and ]. Magnetic resonance imaging of the patient confirmed anterolateral disc dislocation without reduction.\nThe treatment planning included patient education and motivation, supportive therapy, stabilization appliance, and arthrocentesis of TMJ followed by an orthodontic correction.\nThe following steps were used to fabricate a stabilization appliance:\nDiagnostic casts were obtained, and a 2-mm thermoplastic sheet was vacuum formed on it [] The patient was guided in centric relation, and anterior centric indentations were obtained by adding acrylic on the palatal aspect of the upper anterior teeth such as to increase the inter-occlusal distance by 4–6 mm so that it produces 1–1.5 mm space in the molar region [] Now, clear acrylic was added in the posterior region while the patient is asked to close on the anterior centric indentations [] At this step, we need to mark all the centric indentations on buccal cusp tips of the posterior teeth and incisal edge of the anterior teeth to flatten the rest of the appliance [] The appliance was then inserted in the patient's mouth and was checked for the centric and eccentric path. The eccentric path should exclude any other path than the canine. In the present case, as 32 was an interference that is to be corrected orthodontically, the appliance had left-sided eccentric path from 32 [] The final insertion was done, and the patient was asked to wear the appliance at least for 6 weeks [Figures –].\nPostinsertion instructions were to wear the appliance throughout the day and night excluding the mealtimes and teethbrushing.[] In case of exaggeration of symptoms, the patient must report back. Examination was done on follow-up at each week until 6 weeks.\nAt the end of 2 weeks, arthrocentesis of both the TM joint spaces by irrigating with 50 ml of ringer lactate solution and then treating by 0.6 ml of platelet-rich plasma (PRP) was performed at the department of oral and maxillofacial surgery.\nAt the end of 6 weeks, the interincisal opening was 40 mm. Pain while chewing had reduced significantly, and clicking of the right TMJ was absent. On examination of muscles and TMJ, the pain had subsided []. The patient was then sent for orthodontic correction of her existing malocclusion. |
The second case of single intracranial AVM with concurrent spinal AV fistula involves a 10-year-old girl who presented to us with headaches and progressively increasing weakness of all four limbs for 2½ years. On examination, she had decreased bulk of muscles and increased tone in both upper and lower limbs. She also had brisk reflexes, upgoing planters, and clonus. She was quadriparetic with power of 3/5 in the right upper and lower limbs and 4/5 in the left half of body. She was otherwise conscious and well oriented to time, place, and person, and her sensations were intact. Her past history was unremarkable and there was no significant family history of any hereditary vascular disorder or AVMs. Her brain MRI and cerebral angiography revealed left parasagittal AVM of medium size in the parietal region with superficial venous drainage [Figure and ]. Craniotomy and excision was done in another institute. Postoperatively, she was doing well with no residual AVM on her cerebral angiography [].\nAfter her cranial surgery, her symptoms recurred and she showed progressive weakness and signs of myelopathy in all four of her limbs. Angiography was repeated, which revealed an AV fistula in the cervical region []. Multiple feeders were observed that included bilateral vertebral arteries, bilateral posterior inferior cerebellar artery (PICA), and left costocervical artery from subclavian artery. Some tortuosity was observed in the previously treated AVM in the left parietal region, but no arteriovenous (AV) shunting was seen. Few tortuous abnormal vascular channels were seen in venous phase. MRI also showed a cervical aneurysm [Figure and ]. There was no hemorrhage from the lesion and her symptoms were probably because of ischemia secondary to steal phenomenon.\nAngiographic coiling of aneurysm was done and feeders from vertebral arteries and PICA were embolized. Tiny feeder from left costocervical artery was embolized with histoacryl glue. No residual AV fistula was seen in post-procedural angiography [Figure and ] and no postoperative complications occurred; patient was stable and got discharged.\nOn follow-up, the patient was doing well and the treatment of AV fistula seemed to be adequate. However, after 1 year and 4 months, the patient showed signs of mild weakness in left-sided upper and lower limbs. She was able to carry out most of the tasks independently; however, she limped while walking and had weakness of left hand and fingers. The patient refused further evaluation and management. |
Our patient is a 69-year-old Caucasian female who underwent an elective left total knee replacement. There were no intraoperative or postoperative complications with documented stability in her routine laboratory work (Table ). On postoperative day (POD) 2, the patient was discharged on Eliquis 2.5 mg BID for deep vein thrombosis (DVT) prophylaxis. On postoperative day 10, the patient presented to the ED for right flank pain and emesis. She was readmitted to the hospital and diagnosed with a postoperative ileus and urinary tract infection. At the time of the second admission, our patient had developed thrombocytopenia and an elevated D-dimer (Table ). No workup for her thrombocytopenia was performed at this second hospitalization. During the second hospitalization, she received enoxaparin for routine DVT prophylaxis. She spent four days in the hospital for supportive care and IV antibiotics. At discharge, all anticoagulants were discontinued.\nAfter discharge, the patient had a waxing and waning mental state, progressive weakness, and headache. On POD 16, she presented to the hospital with sharp right-sided chest pain. Her physical examination showed a focal neurological weakness of the left face, arm, and leg. Laboratory work showed thrombocytopenia (Table ). Her initial imaging was significant for multiorgan thrombosis and hemorrhage (Figure ). CT head showed a right cerebellar hemorrhage (Figure ). MRV brain showed multiple venous sinus thromboses in the right sigmoid sinus, right transverse venous sinus, posterior superior sagittal sinus, and straight sinus (Figure ). Venous dopplers showed a left posterior tibial venous thrombus (Figure ). MRI brain showed a right thalamic hemorrhage and an acute infarct of the right cerebral peduncle (Figure , ). CTA chest showed left pulmonary artery embolism and bilateral segmental/subsegmental pulmonary emboli (Figure , ). CT abdomen showed a subacute right adrenal hematoma (Figure ).\nThe patient was admitted to the ICU and initially started on a heparin infusion for acute thrombosis, which resulted in further worsening of her thrombocytopenia. Laboratory workup results for thrombocytopenia were obtained (Table ). Heparin infusion was subsequently discontinued. A serotonin release assay and heparin/PF4 complex were positive, confirming the diagnosis of HIT (Table ). Our patient was switched to argatroban but continued to have persistent thrombocytopenia. She received three units of platelet transfusions during this hospitalization. Blood cultures were drawn and remained negative; no antibiotics were administered. After five days of stability on argatroban, the patient was transferred from the ICU and continued to improve cognitively and functionally. Her thrombocytopenia stabilized with a platelet count of 88-89 × 103/mcL. She was transitioned from an argatroban infusion to warfarin with INR at goal before she was ultimately discharged to an inpatient rehabilitation facility. |
A 24-year-old Japanese woman presented to our hospital with complaints of recurrent fever and subcutaneous abscess on her left upper limb. She had been previously healthy and worked as a nurse in a general hospital. She had a history of multiple subcutaneous abscesses in a year, all occurring after surgeries of the shoulder and forearms performed for bone fracture and impingement syndrome. For 3 months before admission she had repeated episodes of subcutaneous abscess on her left forearm, which was the site of blood collection and arterial line puncture. Cefazolin, cefotiam, piperacillin, sulbactam/ampicillin, clindamycin, ceftazidime, gentamicin, and meropenem were sequentially administered, but the problem was not resolved. The cellulitis worsened in spite of these antibiotic treatments, and finally she developed bacteremia. Incision and drainage of her left upper limb was conducted 1 week before admission, and she was referred and admitted to our hospital for further investigation and treatment.\nShe did not have underlying diseases or any family members with psychiatric disorders, autoimmune diseases, or malignancies. Her social history revealed no trouble with her surroundings, including her workplace. Her physical characteristics on admission were as follows: height, 155 cm; weight, 45 kg; body temperature, 37.4 °C; blood pressure, 122/76 mmHg; heart rate, 70 beats/minute and regular; and respiratory rate, 15/minute. A physical examination on admission did not reveal any focus of infection other than her left forearm. The skin of her left upper extremity had been incised at two points. Redness, swelling, and induration of the skin by cellulitis were seen around the incision (Fig. ). Laboratory data suggested systemic inflammation and mild liver dysfunction (Table ).\nOn admission, we considered the possibility of immunodeficiency, but there were no abnormalities of her immune system, such as changes in the number of lymphocytes and neutrophils, neutrophil phagocytosis disinfection capacity, or natural killer (NK) cell activity. She was also negative for human immunodeficiency virus (HIV) infection (Table ). Contrast-enhancedcomputed tomography (CT) of her whole body, transesophageal echocardiography, magnetic resonance imaging of her heart and spine, and bone marrow puncture all failed to detect any focus of infection except her left upper limb. Microbiological examinations, including a culture for anaerobic bacteria using an anaerobic porter, detected Streptococcus mitis, β-Streptococcus, genus Mobiluncus, and Prevotella buccae from the wound, but no microorganisms were detected in her blood. At first, she was treated with meropenem (2 g/day) and linezolid (1200 mg/day) intravenously, and the cellulitis was promptly resolved. However, unexpectedly, she suddenly developed high fever and complained of severe pain at the site of the cellulitis. We made an emergency incision of her left upper limb for diagnosis of compartment syndrome and observed a jet of pus out of the supinator muscle (Fig. ). As a result, the antibiotic was switched to garenoxacin (400 mg/day). Stenotrophomonas maltophilia was detected in blood and pus cultures, so sulfamethoxazole-trimethoprim was added. However, an allergic reaction to sulfamethoxazole-trimethoprim, such as acute fever and extensive skin eruption, occurred, so we switched to minocycline instead. Hyperbaric oxygen therapy was also performed nine times against the refractory soft tissue infection to improve neutrophil functions []. At the end, these multidisciplinary treatments allowed her to consider discharge, however, high fever and subcutaneous abscess of the same limb suddenly developed again.\nAs a result of this puzzling clinical course, especially the fact that multiple species, including oral indigenous bacteria with a polymicrobial pattern, were detected in cultures of blood and the wound abscess (Fig. ), we finally suspected the possibility of self-injury. A psychiatrist was consulted and gave sufficient explanation to our patient and her family to persuade them to cooperate in diagnosing and treating her. Her sister found three syringes with needles in her bag, and one of these syringes contained a turbid liquid (Fig. a). Enterobacter cloacae and Enterococcus faecalis were detected in the liquid, with identical susceptibility to that of the bacteria detected in pus from the muscle. An analysis by repetitive element sequence-based polymerase chain reaction (PCR) determined that Enterococcus faecalis from the wound and the syringe contents were genetically identical (Fig. b). Finally, a diagnosis of Munchausen syndrome was confirmed.\nNo further episodes of fever and cellulitis occurred after the start of monitoring by camera and restraint of both upper and lower limbs but, 3 weeks later, our patient suddenly left our hospital and attempted suicide. An order was obtained for compulsory hospitalization for medical care and protection, and she was admitted to the psychiatric department. Two weeks later she confessed that she had injected her saliva or toilet water into a drip bag and into her wound with a syringe. She expressed feelings of extreme loneliness when deprived of care by medical staff. Her mental condition has gradually improved. She was discharged after 2 months of treatment in the psychiatric ward and was followed up in out-patients for a few years. |
An apparently healthy 23-year-old female patient reported with the chief complaint of pain in upper incisor teeth region since 1 year. There was occasional bleeding from the affected teeth while brushing. Examination of the maxillary incisors revealed the presence of palatogingival groove on distopalatal aspect of lateral incisor on the right side []. Gutta percha (GP) point was used to delineate the course of groove on the tooth and radiographic examination after placement of GP point was carried out to delineate course and extent of the groove []. On probing, 10-mm deep pocket was present on the palatal aspect of affected tooth [], with no mobility. The pocket was present only along the groove. Radiographic examination revealed angular bone loss in relation to the distal aspect of the right lateral incisor. Thermal and electrical pulp testing showed normal response. Thus endodontic treatment was not indicated.\nIn the initial phase of the therapy, thorough scaling and root planing were carried out. Evaluation of the patient after 6 weeks showed a reduction in the probing depth to 8 mm. Following local anesthesia, a full-thickness mucoperiosteal flap was reflected []. Flap reflection and debridement allowed the complete visualization of the groove. The groove extended up to 5 mm on the root surface from the cemento-enamel junction []. Root planing was performed and odontoplasty was carried out with a high-speed diamond bur to eliminate the PRG completely. No pulpal exposure was observed. The exposed dentinal surface was coated with citric acid (pH 1) for 3 minutes and then gently irrigated with normal saline. Also a deep intrabony defect was observed on the distopalatal aspect of the right lateral incisor. The bony defect was filled with a hydroxyapatite graft material, followed by placement of a resorbable membrane [Figures and ]. Flaps were approximated with the interrupted sutures of 3-0 black silk (Ethiprime, Mersilk, Ethicon, Johnson and Johnson ltd., Somerville, NJ, USA) and periodontal dressing (Coe-pak, GC America Inc., Chicago, IL, USA) was placed. Sutures were removed 10 days postoperatively. The postsurgical healing period was uneventful. At 1-year recall appointment, reduction in probing depth to 3 mm was noted in the treated area []. Radiograph showed improvement in the bony defect and a bone fill of approximately 30 - 40% []. |
A 35-year-old man presented at a local hospital with epigastric pain. Esophagogastroduodenoscopy (EGD) showed that an IVC filter strut had penetrated the third portion of the duodenum (arrow, Fig. ), and this was confirmed by computed tomography (CT) (arrow, Fig. ). In order to retrieve the IVC filter, the patient was referred to our department. He had a history of testicular cancer with para-aorta lymph node metastasis. Left renal vein thrombosis developed because of neoadjuvant chemotherapy before RPLND, and anticoagulants were administered before RPLND. Three years previously, he had undergone left orchiectomy, retro-mediastinal lymph node dissection, and RPLND at the previous hospital. The left common iliac vein was intraoperatively damaged during RPLND. Because the previous surgeon was worried about the high incidence of postoperative DVT and PTE, anticoagulant therapy was continued after RPLND. However, because DVT developed in the left common iliac vein after the initial surgery, a retrievable IVC filter (ALN, France) was placed in the IVC caudal to the renal vein to prevent PTE, and the patient had been receiving anticoagulant therapy. Because follow-up CT after IVC filter placement showed that DVT persisted at the left common iliac vein despite anticoagulant therapy, the IVC filter could not be retrieved at the previous hospital.\nEnhanced CT also revealed that DVT remained in the left common iliac vein. Because ultrasound examination showed organized DVT, an IVC filter was considered unnecessary. An endovascular approach was considered unfeasible for retrieval because two of the filter struts had penetrated the duodenal wall. An extensive discussion with an internal medicine specialist was performed. Because DVT remained with no remarkable changes in CT images for 3 years and the incidence of PTE caused by DVT in the left common iliac vein would be low, we chose surgical treatment for this patient in order to prevent bleeding at the duodenum.\nThe IVC filter was retrieved through cavotomy, and the duodenal penetration site was repaired using intraoperative EGD clipping. The operation lasted 5 h and 54 min, and the intraoperative bleeding volume was 1172 mL. Because it was not possible to mobilize the duodenum due to adhesions resulting from the previous surgery, the IVC at the sites caudal to the renal vein could not be explored. However, a mesenteric incision caudal to the third portion of the duodenum enabled encircling and taping of the IVC (Fig. ). After clamping the IVC cranial and caudal to the duodenum, a 5-cm vertical incision was made on the IVC cranial to the duodenum and the IVC filter was retrieved (Fig. a). Although the head of the IVC filter had penetrated into the IVC intima, we were able to bluntly peel the filter head from the intima. The IVC incision was closed using a continuous 5-0 Prolene suture (arrow, Fig. b). The IVC clamping time was 22 min. Intraoperative EGD revealed no bleeding at the duodenal penetration site (Fig. ). To prevent duodenal bleeding or perforation, the penetration site was repaired by EGD clipping. Retrieved IVC filter was presented as (Fig. ). The patient did not develop any postoperative complications and was discharged on postoperative day 16. |
A 13-year-old male patient presented to the Department of Pediatric Dentistry, Maulana Azad Institute of Dental Sciences, New Delhi, with a chief complaint of pain in left lower back tooth since past few days. Familial, medical and dental history was noncontributory. Extraoral examination did not reveal any abnormality. On intraoral examination, a carious lesion was present in relation to left mandibular first permanent molar; however, there was no tenderness on vertical percussion (). A well-aligned full complement of teeth up to second molar was present in all quadrants with fair oral hygiene. A routine intraoral periapical radiograph of the tooth was advised to assess the extent of carious involvement. The radiograph () revealed the carious lesion involving dentin, but not approximating the pulp, with an associated finding of a calcified structure resembling the crown of a developing premolar close to the root of left mandibular first premolar. The calcified structure was surrounded by a radiolucent halo of the dental sac. A follow-up orthopantomograph () was then advised to rule out presence of other supernumerary teeth elsewhere in the jaws. A distinct, yet similar appearing calcified structure was observed lying in its respective dental sac in relation to the root of right mandibular first premolar. The patient, however, was unaware of this and was completely asymptomatic. Hence, the left mandibular first molar was restored, while no invasive procedure was considered for the supernumerary teeth. The patient and his mother were educated about the radiographic finding and adequately counseled. The patient also had ankyloglossia though there was no restriction of tongue movements or difficulty in speech. When explained about the treatment for it, the patient’s mother was reluctant to get any surgical intervention done since she had a similar problem () without any complaint. Hence, a decision was made to keep the patient on regular follow-up. After 6 months, another orthopantomograph () was taken and compared with the earlier one. There was no evidence of any radiolucency or increase in size of the follicular space of the impacted teeth. The patient is still on 6-monthly follow-up. |
A 64-year-old male patient with no medical or coronary heart disease history referred to a public hospital for acute abdominal pain. A specialist in the emergency department clinically examined the patient's abdominal pain, weakness, and numbness. The patient had abdominal pain, weakness, numbness, sweating, dryness, and burning mouth for 1 hour. The abdominal pain was localized to the epigastric region spread to the back and got worse with 7/10 intensity after lying down. The patient reported no change in his bowel habits, no nausea and vomiting, and no history of trauma to his chest or abdomen. He also had no history of substance or alcohol use.\nAbdominal pain and its symptoms appeared after drinking medicinal herbs. The abdomen was soft without tenderness and rebound tenderness. Gastrointestinal consultation showed no abnormality. The patient had a blood pressure of 80/50 and a heart rate of 115 beats per minute (bpm). The lung and heart sounds were normal and the pulse of the distal organs was full and symmetrical. The patient was also evaluated for myocardial infarction (MI), and the troponin test was negative. An irregular heartbeat was observed in the patient's ECG and cardiac monitoring (). Ventricular arrhythmia (an automatic focus, monomorphic, variable cycle length, and nonsustained VT) with a stable hemodynamic state was diagnosed. Oxygen therapy and antiarrhythmic treatment were prescribed in the emergency department. 150 mg amiodarone was used to control the patient's arrhythmia. The patient was referred to the Coronary Care Unit(CCU) for further evaluation. He was hospitalized in the CCU for 48 hours, evaluated for any arrhythmia, and received cardiac medication. One mg/min amiodarone was used to control the patient's arrhythmia for 6 hours, and then, 0.5 mg/min amiodarone was used for 18 hours. The probabilities of cardiomyopathy as well as toxicity were rejected. Finally, the patient was discharged after 72 hours after taking the necessary examinations. |
A 20-year-old man presented to the nephrology department with acute pain of both flanks which started after a physical exercise at school. His past medical history was unremarkable. He was not taking any medications or illicit drugs, and he denied smoking and consuming alcohol.\nThere was no abnormal finding in his physical examination. Vital signs were normal. Laboratory tests on admission showed acute kidney injury with a creatine kinase level of only twice the upper limit of the normal (). Urinalysis was bland, and the proteinuria was within the range (). Contrast-enhanced computed tomography showed decreased enhancement of the kidneys without any abnormality of the vessels and showed no urolithiasis. After conservative therapy including intravenous fluid administration, the renal function recovered gradually and returned to the normal at the fourth day after admission.\nThe patient reported that he was admitted 4 months ago in the emergency room of another hospital for a similar pain which occurred also after a strenuous exercise. Although laboratory tests showed acute kidney injury, he was discharged without any further investigation.\nLaboratory tests were marked by severe hypouricemia. The elevated fractional excretion of urate (119%; N < 10%) suggested a highly increased urinary excretion of uric acid with tubular secretion. Blood and urinary tests showed no argument for Fanconi syndrome or syndrome of inappropriate antidiuretic hormone. Interesting enough, severe hypouricemia was also found in the laboratory tests of his mother and one of his maternal aunts. The occurrence of two episodes of acute kidney injury following intense exercise with the presence of hereditary severe hypouricemia highly suggested exercise-induced acute kidney injury due to familial renal hypouricemia.\nThis index patient (V6) is a member of a highly consanguineous Pakistani family with cousin marriages over two successive generations (). DNA sequencing by next-generation sequencing identified an homozygosity for a class 5 variant of the SLC2A9 gene (NM_020041.3):c.646G > A, p.(Gly216Arg) confirmed by the Sanger technique []. Genetic testing of the patient's mother revealed the same homozygous mutation. Severe hypouricemia with the familial homozygous mutation was found in two of his clinically unaffected sisters (V7 and V9). The third sister (V8) is heterozygous for the mutation and is presenting with normal uric acid level. Profound hypouricemia was also measured in one of his aunts (IV2) and one of his cousins (V12). All of these analyses confirmed an autosomal recessive mode of inheritance. Besides the index patient, no medical history was known in all of the family members except his grandfather (III5) who suffered from kidney stones. |
A 52-year-old male patient was admitted to our interventional radiology unit with pain, swelling and color change in the left inguinal region following therapeutic angiography. Laboratory tests showed progressive reduction in hemoglobin levels. In the diagnostic angiography that was performed two days earlier, partial occlusion in the left anterior descending artery was observed and 1 day before the patient was referred to us, he had undergone percutaneous transluminal coronary angioplasty with stent placement. The patient was under thrombolytic-anticoagulant therapy at the time of angioplasty. He had been smoking one-pack-cigarette/day for the last 40 years and he had high blood pressure. No additional important information was detected in his medical history.\nIn the gray scale ultrasonographic examination, a 43×26 mm pulsatile cystic lesion was observed in the left groin. Doppler ultrasound (US) examination showed “to and fro blood flow” inside the lesion. Based on these findings, the patient was diagnosed as IPA, but IPA and the morphology of its neck could not be demonstrated clearly with US. To detect the morphology, the origin, and the neck of IPA, multidetector computed tomographic angiography (MDCTA) was recommended. US-guided compression was done for 20 minutes, as well. Following compression treatment, a thrombus that formed one third of the size of the IPA was detected. The patient was recommended to sleep and bed rest in the supine position, and pressure was applied on his left groin and he was observed for one day.\nIn 64-MDCTA (Aquilion, Toshiba Medical Systems, Tokyo, Japan) and control Doppler US examination, IPA was originated from SEPA and the common femoral artery was normal (). In addition, an inguinal hernia containing small intestinal loops was detected in the right inguinal region (). Iatrogenic pseudoaneurysm was 36×23 mm in size and there was a 10 mm thrombus inside it. US-guided compression treatment was applied for the second time for the patient. In US Doppler examination, IPA was completely thrombosed following 20 minutes of compression therapy. There was no recanalization observed in the control Doppler US that was performed 2 days after the first compression procedure and the patient’s complaints regressed (). In the control Doppler US performed 3 months later, the sac of IPA was significantly regressed and the patient recovered completely without any complication. |
This report presents a case of a 71-year-old Caucasian male admitted to the ICU with AMS, lower extremity weakness, and worsening diarrhea. The patient had aggressive, castration-resistant prostate cancer with a Gleason score of 4 + 4 and known metastases to the bones, bone marrow, and bladder. The patient was being treated with leuprolide and denosumab at the time of admission, and his previous treatment regimen included leuprolide, bicalutamide, abiraterone, and prednisone. During the patient's hospital course, consent for discussion and report of findings related to his care was obtained from family members.\nThe patient suffered from diarrhea for two weeks prior which resolved after seven days of metronidazole; however, his diarrheal condition resurfaced and worsened when he was given additional antibiotics for suspected UTI. The family reported that in the week prior to admission the patient's mental status had slowly worsened, his lower extremities became increasingly weak which rendered him unable to walk independently, and his overall functional status deteriorated. The family reported that these represented significant decline when compared to his previous condition. Of note, at time of presentation, his measured serum PSA was 5.82 ng/mL.\nIn order to further evaluate his AMS, a CT scan of the head without contrast and a follow-up MRI scan were ordered. Although the CT scan was negative, the follow-up MRI of the brain revealed a diffuse enhancement of the meninges which was highly suggestive of LC (Fig. , ). MRI of the cervical and lumbosacral spine showed evidence of diffuse osseous metastatic disease without a bulky focus of meningeal disease.\nThe CSF cytology obtained through a lumbar puncture showed no malignant cells and further studies of the CSF revealed glucose < 1 mg/dL, protein 8 mg/dL, 0 white blood cells/mm3, 1 red blood cell/mm3, PSA 0.64 ng/mL, negative ACE, negative VDRL, and negative PCR testing and cultures for routine bacterial, viral, and fungal pathogens.\nThe patient was diagnosed with LC from malignant prostate cancer based on the known history of the aggressive disease, clinical presentation, and highly suggestive MRI findings despite CSF studies which were largely unrevealing. The results, further diagnostic options including repeat CSF studies, and prognosis were discussed with his family as the patient's mental status continued to decline. Although further diagnostic and subsequent treatment options were offered, the family collectively elected to withdraw care. |
A 72-year-old female patient was referred to our department due to a mass which was incidentally found in the RV during workup for occasional palpitations. The 24-h tape revealed frequent episodes of ventricular tachycardia which were later attributed to the tumor. The patient was on medication for hyperlipidemia and hypertension, and her past medical history was free. Her physical examination, vital signs, and laboratory profile were normal, and the patient had a normal sinus rhythm. The surface echocardiogram was suggestive of a good LV function, while a homogenous and mobile RV mass was identified to extend from the IVS to the right ventricular outflow tract (RVOT). The patient underwent magnetic resonance imaging (MRI) which identified the mass as a cardiac lipoma [Figure –]. The patient also had a coronary angiogram which was free of coronary artery disease. The case was planned on an elective basis. Once the patient was intubated, a transesophageal echocardiography was done []. After median sternotomy and establishment of cardiopulmonary bypass, the tumor was approached through a 4-cm transverse incision on the anterior wall of the RVOT. The mass was lobulated and yellowish. It had a broad base attached to the ventricular septum, occupying the right ventricular cavity and extending to the RVOT without interfering with the tricuspid valve apparatus []. The mass dimensions were 3.5 cm × 2 cm × 1 cm []. The tumor was not strongly adhered to the IVS, and it was easily resected in one piece leaving no residual mass behind. The remaining defect on the RV was closed using a Prolene 4–0 suture. The patient was easily weaned from cardiopulmonary bypass and had an uneventful in-hospital recovery. She was discharged on the 5th postoperative day. Pathology report confirmed the initial diagnosis of a benign, cardiac lipoma [Figure and ]. On a 3-month follow-up, she remains asymptomatic with no recurrent arrhythmias. |
A 68-year-old male diabetic presented with corneal scarring due to recurrent corneal microbial and herpetic infection with upper lid entropion secondary to stage IV trachoma in left eye (OS). The patient was blind in the right eye since childhood. The patient had undergone extracapsular cataract extraction in the left eye for an immature cataract, many years prior to presentation. One year postoperatively, the patient developed two episodes of recurrent herpetic keratitis and was managed successfully with topical acyclovir ointment and was instructed to continue a maintenance dose of prophylactic oral acyclovir 500 mg once daily.\nConcurrently, the patient developed glaucoma which was controlled with topical antiglaucoma medications. Ten years later the patient underwent penetrating keratoplasty (PKP) secondary to visually significant corneal scaring which failed due to recurrent herpetic keratitis. Once the eye was free of infection for 1 year, PKP combined with trabeculectomy was successfully performed. Three years after combined PKP and trabeculectomy, the graft failed and recurrent herpetic keratitis was successfully treated. The vision was count fingers at one foot with dense corneal scar and deep vascularization. The patient was instructed to start a maintenance dose of prophylactic valacyclovir 500 mg once daily. The eye remained quiet and he discontinued valacyclovir for 4 years.\nIn April 2009, patient underwent an uneventful Boston Type I keratoprosthesis procedure. His best-corrected visual acuity improved to 20/80 and he was maintained on a megasoft bandage contact lens with topical moxifloxacin four times a day, topical fortified vancomycin 25 mg/ml four times a day, and topical antiglaucoma [].\nThree weeks after the keratoprosthesis procedure, patient presented to the emergency room complaining of painless and gradual decrease in vision over 3 days. The presenting vision was hand motion. Anterior segment examination revealed clear graft and the keratoprosthesis was in place. Fundus examination showed 3+ vitritis and large, demarcated areas of hemorrhagic necrotizing retinitis involving all quadrants, encroaching the optic disc and the macula []. Fluorescein angiography demonstrated a typical peripheral vascular leakage related to vasculitis with evidence of ischemic optic neuritis.\nThe patient was diagnosed with ARN secondary to herpetic infection. Serology testing indicated high titers of herpes simplex virus type 2 which confirmed the diagnosis. The patient was immediately admitted and started on intravenous acyclovir 10 mg/kg every 8 hours with topical prednisolone acetate 1% four times a day with close monitoring of his renal functions. Forty eight hours after admission the patient was also placed on oral prednisolone 100 mg (1 mg/kg) once daily. After 11 days, intravenous acyclovir was stopped in favor of oral valacyclovir 1 g every 8 hours due to mild to moderate changes in the renal function tests. During this time, there was marked improvement in visual acuity from hand motion to 20/200 and regression in the size of retinitis []. Prophylactic laser retinopexy was performed [].\nFour weeks later, there was a marked reduction in vision to light perception, with a soft eye and choroidal detachment without RD on B-scan ultrasound. |
A 19-year-old male patient complained of shoulder pain with no trauma history. He reported that the pain started about 6 months before, with progressive worsening. Pain was present in elevation and abduction, especially above 90°. The pain caused him to interrupt his physical activities, although he did not notice worsening during bodybuilding.\nA winged scapula was identified in physical exam, with scapulothoracic grade III dyskinesia, according to Kibler et al. []. He presented infraspinatus atrophy, complete range of motion, preserved upper limb strength, and discrete paraesthesia at the region of the medial border of the right scapula, with no other signs. There were no clinical signs suggestive of rotator cuff injury or glenohumeral instability ().\nThe imaging exams did not show significant changes. Shoulder MRI showed no rotator cuff lesion, labral lesions, cysts, or other soft tissue involvement. The cervical spine MRI did not show cervical discopathy or syringomyelia. Electroneuromyography with evoked potential of the scapular girdle evidenced diffuse axonal involvement of the long thoracic nerve, without other alterations.\nThe patient was then referred to the physiotherapy service, where he initiated a program of shoulder girdle rehabilitation focused on analgesia and passive mobilization. During ten weeks, he remained under the care of physiotherapists twice a week, but he did not notice an improvement in the pain. When he returned to the orthopedic clinic, he was informed about the possibility of surgical treatment.\nThe patient insisted on conservative treatment. We recommended strengthening of the shoulder girdle and swimming. During three months, he practiced swimming three times a week under the guidance of a physical education professional with experience in athlete training. In order to strengthen the periscapular muscles, he tried to practice the four classic styles of swimming, using floats in the lower limbs and increasing the demand on the upper limbs. Progressively, he noticed an improvement in his pain.\nThe swimming program consists of a 60-minute pool training three times a week, with increasing distances. The front crawl, breaststroke, and backstroke were alternated during training. The main set was a target mile, split in 200 m lengths with 1-minute rest (in a 25 m pool) and alternating strokes.\nHe returned after 90 days, free of pain. He presented dynamic stabilization of the scapula during elevation, and dyskinesia was no longer perceived. The force remained unchanged, but atrophy was no longer identified. The patient was satisfied with the progress made and was encouraged to stay in the muscle strengthening program in aquatic activities (). |
The patient was an 87-year-old healthy female with only a medical history of hypertension. One month earlier, she experienced blunt chest trauma. During this period, general malaise and intermittent fever occurred.\nInitially, the patient was admitted to another emergent hospital because of excruciating chest pain and severe dyspnea. Emergent computed tomographic (CT) scan demonstrated a transverse mid-body fracture of the sternum with surrounding fluid collection. The patient was transferred to our institution with a diagnosis of traumatic sternal fracture with mediastinal abscess.\nOn admission to our institution, the patient had high grade fever. Initial laboratory data demonstrated a white blood cell (WBC) count of 25,100/uL and C-reactive protein (CRP) of 340 mg/L. Blood culture was positive for Gram-positive cocci in clusters and vancomycin was immediately started. A few days after admission, her inotropic requirement increased progressively as her hemodynamic status deteriorated. She was endotracheally intubated and follow up images showed progressive changes. The abscess could be seen extending to both the anterior and posterior aspects of the fractured sternum and to the left pectoralis muscles and subcutaneous tissues (Fig. ). Moreover, transesophageal echocardiography was done because of her bacteremia and it showed a large vegetation in the mitral valve with moderate insufficiency (Fig. ). We believed that definitive surgical treatment involving aggressive debridement, probably followed by vegetectomy, is the only effective method of saving our seriously co-infected patient with extensive mediastinitis and concomitant mitral valve endocarditis with large vegetation.\nThe patient was taken to the operating theater the next day. We performed a median incision and drained the mediastinal abscess, debrided her fractured sternum, including the cartilaginous portions of the transverse second and third ribs, the left internal mammary artery and the left pectoralis muscles with necrotic subcutaneous tissues. The manubrium of the sternum was spared from debridement since there was no gross evidence of infection, leaving a 12-cm defect in the mid-portion of the sternal body. After the debridement, vacuum-assisted closure (VAC) therapy with continuous saline washing was performed. The first wound culture demonstrated the presence of Methicillin-sensitive S. aureus (MSSA), and the antibiogram was identical to that of the MSSA isolate from the blood. Intravenous vancomycin was changed to intravenous cefazolin.\nOver the next week, she underwent two wound irrigation procedures. Although subsequent cultures at dressing changes were all negative in this period, she had continuously high grade fever and her laboratory data still demonstrated high. After extensive discussion of the benefits and risks of the surgical intervention because of the significant co-morbidities, we decided to perform the vegetectomy and mitral valve repair. The cardiopulmonary bypass was established through lower partial sternotomy. Surgery revealed perforation with a large vegetation of the posterior mitral leaflet, which was completely resected and successfully repaired with an autologous pericardial patch (Fig. ). The aortic crossclamp time was 98 min and cardiopulmonary bypass time was 132 min. After 4 days, the lower sternum was apposed using sternal wires and the chest wound was completely closed with an omental flap (Fig. ).\nThere were no pulmonary complications and the patient received intravenous cefazolin for 8 weeks. At the 8th month follow-up period, the patient showed no signs of infection and was doing well without any significant mitral insufficiency.\nThere are some reports of sternal fractures with osteomyelitis as a complication of closed chest cardiopulmonary resuscitation [], and are not uncommon in the setting of antecedent chest wall trauma, such as thoracic surgery [, ]. However, a mediastinal abscess because of a primary traumatic sternal fracture is uncommon [, ]. Cuschieri et al. identified the presence of hematoma, intravenous drug use, and a source of staphylococcal infection as risk factors for developing a posttraumatic mediastinal abscess []. Of note, the majority of these patients have an underlying systemic disease such as diabetes mellitus, liver dysfunction, or compromised host []. In our case, the patient had no remarkable co-morbidity, experienced only a traumatic episode 1 month earlier and she was accompanied by the native valve endocarditis. To the best of our knowledge, this catastrophic situation is extremely rare and has not been previously reported, nor has a successful strategy been employed to manage this situation; hence, we described this case.\nOnce diagnosed this serious co-infected case, the primary concern of any surgical procedure is to control the bacteria infection []. Treatment of mediastinitis may depend on the degree severity of the infectious; conservative therapy for mild infections, or single staged intervention such as debridement and primary wound closure for moderate infections, or leaving the wound open and second-stage closure using the several closure techniques available for severe cases [, ]. Urbanski et al. reported a case of chronic sternal osteomyelitis after radiation and severe aortic valve stenosis []. The patient was treated simultaneously by sternal debridement and concomitant aortic valve replacement, and single-stage primary wound closure. This was a case of chronic non-severe mediastinitis; therefore, the single staged intervention could be performed. In our case, the mediastinal abscess was already extensive and progressed in a few days; furthermore, the patient had concomitant endocarditis with uncontrolled infection and considerable risks of embolism from the lerge vegetation. Thus, we decided on a staged strategy: first, open drainage for mediastinitis; second, cardiac operation for endocarditis; and finally, primary wound closure with omental flap.\nIt remains unclear when the endocarditis with the vegetation should be treated in this co-infected situation. For example, Kang et al. demonstrated that early surgical intervention in patients with large vegetation (a diameter greater than 10 mm) may significantly decrease embolic events and in-hospital mortality []. Moreover, the risk of embolism has been reported to be particularly high during the first week after diagnosis. However, cardiac operations performed too early had the disadvantage of increasing the risk for recurrent mediastinitis. In our patient, although she had VAC therapy for about 2 weeks and underwent four procedures from initial debridement to cardiac operation, the infection was not controllable because of the large vegetation. We thought this timing was best to perform the vegetectomy.\nIn treating the mitral valve endocarditis, several surgical approaches may be employed. The right thoracotomy approach could avoid going through the infected field and decrease the recurrence of the bacteria. However, the major disadvantage is the need for an additional right-sided thoracotomy incision, which requires a longer mechanical ventilation period, as is often the case with our older patients after aggressive debridement of the sternum. With partial sternotomy, Kaneda et al. reported the usefulness of a lower partial sternotomy for acutely infected endocarditis in patients with tracheostoma []. When the manubrium of the sternum could be spared from debridement, the lower partial sternotomy approach was associated with benefits, including thoracic stability because of preservation of the sternoclavicular joint and the upper ribs. In our case, we believe that lower partial sternotomy could be performed and sparing the manubrium of sternum led to a favorable result. |
A 43-year-old Caucasian man was admitted to the emergency department of our hospital for headache associated with weakness of the left limbs. He had no medical history, he denied even slight trauma of the neck, and his only cardiovascular risk factor was tobacco smoking for 10 years. Neurological examination revealed verbal aphasia and left hemiparesis.\nDuring CT angiography (TK–64-row MDCT scanner, LightSpeed VCT, GE, Waukesha, WI) of the neck and head, segmental dissections of the right internal carotid artery (ICA) at the level of the cranial basis were discovered (Fig. a). The lumen of the ICA was decreased to 4 × 2.5 mm (60 % of normal flow). Cerebral CT showed a recent infarction involving the medial part of the right temporal lobe and deep structures of the right hemisphere including the thalamus, the genu and the posterior limb of the internal capsule (Fig. b).\nCT angiography of the neck and head also showed a duplication of the left vertebral artery (LVA) with two origins, both from the left subclavian artery (Figs. , ). The duplicated segments were fused at level of the C5–6 vertebrae into a single vertebral artery, which then entered the transverse foramen of C5 (Fig. ). All measurements were taken on an Advantage Workstation (GE).\nThe first LVA originated from the left subclavian artery at a distance of 7 mm from the aortic arch. It measured 2.9 and 2.8 mm in diameter at the points of origin and just before fusion, respectively (Fig. ). The course of the artery was 101 mm. The second LVA arose from the left subclavian artery 37 mm distal to the first (44 mm from the aortic arch). It measured 2.4 and 2.2 mm in diameter at the points of origin and just before fusion, respectively (Fig. ), and its course was 89 mm. Both vertebral arteries possessed a regular lumen. The diameter of the LVA after fusion was 3.3 mm (at level C5–6) (Fig. ).\nThe right VA arose as the first branch from the left subclavian artery, 43 mm from the aortic arch. Its course was normal. The diameters of the right VA were 4 mm at the points of origin and 3.8 mm at the C5–C6 vertebrae (Fig. ). Both thyrocervical trunks were seen to originate separately from the subclavian arteries.\nAdditionally, complementary but independent to CT angiography Doppler Sonography B-flow mode (Vivid 7 Pro, GE) of the vertebral arteries also revealed a duplicated LVA (Fig. ).\nAfter all procedures were completed, Ehlers–Danlos syndrome type IV was diagnosed. |
A 26-year-old man was admitted with a 6-hour history of rapidly developing erythematous, severely painful, mildly to moderately edematous skin rash. The rash started from his right lower back and spread within hours to include the right hip, abdomen, and groin, and down to the proximal third of the anterior lateral aspect of right thigh. At the time of initial infectious diseases consultation, the skin lesion had further spread from the upper third of his right thigh to the upper edge of his right knee over the course of 40 minutes. The patient complained of extreme fatigue; however, he denied any fever, nausea, vomiting, coughing, shortness of breath, dysuria, or sick contacts. He also denied any history of allergy, tobacco use, alcohol consumption, or intravenous drug use. His medical history revealed a history of a mixed connective tissue disease (limited systemic sclerosis overlapping with polymyositis and SLE which had been diagnosed one month previously). He was currently being treated with intravenous immune globulin (IVIG) every three months and prednisone 40 mg daily. He had received a pulse infusion of 1 gram of cyclophosphamide 18 days prior to the current presentation.\nHis initial vital signs were stable, with temperature of 36.2°C, blood pressure of 123/82 mmHg, heart rate of 114/min, respiratory rate of 22/min, and oxygen saturation of 92% on room air. The patient was alert and oriented. Further physical examination revealed marked erythema over his right lower back, right hip, abdomen, and groin area, as well as the anterior lateral aspect of right thigh. The overlying skin was warm, mildly edematous, and extremely tender when palpated. No sign of crepitus was identified. The patient was neurovascularly intact in both lower limbs. Laboratory studies included a white blood cell count of 6.0 × 109/L, absolute neutrophil of 5.8 × 109/L with left shift noted, hemoglobin of 94 gm/L, platelets of 174 × 109/L, sodium of 130 mmol/L, potassium of 4.1 mmol/L, creatinine of 58 μmol/L, lactate of 2.9 mmol/L, creatine kinase of 661 U/L, and C-reactive protein of 99 mg/L. His hepatic profile revealed elevated alanine aminotransferase of 83 U/L and alkaline phosphatase of 321 U/L. Coagulation study showed normal prothrombin time and an elevated partial thromboplastin time of 47 seconds (22–35 seconds).\nThe patient was empirically started on piperacillin-tazobactam 4.5 gm IV every 6 hours and vancomycin 500 mg IV every 12 hours which cover broadly for Gram negative and Gram positive organisms including methicillin-resistant Staphylococcus aureus (MRSA), along with clindamycin 600 mg IV every 8 hours. Clindamycin was given to inhibit the toxin production from possible invasive Streptococcus pyogenes based on available human and animal studies [–]. One dose of IVIG was given since he was due for his routine dose for his rheumatological disorders, and given that IVIG may improve outcomes of necrotizing fasciitis from GAS infection []. Tissue biopsy of fascia from the anterior and lateral side of right thigh was performed by the orthopedic surgery team at the bedside under local anesthesia and was sent for urgent frozen section pathology and microbiology. Both tissue and blood sample cultures grew Streptococcus pneumoniae sensitive to penicillin. Pathological findings with heavy neutrophilic infiltration and abundant necrosis within fat and fascia were consistent with necrotizing fasciitis. Contrast computerized tomography of the abdomen and pelvis was performed after tissue biopsy suggesting postbiopsy changes without obvious infection involving fascia or muscles. Based on the patient's rapidly developing clinical symptoms and evidence of necrotizing fasciitis on tissue biopsy, he was taken to the operating room urgently. During surgery, he was found to have extensive edema of his subcutaneous tissue, but with no obvious fascial or muscle necrosis. Therefore, no debridement was performed. Fascial tissue from the anterior and lateral side of right thigh was randomly sampled intraoperatively and clear evidence of necrotizing fasciitis with significant neutrophilic infiltration and necrosis in fat and fascia was confirmed by pathological results, as shown in . Clindamycin and vancomycin were discontinued on the second day after the microbiology culture results became available. Piperacillin-tazobactam was switched to ceftriaxone which was continued to complete the course of 6 weeks of treatment. The patient required 8 days of postoperative ICU stay which was complicated by methicillin-sensitive Staphylococcus aureus (MSSA) pneumonia, coagulopathy with factor XI deficiency, and a flare-up of his rheumatologic condition. He recovered well with subsequent physiotherapy on the medicine ward and later in the rehabilitation unit. |
A female 75-year-old farmer, while working in her field, accidentally fell onto a piece of wood that pierced through her skull [] and was directly brought to our emergency department about 8 hours following the alleged trauma. She was conscious throughout the incident with a brief history of vomiting but no seizure episode was present. During admission, her Glasgow coma scale (GCS) was 15 with both pupils equal and reactive. A large open wound at her left frontal region size 5×4 cm with a penetrating wood was visible.\nA CT scan of her brain was performed emergently. The bone window CT demonstrated bone fragments penetrating her brain with underlying hematoma [Figure and ]. Upon completion of all the necessary diagnostic studies, she was taken promptly to the operating room.\nPatient was positioned supine with her head slightly turned to the right. The same wound was used as one of the margin of a ‘S’ shape incision and scalp flap was retracted. On raising the flap, a large wooden object size 4×3 cm was found piercing the bone and surrounding tissue. A craniotomy was undertaken using four burr holes surrounding the object for better operative exposure []. During careful removal of the object, dural tear size 3×2 cm was found. We carefully performed necrotic tissue debridement and removal of bone fragments piercing the brain matter []. There was significant bleeding during hematoma evacuation which was effectively controlled using gel foam and pressure. Wound was irrigated with saline and gentamicin antibiotic. The dural defect was then covered with autologous graft [].\nThis surgical procedure was considered as a successful surgery. The patient was allowed to be awakened from general anesthesia in the operating room. Post operative recovery was good and patient was discharged on the tenth day after the surgery. During 3-month follow-up, patient is doing well without any neurological deficits. |
A 26-year-old male, diagnosed with schizophrenia six years earlier, was admitted to the Abarbanel Mental Health Center, Israel, during an acute psychotic event. While being admitted, the patient deliberately injured his right eye with a ballpoint pen. A quick response from the medical staff prevented him from self-enucleating and he was transferred to our ER for ophthalmologic evaluation. A bag containing a broken pen with a missing tip accompanied him. The patient was catatonic and noncooperative, so no history could be obtained. After administering Zyprexa 10 mg and Clonex 1 mg, we were able to examine the patient. On examination the best corrected visual acuity (BCVA) in his right eye was 20/150 and intraocular pressure (IOP) was 20 mmHg. The patient had right periorbital edema and ecchymosis, severe conjunctival chemosis, corneal erosions, and a clean anterior chamber. The pupil was dilated with no direct or consensual response to light. The vitreous was clear and fundoscopy showed a completely normal disc and retina. In addition, the patient had positive right relative afferent papillary defect (RAPD) and a complete ophthalmoplegia of the right eye. Examination of the left eye was normal, with visual acuity of 20/20 and IOP of 14 mmHg.\nOn orbital CT there was no sign of perforation. It did show, however, a metallic foreign body in the right orbital apex close to the optic nerve and adjacent to the superior orbital fissure ().\nBecause the patient displayed signs of traumatic orbital apex syndrome, we planned an urgent surgery to remove the foreign body. However, it was clear that the patient, suffering from acute psychosis, is unable to give an informed consent. Therefore, the decision to operate was made and the signatures of three physicians were obtained, as dictated by the Israeli Patient's Rights Law []. Intravenous cefazolin and parenteral cotrimoxazole were administered to protect against aerobic and anaerobic bacteria. A lateral orbitotomy through the superior lid crease incision was performed. A 1.2-centimeter long metallic pen tip was located and retrieved from the apex (). After removing the object, drain was left and the orbit was repaired.\nThe following week, there was no sign of infection and the patient was discharged with BCVA of 20/60, a dilated pupil slightly reactive to light, and complete ophthalmoplegia. Sixty-six days after surgery the visual acuity had improved to 20/20. The patient had slight ptosis, slight restriction in RE adduction with diplopia, and a pupil responsive to accommodation but only slightly responsive to light, with no RAPD (). The patient's eye exam during his follow-up period is described in . |
The patient was a 38-year-old woman of childbearing age with a history of regular menstruation, gravidity 3, and parity 1. She underwent a B-mode ultrasound examination at a local hospital to evaluate the complaint of prolonged menstruation and increased menstrual bleeding of a duration exceeding 2 months. The results of the examination indicated the presence of a lesion in the intrauterine space. The patient underwent diagnostic curettage, and a pathological examination of the tissue specimen indicated the possibility of sarcoma. Accordingly, the pathological specimen was sent to the Department of Pathology of our hospital, and the pathological report indicated the presence of a mixed-type endometrial adenocarcinoma (approximately 80% clear cell carcinoma and 20% poorly differentiated endometrioid adenocarcinoma) accompanied by mucinous adenocarcinoma.\nA pelvic examination revealed uterine enlargement at approximately 50 days after conception. The uterine surface was soft and smooth without tenderness, and no other abnormalities were observed. Tumor marker evaluations revealed a high level of carbohydrate antigen (CA) 125 (58.5 U/mL), and normal levels of AFP, carcinoembryonic antigen (CEA), CA199, and human chorionic gonadotropin (HCG). A magnetic resonance imaging examination indicated a slight expansion of the uterine cavity and a patchy area of abnormal signal intensity suggestive of uterine bleeding. The left obturator lymph node, which was approximately 0.8 cm × 0.5 cm in size, was relatively larger than the right node, and no other abnormalities were found. The patient was diagnosed with mixed-type endometrial adenocarcinoma.\nOn December 9, 2016, the patient underwent a laparoscopic extrafascial hysterectomy, bilateral adnexectomy, pelvic lymphadenectomy, abdominal para-aortic lymphadenectomy, omentectomy, and appendectomy. Intraoperatively, the uterus remained enlarged at approximately 50 days after pregnancy, and a 0.3-cm nodule was observed on the left side of the peritoneal reflection of the bladder. No abnormalities were observed in the bilateral ovaries or oviducts, and no enlargement or hardening of the pelvic and abdominal para-aortic lymph nodes was observed. However, scattered hard nodules (size: 0.5–0.8 cm) were observed in the greater omentum. Furthermore, a postoperative uterus evaluation observed hard nodules (size 2.5 cm × 1.2 cm) in the left posterior wall of the uterine cavity. Postoperative pathology revealed a stage IVB endometrial YST with tumor metastasis to the greater omentum.\nPostoperatively, the patient was administered 6 courses of chemotherapy with the bleomycin–etoposide–cisplatin (BEP) regimen. Although she developed adverse reactions, such as grade IV neutropenia and vomiting, during chemotherapy, these symptoms improved after treatment. She was followed up regularly for 24 months after completing chemotherapy, and no tumor recurrence occurred during this period. |
A 40-year-old man with scoliosis and developmental delay presented for outpatient evaluation prior to retinal surgery. His physical exam showed a heart rate of 110 bpm, blood pressure of 160/70 mmHg, respiratory rate of 16/minute, oxygen saturation of 84%, clubbing, elevated jugular venous pressure, and a multifocal V/VI systolic murmur radiating to the back. Laboratory tests were remarkable for hemoglobin of 18 g/dL. An echocardiogram demonstrated biventricular hypertrophy, preserved biventricular systolic function, an overriding aorta (), and a right-to-left interventricular shunt.\nA ventriculogram, right heart catheterization, and oximetry were performed. After initial femoral venous access, there was difficulty passing a J wire and angiography showed an interrupted inferior vena cava (). Therefore, access was changed to the internal jugular vein and right heart catheterization demonstrated severely elevated right atrial and ventricular pressures (). There was an inability to obtain pulmonary artery waveforms, and a right ventriculogram demonstrated pulmonary atresia. A pigtail catheter was introduced via femoral arterial access, and simultaneous right and left ventricular pressures were obtained. Ventriculography confirmed a membranous VSD. Subsequent root and thoracic aortography showed MAPCA from the lower thoracic aorta connecting to the right upper pulmonary artery branch (, ). The main communication to the right lung was cannulated via the MAPCA, and a pulmonary angiogram was performed. There were two areas of stenosis. The pressures in the right upper pulmonary artery branch were measured at 120 mmHg proximal and 18 mmHg distal to the areas of stenosis. Next, the left subclavian artery was engaged, and an angiogram was performed, revealing MAPCA between the left subclavian artery and left and right lower pulmonary artery branches (, ).\nA discussion was held with the patient and his family including surgical options; however, due to the potentially high operative risks and poor baseline functional capacity, they decided to pursue medical management and focus on quality of life. |
An 11-year-old girl presented to department of oral and maxillofacial surgery, Yenepoya dental college and hospital, Deralakatte, Mangalore, with the complaint of swelling on the left side of the face [].\nThe swelling started 6 months back which gradually enlarged to present size. On examination, extra orally a diffuse swelling was seen on left side of face which extended superiorly up to ala tragus line, anteriorly from about 2 cm posterior to commisure of mouth to angle of the mandible. Intra oral examination revealed mixed dentition and swelling extending from left mandibular canine to left mandibular second molar [].\nOn palpation, the swelling was non-tender and hard in consistency. Bi-digital palpation revealed expansion of the buccal plate, no perforation of buccal/lingual cortex and no paresthesia of mucosa or lower lip were seen. Grade III mobility was seen on all the teeth from left mandibular canine to left mandibular second molar, which were non tender on percussion.\nThe intra oral periapical radiograph shows radiolucency near the mandibular teeth [].\nThe CT scan findings revealed the following: an ill-defined expansile cystic lesion was seen in the body of the mandible on left side and also thinning and erosion of buccal cortex was also noticed [].\nThe results of hematological and biochemical investigations were within normal limits. Under local anesthesia, extraction of the mobile teeth left mandibular first molar was performed and the tissue sample obtained through the extraction socket was sent for histopathological examination. [Figures and ].\nOn microscopic examination, H and E stained sections showed sheets of uniform, small, round cells arranged in diffuse pattern, with indistinct outline, scanty cytoplasm, and well-defined nuclear outline, with round to oval nucleus and inconspicuous nucleoli. []. Mitotic figures were rare. A diagnosis of malignant small round cell tumor was made based on the above histopathological findings.\nA panel of immunohistochemical markers namely CD99, CD3, CD20, CHR, MPO, desmin, and SYN were used to rule out other small round cell tumors. Tissue sections showed positive expression for CD99 (MIC2), with characteristic membranous pattern, confirming the diagnosis of Ewing's sarcoma\nBone scan was also advised which revealed reduced uptake over the body of left mandible without rim of enhanced uptake. No evidence of distal bone involvement was seen [].\nA surgical procedure was performed to resect the malignant tissue with generous surgical margins and to reconstruct the resulting defect. The patient underwent a segmental resection of left side including the body of mandible. The bony defect was reconstructed using titanium reconstruction plate. [].\nThe postoperative panoramic radiograph revealed a well-aligned reconstruction with intact plating (6 months post operative orthopantamograph)\nAdjuant chemotherapy comprised 20 weeks (3 cycles) of treatment with a four-drug regimen which were Vincristine [VC], Dactinomycin [AC], Cyclophosphamide [CP] and Doxorubicin [AD] after surgery. |
A 5-year-old girl presented with an acute onset fever, for about 4 days. She was seen initially in her local emergency room and a chest X-ray was done to rule out pneumonia. This showed a marked, diffuse reticulonodular pattern of parenchymal opacity bilaterally (), incongruous with her clinical appearance. In reviewing the history, she was well until about 4 days before her presentation with a dry cough, night sweats, and daily fevers. During this time, she had no other significant respiratory symptoms, including no chest pain, no shortness of breath, no tachypnoea, and no haemoptysis.\nHer medical history included a possible episode of pneumonia at one year of age, diagnosed and treated as an outpatient. A chest X-ray done at this time showed the beginning of a reticulonodular pattern that was considered to be due to infection and not followed up, as she recovered and remained clinically well thereafter. She had no hospital admissions or surgical procedures done. She received all of her immunizations. She had received salbutamol and fluticasone inhalers for an episode of wheezing at 3 years of age but had not used these thereafter. She had no atopic features. She was born premature, at 32 weeks, and required NICU admission for 2 weeks to establish feeding; there were no reported respiratory issues during the admission. She has a twin sister who has had no medical concerns. The family history was noncontributory but the parents were of Middle-Eastern descent and consanguineous, being first cousins. The patient had not travelled outside of the country and there was no obvious tuberculosis contact.\nShe had normal vital signs and normal oxygen saturations in room air (heart rate 100/min, respiratory rate 22/min, saturation 97%, temperature 36.1 degrees Celsius, and blood pressure 90/51 mmHg). Her height and weight were on the 3rd percentile. She had mildly increased work of breathing but was otherwise comfortable at rest. Her chest had bibasal inspiratory crepitations, bronchial breath sounds in the midzones bilaterally, and clear upper zones. Her cardiovascular, gastrointestinal, and head/neck examination was normal.\nHer nasopharyngeal swab was found to be positive for influenza A. Her complete blood count was unremarkable with a white cell count of 7.2 × 109/L, haemoglobin of 123 g/L, and platelets of 223 × 109/L. Her inflammatory markers were done, with a C-reactive protein of 15.9 mg/L and an erythrocyte sedimentation rate of 41 mm/hr. Immunoglobulins were sent showing a normal IgG of 10.5 g/L, normal IgM of 1.9 g/L, and slightly elevated IgA of 2.4 g/L. She had normal electrolytes, including normal serum calcium and magnesium. Her liver enzymes were also normal.\nA wide differential that included rheumatological, infectious, oncological, respiratory, and immunological causes was considered and so multiple investigations were then arranged. She had gastric aspirates and a tuberculin skin test to rule out tuberculosis, all of which were normal. Autoantibodies were sent and she was found to be mildly antinuclear antibody (ANA) positive (titres of 1 : 160) but her other autoantibodies were negative. She had adenine deaminase (ADA) levels and lymphocyte immunophenotyping done to rule out immune deficiency, which were normal. She had a normal sweat chloride. Bronchoscopy with bronchoalveolar lavage (BAL) was done: there was no bacterial or fungal growth on culture and the fluid was normal in appearance; BAL cell count showed WBC 127 × 106/L (3% neutrophils, 2% lymphocytes, and 95% macrophages) and RBC 303 × 106/L.\nIn regard to other imaging, she had a normal abdominal and thyroid ultrasound. Her chest CT showed diffuse interstitial lung disease, essentially characterized by interstitial lobular septal thickening (Figures , , and ). This raised possible diagnoses of pulmonary alveolar microlithiasis, dendritic pulmonary ossification (DPO), and pulmonary alveolar proteinosis (PAP).\nAs the imaging was not definitive, a lung biopsy was done; tissue was obtained from the lingula, which was sent for microbiology and pathology. The microbiology was negative. Pathology demonstrated presence of calcium concretions within the alveolar spaces, with blue discoloration at the edges and an eosinophilic centre on hematoxylin and eosin (H&E) stain; some structures had a concentric lamellar arrangement on trichrome staining (Figures and ). These findings were consistent with the diagnosis of pulmonary alveolar microlithiasis, with an incidental influenza infection explaining her acute symptoms.\nThe patient was then discharged home after recovery from the biopsy. Genetic testing showed homozygous pathogenic mutations in the SLC34A2 gene (NM_006424.2:c.226C>T [p.Gln76∗] in exon 03). Her sister was then brought for chest X-ray and CT, which showed similar findings, and was also positive on genetic testing. |
The patient, a 38-year-old male previously undiagnosed with FAP despite a strong family history, initially presented to the surgical service following a syncopal episode in December of 2017. Workup revealed severe anemia and a hemoglobin level of 3.8 g/dL (reference range: 12–16). Colonoscopy revealed innumerable colonic polyps beginning at the cecum and extending to the sigmoid colon, with sparing of the rectum, and the patient was diagnosed with FAP. A total abdominal colectomy with ileorectal anastomosis was performed without complication (Fig. ). Pathology subsequently revealed a T3 adenocarcinoma of the ascending colon with 1 positive lymph node out of 75 examined. The patient subsequently received 12 cycles of adjuvant FOLFOX.\nSurveillance CT scanning in late 2019 revealed a suspicious mass in the superior perirectal soft tissue (Fig. ). The patient exhibited no related symptoms at that time. Follow-up PET scanning revealed a PET-avid mass within the left superior mesorectal soft tissue, corresponding to the mass noted on the CT scan (Fig. ). No other suspicious lesions were identified. An examination under anesthesia was conducted, revealing an extrinsic nodular mass in the area of the left superior rectal wall, approximately 2 cm below the level of the ileorectal anastomosis. Colonoscopy and biopsy revealed 2 tubular adenomas, but no mucosal mass corresponding to the lesion noted on imaging. The patient underwent completion proctectomy with an ileoanal anastomosis and diverting loop ileostomy in January of 2020. The patient's postoperative course was uneventful, and he was discharged without complication. Pathology revealed a metastatic tumor deposit within the mesorectum 1.5 cm distal to the ileorectal anastomosis, with no evidence of mucosal or nodal involvement (Fig. ). The lesion had been resected in its entirety. There was no evidence of a primary rectal cancer, nor was there evidence of involvement of the ileal mesentery. After discussion at a multidisciplinary tumor board, the patient was subsequently returned to surveillance without further plans for adjuvant therapy. |
A 71-year-old non-Marfan male with a history of severe coronary artery disease, chronic obstructive pulmonary disease, and hyperlipidemia was referred to our department for evaluation of pulsatile masses in both groins and disabling (<20 m) intermittent claudication of his left limb. The CT angiogram revealed an infrarenal abdominal aortic aneurysm 5 cm in diameter, bilateral common femoral artery aneurysms (CFAAs) 2.8 cm (left) and 2.3 cm (right) in diameter, a thrombosed aneurysm of the right popliteal artery (PAA), and an aneurysm of the left popliteal artery 2.9 cm in diameter. Additionally diffuse stenotic lesions of the left SFA were noted (). Distal runoff was adequate without any significant stenosis. After we took into consideration all the potential treatment options and given the high risk status of the patient, we decided to repair all four aneurysms and revascularize the left limb in a single session using a hybrid approach. The anatomic configuration of AAA fulfilled the requirements for EVAR. Based on this approach, under general anesthesia, the distal external iliac artery, the common femoral artery, and its bifurcation were exposed bilaterally through vertical incisions. Through direct puncturing of CFA's aneurysms, an endovascular bifurcated graft of 23 mm in main body diameter, 20 mm in limb diameter, and 14 cm in length was implanted (Excluder, W. L. Gore & Associates, Inc, Flagstaff, AZ, USA). Balloon remodeling was performed to the proximal and distal landing zones as well as to all overlapping sites according to the manufacturer's recommendations. Completion angiography revealed adequate graft deployment with exclusion of the aneurysm sac, maintenance of internal iliac arteries' perfusion, and no signs of endoleak. After removing the sheath from the right CFA to restore the flow in the right limb, we proceeded with open repair of the left CFA's aneurysm combined with the revascularization of the left leg. Initially resection of the CFA aneurysm was accomplished in combination with completion of the proximal anastomosis of a PTFE graft 6 mm in diameter. Afterwards, under direct catheterization and insertion of an 8F sheath, we proceeded with the deployment of a covered stent 7 mm in diameter and 10 cm in length (Viabahn, W. L. Gore & Associates, Inc, Flagstaff, AZ, USA) for the exclusion of the aneurysm of the left popliteal artery. Then, we proceeded with angioplasty and stenting of the stenoses of the SFA using two self-expandable covered stents 8 mm in diameter and 10 cm in length (Viabahn, W. L. Gore & Associates, Inc, Flagstaff, AZ, USA). At last we completed the distal anastomosis of the PTFE graft and restored the flow of the left leg. Finally, we proceeded in the resection of the right CFA aneurysm and accomplished the interposition of a 6 mm PTFE graft with restoration of the flow to the profunda femoral artery. The whole duration of the operation was 210 min, the amount of contrast media given was 320 mL (Optiray 320, Mallinckrodt Inc, St. Louis, USA), while the radiation burden was 72.1 mGy.\nThe patient experienced an uneventful recovery and was discharged at the 3rd postop day. At 6-month followup, the CT angiogram revealed no signs of endoleak and the patient was well having a normal life with no symptoms of intermittent claudication in his left leg anymore (Figures and ). |
A 69-year-old man developed a sudden epigastric pain. He was presented at this hospital as an emergency outpatient. Six years earlier, he underwent laryngoesophagopharyngectomy, bilateral lymph node dissection for hypopharyngeal cancer, and esophageal reconstruction with a free jejunum flap. On physical examination, the abdomen was flat and soft with tenderness in the epigastric region, but no sign of peritoneal irritation. Blood biochemistry findings revealed elevated values: creatinine, 1.16 mg/dl; lactate dehydrogenase, 364 U/l; and creatine phosphokinase, 622 U/l.\nAbdominal contrast computed tomography (CT) revealed twisted mesentery with the small intestine around the point of torsion (whirl sign) and the superior mesenteric artery as the axis. Contrast enhancement was weakened in the same area of the small bowel (Fig. ). Given this information, we suspected small bowel volvulus and performed emergency surgery on the same day.\nA 5-mm camera port was placed in the umbilicus and 5-mm ports in the lower and right lower abdomen. During laparoscopic examination, the upper jejunum adhered to the small bowel close to the terminal ileum with overlapping of the small bowel. The entire part from the upper jejunum to the terminal ileum was twisted clockwise with the superior mesenteric artery and vein as the axes and the adhesion site as the starting point. There were areas of poor color enhancement throughout the twisted section of the small bowel (Fig. ). We laparoscopically separated the adhesion between different sections of the intestinal tract and traced the bowel from the small bowel in the region of the ligament of Treitz toward the anus to confirm the absence of adhesions or torsion up to the terminal ileum. The color of the small bowel improved; hence, the surgery was completed without resecting any part of the intestine.\nPostoperatively, the patient made good postoperative recovery, resumed oral intake on day 2, and was discharged on day 5 after surgery. No recurrence has been reported 1 year postoperatively. |
A 12-year-old healthy male child with no contributory medical history visited the Dept. of Pediatric and Preventive Dentistry, Dr DY Patil Dental College Pune, with the chief complaint of pain in the upper front tooth since 15 days and history of intermittent dull ache in upper right and left back region for a couple of months.\nOn examination, the upper left lateral incisor showed a gross carious involvement of crown. Upper right and left second premolars showed severe carious destruction of crown rendering them clinically nonrestorable (Figs 1 and 2). The upper left lateral incisor was tender on vertical percussion. The child had mandibular prognathism and angles class III molar relation ship (Fig. 3).\nRadiographic examination of the upper anterior region revealed tortuous root canals suggestive of severe root dilacerations of upper left central and lateral incisor. In case of lateral incisor the root tortuousity does not seem to be as complicated as of a central incisor. This is because of the buccopalatal angulation of the root of lateral incisor which is not evident on radiograph very clearly (Fig. 4).\nPatient’s parents revealed history of traumatic episode at the age four which involved avulsion of two deciduous teeth from upper front region.\nElectric pulp testing of the involved lateral incisor exhibited delayed response compared to the contralateral tooth.\nVarious treatment modalities were explained to the parents of the child with their pros and cons and it was finally decided\nto go ahead with the present treatment approach.\nAs the pulp chamber was calcified and root canal was patent only in the middle and apical third, the negotiation of the root canal was not an easy task (Figs 5 A and B).\nProfound anesthesia was achieved by administering 2% Lignocaine with Adrenaline (1:200000) and access cavity preparation was done using round carbide bur.\nIn an attempt to gain an access to radicular portion, a perforation occurred in coronal one third of the distal aspect of root.\nUpon further exploration, a very unusual location of the root canal was found on the palatal wall of the root (Figs 6 A and B).\nAn immediate diagnostic radiograph was made to ensure the correct positioning and the length of the root canal (Fig. 7).\nUpon confirming the canal position, thorough biomechanical preparation was carried out using rotary protaper system and pulp space was obturated with gutta percha points using AH-26 (Dentsply, USA) as a root canal sealer (Figs 8 and 9).\nThe perforation on coronal one third of the distal aspect of root was sealed using mineral trioxide aggregate (MTA - Angelus, Brazil) (Fig. 10).\nThe affected tooth was kept isolated using cotton rolls, 2 × 2 gauze and high vacuum suction all throughout the procedure.\nThe entire endodontic treatment and sealing of the perforation was carried out in a single visit. In the subsequent visit the post space was prepared using peeso reamers. Considering the length of the root canal and crown-root ratio and the disadvantages associated with cast posts, the light transmitting fiber post (Angelus, Brazil) was used (Fig. 11).\nIt was cemented using dual cure adhesive cement (Panavia F 2.0 Kuraray Dental products Japan) (Figs 12 A and B).\nThe core structure was built around the post using nano filled composite resin (Z-350 3M ESPE USA) (Figs 13 A and B).\nFinishing and polishing of the composite was done using contouring and polishing discs (Sof-Lex-3M ESPE USA) which ultimately resulted into a fairly acceptable esthetics (Figs 14 to 16).\nConsidering the dental and chronological age of the child the decision of giving a final extracoronal ceramic restoration is deferred until the eruption of all permanent teeth. |
A 5 year old girl of Indo-Aryan origin presented with one and a half month old neglected trauma left leg, managed by a local bone setter (quack). Examination revealed swelling from knee to ankle, 5cm×5cm ulceration over proximal part of shin, purulent discharging sinus and deformity. X-ray revealed non-union with exuberant callus at the fracture site.\nAt the age of 2 years patient developed ulcers of multiple fingers and toes followed by their self amputation (, ). Finger and toe tips showed multiple healed scars. Radiographs revealed osteoacrolysis (). In the same year, patient had trauma to left thumb which healed with a deformity (). There had been trauma to left forearm in childhood; X-ray revealed healed fracture of shaft of ulna. Parents mentioned all the above traumatic events were not associated with pain.\nAround the age of 2 and half years patient developed blisters over right lower extremity, which healed leaving scar marks ().\nThere is history of recurrent episodes of high grade fever since childhood. There was one such episode in the hospital lasting 4 days. There is history of lack of sweating since birth. Physical and laboratory tests revealed anhidrosis. Patient feels very uncomfortable during hot weather and comforts herself by taking a cold water bath which gives temporary relief.\nShe is born of non-consanguineous marriage. Family history was insignificant. Motor and mental milestones were delayed. Intelligence is low as compared to other siblings. There is history of repeated respiratory tract infections in childhood.\nNeurological examination was normal except inability to perceive painful stimuli. No pain was perceived even at the time of intravenous and intramuscular injections. Nerve conduction study revealed normal median nerve conduction velocity. There is history of several episodes of biting of tongue. The patient had normal eruption of teeth but had lost most of them; all the incisors and canines were absent.\nCRP was elevated. Culture of pus from discharging sinus cultured S. aureus. Serum calcium, serum phosphorus, renal function parameters, thyroid function tests, parathormone levels, serum uric acid were in normal range. Infected non-union was managed by curettage and debridement. Reduction was achieved and fixed using biplanar external fixator (). At 3 months follow up pus discharge had subsided with abundant granulation tissue at wound site. There was callus formation at fracture site and features of union on radiograph. |
A 51-year-old drunken male lost his balance and went fell down on the ground with a wooden stick in his hand. The pointed end of the wood impaled through the right lower vestibule of his mouth, traveled subcutaneously in the neck and was stuck in the clavicle. There was no excessive bleeding, vomiting or loss of consciousness and respiratory distress. The relatives of the patient did not make any attempt to remove the protruding wooden piece. Within 3 h of the primary incidence, the patient was transported to the emergency department of the B P Koirala Institute of Health Sciences, Dharan, Nepal. The patient did not have any history of dyspnea, dysphagia, oronasopharyngeal bleeding and neurological deficit. Mild trickling of blood from the entry site was noticed in the hospital. The arterial pressure was 130/86mmHg and the pulse rate was 80/min. The bamboo stick was seen protruding through his mouth []. The entry point was 3cm longer in transverse diameter in between the mandible and the lower lip toward the right side. A hard 13cm long and 4cm wide longitudinal swelling extending from the under lip to the clavicle was palpated []. It was found that the distal end of the wooden stick moved with movement of the proximal end. No other swelling was revealed in other part on the neck. A further examination of the neck revealed the absence of subcutaneous emphysema, bruit, thrill, stridor and respiratory distress. The pulsation of the carotid and superficial temporal artery was intact bilaterally and was also absolutely symmetrical in radial and brachial pulse. The expansion of the chest and the bilateral air entry in the chest were normal. The remaining ENT and systemic examinations were within the normal limit.\nImmediately, non-contrast CT followed by contrast-enhanced CT (CECT) scan of the head and neck region were performed, which revealed a low-density area with a density (Hounsfield Unit, -1003) equivalent to air under the soft tissue and lateral to the mandible on the right side []. The mandible, hyoid, clavicle and cartilaginous framework of the larynx and trachea were normal in texture. No fracture in related bones and cartilages was revealed by the CT scan. There was no evidence of vascular injury and hematoma formation. The contrast CT angiography revealed normal integrity of the major vessels. The CT scan of the thorax was absolutely within the normal limit.\nIn the operation theater and under general anesthesia, the wound was explored by a vertical incision along the long axis of the foreign body. The bamboo stick was removed completely, a corrugated drain was placed and the wound was closed in layer after securing hemostasis. The foreign body did not damage any major structures, including the major blood vessels. Intravenous antibiotics, anti analgesic and prophylaxis of tetanus were given in appropriate doses. The drain was removed on the third postoperative period and the patient was discharged on the seventh day. Follow-up after the fourth week revealed a thick fibrous band and wound contracture along the line of incision []. The wound was re-explored and the fibrous tissue and scar were completely removed. The subsequent follow-up revealed complete healing of the wound. |
A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis (Fig. , ). The major findings of EPS, such as peritoneal thickening and calcification, were not noted on CT.\nOn physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction (Fig. ). As we suspected that the peritonitis might be associated with bacterial translocation from the dilated intestine, he was advised to stop eating and was switched from CAPD to hemodialysis. Additionally, he was treated with intravenous vancomycin and cephtazidime. The PD catheter was flushed once a day to prevent catheter obstruction with fibrin, and the characteristics of the peritoneal fluid were monitored. His abdominal pain was resolved and peritoneal fluid cell count decreased to < 30/mL, and thus, he resumed oral intake on day 8.\nAfter resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells (Fig. ). Although he stopped eating again, his abdominal pain did not improve, and fecal material with foul smell was identified from the PD catheter on day 23 (Fig. ). Culture of peritoneal dialysate on admission was negative; however, culture of peritoneal dialysate on hospital day 23 was positive for Enterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. ). Considering these facts, intestinal perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. Although intra-abdominal adhesion was severe, fibrinous encapsulation of the bowel, which would suggest EPS, was not detected macroscopically during surgery (Fig. ). As indicators of EPS were not evident, the PD catheter was removed. The perforation site was located at the adhesive intestine. The tip of the peritoneal catheter was located in Douglas’ pouch, and it did not injure the adhesive intestine. Pathological examination of the resected specimen revealed inflammatory cells associatet with the peritonitis in the intestinal wall. Intestinal fibrosis, arterial alteration, and tissue calcification were not evident pathologically (Fig. , ). Although his serum beta-2 microglobulin (B2M) level was high (41.05 mg/L), amyloidosis and deposition of B2M were not observed (Fig. -). The postoperative course was uneventful and left arteriovenous fistula surgery was performed on day 42. Since then, he has been on maintenance hemodialysis with no recurrence of peritonitis. |
A 45 year old female with the diagnosis of 10 years' progression of definite () bilateral type I Menière's Disease, according to the Lopez-Escamez classification (), with recurrent vertigo and hearing loss attacks symptomatically controlled with sulpiride (50 mg) on demand, was referred to the cochlear implants unit of a tertiary hospital center as a possible candidate for cochlear implantation, because of profound hearing loss (1997 BIAP) in the left ear and fluctuating moderate-severe (1997 BIAP) hearing loss in the right ear. The most recent vertigo episode occurred 10 days prior to her visit to the cochlear implant unit. No visual impairment or other alterations were found on otoneurotologic examination; normal ocular movements, normal smooth pursuit, and saccadic movement, absence of ocular misalignment on skew deviation test and no spontaneous nystagmus were also found, despite the recency of the last vertigo attack. Other neurological examinations and standard cerebral MRI were also normal for this patient.\nIn addition to the same tests as for case I, because of the bilateral MD diagnosis, the patient had received 3 months prior a 3 Tesla MRI examination of the inner ear using the HYDROPS MRI sequence (): this sequence is based on the digital subtraction of images produced by the different time diffusion of gadolinium along the inner ear fluids. This MRI showed bilateral cochlear and vestibular endolymphatic hydrops with left side hydrops predominating, as can be observed in Figure .\nThe instrumental vestibular testing was performed by the same senior neurotologist using a vHIT ICS Impulse™ USB hardware version with software version 4.1: this device was a different unit from the device used in case I. Figure shows enhanced vHIT eye velocity responses for both sides, with an aVOR gain value of 1.14 on right horizontal canal function test and 1.05 for left side. In this case, the vHIT calibration was repeated four times with similar enhanced gain values obtained, and a fifth calibration was done with the default system calibration parameters, also yielding similar aVOR gain values. The VVOR test was also performed on this patient using the vHIT ICS Impulse™ device, finding an enhanced eye velocity response during the VVOR test with a measured VVOR gain () value of 1.39 for left side and 1.35 for right side.\nFor both cases presented in this paper, the patients gave written consent to publish the results obtained from their clinical examinations and instrumental tests. |
The patient was a third child born after normal pregnancy to a 32-yr-old mother. Apgar scores were 9 and 9 at 1 and 5 min, respectively, and birth weight was 6 pounds and 14 ounces (2785 g). The patient has had problems with feeding since birth and his psychomotor development was delayed. At age 1 yr the patient was functioning at a 2-month developmental level. One episode of infantile spasms was reported at 6 months that resolved with a single dose of ACTH; prophylaxis was not begun. He underwent bilateral inguinal herniorraphy at 10 months and subsequently received a gastrostomy tube to facilitate feeding. He was evaluated in the genetics clinic at 14 yr of age. His weight was 27.60 kg, height was 147 cm, and head circumference was 55 cm. Facial features were notable for mild dolichocephaly, mild midface hypoplasia, deep-set eyes with short palpebral fissures, large protruding ears with simplified helix and deficient lobuli, bilateral skin tags on the posterior aspect of the ear lobes, and micro- and retrognathia with large appearing teeth (A). The patient was nonverbal with very limited social interaction and needed assistance with ambulation. Self-stimulating behavior was observed in the form of constant rocking back and forth and repetitive movements of his arms. Mild facial weakness, global hypotonia, diminished muscle mass, and motor apraxia were evident. Deep tendon reflexes were bilaterally brisk. The patient was able to localize touch and there was no cerebellar involvement. Short stature, both height and weight consistently below the third percentile, was recorded between 14 and 19 yr (B,C). Bone age was ∼12.5 years at a chronological age of 15 yr, representing a delay of greater than 3 SDs. Brain magnetic resonance imaging (MRI) was unremarkable and limited ophthalmologic evaluation disclosed mild optic nerve hypoplasia bilaterally. An awake and asleep computer-assisted prolonged video EEG recorded left occipital onset seizure with secondary generalization, dysrhythmia grade 3, generalized and multifocal spikes and sharp waves, and right temporal intermittent rhythmic delta activity. Cerebrospinal fluid (CSF) examination was significant for a very low level of hydroxyindoleacetic acid (5-HIAA) of 19 nmol/l (normal range 67–140 nmol/l) and a low level of homovanillic acid (HVA) of 68 nmol/l (normal level of 145–326 nmol/l), whereas concentrations of 3-methyldopa, tetrahydrobiopterin, neopterin, and 5-methyltetrahydrofolate reductase were within the laboratory reference range. The overall phenotype was significant for optic nerve hypoplasia, severe intellectual disability with absent speech and limited ambulation, hypotonia, dysmorphic facies, short stature with delayed bone age, and low levels of 5-HIAA and HVA in CSF. |
A 66-year-old man was referred with a diagnosis of hyperparathyroidism, mild renal failure, peptic ulcer and large suspicious cervico-mediastinal goitre. The patient had fatigue, anaemia, anorexia and bone pain for several months. The fine needle aspiration of the neck mass, performed before the admission showed the presence of malignant cells. Preoperative laboratory findings are reported in table .\nUltrasonography of the neck showed an enlarged thyroid gland with substernal extension; many solid nodules and some calcifications were present. The computerised tomographic (CT) scan showed a large solid mass in left neck, with extension to the mediastinum, and displacement of the trachea, oesophagus and great vessels (Figure ). Tracheobronchoscopy demonstrated substenosis of the proximal trachea due to extrinsic compression.\nTc-99m sestamibi scintigraphy demonstrated diffuse radiopharmaceutical uptake within the thyroid region and the lower portion of the neck. This tracer accumulation corresponded to the mass and it was persistent during the different phases of the scintigraphy without significant changes (Figure ). The lack of a spot area of increased MIBI uptake ruled out the diagnosis of adenoma. The scintigraphic pattern was classified as due to a multinodular goitre.\nThe patient was vigorously hydrated and bisphosphonates were given preoperatively to control hypercalcemia. Eight days later the serum calcium level decreased to 10.4 mg/dl. With the above clinical findings, the patient underwent surgical neck exploration.\nAt surgery a voluminous cervicomediastinal firm mass was found that could not be removed via the superior thoracic strait; therefore a complete sternotomy was performed. The surgical findings revealed an ovoid, grayish-yellow, hard mass, measuring 12 × 9 × 8 cm, adherent to the base of the left lobe of the thyroid, extending to the anterior upper mediastinum; the recurrent laryngeal nerve was laterally displaced but not invaded. Therefore en bloc resection of the mass along with the total thyroid gland and the connective tissue apparently involved in the tumour was performed. All the lymphoid tissue from the ipsilateral tracheoesophageal groove to the upper mediastinum was removed\nThe histological report described a parenchymatous thyroid gland, with a subcapsular gelatinous nodule (1 cm diameter). The voluminous tumour adherent to the base of left lobe was multinodular, greyish-yellow. Histologically many fibrous bands of acellular collagenous tissue extending from the thickened capsule, subdivided the neoplasm into irregular compartments. The tumour cells, arranged in trabecular and solid sheets, were homogeneous; they showed round – ovoid nuclei, with evident nucleoli, and clear cytoplasm; occasionally cytoplasm was abundant, eosinophilic and granular; nuclei were pleomorphic; mitoses were present (3 per 10 HPF). Rare multinucleated osteoclastic-like giant cells were found around foci of haemorrhage; necrosis and coarse calcifications were observed. Both capsular and vascular invasion were present at the periphery of neoplasm. In the infiltrated adjacent adipose tissue, well-circumscribed, encapsulated nodules of tumour, with a peripheral rim of lymphoid tissue were found; they were considered to be metastatic lymph nodes. Final diagnosis was a parathyroid carcinoma (Figure ). The postoperative course was complicated by transient oligouria, with worsening the chronic renal failure.\nThe patient also had severe postoperative hypocalcaemia, necessitating calcium and vitamin D therapy. The postoperative laboratory values at hospital discharge are shown in table . Figure shows the change of serum Calcium and PTH levels during patient's hospitalization\nAt follow-up, three, six, nine and 12 months after surgery, the patient was disease-free with normal levels of PTH and calcaemia and improvement of the bone disease symptoms. Whole body positron emission tomography (PET) performed one year after surgery was negative. |
We report the case of a 41-year-old male who presented to our outpatient department with minor symptoms, including dyspnea during heavy activities. The patient did not have a history of fever, weight loss, malaise, or carotid tenderness but had a 1-year-old history of dyspnea on exertion. The patient never had any previous indications of autoimmune diseases or infections. Approximately 17 years earlier, at the age of 24, the patient experienced a frequent loss of consciousness during exercise. He had no history of hypertension, diabetes, smoking, dyslipidemia, or obesity. The patient was diagnosed with dilated cardiomyopathy a year ago, and echocardiography showed that all chambers were dilated with a left ventricular ejection fraction (LVEF) of 39%. The patient had received an angiotensin-converting enzyme inhibitor, a beta-blocker, and spironolactone and had good compliance and functional outcomes as the patient has presently been classified as New York Heart Association functional class I. Physical examinations revealed blood pressure of < 120/80 mmHg and a heart rate of 60 beats per minute (bpm) because of the optimal medical treatment he had received since last year. The patient had left carotid bruit and an enlarged heart with a grade IV/VI holosystolic murmur on the apex radiating to the axilla. Chest X-ray showed cardiomegaly, and electrocardiography revealed sinus rhythm with a heart rate of 64 bpm, poor R wave progression, and left ventricular hypertrophy (LVH) (Fig. ). Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) showed eccentric LVH with reduced ejection fraction and some hypoechoic lesions resembling cysts at the anterior, anteroseptal, and anterolateral walls, suggestive of myocardial detachment. Both ventricles were dilated with reduced LVEF (31%). A severe functional mitral regurgitation secondary to increased left ventricular end diastolic volume and mild aortic regurgitation due to aortic root dilatation and uncoaptation of the left coronary cusps were detected. Moreover, TTE and TEE data obtained last year showed similar results. The patient underwent cardiac computed tomography (CT), which revealed a large calcified LSVA protruding into the left ventricular anteroseptal, anterior, and anterolateral walls, causing MD. The patient had no plaque, stenosis, or intimal wall thickness on the coronary arteries. CT angiography (CTA) showed diffuse calcification from the aortic arch and main branches of the aorta, extending to the descending thoracic and abdominal aortae. The patient had mural thickness at the left carotid artery as a sign of active disease, and no calcification or thickness was observed on either renal artery on CTA. We performed duplex ultrasonography to assess the involvement of aortic branch arteries. A long-diffuse-homogenous-concentric type IV plaque was detected along the left common carotid artery without the participation of other aortic branch arteries. The venereal disease research laboratory test, treponema pallidum hemagglutination assay, anti-hepatitis B surface antigen, anti-human immunodeficiency virus (anti-HIV), and anti-streptolysin titer O were nonreactive; inflammatory markers were slightly increased, including the C-reactive protein (CRP) (0.51 mg/dL) and erythrocyte sedimentation rate (ESR) (25 mm/h).\nThe patient was diagnosed with TA with MD complicating LSVA and dilated cardiomyopathy and received the treatment for chronic heart failure treatment: a beta-blocker, an angiotensin-converting enzyme inhibitor, and a mineralocorticoid antagonist, which were administered at the optimal dose. The patient has also received a high-dose steroid (40-mg methylprednisolone) as the initial therapy for TA and aspirin (81 mg, daily). Later, azathioprine (50 mg, b.i.d.) was added to further reduce inflammation. The symptoms and signs were improved, and inflammatory markers decreased after 3 months of therapy. Follow-up ESR was 17 mm/h, and the CRP level decreased to 0.3 mg/dL. The patient experienced no adverse events owing to the medications. |
A 73-year-old woman with no previous comorbidities or family history of hematological disorders or hypercoagulability was admitted to the Infectious Disease Clinic due to suspected HFRS and dehydration. Two weeks prior to disease onset, she had been exposed to bank voles while cleaning out a cabin. For 6 days following disease onset, she had been ill with fever, chills, weakness, low urine production, and difficulties eating and drinking. The patient had positive serology for Puumala virus thereby confirming the HFRS diagnosis. Laboratory tests taken the day before admission revealed thrombocytopenia (platelet count: 48 × 10\n9\n/L), impaired renal function (creatinine: 278 μmol/L), and leucocytosis (white blood cell count: 14 × 10\n9\n/L). Upon admission to the hospital, the platelet count had increased to 61 × 10\n9\n/L and creatinine increased to 370 μmol/L indicating clinical progression to the oliguric stage of HFRS. However, the platelet levels decreased to 12 × 10\n9\n/L on days 8 to 9. The treating physicians decided to transfuse platelets on days 8, 9, and 10 due to the high risk of spontaneous bleeding. Despite transfusion with three platelet units, the patient remained severely thrombocytopenic with platelet counts below 50 × 10\n9\n/L during days 8 to 13. The case is summarized in\n. Criteria for disseminated intravascular coagulation (DIC) were fulfilled from day 8 (see\nfor an overview of criteria).\nOn day 13 (2 days after the last platelet transfusion and a platelet count of 27 × 10\n9\n/L), the patient falls ill with abdominal pain which increases in severity during the evening. An abdominal computed tomography (CT) shows congestion and ischemia in the terminal ileum due to a thrombus in the superior mesenteric vein (SMV) reaching up to the portal vein (PV). The hematologist advised against thrombolysis due to thrombocytopenia in combination with a known mild VHF, which could increase the risk of bleeding. A national coagulation expert is consulted for further advice, who recommends anticoagulant treatment with heparin in a “careful” dose. Heparin at a dose two-thirds the national recommended dose is initiated with the aim of APTT 1.5 times the baseline value (40–50 s). The patient therefore receives a bolus dose of 4,000 units heparin followed by transfusion of 24,000 units heparin per day. The following day (15), the patient has gastrointestinal bleeding and a decrease in hemoglobin values from 100 to 89 g/L. According to the surgeon consultant, the stasis caused by the SMV and PV thrombus damages the intestinal mucosa leading to the observed gastrointestinal bleeding. Heparin is therefore continued in the same careful dose and the patient receives one unit red blood cells (RBCs) that day and the following day (16). The following criteria had to be fulfilled before mesenteric phlebography and thrombolysis via catheter could be considered: (1) platelet levels greater than 100 × 10\n9\n/L, (2) no bleeding, and (3) the patient can tolerate a full-dose heparin. On day 19, the patient fulfilled these criteria and underwent mesenteric phlebography via interventional radiology. Using the percutaneous transhepatic route to the PV, a hydrolyser 7F, double lumen, over-the-wire thrombolysis (Hydrolysis, Cordis Europe NV, Roden, the Netherlands) was used to perform a mechanical thrombolysis of the PV followed by pharmacological thrombolysis with tPA (Actilyse infusion: 0.8 mg/hour). A notation from the surgery department states that the previous heparin treatment aiming for 1.5 times APTT had been unsuccessful in decreasing the size of the SMV and PV thrombus. A control angiography 6 hours postsurgery shows that the thrombus distally in the SMV has been removed. There is still a thrombus between the portal and the splenic vein; therefore, the catheter is moved further into the area of thrombosis and thrombolysis by Actilyse administration is continued. At this time, there is no contrast leakage as a sign of hepatic bleeding. The patient stays in the intensive care unit (ICU) with local hydrolysis via the catheter. The following day (20), the patient becomes hypotensive with systolic blood pressure down to 75, and has signs of peritonitis. The levels of the fibrin degradation product D-dimer increases to 20 mg/L and fibrinogen decreases to 0.69 g/L. A CT thorax/abdominal scan shows an ongoing expanding hepatic intraparenchymal arterial bleeding. In addition, the CT scan shows presence of pulmonary emboli. Since the patient has a propensity for bleeding and thromboembolism, arterial intervention via the femoral artery into the aorta and then out into the common hepatic artery with coiling was not an option. Instead an emergency surgery procedure is performed with ligation of the right hepatic artery in the hepatoduodenal ligament, which stops the bleeding. In addition, the anticoagulant therapy is discontinued that day and the patient is tended in the ICU in a respirator. On day 21, the anticoagulant therapy is readministered at a low dose. The patient is extubated on day 22 and the anticoagulant therapy is increased to a target of APTT 60s due to remaining portal thrombi and peripheral pulmonary emboli. On the evening of day 25, the patient develops acute dyspnea, and oxygen saturation decreases to 88% with 4 L of oxygen and tachycardia. A CT pulmonary angiography shows pulmonary emboli in the right and left pulmonary arteries and peripherally in the pulmonary lobe arteries. The patient is transferred that evening to the ICU with heparin treatment at a target of APTT 85s. An ultrasound of the peripheral extremities (day 26) shows bilateral deep vein thrombosis in the posterior tibial veins. Since the APTT remains difficult to adjust (ranging from over 180s to the therapeutic target of 80s) and the propensity to develop thrombosis despite anticoagulation, it is decided to change the treatment from heparin to low-molecular-weight heparin (Fragmin 16,000 IU/day) on day 32. After day 55, the patient receives warfarin as prophylaxis against further thromboses and is discharged to her home on day 61. To rule out other causes for the thromboembolic complications, she was tested and found negative for activated protein C resistance. |
A16-year-old boy sustained injury to his left knee while playing. The patient had severe pain and swelling and was unable to bear weight in the affected knee. X-rays revealed displaced tibial spine fracture [, ], and the young boy was treated with cast. However, the patient was uncomfortable with the cast and came to our institute. It was displaced fracture. We did MRI to rule out other injuries [].\nPatient was treated arthroscopically under spinal anesthesia in supine position. Anterolateral and anteromedial portals 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 shaver. It also involved significant portion of medial tibial platue [, ]. Fracture was reduced with 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 was not sitting 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 stable []. Post-operatively, patient was kept in a knee brace [Fig. 10]. Post-op X-rays showed proper postion of both the screws [Fig. 8, 9]. He was allowed to walk full weight bearing with the knee brace for support. Knee range of motion was allowed from 0-90 for 2 weeks than 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. |
A 71-year-old female was referred to our quaternary referral center with a hilar biliary stenosis suspicious for a hilar cholangiocarcinoma (Bismuth classification 3A). She suffered from progressive jaundice, itching, and weight loss (6 kg) for the last 4 weeks. Blood test revealed an elevated bilirubin of 256 µmol/L. She had a history of clinically stable Crohn's disease treated with infliximab. In 2013, she underwent curative treatment for a pT1cG1No infiltrating ductal adenocarcinoma of the left breast. Computed tomography (CT) scan revealed a tumor mass with encasement of the right hepatic artery and right portal vein, which was considered surgically resectable by an extended right hemihepatectomy after internal biliary drainage of the future liver remnant. Due to an insufficient volume of the left lateral liver remnant (22% of the total liver volume), we performed a percutaneous ipsilateral embolization of the segmental right portal vein branches, using polyvinyl alcohol embolization particles (ContourTM PVA Embolization Particles 45–150 μm, Boston Scientific) and interlock coils (Interlock-18 Fibered IDC occlusion system, Boston Scientific). A postprocedural control angiography showed coiled right portal veins with a patent common and left portal vein (Fig. ). Two days after this uneventful procedure, she presented at the emergency room with fever (39.5°C) combined with nausea and headache. A CT scan was performed and revealed a successful embolization of the right portal vein; however, also a detrimental total occlusion of the common and left portal vein due to a massive thrombosis (Fig. ). She was admitted to the hospital and treated with antibiotics intravenously (Clindamycin 600 mg 3 times daily and ciprofloxacin 400 mg twice daily). Immediate portal vein thrombolysis was attempted by continuous intra-arterial infusion of the thrombolytic agent urokinase into the selectively catheterized superior mesenteric artery. Unfortunately, after 3 days of intra-arterial infusion of urokinase, no signs of improvement were seen on the control CT scan. A transhepatic or transjugular retrograde cannulation of the portal vein was considered to be a nonrealistic treatment option due to the extent of thrombosis. No other minimally invasive treatment options were left to solve this major complication. Therefore, we attempted revascularization of the future liver remnant portal vessels by combining a mechanical thrombectomy of the left and common portal vein together with intraluminal catheter-based thrombolysis via a surgically reopened umbilical vein.\nAfter induction of general anesthesia, we performed a (limited) bilateral subcostal laparotomy. No intra-abdominal signs of metastatic disease were found. After ligation of the umbilical ligament, we re-opened the obliterated umbilical vein with surgical forceps in a retrograde fashion until some retrograde backflow from the left portal vein was obtained. Next, the reopened umbilical vein was used to introduce a Fogarty balloon catheter into the left portal vein, and a significant amount of thrombus was removed from the common and left portal vein. Finally, we introduced a central venous catheter (CVC) through the umbilical vein and positioned the tip of the CVC at the base of the portal vein. We fixated the CVC to the umbilical ligament, peritoneal side of the abdominal wall and skin. After the operation, thrombolysis was initiated via the CVC with strict observation of the hemodynamics, plasma fibrinogen concentration and daily angiography via the CVC. Pathologic report of the removed thrombus showed a fresh thrombus with no signs of malignancy (Fig. ). Control angiography after 1 day of thrombolytic therapy revealed recanalization of the left portal vein and portal vein main stem, but also remnant mural clots (Fig. ). Intraportal thrombolytic therapy was continued for another 2 days, after which control angiography demonstrated complete resolution of the PVT and the branches of the left portal vein to segment 2 and 3 of the liver (Fig. ). Thrombolytic therapy was discontinued, and a therapeutic dose of low-molecular-weight heparin was started. The same day, the patient developed abdominal pain, and a CT scan revealed a subscapular hematoma at the right side of the liver as complication of the thrombolysis and full-dose heparin treatment (Fig. ). The therapeutic dose of low-molecular-weight heparin was discontinued, and fortunately repeated imaging showed no progression of the subcapsular hematoma. After 4 weeks, CT volumetric analysis revealed a future liver remnant volume of 31% of the total liver volume. Accordingly, the patient underwent an uncomplicated extended right hemihepatectomy, including resection of the caudate lobe and extrahepatic bile ducts, followed by a hepatico-jejunostomy reconstruction. Because of a small persistent partial common PVT, we resected 1 cm of portal vein and reconstructed the vein by an end-to-end anastomosis between the portal vein main stem and the left portal vein. Pathology report stated a 4-cm hilar cholangio-adenocarcinoma with invasion of the gallbladder with free resection margins and metastases in 3 out of 10 resected hilar lymph nodes (Fig. ). After 19 days of an uneventful postoperative stay, the patient left the hospital in a relatively good clinical condition. She remained well for 2 years after surgery without any signs of recurrent PVT. Unfortunately, a CT scan performed due to abdominal complaints 2 years after the surgery revealed local tumor recurrence for which palliative care was started. |
A 28-year-old male presented with a dull ache and photophobia in the right eye following blunt ocular trauma. Three days earlier he had been hit in the right eye with a cricket ball. There were no other ocular complaints or significant past ocular history. The patient was in excellent general health and was not taking any medications.\nOn examination, his corrected visual acuities were 6/12 and 6/6 in the right and left eye respectively. Intraocular pressures were 19 mm Hg in each eye. Biomicroscopy revealed a moderate traumatic iritis in the right eye. After pupillary dilatation, ocular examination revealed a clear lens with an annular pigment band on the posterior capsule in the right eye. No other signs of blunt trauma were detected on a detailed examination of the anterior and posterior segments. The examination of left eye was within normal limits. There was no evidence of any pigmentation on the posterior capsule in the left eye. The patient was treated with mild steroids (prednisolone acetate 0.5% q.i.d) and cycloplegics (cyclopentolate 1%) for a week.\nDuring follow-up evaluation of the patient after two weeks, visual acuities were 6/6 in both eyes. The traumatic iritis in the right eye had resolved. On a detailed evaluation of the right eye, no sequelae of blunt trauma were found. However, the annular pigment band on the posterior capsule of right eye persisted. A directed examination was performed in both eyes for signs of pigment dispersion syndrome. A re-examination of posterior corneal surface did not reveal Krukenberg's spindle or any other pigment deposition patterns in either eye. In both eyes, the anterior chamber was of normal depth with no noticeable concavity of the peripheral iris. There were no transillumation defects in the iris on careful retroillumination. Gonioscopy revealed open angles without any evidence of hyperpigmentation or angle recession in either eye. The cup to disc ratios were 0.3 in both eyes with a healthy neuroretinal rim. On direct questioning, there was no significant family history of glaucoma or other ocular disorders. |
A 55-year-old non-diabetic, non-hypertensive male with a history of recurrent colicky left lumbar pain presented with acute urinary retention. Catheterization was attempted; however, it was unsuccessful. Ultrasound revealed an overdistended urinary bladder with a normal-sized prostate and scarring and focal caliectasis in the left kidney (). The right kidney was normal and no calculi were seen on either side on ultrasound. Serum electrolyte, renal and liver functions were normal. The haemogram revealed neutrophilia. The prostate-specific antigen was within normal limits. A rigid urethroscopy was performed owing to suspicion of a left urethral calculus and a 11-mm size calculus was removed from the posterior urethra. The patient was discharged and had no difficulty with micturition for 2 weeks thereafter. The patient subsequently developed burning micturition with hesitancy and induration in the perineal region. The urine was turbid and microscopy revealed the presence of Escherichia coli. A perineal ultrasound revealed an abscess in the perineum, which extended to the proximal parts of the corpus spongiosum (). This abscess was drained under saddle block. The patient subsequently developed a discharging sinus at the operative site () leaking purulent fluid. A retrograde urography revealed periurethral extravasation of the injected contrast material with a lytic lesion in the left pubic ramus (). A repeat perineal ultrasound revealed a linear hypoechoic tract leading from the skin surface to the corpus spongiosum (). A retrograde CT urography was performed to look for the extent and ramifications of the abscess. On the non-contrast CT scan, a lytic lesion was seen involving the left inferior pubic ramus (). The pubic symphysis and bodies of both pubic bones were normal. A proximal femoral nail was noted in situ on the left side, which was inserted 10 years before for fracture of the proximal shaft of the femur owing to accidental trauma. On injecting iodinated contrast into the urethra, there was extravasation of the contrast in the periurethral region in the soft tissues surrounding the bulbar and the posterior penile urethra. An extension of the contrast through the external anal sphincter into the intersphincteric plane () with inflammatory stranding in the ischioanal fossae was seen. The contrast also extended into the lytic lesion present in the left inferior pubic ramus (). The contrast also extravasated through the cutaneous opening in the perineum and the natal cleft (). E. coli were isolated on pus culture. The patient was treated with intravenous antibiotics and suprapubic cystostomy was performed. The patient is presently being considered for elective urethroplasty. |
A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition. |
A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points (). |
Patient 1: A 31-year-old gravida 5 para 4 woman at 24 weeks and 4 days of gestation presented to the emergency department after having suffered 5 gunshot wounds to her bilateral lower extremities. On admission, her Glasgow Coma Scale Score was 15 and she was hemodynamically stable. Plain film radiography of the pelvis and left femur was performed. There was a comminuted, segmented, displaced fracture of the left mid-femoral diaphysis with multiple osseous and metallic fragments as well as a nearby dominant bullet fragment (). There were no additional injuries. Her obstetric history consisted of four previous pregnancies with uncomplicated term spontaneous vaginal deliveries. The patient reported no additional medical problems or prior surgical history. Laboratory evaluation was significant for a low serum calcium level of 8.2 mg/dL.\nThe patient underwent retrograde intramedullary nailing under general endotracheal anesthesia. Intraoperative fetal monitoring demonstrated moderate variability with occasional shallow variable decelerations. The total radiation dosage both preoperatively and intraoperatively was 20.1 milligray (mGy). A lead apron was used to shield the maternal abdomen during all images required pre- and- intraoperatively. In the postoperative care unit, uterine tocometry showed contractions every 3–5 mins that the patient stated were non-painful. Cervical examination was performed due to continued non-painful contractions, and her cervix was closed. She was transferred to labor and delivery for the remainder of her postoperative care.\nThe patient received betamethasone and magnesium postoperatively due to concern for possible preterm delivery due to initially non-reassuring fetal heart tones. With intrauterine resuscitation, delivery was not required and contractions spaced out. The patient was started on 5,000 units of heparin three times daily for VTE prophylaxis. Physical therapy was initiated while inpatient. She was discharged on postoperative day five with a 30-day supply of 30 mg twice daily low-molecular-weight heparin (LMWH) after ambulating with a walker and had a spontaneous vaginal delivery at term. |
A 62-year-old female with no significant past medical history presented to the emergency department in November of 2017 with complaints of arthralgias, most notably in her right knee, left shoulder, and bilateral thighs that made it difficult for her to ambulate. She was also admitted due to a headache that was triggered primarily by coughing. Vital signs on admission were as follows: a blood pressure of 202/90 mmHg, a heart rate of 137 bpm, a respiratory rate of 20, and a temperature of 36.6 Celsius. Physical exam revealed Janeway lesions. She was found to have a neutrophilic leukocytosis, with white blood cell count at 20.4 cells/mm3 and neutrophils at 17.4 bil/L. Troponin was elevated at 1.85; this was deemed to be noncardiac in nature as the patient's pain was relieved with ibuprofen and her EKG showed no acute findings. ESR and CRP were elevated at 95 mm/hr and 24.8 mg/dL, respectively. A computed tomography of the brain showed a high-density mass in the right occipital lobe, with surrounding vasogenic edema. The patient continued to deny any visual changes or symptoms other than what was discussed above. An ophthalmologist was consulted to perform a dilated fundus exam, which was positive for small intraretinal hemorrhages that were deemed to be secondary to the patient's hypertension and less likely positive for Roth's spots. There was no evidence of disc edema. A brain MRI with and without gadolinium showed multiple small punctate bilateral areas of acute or subacute infarctions indicative of embolic phenomenon. The hemorrhagic area in the right occipital lobe was again identified, with subtle surrounding enhancement; the differential diagnosis consisted of neoplasm, vascular malformation, or embolic infarction with hemorrhagic conversion. A transthoracic 2D echo was without vegetation, so a transesophageal echo was ordered, and vegetation was shown on the posterior leaflet of the mitral valve. Two blood cultures from admission then came back positive for Rothia dentocariosa. Infectious disease was confirmed, and the patient's current antibiotics, which consisted of vancomycin and ceftriaxone, were switched to penicillin G on a continuous pump. The patient remained largely asymptomatic during her admission and was deemed to be stable for discharge from the hospital after a nine-day stay with penicillin G via a continuous pump for a total of six weeks and was planned for a follow-up MRI in three weeks. The repeat MRI came back showing new subacute strokes. The patient was reported, again, to be asymptomatic but was directed to come straight to the emergency department. A repeat transesophageal echo was done and showed the known vegetation on the mitral valve with new vegetation seen on the PICC line and an abscess between the mitral and aortic valves extending into the ascending aorta. The patient then requested transfer to another institution for further evaluation. A repeat transesophageal echo was completed at this outside institution which showed small anterior and posterior mitral leaflet vegetation with no significant destruction and no abscess. A cardiac MRI was then performed which showed a focal delayed enhancement in the apical inferior and lateral wall, likely secondary to coronary arterial embolization. The patient went on to complete the full six weeks of penicillin therapy, remained asymptomatic, and refused a mitral valve replacement. Her follow-up was continued in the cardiology clinic. |
A 23 year old married female presented in the surgical OPD with complaint of pain in abdomen for past 1–2 years, and loss of appetite and vomiting post meals for 6 months. On observation, the patient had thin built and appeared pale with body mass index (BMI) of 13.3 kg/m2. On clinical examination, she was found to have a 7 × 3 inches large lump in the epigastric region which was freely mobile. Recent blood investigations revealed Hemoglobin to be 11.6 gm/dL with no electrolyte abnormalities. USG epigastrium revealed a well-defined mixed echogenic mass lesion predominantly hyperechoic in echotexture of size ~ 65 × 75 mm. A contrast enhanced CT scan of whole abdomen was performed which revealed over-distended stomach and large heterogeneous intraluminal mass with mottled appearance and small air pockets. Smooth wall thickening of stomach was also seen. A contrast enhanced CT scan of whole abdomen was performed which revealed over-distended stomach and large heterogeneous intraluminal mass with mottled appearance and small air pockets. Smooth wall thickening of stomach was also seen []. On interviewing, her mother mentioned about patient's habit of eating her hair since the age of 3 years. Hence, a diagnosis of trichobezoar was formed and patient was taken for surgical treatment. Laparotomy with gastrotomy and removal of trichobezoar under general anaesthesia was performed. Laparotomy with gastrotomy and removal of trichobezoar under general anaesthesia was performed. []. Postoperatively, patient was sent for psychiatric consultation.\nOn psychiatric evaluation, patient reported history of eating hair since childhood. She would keep hair in mouth when stressed but would not realize that she was swallowing the hair too. Parents made her aware about her hair eating behavior when she was around 6 years old. This behavior would increase whenever she was scolded or had an argument with her family. She also provided history of fainting always following stress without any associated injury, tongue bite or urinary incontinence; weakness and; off and on mood symptoms during childhood. Temporal lobe epilepsy was also ruled out. Childhood history points towards her being stubborn and short-tempered. However, no such symptoms were reported currently. Physical examination showed no bald patches on scalp suggestive of hair pulling.\nThe patient remembered her family environment during childhood as hostile. She remembered her father returning drunk from work and beating up her mother. She experiences similar behavior in her married life too; her husband and in-laws being unsupportive and criticizing her on small issues. Currently, the patient complained of sad mood, low interest in pleasurable activities, sleep disturbances, excessive worry for almost more than 6 months which has gradually been worse over a period of time, and during interview she was found to have depressed mood and affect with depressive cognition. A diagnosis of moderate depression was considered under psychiatry. She was prescribed fluoxetine 20 mg and low dose of clonazepam and was informed about her illness. She was counselled for developing healthy coping skills to deal with the stressful situations. Family members were advised to remain observant of her hair eating or any other unhealthy behavior. |
A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event. |
A 67-year-old man was admitted to our hospital because of liver dysfunction during a screening examination. Enhanced abdominal computed tomography (CT) revealed a hypervascular mass of 35 mm in diameter in the descending portion of the duodenum (Fig. ), and the left three sections of the liver were occupied by multiple cystic tumors with contrast enhancement of the cystic wall, 13 cm in diameter (Fig. ). A duodenal tumor was identified on gastrointestinal endoscopy (Fig. ), and a biopsy revealed a NET. The serum levels of insulin, gastrin, and glucagon were within normal ranges. CT did not initially reveal evidence of pancreatic invasion between the tumor and the pancreas; however, irregularities of the duodenal wall and swelling of the lymph nodes around the pancreatic parenchyma were observed. Thus, the patient was diagnosed with non-functional duodenal NET with multiple liver metastases, T2N1M1 stage IV (UICC 8th). In addition, CT revealed the anatomical variation of the CHA, which branched from the SMA and ran fully through the head of the pancreatic parenchyma (Fig. , Additional file Figure S1). The CHA branches into the left hepatic artery (LHA), the middle hepatic artery (MHA), and the right hepatic artery (RHA) (Fig. a, b). Furthermore, a developed gastric arterial arcade, 4 mm in diameter, was found between the left gastric artery (LGA) and the right gastric artery (RGA). The RGA was branched from a distal portion at a distance of 10 mm from the root of the LHA (Fig. ). Incidentally, we did not observe stenosis of the celiac axis due to compression by the median arcuate ligament. We planned PD and left trisectionectomy with caudate lobectomy combined resection of the tp-CHA with the preservation of the gastric arterial arcade in order to maintain arterial flow of the remnant liver, preserving the route of the celiac artery to the right posterior hepatic artery (RPHA) via the gastric arterial arcade from the LGA to the RGA, LHA, and RHA. If the hepatic arterial flow could not be maintained by this route, the preservation of the tp-CHA by separating from pancreatic parenchyma or arterial reconstruction using radial artery graft between CHA and RHA was planned. Four weeks after percutaneous transhepatic portal embolization, surgery was carried out.\nAfter laparotomy, the gastric arterial arcade was exposed and encircled, and the LHA, RHA, and proper hepatic artery (PHA) were encircled (Fig. ). The LHA was divided at the distal side of the origin of the RGA. The MHA and the right anterior hepatic artery (RAHA) were also divided. The left portal branch and the right anterior portal branch were divided (Fig. ). The liver was transected, and the left hepatic duct and right anterior hepatic duct were divided. The left trisections and caudate lobe were anatomically resected. After clamping the PHA, the hepatic arterial signals of the RPHA via the gastric arterial arcade were confirmed by intraoperative Doppler ultrasonography (Fig. ). After trisectionectomy and caudate lobectomy, PD was performed. The pancreatic head was dissected from the SMA after the upper jejunum was divided. The pancreas was divided in front of the SMV. Finally, the specimen was only connected by the tp-CHA and the common hepatic duct (CHD) (Fig. ). The hepatic arterial signals of the RPHA was maintained after clamping the PHA. The PHA and the origin of CHA were divided, and the tp-CHA was taken out with the pancreatic head (Fig. ). The CHD was divided, and the specimen was removed (Fig. ). Reconstruction was performed via modified Child’s method. The operative time was 1072 min and the intraoperative blood loss was 3052 ml, and red blood cell transfusion was performed (1680 ml).\nPostoperatively, the patient developed pancreatic fistula (Clavien-Dindo IIIa) and biliary leak (Clavien-Dindo IIIa), and these complications were treated conservatively. There were no signs of hepatic ischemia. The patient was discharged on postoperative day 39. The pathological diagnosis was duodenal neuroendocrine tumor G2 with multiple liver metastases. The Ki-67 labeling index was < 20%, and staining for chromogranin A and synaptophysin were positive. There was no evidence of invasion of the pancreatic parenchyma; however, the duodenal tumor was confined to the MP layer, and one of the 25 examined lymph nodes was positive, and moderate lymphovascular invasion was observed. The final diagnosis was pMP, med, INFa, ly1, v2, pPM0, pDM0, and pEM0. The patient has shown no recurrence in the 22 months since the operation. Enhanced abdominal CT at 4 months after surgery revealed the blood flow of the RPHA via the gastric arcade (Fig. ).\nOver the years, several authors have described variations in the hepatic arterial anatomy; a CHA arising from the SMA—called the hepatomesenteric type—is a rare clinical entity. Yang et al. and Hiatt et al. reported that this condition was observed in only 31 of 1324 patients and 15 of 1000 patients, respectively [, ]. A CHA passing through the pancreatic head parenchyma, tp-CHA, is even rarer; Yang et al. [] reported that among 31 patients with the hepatomesenteric type, only 3 had this condition.\nWhen PD is scheduled in such patients with tp-CHA, it is important to maintain the arterial supply to the liver. Surgeons should preoperatively determine whether to preserve or perform combined resection of the tp-CHA. Tp-CHA preservation was selected in several previous reports [, , ]. This surgical procedure is technically feasible; however, there is a risk of a positive surgical margin or insufficient lymph node dissection and a tendency for increased intraoperative blood loss during the separation of the pancreatic parenchyma. If the tp-CHA is resected, reconstruction is usually necessary in order to maintain the hepatic arterial flow. Previous reports [, , ] have described successful arterial reconstruction after CHA resection during PD; however, such procedures are associated with an increased risk of thromboembolism, which can lead to a fatal outcome, especially in HPD []. In contrast, when collateral circulation develops, surgeons can perform combined resection of the tp-CHA, preserving the collateral circulation without arterial reconstruction. Several reports have recommended preoperative embolization of CHA in order to maintain the hepatic arterial flow through enlarged collateral arteries []. Although preoperative embolization can increase the liver arterial flow through collateral arteries, it is not routinely recommended because of the risk of complications, which includes the migration of embolic material [, ].\nA developed gastric arcade or pancreaticoduodenal arcade is frequently seen in patients with the stenosis of the CHA due to factors such as compression by the median arcuate ligament []. There are only a few cases in which the hepatomesenteric trunk and the tp-CHA and the association between the tp-CHA and the development of a gastric arterial arcade have not been reported. On the other hand, Miyamoto et al. reported the case of a patient with pancreatic head cancer with a CHA arising from the SMA who underwent radical PD combined with the resection of the CHA, in which the hepatic arterial flow was maintained via the gastric arterial arcade []. In this report, the patient did not have a developed gastric arterial arcade; however, the hepatic arterial flow via the gastric arterial arcade was sufficient and hepatic ischemia was not detected after the operation. Considering this case, even if the patients with tp-CHA do not have a developed gastric arterial arcade, surgeons may be able to preserve hepatic arterial flow via the gastric arterial arcade alone. If the hepatic arterial flow via the gastric arterial arcade alone is adequate after clamping the PHA, the combined resection of the tp-CHA can be considered, even if the gastric arcade is not developed before surgery. In cases in which the hepatic arterial flow is not adequate, the preservation of the tp-CHA or arterial reconstruction should be considered.\nWhen performing HPD, a PD-first procedure before hepatectomy is generally performed, as this approach is anatomically rational []. However, in the present case, performing hepatectomy after PD carried a risk of the arterial supply to the liver being reduced during hepatectomy. Had we chosen a PD-first procedure and the hepatic arterial flow not been maintained after CHA resection, it would have been necessary to perform arterial reconstruction before liver transection. This method is associated with a risk of injury to the reconstructed artery and thrombosis during liver transection. Given the above, we opted to perform hepatectomy before PD in our patient with a tp-CHA undergoing HPD.\nIn the procedure for separating the tp-CHA from the pancreatic parenchyma entirely, the surgeon should be concerned about the increasing rate of hemorrhage, surgery time, and the risk of injury to the tp-CHA. The surgical reconstruction of the hepatic artery when performing HPD is also associated with a high degree of risk. The association between tp-CHA and gastric arterial arcade was recognized on preoperative CT scans; the development of this collateral circulation may have the potential to prevent ischemia-related liver complications. From these points of view, the preoperative identification of the developed arcade of the gastric arteries helps in planning an appropriate operative procedure, and this procedure seems to be a viable and simple option. To our knowledge, this is the first report of PD combined with resection of a tp-CHA without preoperative embolization. Furthermore, this is also the first report of HPD for a patient with a tp-CHA. The preoperative identification of the developed arcade of the gastric arteries helps in planning the appropriate operative procedure when PD is scheduled for patients with a tp-CHA. |
The patient was a 21-year-old woman who suffered from numbness and weakness of the limbs. She had no abnormalities on blood tests or physical examination and was accordingly suspected of having a psychological disorder. On the same day, she was examined at a psychiatric clinic. She was diagnosed with a functional neurologic disorder triggered by psychological stress, such as conflicts with her supervisor at her workplace and her parents. She was treated with the anxiolytic alprazolam (1.2 mg). She then developed symptoms such as numbness of the tongue, headache, and difficulty opening her eyes. She also complained that her vision was unfocused and that she had noticed circular eye motions (nystagmus) in the mirror. The next day, she suddenly felt exhausted and was unable to walk unaided, whereupon she presented at the Department of Psychiatry. On examination, she sat slumped backward in a wheelchair and responded to questions with a slight nod of the head. When asked if there were any people she found disagreeable at her workplace, she suddenly raised her voice. She was thus provisionally diagnosed as being catatonic due to a functional neurologic disorder triggered by psychological stressors from her medical and occupational history. We admitted her to our hospital on the same day for a detailed evaluation. Immediately after admission, she responded only by nodding when spoken to, until her elder sister arrived to visit, whereupon she suddenly became excited and started crying and talking loudly. She later became unresponsive and started groaning as if she was in pain, crying out in a loud voice, and making backward jerking motions with her body. She had a temperature of 37.2°C, a pulse of 72 beats/min, and blood pressure of 106/67 mmHg. Her pupillary diameter was 5 mm, and the light reflex was normal bilaterally. The deep tendon reflex was normal, and the Babinski sign was negative. Blood tests revealed no abnormalities in liver or kidney function or electrolytes. Thyroid function indices (free thyroxine, free triiodothyronine, thyroid-stimulating hormone and thyroid-stimulating hormone receptor antibody (TRAb)) were within the normal range; however, serum anti-thyroglobulin antibody (TgAb) and anti-thyroid peroxidase antibody (TPOAb) were positive (630.0 IU/mL and 15.4 IU/mL). Magnetic resonance imaging (MRI) of the head region revealed no obvious abnormalities in the brain parenchyma, cerebral blood vessels, or intracranial structures. Electroencephalography (EEG) revealed widespread, low-voltage, slow waves between 5 and 7 Hz, with 4- and 15-Hz spindle waves concentrated in the frontal, parietal, and occipital regions. The frequencies of these waves remained the same after photic stimulation. Five days after admission, she showed no response to stimulation. We concluded that her altered consciousness was more likely to be due to an organic disease than catatonia; thus, she was referred to a neurologist. The patient also had pyrexia and nuchal rigidity after admission. Therefore, we performed cerebrospinal fluid (CSF) analysis, which showed a slight increase in monocytes (7 cells/µL) and normal glucose (0 mg/dl) and protein (41 mg/dl) levels. Herpes simplex virus antibody and herpes simplex virus DNA testing were not performed because we thought that the possibility was low due to the cell count, glucose and protein levels in CSF. TPOAb and TgAb in CSF were negative. CSF did not show albuminocytologic dissociation, with 7 cells/µL and protein at 41 mg/dl. Oligoclonal bands were not evaluated. Compound motor action potentials of the median and ulnar nerve were in the normal range, and sensory conditions of the median and ulnar nerve were in the normal range in a nerve conduction velocity examination (). Ultrasonography revealed distinct thyroid enlargement. Consequently, Hashimoto’s encephalopathy was initially suspected as more likely than infectious brain inflammation, such as herpetic encephalitis, and the patient was started on steroid pulse therapy (methylprednisolone at 1 g for 3 days). Her consciousness level improved soon after completing steroid pulse therapy, and by day 4 posttreatment (day 10 after admission), she was able to converse and eat. She underwent further neurological examinations because of the improved state of consciousness and complained of double vision and numbness of the left arm from the shoulder to the fingertips. She also had abductive movement disorder of the right eye, reduced grip strength (9.6/8.2 kg), and cerebellar ataxia (clumsiness while performing rapid finger-nose, heel-knee, and hand pronation/supination movements). At that point (11 days after admission), we started another round of steroid pulse therapy (methylprednisolone at 1 g for 3 days), which resulted in almost total amelioration of the abductive dyskinesia of the right eye (with just a small degree of double vision in the right field). Subsequently, she underwent rehabilitation, regained the ability to walk and was discharged from the hospital 35 days after admission. At discharge, she had improved and had no ataxia and only residual numbness in the left index finger. Her grip strength had also improved to 21.6/21.4 kg. Subsequently, CSF was negative for anti-N-methyl-D-aspartate receptor (NMDAR) antibody. However, serum anti-GQ1b and anti-GT1a antibodies were positive; therefore, the patient’s final diagnosis was BBE. She has not undergone chronic immunosuppressive treatment; however, she has not had a recurrence during the follow-up period (approximately 1 year). |
A 58-year-old male patient was admitted to our clinic with the diagnosis of remnant gastric cancer. Nine years ago, the patient underwent open subtotal gastrectomy and Billroth II reconstructive surgery due to stomach cancer. A biopsy of a suspected ulcerous lesion of the remnant stomach was made in the esophagogastroscopy for the evaluation of the patient who had radiotherapy and chemotherapy story after surgery. Laparoscopic complementary gastrectomy was planned in the patient whose biopsy pathology result was poorly cohesive carcinoma. The patient in the ASA-3 class with a history of diabetes mellitus and oral antidiabetic use was operated on. Pneumoperitoneum was formed with Veress needle from the left upper quadrant in the patient with a median incision on the upper abdomen. The 10-mm working trocars were placed under the umbilicus, the lower right quadrant and the lower left quadrant; the 5-mm working trocar was placed in the upper right quadrant. Because the liver was sticking to the diaphragm, the liver retractor was not used. Adhesions to the anterior abdominal wall were removed. The intestinal loop of antecolic Billroth II gastroenterostomy anastomosis was cut and sealed with linear staples. The greater curvature was released along with the remaining omentum. Lesser curvature lymph nodes were dissected and included in specimen. The oesophagus was cut and closed with a linear stapler. Subsequently, antecolic oesophagojejunostomy was performed intracorporeally with single layer 3/0 prolene. The jejunojejunostomy with linear stapler was performed between the place that was 100 cm distal after oesophagojejunostomy anastomosis and the loop that was 15 cm away from the ligament of Treitz. The specimen was removed by suprapubic route. The operation time was 390 min with bleeding 100 cc. The patient was discharged without any problems on the 10th day. The pathology was reported as a 3 cm × 1.5 cm poorly cohesive carcinoma type (signet ring cell form) lesion with a proximal margin of 2 cm and a distal margin of 7 cm. Proximal distal and radial surgical margin was reported as negative. There was extensive lymphovascular invasion. In total, 25 lymph nodes were removed from the patient, and 11 of these lymph nodes were positive. The medical oncology clinic initiated chemotherapy treatment for the patient. Chemotherapy was continued, and the patient was admitted to the emergency department due to the complaints of abdominal pain, nausea and vomiting in the 2nd post-operative month. Subsequently, computed tomography showed dilated terminal ileum, ascending colon, transverse colon and air–fluid levels. Diameter increase of 7 cm at its widest point in transvers colon was seen []. Laparotomy was performed due to the diagnosis of ileus. At the level of the splenic flexure at the colon, there was severe stenosis. The transverse colon near the splenic flexure narrowed in the shape of beak. There was no adhesion or abscess. When splenic flexure was mobilised, an iatrogenic colostomy occurred distal to the stenosis. The transverse colon was transected just before the colon stenosis, and the end colostomy was fashioned. The patient was discharged on the 17th post-operative day after discontinuation of abdominal drainage on conservative follow-up. The chemotherapy of the patient with good general condition is continuing during the 2-month follow-up. |
A 73-year-old woman with no previous comorbidities or family history of hematological disorders or hypercoagulability was admitted to the Infectious Disease Clinic due to suspected HFRS and dehydration. Two weeks prior to disease onset, she had been exposed to bank voles while cleaning out a cabin. For 6 days following disease onset, she had been ill with fever, chills, weakness, low urine production, and difficulties eating and drinking. The patient had positive serology for Puumala virus thereby confirming the HFRS diagnosis. Laboratory tests taken the day before admission revealed thrombocytopenia (platelet count: 48 × 10\n9\n/L), impaired renal function (creatinine: 278 μmol/L), and leucocytosis (white blood cell count: 14 × 10\n9\n/L). Upon admission to the hospital, the platelet count had increased to 61 × 10\n9\n/L and creatinine increased to 370 μmol/L indicating clinical progression to the oliguric stage of HFRS. However, the platelet levels decreased to 12 × 10\n9\n/L on days 8 to 9. The treating physicians decided to transfuse platelets on days 8, 9, and 10 due to the high risk of spontaneous bleeding. Despite transfusion with three platelet units, the patient remained severely thrombocytopenic with platelet counts below 50 × 10\n9\n/L during days 8 to 13. The case is summarized in\n. Criteria for disseminated intravascular coagulation (DIC) were fulfilled from day 8 (see\nfor an overview of criteria).\nOn day 13 (2 days after the last platelet transfusion and a platelet count of 27 × 10\n9\n/L), the patient falls ill with abdominal pain which increases in severity during the evening. An abdominal computed tomography (CT) shows congestion and ischemia in the terminal ileum due to a thrombus in the superior mesenteric vein (SMV) reaching up to the portal vein (PV). The hematologist advised against thrombolysis due to thrombocytopenia in combination with a known mild VHF, which could increase the risk of bleeding. A national coagulation expert is consulted for further advice, who recommends anticoagulant treatment with heparin in a “careful” dose. Heparin at a dose two-thirds the national recommended dose is initiated with the aim of APTT 1.5 times the baseline value (40–50 s). The patient therefore receives a bolus dose of 4,000 units heparin followed by transfusion of 24,000 units heparin per day. The following day (15), the patient has gastrointestinal bleeding and a decrease in hemoglobin values from 100 to 89 g/L. According to the surgeon consultant, the stasis caused by the SMV and PV thrombus damages the intestinal mucosa leading to the observed gastrointestinal bleeding. Heparin is therefore continued in the same careful dose and the patient receives one unit red blood cells (RBCs) that day and the following day (16). The following criteria had to be fulfilled before mesenteric phlebography and thrombolysis via catheter could be considered: (1) platelet levels greater than 100 × 10\n9\n/L, (2) no bleeding, and (3) the patient can tolerate a full-dose heparin. On day 19, the patient fulfilled these criteria and underwent mesenteric phlebography via interventional radiology. Using the percutaneous transhepatic route to the PV, a hydrolyser 7F, double lumen, over-the-wire thrombolysis (Hydrolysis, Cordis Europe NV, Roden, the Netherlands) was used to perform a mechanical thrombolysis of the PV followed by pharmacological thrombolysis with tPA (Actilyse infusion: 0.8 mg/hour). A notation from the surgery department states that the previous heparin treatment aiming for 1.5 times APTT had been unsuccessful in decreasing the size of the SMV and PV thrombus. A control angiography 6 hours postsurgery shows that the thrombus distally in the SMV has been removed. There is still a thrombus between the portal and the splenic vein; therefore, the catheter is moved further into the area of thrombosis and thrombolysis by Actilyse administration is continued. At this time, there is no contrast leakage as a sign of hepatic bleeding. The patient stays in the intensive care unit (ICU) with local hydrolysis via the catheter. The following day (20), the patient becomes hypotensive with systolic blood pressure down to 75, and has signs of peritonitis. The levels of the fibrin degradation product D-dimer increases to 20 mg/L and fibrinogen decreases to 0.69 g/L. A CT thorax/abdominal scan shows an ongoing expanding hepatic intraparenchymal arterial bleeding. In addition, the CT scan shows presence of pulmonary emboli. Since the patient has a propensity for bleeding and thromboembolism, arterial intervention via the femoral artery into the aorta and then out into the common hepatic artery with coiling was not an option. Instead an emergency surgery procedure is performed with ligation of the right hepatic artery in the hepatoduodenal ligament, which stops the bleeding. In addition, the anticoagulant therapy is discontinued that day and the patient is tended in the ICU in a respirator. On day 21, the anticoagulant therapy is readministered at a low dose. The patient is extubated on day 22 and the anticoagulant therapy is increased to a target of APTT 60s due to remaining portal thrombi and peripheral pulmonary emboli. On the evening of day 25, the patient develops acute dyspnea, and oxygen saturation decreases to 88% with 4 L of oxygen and tachycardia. A CT pulmonary angiography shows pulmonary emboli in the right and left pulmonary arteries and peripherally in the pulmonary lobe arteries. The patient is transferred that evening to the ICU with heparin treatment at a target of APTT 85s. An ultrasound of the peripheral extremities (day 26) shows bilateral deep vein thrombosis in the posterior tibial veins. Since the APTT remains difficult to adjust (ranging from over 180s to the therapeutic target of 80s) and the propensity to develop thrombosis despite anticoagulation, it is decided to change the treatment from heparin to low-molecular-weight heparin (Fragmin 16,000 IU/day) on day 32. After day 55, the patient receives warfarin as prophylaxis against further thromboses and is discharged to her home on day 61. To rule out other causes for the thromboembolic complications, she was tested and found negative for activated protein C resistance. |
An 83-year-old female presented to the cardiac clinic with complaints of neck pain and upper back pain for the past few weeks, associated with exertion and difficulty walking on a treadmill. She had no associated chest pain or shortness of breath. Her initial physical examination was normal, with a blood pressure of 110/60mmHg and a heart rate of 60 beats/min. Her physical examination was unremarkable. Her past medical history included diabetes and hypertension, treated with antihypertensive and oral hypoglycemic agents. Her family history was significant for a daughter with myocardial infarction at 59 years and diabetes.\nHer EKG showed normal sinus rhythm with no ST-T changes. Laboratory evaluation mild anemia with a hemoglobin of 9.7 g/dL (normal range: 12 to 15.5 g/dL), urea 52 mg/dL (normal range: 6 to 24 mg/dL) and normal creatinine levels. An echocardiogram showed no regional wall motion abnormality, mild concentric left ventricular hypertrophy, mildly dilated left atrium, mild to moderate mitral regurgitation, mild tricuspid regurgitation, and normal left ventricular systolic function with an ejection fraction of 55%-60%. The patient was provisionally diagnosed with Angina on exertion and essential primary hypertension. Since the patient could not walk on the treadmill, a pharmacological stress test with myocardial perfusion imaging was performed, which showed evidence of moderate to severe ischemia in the mid inferior region. Post-stress imaging, LV function was normal. Left heart catheterization was done. The origin of LAD was seen from the right ostium with 75% stenosis at the origin and then continued after traversing to the left side as a mid and distal LAD without any stenosis in mid and distal LAD. Right coronary arteriogram performed showed medium-sized codominant right coronary artery (RCA) multiple lesions (Figure ).\nDistal LAD was providing some collateral to the right coronary. The left main coronary artery (LMCA) is divided into LAD and circumflex (LCx). LAD was only seen up to the first septal, and then there was a diagonal branch with 90% stenosis (Figure ).\nThe patient was diagnosed with severe triple vessel coronary artery disease with an abnormal origin of LAD from the RCA. The patient was referred to cardiac surgery for coronary artery bypass graft (CABG). After the thorough preoperative assessment, the patient underwent CABG with four grafts. The postoperative period was uneventful. |
An 11-year-old boy, otherwise healthy (height 136 cm, weight 33 kg), presented with fever and headache of 5 days duration. He had purulent discharge from the right ear for the last 6 months. There was no history of contact with active tuberculosis. He was fully immunized for age and the BCG scar was present. He was gaining weight normally and was free of any significant illness, except for the chronic otitis media.\nOn examination, the child was drowsy, but comprehending well and verbalizing normally. His BP was 130/90 mm of Hg. The fundus was normal and there were no focal motor/sensory deficits. He had neck stiffness, but Kerning's sign was negative. There was attico antral perforation with purulent discharge from the right ear (unsafe chronic suppurative otitis media). The purulent discharge did not grow any microorganisms on Gram stain or culture (staining for acid fast bacilli was not done at this time). Blood counts showed a polymorphonuclear leucocytosis with an ESR of 100 mm in the first hour. Mantoux test was negative. His liver function and renal function tests were within normal limits, HIV was negative and hepatitis B surface antigen was negative. A provisional diagnosis of pyogenic meningitis/brain abscess was made. Lumbar puncture yielded clear fluid with normal tension, with total cell count of 335/mm, polymorphs 65%, lymphocytes 35%, protein 23 mg% and sugar 50 mg%. The cerebrospinal fluid culture was sterile. Contrast-enhanced computerized tomography (CT) scan of the head showed subdural empyema of 5-mm thickness in the right parafalcine region and evidence of right-sided mastoiditis. The temporal abscess was not evident on CT scan.\nThe child was given injection ceftriaxone and other symptomatic and supportive measures. After 2 days, weakness of the left lower limb was noticed (power Grade 2) with mild weakness of the left upper limb (grade 4) and left-sided pyramidal signs. Magnetic resonance imaging (MRI) brain with gadolinium contrast showed a peripherally enhancing lesion with thin walls in the right temporal lobe measuring 28 mm x 17 mm x 17 mm with surrounding perilesional edema with restriction of diffusion and a peripherally enhancing multiloculated subdural empyema in the right parafalcine region with a maximum thickness of 9 mm and a smaller collection in the right temporo-occipital region and evidence of right mastoiditis [Figures and ]. Antibiotics were changed to ceftazidime, vancomycin and metrogyl and a neurosurgical opinion was sought. The surgeon deferred active surgical intervention as the child appeared to improve with antibiotics. He became afebrile after 5 days of treatment and the lower limb weakness improved. The child developed persistent headache and low-grade fever on the 10th day and became drowsy. MRI repeated on the same day showed increase in the size of the temporal lobe abscess (48 mm × 46 mm × 43 mm) with surrounding edema and mass effect compressing the right lateral ventricle. The subdural collection also increased in size (17 mm) with midline shift. An emergency craniotomy was performed and 25 ml of brownish pus was drained and the lateral wall of the abscess was resected and sent for histopathologic study, Gram stain, AFB stain and culture. Aspiration of the subural empyema was not attempted as it was on the medial aspect, and the neurosurgeon opined that it was difficult to approach.\nGram stain did not show any organism and the bacterial culture was sterile. AFB staining showed 3+++ acid fast bacilli. The quantiferon gold test was found to be negative. The polymerase chain reaction for Mycobacterium tuberculosis could not be carried out due to technical reasons. Histopathology of the abscess wall showed granulation tissue formed by inflammatory cells, predominantly polymorphs with no evidence of granuloma.\nThe child was put on anti-tubercular treatment with 5 drugs (INH, rifampicin, ethambutol, pyrazinamide and streptomycin) along with steroids. Radical mastoidectomy was performed for the chronic mastoiditis, which showed partial erosion of stapedius. There was no pus and the ear was completely dry. The specimen was sent for AFB stain and culture, but did not show acid fast bacilli. On antituberculous treatment the patient became afebrile after 1 week and is without any neurologic deficits at present. MRI was repeated after 4 weeks, which showed a significant decrease in the size of the abscess []. The subdural abscess also became smaller, but was still visible on MRI []. |
A 41-year-old woman was admitted to our hospital with a complaint of severe headache. She had headache for 3 years and expressed it as throbbing pain in both temporal regions accompanied by nausea. This pain was exacerbated by physical activity and under sunlight. Symptoms tended to occur once in 1~2 months, lasted for 1~3 days and then disappeared. A week before admission to our hospital, her headache symptoms were different from those in the past. She felt a twinge it the right posterior neck for the first time, and subsequently experienced severe pain in the entire head, but no nausea, vomiting, or fever. She was admitted to the neurology department of an outside hospital and underwent computerized tomography (CT) angiography 2 days after the headache developed; no bleeding was noticed yet multifocal vasospasms of intracranial arteries were revealed (). She was diagnosed with status migrainosus; hence, steroid pulse therapy with oral beta-blockers and non-steroidal anti-inflammatory drugs were given to relieve headache. However, her symptoms did not improve but rather worsened. Several hours before admission to our hospital, she started complaining of nausea and vomiting as well as very sharp tearing pain in the neck and back. According to the analysis of a cerebrospinal fluid (CSF) specimen that was obtained via lumbar puncture in an outside hospital 3 days after symptom onset, the levels of white blood cells (WBC), protein, and glucose were 4/µl, 62 mg/dl, and 46 mg/dl, respectively. Other CSF profiles were not provided. She had a history of hypertension and her blood pressure was well controlled with regular antihypertensive medication. She denied any history of auto-immune or cerebrovascular diseases. There was also no special family history of migraine or autoimmune disease. On admission to our hospital, her blood pressure was 195/114 mmHg; pulse, 60 beats/min; respiration, 20 breaths/min; and body temperature, 36.6℃. Mental status was alert and oriented. Cranial nerve examination and motor and sensory functions were normal. No pathologic reflexes were found, yet neck stiffness was suspected. According to the routine blood tests, the only abnormality was that the WBC count was increased to 18000/µl. In the blood coagulation test, prothrombin time-international normalized ratio and activated partial thromboplastin time were 0.99 s (normal range, 0.00~1.20 s) and 28 s (normal range, 20.0~36.0 s), respectively; both values were within the normal range. Aortic CT angiography revealed no evidence of aortic dissection. We started steroid pulse and mannitolization therapy. On day 2 after admission, her headache was alleviated, yet pain in the neck and back rapidly worsened and subsequently spread to the whole spine. Additionally, marked neck stiffness was observed. On day 3 after admission, brain magnetic resonance imaging (MRI) showed no intracranial hemorrhage, such as SAH (). On the same day, transfemoral cerebral angiography (TFCA) revealed multiple ICVSs (). Two hours after TFCA, weakness of both legs [Medical Research Council (MRC) grade 0 or I], bladder distension, and sensory deficit below both nipples developed. On spinal cord MRI, we found spinal cord compression by SDH, which was located from the ventral lower cervical to mid-thoracic level (C7 to T6) (). In subsequent CSF examinations, the red blood cell count was 340000/µl and WBC count was 30/µl (polymorphonuclear leukocytes, 59%; lymphocytes, 29%; and mononuclear cells, 12%), CSF was red, and no color change was observed in the three-tube test. Emergent total laminectomy was performed through the posterior approach, which was started 7 h after paraplegia. While incising the dura, we found that the spinal cord was swollen and large hematoma was present in the subdural and subarachnoid spaces. The hematoma was evacuated and no vascular malformation was found. After surgery, pain was relieved. On postoperative day 5, sensory symptoms and muscle power of the left leg (MRC grade II) partially improved.\nUpon the request by the patient's guardian, the patient was transferred to another hospital on postoperative day 6. Three days after the transfer, the patient had weakness and alien hand syndrome of the left arm. Brain MRI showed bilateral multifocal acute infarction of the corpus callosum; the right side of the lesion was larger than the left side (). On CT angiography performed at the other hospital at 8 days after the transfer from our hospital, more exacerbated vasospasms were found in the anterior, posterior, and middle cerebral arteries than initial CT angiography after the headache onset. Upon medical treatment, symptoms in the left arm gradually improved. Four months after discharge from our hospital, she visited our out-patient clinic and no longer had any pain including headache. She did not complain of urinary symptoms yet complained of a tingling feeling in both legs. The muscle power of both legs and the left arm was approximately MRC grade IV, while the power of the right arm was MRC grade V, which represented considerable improvements in comparison with those parameters at the time of discharge from our hospital. Alien hand syndrome was also no longer observed.\nAbout 1 year after symptom onset, muscle power recovered to an almost normal level in both legs and arms. Follow-up magnetic resonance angiography revealed no vasospasms in any of the intracranial arteries (). |
Case 1. A 78-year-old man experienced a fall and hyperextension injury to his neck. Traumatic disc herniation caused central cord syndrome, the predominance of the arms weaker than of the legs. He underwent anterior discectomy. Postoperatively, his arm weakness resolved well. He was able to walk independently and use his arms purposely at time of discharge from the rehabilitation unit. Four months after injury, he developed painful swelling of the right hand. He reported severe pain, swollen joints, and the inability to use the right hand. His neurosurgeon ordered urgent magnetic resonance imaging when notified of his symptoms, which was reported as unremarkable. A chance meeting between the patient and the present author at the hospital led to the recognition of the acutely inflamed hand. C-reactive protein level and white blood cell count were normal. An urgent triple-phase bone scan revealed diffuse hyperemia in the right hand and wrist, with the left arm being normal. Clinical examination revealed a grossly swollen hand. The MCP and interphalangeal joints were contracted. He was unable to tuck and fist his fingers or passively extend to neutral at any of these joints. His shoulder range was restricted with a capsular pattern on the right. There was hypersensitivity to touch at the hand, with temperature fluctuations of hot and cold. There was pain with compression of the joints, the greatest at the MCP joints. The decision to treat immediately with prednisone was made. The patient kept a journal of his findings. At one week, the hand had returned to its normal morphology with no pain. The shoulder range of motion also improved. He tapered his analgesia until it was fully weaned over a short time period. After review, the patient was weaned off the prednisone over the next two weeks. The patient's hand remained normal, and swelling and pain did not reoccur (see ). |
A 60-year-old-male patient presented to the dermatology clinic due to a cutaneous lesion in the left iliac region. His history was insignificant and he had no family or genetic history of leiomyosarcoma. In present history, the lesion was itchy without any other symptoms and existed 20 years before the attendance. In the physical examination, we noted an ulcerative nodule with a 3 cm diameter, moveable with the skin and non-stick with the layer underneath (the muscles) [].\nSystemic investigations, including complete blood count (CBC), liver and renal function test, and blood sugar level were normal. With these clinical findings, Leishmania and squamous cell carcinoma were considered and a biopsy was required. An excisional biopsy was performed and sent to the pathology department, where one section measuring 3 × 9 × 9 was studied, Hematoxylin and eosin (H&E) sections showed ulcerated epidermis. Interlacing fascicles of elongated spindle-shaped cells with blunt-ended (cigar-shaped) nuclei invading the dermis and parts of subcutaneous adipose tissue. The nuclei revealed variable pleomorphism & hyperchromasia with scattered atypical mitosis. All the deep and lateral surgical borders were free of malignancy []. The section was also stained with (SMA) which is more sensitive to leiomyosarcoma and the result was positive []. Negative immunohistochemical staining for S100 and CD34 ruled out malignancies of nervous and vascular origins, respectively [, ]. Chest, abdomen, and pelvis CT with contrast were performed to investigate metastasis and they was normal [].\nWe referred the patient was referred to a senior surgeon to excise the nodule. The pathological examination and the surgical operation have taken place in Aleppo University Hospital. The lesion was eradicated with 3cm safety margins. The excision also included the major iliac muscle. Two localized transposition slides were made, after that, a drainage tube was put, and finally, it was closed by simple sutures []. The surgery was successful; the patient received the usual postsurgical antibiotics, anti-inflammatories and painkillers. And had been covered with antibiotics, anti-inflammatories, and painkillers after it []. The follow-up was twice a year in the first year and then once a year until now and no metastatic nor recurrence has been noted so far.\nA general physical examination of the patient including the surgical site to monitor any changes after surgery was performed at each follow-up visit. |
A 10-year-old boy presented to a retina specialist with symptoms of nyctalopia and decreased vision. He was referred by an optometrist after an evaluation that included an abnormal optical coherence tomography (OCT) scan. He was followed since the age of two by the optometrist and his examination was limited to refractive evaluation prior to this scan. His parents became concerned when he could not see objects directly in front of him and he frequently ran into household objects. They noted that he even ran into a tree while at a birthday party at the age of six, and has been followed by his primary care physician for multiple complaints. This history prompted further workup by the optometrist.\nThe patient had a relatively complex medical history, starting during gestation. During pregnancy, his mother had oligohydramnios and decreased fetal movements. At birth, the patient had joint laxity with associated hypotonicity. Shortly after an in-hospital birth, he required resuscitation due to a nasolacrimal cyst obstructing his airway, which was subsequently removed. Both physical and mental development were delayed, as he did not sit until eight months of age, the cruise until 14 months, and walk until 17 months. He had a history of growth hormone (GH) deficiency and delayed bone age, which, after discussion of the risks and benefits with the parents, were not treated with GH replacement. MRI of the brain at age two for GH deficiency revealed mild vermian hypoplasia among other non-specific findings. He had also had frequent illnesses, particularly ear infections with high fevers. He recently failed his hearing test at school but had not yet had a formal hearing evaluation.\nOn physical examination, visual acuity was 20/50 right eye (OD) and 20/40 left eye (OS) with an intraocular pressure (IOP) of 18 and 19, respectively. His refractive error was -2.25 OD and -2.50 OS. An informal illustration of visual field testing is shown in Figure . Blunted focal light reflexes were noted bilaterally. Trace posterior subcapsular cataracts were also noted, along with 1+ cells in the vitreous and optic nerve pallor. The cup-to-disc ratio was 0.2. The retinal vessels had 2+ arterial attenuation, parafoveal pigmentation with “bulls-eye” maculopathy, and diffuse retinal pigment epithelial mottling peripherally, as seen in Figure . OCT showed CME bilaterally, for which dorzolamide 2% gtt t.i.d. was prescribed (Figure ).\nApproximately six months after evaluation by a retina specialist and completion of a retinal dystrophy panel, the patient was referred to a pediatric genetic ophthalmologist. During the evaluation, the genetic pediatric ophthalmologist noted the patient's somatic features. The patient had downslanting palpebral fissures, a short upturned philtrum with a grimacing smile, and long eyelashes. Other somatic features included truncal obesity, hypotonia, and joint hyperextensibility. Despite previous delays, the patient had a period of catch-up growth and was intellectually high-functioning. Intelligence quotient (IQ) and other cognitive functions were intact and normal.\nInitial genetic studies showed a single gene variant of undetermined significance associated with Cohen syndrome. A more thorough evaluation to detect a second mutation including deletion/duplication variants was ordered, but was negative. |
A 49-year-old man was referred to us with a 3-day history of headache, pain and fluctuating weakness in the right arm, and a one-day history of acute dizziness, unsteadiness, and transient loss of vision in the right hemifield. He had hypertension, dyslipidemia, and 27-pack-year history of smoking. Six months earlier he was admitted to a local hospital due to pain and weakness when elevating his right arm. MR angiography showed a right brachial artery occlusion. Conservative treatment with aspirin 75 mg was initiated and the arm symptoms diminished.\nUpon arrival at our hospital, the patient stated that the pain in the right arm was exacerbated by elevation, such as sleeping with his arms overhead. There was no history of neck trauma. His additional medications included irbesartan in combination with hydrochlorothiazide 300/25 mg and simvastatin 20 mg.\nBlood pressure was not measurable and there was no detectable pulse in the right upper limb. On the left side, the blood pressure was 147/83 mmHg with a regular radial pulse of 72 beats per minute. Apart from left-sided hyperreflexia without Babinski sign, general medical and neurological examinations were unremarkable. Biochemical and hematological blood samples were normal.\nCT angiography showed a right-sided subclavian thrombus extending retrograde into the right carotid artery bifurcation and into the proximal segment of the vertebral artery, consistent with artery thrombosis (Figure A, B). The aortic arch was without signs of dissection or atherosclerosis. Brain MRI showed acute/subacute infarctions within the right cerebellum and in the superior parietofrontal lobe (Figure C, D), suggestive of emboli originating from the occluded subclavian artery. Imaging also showed bilateral cervical ribs (Figure A) with the right longer than the left, leading to reduced space between the cervical rib and anterior scalene muscle and compression of the right subclavian artery. Other possible explanations for cerebral thromboembolism were excluded by normal thrombophilia screening and transoesophageal echocardiography. Carotid Doppler examination showed that clots in the carotid were from propagating subclavian thrombus and were not anchored by plaque. Vessel wall inflammation and malignancy-predisposing thrombosis were excluded by 18 F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT).\nBased on the suspicion of cerebral thromboembolism originating from the occluded subclavian artery, low molecular weight heparin was initiated at 10000 units twice a day, followed by warfarin (target International Normalized Ratio 2,0-3,0).\nCT angiography performed twice over a period of 3 months following the initiation of anti-coagulation showed partial recanalisation in all affected arteries. Thrombus extension retrograde into the right carotid artery bifurcation was not present at the second CT angiography prior to surgery. Four months after the cerebral thromboembolism the patient underwent resection of the right cervical rib and first costa through a transaxillary approach. The cervical rib was attached to the upper surface of the first costa by a pseudoarticulation. Both the surgery and postoperative period were uneventful, and the combined anti-coagulant and anti-platelet therapies were continued postoperatively. |
A 27-year old male complaining of a dull pain in his lower left jaw referred to a private dental clinic. On clinical inspection, no swelling was detected on his face. Intra-oral evaluation showed a severely decayed second mandibular molar that was mesially tilted and had an occlusal amalgam filling; the tooth was strategic, as its absence would lead to free-end edentulism. The tooth was tender on palpation but was not responsive to cold and electric pulp testing. On periapical radiography fading of the lamina dura and periradicular bone trabeculation was evident around the apex of the severely curved roots and in the middle area surrounding the mesial root (); periradicular periodontitis subsequent to pulpal necrosis was the final diagnosis.\nThe treatment option was discussed with the patient; RCT encompassing the high possibility of instrument fracture due to the severely curved roots. He was informed that in case of instrument separation, periapical surgery should be planned which was also unpredictable because of the long roots, proximity of root apices with mandibular canal and low depth of the vestibule. He chose coronal sealing so that in case of failure, other low-prognosis options would be tried.\nAfter local anesthesia, all caries were removed and an access cavity was prepared. Then the tooth was isolated and the canal orifices were located. The distal canal was navigated with a #10 hand NiTi K-file (Maillefer, Ballaigues, Switzerland) and its working length (WL) was determined with an electronic apex locator (Root ZX, J. Morita USA, Inc., Irvine, CA, USA). Although the mesial canals were navigated to the similar WL as the distal canal, due to the relatively higher root curvature in the apical area and also the obtuse curvature of the mesial canals branching from the chamber floor, the largest file that could penetrate the mesial canals was #25. Even troughing of the mesial part of the chamber floor did not facilitate negotiating the canals; the anatomy condemned these canals untreatable. All canals were irrigated with 5.25% NaOCl solution and the apical part of the distal canal was prepared to #25 with inter-instrumentation irrigation. For disinfection, canals were filled with a creamy paste consisting of minocycline (Razak, Tehran, Iran), ciprofloxacin (Amindaru, Tehran, Iran) and metronidazole (Parsdaru, Tehran, Iran) in equal proportions mixed with saline.\nTwo weeks later the patient was asymptomatic. The canals were irrigated, cleaned and dried; distal canal was obturated with #25/0.04 master gutta-percha cone and lateral condensation technique using Roth 801 root canal sealer (Roth international LTD, IL, USA). For the mesial canals, calcium-enriched mixture (CEM) cement (BioniqueDent, Tehran, Iran) was mixed according to the manufacturer’s instructions and then placed in the coronal part of the mesial canals. The tooth was temporarily restored with Cavit (ESPE-Premier, Norristown, PA, USA) which was later replaced with amalgam permanent restoration ().\nDuring the next 12 months, the tooth was asymptomatic and functional; moreover, radiography displayed the reestablishment of the periodontal ligament and lamina dura (). |
An eight-year-old male presented to his pediatrician with a four-month history of wheezing and dyspnea on exertion. He was prescribed antibiotics and albuterol for presumed asthma with no improvement in symptoms. Following a failed inhaled corticosteroid treatment at his second outpatient visit, his pediatrician ordered posteroanterior (PA) and lateral chest radiographs. These revealed a radiopaque spring in the left main bronchus (Figures , ). The patient was transferred to the emergency department via private automobile by his mother and directly admitted to the pediatric service, after which otolaryngology was consulted.\nThe patient confirmed an unwitnessed aspiration of the metallic spring from a toy approximately four months prior to admission, although he did not alert his mother or pediatrician at the time. Immediately after the aspiration event, he did not experience respiratory symptoms and was able to perform his daily activities. His only complaint for some time was difficulty breathing with physical activity. Vital signs upon arrival to the hospital were as follows: heart rate was observed to be 82 beats/minute, respiratory rate was 20 breaths/minute, oxygen saturation was 100% on room air, and temperature was 36.8°C. Physical examination revealed symmetric lung inflation and unilateral, biphasic wheezing on auscultation. Coarse breath sounds were auscultated in the left upper lobe, along with decreased movement of air at the left lung base. No tactile fremitus was noted. Neither nasal flaring nor retractions were observed. All other systems were reviewed and found to be negative or non-contributory. The patient remained calm and cooperative throughout the evaluation. Medical history was significant only for attention deficit disorder.\nThe patient was started on intravenous dexamethasone and inhaled nebulized budesonide with the intention of reducing expected edema related to the prolonged retention of the foreign body, along with famotidine for reflux prophylaxis. He underwent direct laryngoscopy and rigid bronchoscopy for foreign body removal on hospital day 4. Pediatric otolaryngology encountered a metallic spring coated in thick, purulent secretions in the left main bronchus (Figure ). The spring was freely mobile and safely removed with optical alligator forceps through the bronchoscope. The extracted spring measured 1.3 cm in length and 0.6 cm in diameter. Small diffuse fragments of black material thought to be debris from the AFB were seen on the airway mucosa after extraction. Remnant mucus and small black particles were evacuated through the rigid bronchoscope using suction under direct visualization. The pediatric pulmonology team followed with a flexible bronchoscopy and left bronchoalveolar lavage (BAL). Fluid obtained during BAL appeared bloody and revealed normal cell count with differential: 68% neutrophils, 1% lymphocytes, 27% epithelial cells, and many red blood cells (RBCs). BAL fluid cell count was as follows: 300 white blood cells (WBCs) and 41,000 RBCs.\nFlexible bronchoscopy revealed extensive granulation tissue and edema deep in the left main bronchus. Stenosis related to edema and granulation tissue was measured to be 1.5 mm (Figure ). The bronchoscope was not passed through the stenotic portion to avoid irritating the granulation tissue and causing hemorrhage.\nAfter the spring was extracted, the patient’s biphasic wheezing resolved and his postoperative chest radiographs demonstrated normal left lung inflation. The patient was continued on IV dexamethasone until discharge on postoperative day 2. Flovent inhaler and famotidine prescriptions were given prior to discharge, and he was instructed to continue their use for six to eight weeks.\nPostoperative follow-up was conducted by pediatric pulmonology. Physical examination at both one month and three months postoperative revealed no wheezes or crackles on auscultation. The patient denied any shortness of breath or exercise intolerance. Follow-up pulmonary function testing (PFTs) indicated no evidence of obstructive or restrictive lung disease. Flexible bronchoscopy performed at three months to evaluate for airway stenosis showed great improvement compared to the previous perioperative flexible bronchoscopy. Only 10% obstruction of the left main bronchus remained. The mucosa appeared normal without any inflammatory changes or postobstructive secretions. No further flexible bronchoscopies were recommended by pediatric pulmonology. |
A 63-year-old man presented, in October 2009, with complaints of right sided hemiparesis. An MRI of the brain showed a large mass in the left frontal area () and a stereotactic biopsy showed that the tumor consisted of a diffuse proliferation of large lymphoid cells with frequent mitotic figures and necrotic cells (). A CD20 immunostain was strongly positive in the tumor cells (). A ki-67 immunostain showed a proliferation index of approximately 90% (). A bcl-2 immunostain was positive whereas immunostains for bcl-1, CD5, and CD10 were negative (not shown). A FISH study was negative for MYC translocation. The overall pathologic findings were diagnostic of a diffuse large B cell lymphoma. Workup for systemic disease including bone marrow biopsy, CT scans, and a lumbar puncture for CSF examination was all negative; therefore, he was diagnosed with primary CNS lymphoma (PCNSL). The workup for HIV disease was negative. The patient was treated with 8 cycles of high dose methotrexate, with significant improvement in his symptoms. He was given adjuvant radiation after completion of his chemotherapy and, in February 2010, acquired complete remission. A posttreatment MRI brain showed no recurrent neoplasm.\nHe stayed in remission until August 2012, when he presented with a palpable 1.5 cm nodule, in the lumbosacral region, the site of a previous lumbar puncture. A biopsy of the lesion showed a diffuse large B cell lymphoma, consistent with recurrence of the original CNS lymphoma. He underwent local excision followed by chemotherapy, in the form of 4 cycles of the R-CHOP (Rituxan, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) regimen, to treat the recurrent nature of his disease. Postchemotherapy consolidation radiotherapy of 3000 Gy in 250 cGy was directed at the lumbosacral spine region and the patient acquired complete remission, with the area showing no palpable mass, hypopigmentation, or wound dehiscence. At the last visit (on 17th of September 2013), the patient continued to be in remission. |
The patient, a 65-year-old woman, came to our private practice in 2010, with the complaint that she could not eat without discomfort in the mental foramen region due to the use of a mandibular complete denture. The patient had been using the same dentures for 30 years (Figures and ). On clinical examination, it was possible to feel the alveolar nerve on the crest of the mandible, and its compression during clenching usually caused the patient to feel pain. After imaging analysis (), a fixed full-arch rehabilitation on 3 straight immediately loaded implants was planned because the interforaminal distance limited the placement of four implants, according to the surgeon's experience.\nBefore the surgery, a new complete denture had to be made, with better and adequate vertical dimension, centric relation, harmonious tooth positioning, and lip support. With these parameters tested and approved by the patient and the operator, the surgical procedures were planned, using a multifunctional surgical guide to determine the best implant placement positions. After anesthesia with Articaine 4% 1 : 100,000 (DFL, Rio de Janeiro, Brazil), a crestal incision was made next to the emergence of the alveolar nerve, which we could feel by touch, to avoid any nerve damage caused by the scalpel. A full thickness incision flap was performed to expose the alveolar ridge; then, with the help of the surgical guide, the implant osteotomies were performed. The implant positions were tested with the parallel pins and the surgical guide before completing the osteotomies (). Three Titamax GT 3.75 × 11 mm implants (Neodent, Curitiba, Brazil) were inserted in the interforaminal region (Figures and ), with an insertion torque ranging between 60 and 80 N/cm.\nThe multifunctional surgical guide was used to help transfer the implant positions and register the vertical dimension and positions of the teeth. The day after surgery, the nickel-chromium metal bar, which had been made according to the position in the wax-up of the future teeth, was tried and sent for mounting the teeth, after being approved by the patient (). On the next day, the esthetic appearance, which provided adequate lip support, smile line, positioning of the teeth, and occlusal guidelines (), was approved. After patient and dentist approval, the prostheses were sent for full laboratory processing with acrylic. Finally, on the third day after surgery, the screw-retained full-arch rehabilitation on the three implants was installed (Figures and ).\nThe patient was instructed not to sleep with the opposing complete denture for 7 days, eat only soft foods, put ice bags on the surgical area for 48 hours, and take analgesic medication if necessary. The sutures were removed after seven days. The patient was recommended to return after 3, 6, and 12 months. The patient returned after one year for a clinical and radiographic evaluation (). After this, the patient returned seven years later, for the second follow-up. This clinical evaluation showed that the fixed restoration, screw-retained on 3 implants, did not show any type of failure in the teeth or the acrylic denture base. There was no bleeding on probing (2 mm probing depths around all implants), the appearance of the gingival tissue around the implants was very good, and there were no complaints about the fixed rehabilitation. In the evaluation by panoramic radiograph, there were no aspects of bone loss or bone remodeling around the implants (). Only a few adjustments on the complete dentures of the opposite arch were made to address minor discomfort and mucosal injuries. |
A 7-year-old male reported to the Department of Pedodontics and Preventive Dentistry, Dr. R Ahmed Dental College and Hospital with the chief complain of pain in the lower right posterior tooth. On examination grossly decayed 85 was seen and erupted 44 was seen [Pics and ]. IOPA X-ray [] of 85 revealed retained roots stumps of 85 with signs of periapical inflammation. The presence of a tooth bud of 45 was seen apical to the roots of 85. Crown formation of 45 was almost complete but root formation had not yet started. In addition, 44 fully erupted but no sign of root formation was seen. Clinically, no sign of mobility was seen in 44. The patient gave a history of intraoral swelling and pus discharge in 44 regions and shedding of the tooth a few months back.\nDiscussion: Rootless teeth are seen in cases with dentin dysplasia and localized odontodysplasia. In dentin dysplasia the condition is generalized and teeth are characterized by pulpal obliteration, normal eruption, multiple periapical radiolucencies, and early exfoliation. In localized odontodysplasia, teeth demonstrate a lack of healthy dental tissues. However, in this case, enamel and dentin were healthy and the condition was localized. The possible explanation as seen from the oral health status of a child could be that the tooth erupted prematurely due to inflammation in overlying bone probably due to decayed primary tooth. Camm and Shuler noted very early eruption of premolar in 5-6-year-old following early loss of abscessed primary molars.[] In these cases, erupted premolars showed mobility. Mulia et al. have stated that the rate of root resorption of the primary teeth affects the eruption rate but not the growth of the permanent teeth.[] Moreover, the acceleration of eruption occurs if there is extensive alveolar bone damage due to chronic inflammation originating from the primary teeth.\nThus, as seen in this condition, the tooth erupted prematurely but root was not formed.\nUnusual finding in this case as compared to other similar cases is the absence of abnormal mobility in spite of the absence of roots. A tooth crown can erupt independent of its root development and subsequently continue its root development independent from the progenitor tissue complex of the dental papilla.[] Since there was no abnormal mobility noted in this case, the decision to retain the tooth was made and the patient was kept on regular follow-up.\nDeviation in eruption timing from normal is usually seen but marked deviation from the normal trend may confuse a clinician and affect the treatment planning if not dealt with proper care. Since such cases are usually associated with systemic conditions, knowledge of such a case where no systemic abnormality is seen can help the clinician in proper diagnosis and thereby deliver appropriate treatment.\nThe authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.\nNil.\nThere are no conflicts of interest. |
A 35-year-old woman had presented in the outpatient services of psychiatry with multiple episodes of seizure for the past 6 years. The episodes of seizure were of generalised tonic-clonic type with a frequency of two to three episodes in a month. She was treated with phenytoin 300 mg/day with partial control of seizures (frequency of seizures was decreased). However, non-adherence to treatment resulted in worsening of seizures. She was diagnosed with hypothyroidism 2 years ago and was on thyroxine supplementation (50 μg/day). There was no history of any other medical illness. There was no history of psychiatric illness or substance use. Her family history was also uneventful. Premorbidly, she was well adjusted to life. She had expressed her concerns and worries related to seizure over the past few months prior to psychiatric consultation. Her distress was not persistent and often increased when seizures occurred more frequently. Current psychiatric evaluation did not reveal any significant psychopathology, other than apprehension about seizures.\nHer general physical examination and systemic examination did not reveal any abnormality. There were no focal neurological deficits. Her cognition was intact. Lobar function tests also did not reveal any abnormality. The patient was investigated for seizure. CT scan of head revealed calcification in bilateral basal ganglia, frontal lobes and cerebellar hemispheres (). On the mental status examination, she had an anxious affect and worries related to persistent seizure. She was diagnosed with Fahr’s disease. Her psychological symptoms were not persistent and mild in severity, so no psychiatric diagnosis was made. It was planned to keep the patient on regular follow-up to see the evolution of symptoms over time. Her routine haemogram, liver function tests and kidney function tests were within normal limits. Her serum calcium (ionic) was 2.84 mg% (normal range: 4.5–5.5 mg%). Parathormone (PTH) level was 14.37 pg/mL (normal range 12–88 pg/mL). There was increased phosphate level (6.43 mg%; normal range 2.5–4.5 mg%) with normal level of serum albumin and alkaline phosphatase. Her seizure was treated with sodium valproate 600 mg/day in divided doses. She was advised to remain adherent to treatment. Supportive counselling was done. The patient was referred to endocrinology unit for management of hypoparathyroidism. During follow-up, she reported adequate control of seizures and her psychological distress was also reduced significantly. |
A 68-year-old male patient with a medical history of long-standing hypertension, ischaemic heart disease, obesity, type 2 diabetes mellitus and rheumatoid arthritis was referred by his general practitioner to the hypertension clinic in May 2007, due to uncontrolled hypertension despite three antihypertensive agents at maximum tolerated doses (ramipril, felodipine and atenolol). His mean daytime blood pressure at the time of referral was 168/99 mmHg. He was thoroughly investigated for secondary causes of hypertension, but none were found. His antihypertensive medication regimen was progressively optimised over many years, taking into account intolerances, adverse effects and co-morbidities. His medications at the time of the addition of canagliflozin can be seen in .\nIn view of apparent resistance to treatment, the patient was admitted to hospital for directly observed therapy in June 2013 to exclude non-adherence. His adherence was also confirmed on two separate occasions in 2014 when urinary drug screening became routinely available. Renal denervation (radio-frequency ablation of the renal sympathetic nerves surrounding the renal arteries) was conducted in October 2013. This had no effect, and in fact his blood pressure in June 2014 was higher with a mean daytime blood pressure of 181/105 mmHg.\nThe patient was started on the sodium glucose co-transporter 2 inhibitor canagliflozin 100 mg in May 2015 in view of a high body mass index of 38 and suboptimal diabetes control (HbA1c of 103 mmol/mol). This was uptitrated to 300 mg daily. A significant reduction in the patient’s blood pressure occurred with the best clinic reading being 137/80 mmHg. The HbA1c also reduced significantly (see ), although this cannot be solely attributed to canagliflozin, as the doses of metformin and gliclazide were also uptitrated. The patient did not experience any unwanted effects although the creatinine measurement did increase over this time. In line with manufacturer recommendations, the dose was reduced to 100 mg in view of the estimated glomerular filtration rate dropping to less than 60 ml/min. Blood pressure recorded in clinic at the end of July 2015 was 121/68 mmHg, which was the lowest it had ever been since 2007.\nAt this point the canagliflozin was stopped as it was not felt necessary for his diabetes control and because of the reduction in estimated glomerular filtration rate. In the next clinic in August 2015, blood pressure measurement was again high, the lowest being 151/87 mmHg. In view of the apparent remarkable effect on the patient’s blood pressure, a decision was made in conjunction with the patient to restart the canagliflozin at a low dose of 100 mg daily and cautiously monitor the kidney function. After re-starting the canagliflozin 100 mg in August 2015, the blood pressure reduced to 138/86 mmHg in the next clinic. The blood pressure remained well controlled at 136/83 at the clinic visit in November 2015. |
A 34-year-old woman with a mass in the right parotid area presented to the department of plastic surgery at our institution. The mass was approximately 1 cm in diameter, and the patient declined surgical excision due to the absence of clinical symptoms. However, during the second and third trimesters of pregnancy, the size of the mass increased to 5 cm (), and it became painful. The growth of the mass stopped after childbirth. Upon palpation of the mass, the patient reported pain radiating from the parotid region to the posterior auricular area. The lesion was warm to the touch, hard, pale-bluish, and lobulated. The patient agreed to undergo surgical excision of the mass due to the exacerbation of her clinical symptoms.\nUltrasonography was performed to determine whether the mass was a parotid tumor, and revealed a heterogenous mass separated from the parotid gland (). Contrast-enhanced computed tomography of the neck and ultrasound-guided aspiration biopsy were performed. At the right mandibular angle, it appeared as a well-defined, lobulated, and heterogeneously enhanced tumor with a calcified portion in the subcutaneous fat layer and the superficial musculoaponeurotic system (). A punch biopsy revealed features consistent with pilomatricoma, and no tumor cells were found upon ultrasound-guided aspiration biopsy of the cervical lymph nodes.\nUnder general anesthesia, total excision of the mass was performed through a skin incision (). The mass was located above the superficial musculoaponeurotic system, and it was measured to be 6.0×5.5×1.5 cm. A histopathological examination led to the diagnosis of pilomatricoma on the basis of characteristic findings, including an encapsulated tumor with a central area of abundant eosinophilic anucleated ghost cells and a peripheral area of blue, round basaloid cells. Furthermore, multinucleated giant cells, along with multifocal dystrophic calcifications and focal foreign body reactions, were observed (). The patient recovered without complications and no recurrence was observed at a 6-month follow-up. |
A 54-year-old man presented to our hospital with redness, pain and blurred vision in both eyes in July 2019. In the previous three months, he was admitted to a local hospital with anterior uveitis and secondary glaucoma in both eyes and was given prednisolone and IOP-lowering eye drops. However, both eyes further deteriorated, and the patient presented with hypopyon and increased IOP. His medical history included a diagnosis of systemic DLBCL based on immunohistochemical analysis of an axillary lymph-node biopsy in December 2018. He has received six courses of chemotherapy (standard rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), and his general condition is now stable.\nHis BCVA was 20/100 in the right eye and 20/200 in the left eye, and his IOP was 42 mmHg and 45 mmHg in the right and left eyes, respectively. Biomicroscopic examination of both eyes showed severe anterior chamber reaction (keratic precipitates 3+, flare 1+, cell 4+) and a pseudohypopyon in the inferior angle (). The bilateral peripheral anterior chamber was obliterated, and the iris appeared thickened with nodular areas of infiltration. Dilation examination revealed an invisible fundus. B-scan demonstrated clear vitreous and an absence of fundus anomalies (). UBM revealed 360° thickening of the iris and ciliary body, plus peripheral angle closure (). Paracentesis of the anterior chamber and diagnostic vitrectomy were performed in the left eye on July 22, 2019. Liquid-based cytology revealed atypical lymphoid cells (), which were IgK and IgH on gene rearrangement analysis (). Brain magnetic resonance imaging was unremarkable. The patient was given a series of 0.4 mg MTX (the specific therapeutic regimen was the same as for Case 1). Following a single MTX injection, the patient felt the symptom of ocular pain was relieved, the BCVA improved to 20/63 and 20/100 in the right and left eyes, respectively, the IOP decreased to 13 mmHg in the right eye and 19 mmHg in the left eye, and the slit-lamp examination revealed the pseudohypopyon had disappeared. After that, the patient received 6 intravitreal injections of MTX and 3 courses of chemotherapy. However, the patient lost to follow-up after 3 months of treatment. |
A 17-year-old female was admitted to the hospital due to severe suicidality. At the time of admission she complained about an irritating feeling in her nose, which made her constantly grimace in the area around the nose. She was excessively worried about having a serious illness of her nose (secondary hypochondriacal delusions) and was suicidal as a consequence. Her belief persisted even after any underlying medical condition of the nose has been ruled out by extensive medical examinations. She also presented with disorganized behavior, stereotypical movements, emotional instability and lability, and a below average level of intelligence during hospitalization. On the PANSS, her symptoms scored 29/23/70 (for the Psychotic, Negative and General Psychopathology Scale, respectively). Brief neurological examination revealed no abnormal neurological signs. As ascertained by the history taken from the patient and her mother, she had a history of school phobia that began at the age of 12 years, emotional disorders, normal cognitive and physical development, and a three-year history of chronic headache. She managed to complete primary and secondary education with the help of school counseling services given to her on account of school phobia. She had not received any psychiatric care before the described admission. A diagnostic evaluation for chronic headache at the University Children’s Hospital was undertaken a year before admission.\nCalcium, phosphate and parathyroid hormone blood levels were normal. Vitamin D levels were decreased with decreased calcium levels in the urine. No signs of calcium depositions in organs other than the described brain regions were determined by ultrasound. Ophthalmological, ear-nose-and-throat examination and electroencephalography were also normal.\nDetailed neurological examination revealed dysfunction of pursuit eye movement, dystonic positioning of both arms when stretched ahead, discrete ataxia of the arms and legs, and a pathological extensor response of the left big toe.\nBilateral symmetrical calcification in head, body and tail of the caudate nucleus and ventral part of the thalamus were determined by computerized tomography (Figs. and ). MRI was preformed twice over a two-year period using the same protocol. Corresponding MR T1 sequences showed hyperintense calcifications in the same regions as those found on CT examination (Figs. and ). Both MR examinations showed a high signal of calcified areas on T1 weighted sequences due to the surface area of calcium crystals. The same areas had an isointense signal on T2 weighted sequences. No other abnormalities of the brain were detected on the MRI.\nDuring psychometric evaluation, the patient’s cognitive abilities were assessed with RPM, TOL-II, d2, CTMT and Stroop tests. The patient performed significantly worse than her normative age group in terms of general cognitive abilities, coming in below the 10th percentile. She was unable to perform problem-solving operations that require abstract thinking. Assessment of her attention performance showed below-average results in scanning and alternating attention. She also showed below-average performance in her sustained and divided attention, with her concentration performance in the 8th percentile. Her planning abilities were significantly worse in comparison with her normative group, where she was unable to construct a problem-solving strategy. Her approach was a trial-and-error strategy and she failed to solve the problem within the time limit. The patient had no significant difficulties with inhibition of dominant response, reaching a borderline average result. We performed a retest after a year of treatment and the results showed no significant changes, although she was slightly better, but unfortunately without significant improvements, in her planning abilities and in her sustained attention.\nA whole blood EDTA sample was used for extraction of genomic DNA according to established laboratory protocols using the FlexiGene DNA isolation kit (Qiagen, Germany). Whole exome sequencing a trio (index patient and her parents) was performed in collaboration with NovoGene Corp. Inc. (Davis, CA, USA) using an Agilent Sure Select Human All Exon V6, 5191–4004 kit for whole exome enrichment preparation together with an Illumina Platform PE150 (Illumina, San Diego, USA) to perform the whole exome sequencing. Genetic variants with coverage >15x were analyzed using Variant Studio 3.0 software (Illumina). Evaluation of variants was firstly restricted to those located in eight genes related to Fahr’s disease (SLC20A2, PDGFRB, PDGFB, XPR1, KRIT1, SLC19A3, TREX1, MYORG). We reached 99.9% with at least 10X coverage for the patient. A search tool for the retrieval of interacting genes/proteins (STRING, ) was used to construct the protein-protein interactions that are involved downstream and upstream in Fahr’s syndrome (GNAS, ERCC8, PDGFB, CYP2U1, GNA11, SLC20A2, IFIH1, PSMB8, PDGFRB, CA2, ERCC6, SAMHD1, TREX1, CASR, TREM2, TYROBP, GJA1, ERCC3, FAM111A, RNASEH2B, SLC46A1, SLC7A7, ATP13A2, PARK7, HMBS, KRIT1). No causative mutations were found in the selected genes in the patient.\nComputerized tomography scans of the heads of the patient’s parents were normal.\nThe patient was treated symptomatically with quetiapine sustained release (initially 200 mg and gradually increasing to 900 mg daily) and sertraline (150 mg daily, gradually increasing to 200 mg daily). We did not observe any side effects with the use of quetiapine, although special attention was given to the possible exacerbation of extrapyramidal symptoms. Psychotic (PANSS scores at discharge were 8/13/27, PANSS scores after two years were 9/16/34 for the Psychotic, Negative and General Psychopathology Scale, respectively), affective and behavioral symptoms were improved; she was no longer suicidal and remained stable on gradually increasing doses of antipsychotic medication within two years of treatment, however, her intellectual abilities were not improved. Even though the patient completed secondary professional education and intense professional rehabilitation efforts were made, she has not been able to start working, mainly due to emotional instability. The patient was transferred to adult psychiatric services at the age of 21. |
An 87-year-old Japanese man complaining of abdominal distention was referred to our hospital. He had been surgically treated for early colon cancer and abdominal aortic aneurysm and nonsurgically for sigmoid colon volvulus. After being aware of insidiously presenting abdominal distention, the patient had failed to defecate for 12 h due to lack of intestinal peristalsis. He had no fever and complained of neither chills nor abdominal pain. Blood tests showed a mild inflammatory response and normal CPK level: white blood cells 10,200/μL, C-reactive protein 0.183 mg/dL, and CPK 89 U/L. Plain abdominal radiograph and enhanced CT showed a markedly dilated colon in the fashion of the so-called coffee bean sign (Fig. ). Blood test and enhanced CT findings (Fig. ) led to the diagnosis of sigmoid volvulus without colon necrosis. The patient underwent initial and careful endoscopic reduction (Fig. ), leading to the successful reduction of the sigmoid volvulus followed by the improvement of abdominal distention. Although pretreatment assessment was sigmoid volvulus without colon necrosis, postreduction endoscopy showed widespread presumed mucosal ischemia (Fig. ). Considerable spread of the affected mucosa made it difficult for us to rule out the presence of transmural ischemia, which could lead to the necrosis of the sigmoid colon followed by peritonitis. However, we judged that the patient lacked the definitive evidence of necrosis of the detorsed sigmoid colon and decided to follow the patient with infusion, nil per os, and antibiotics administration under careful observation. Due both to no relapse of the abdominal symptoms and to the lack of increasing inflammatory response judged by laboratory tests, the patient started to take a meal on the 5th day and was discharged on the 9th day after endoscopic reduction of the sigmoid volvulus. Two months later, i.e., after full recovery of his physical condition, the patient received endoscopic surgery with a 20-cm resection of the redundant sigmoid colon and a functional end-to-end anastomosis as an elective surgery to prevent further recurrence. He recovered uneventfully and was discharged on the 8th day after the operation. He has been well without any events for 20 months. |
The 73-year-old male patient presented to an emergency department with bilateral lower extremity weakness and abdominal pain. He was hospitalized and underwent extensive neurologic workup due to a rapidly progressive sensorimotor neuropathy. Diaphragmatic involvement and subsequent ventilator dependence required tracheostomy placement and prolonged hospitalization. The patient was determined to have an inflammatory neuropathy due to anti-neurofascin 155 antibodies. Treatment options for this pathology are limited and poorly defined, but theoretic benefit could be achieved via anti-CD20 biologic therapy with rituximab. A six-dose course was planned and initiated.\nThe hospitalization was complicated by voluminous respiratory secretions and three separate clinical syndromes consistent with ventilator-associated pneumonia. Despite aggressive nursing care, the patient developed a sacral wound which progressed to stage 3. Given need for multiple antibiotic and vasoactive infusions over the course of his stay, a peripherally inserted central catheter (PICC) was placed on hospital day fourteen. These infections created delays in biologic therapy for his inflammatory neuropathy. However, he was able to receive his second of six planned rituximab infusions on hospital day fifty-eight.\nOn hospital day sixty, the patient developed hypotension, hypoxia, and leukocytosis consistent with sepsis physiology. Two blood cultures were collected peripherally forty minutes apart as part of broad workup for decompensation. Neither was drawn from the PICC line. After twenty-one hours, the first culture resulted as Gram-positive cocci in clusters, and rapid multiplexed polymerase chain reaction (PCR) testing by the FilmArray blood culture identification panel (BioFire Diagnostics LLC, Salt Lake City, UT) suggested coagulase-negative staphylococcus with resistance due to mecA. No change in clinical management was made at that time, as the single blood culture demonstrating CoNS was suspected to represent sample contamination, and the patient was already being treated empirically with a regimen including vancomycin. However, within 72 hours of collection, both blood culture samples demonstrated growth with CoNS, which was identified as S. pettenkoferi by matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS). Both isolates showed susceptibility to vancomycin by Microscan testing (Beckman Coulter, CA, USA; see ).\nThe PICC line was discontinued on hospital day sixty-one with device tip culture also growing S. pettenkoferi. Serial blood cultures were persistently positive for S. pettenkoferi through hospital day sixty-three. He demonstrated clinical stability on vancomycin for treatment of S. pettenkoferi bacteremia, and culture clearance was achieved on hospital day sixty-five. Vancomycin was continued for a fourteen-day course from culture clearance and completed on hospital day seventy-nine.\nUnfortunately, his six-dose rituximab schedule continued to be interrupted by recurrent sepsis secondary to Proteus bacteremia by urinary source. He ultimately completed four doses of his planned six-dose rituximab course in the ICU and was transferred to the general medicine ward. At the time of manuscript submission, he was beyond ninety days of hospitalization and was yet to experience any neurologic recovery. |
A 62-year-old right-handed man presented to our clinic for advanced treatment options for WC. He had no previous injuries of the limb, no family history of dystonia or tremor. However, he did report repetitive use of the right hand with handwriting and shooting firearms. Initial symptoms occurred in his early 30’s and were characterized by hand cramping and abnormal penmanship when writing. The symptoms were intrusive and unremitting, and over the past nine years significantly interfered with his ability to handwrite with his dominant hand. These symptoms progressed over the past 5 years impairing his ability to perform any action that involved gripping objects. Consequently, he rarely used his right hand for daily activities. He had previously been prescribed tetrabenazine, baclofen, and had undergone botulinum toxin injections without significant benefit or unwanted adverse effects therefore medication was discontinued.\nOn examination, there was no abnormal posturing or tremor of the left upper limb, lower limbs, neck, face, and voice. At rest there was no abnormal posturing of the right hand. With the right hand held in pronation and full extension, the 2nd and 3rd digit flexed at the metacarpophalangeal joints. This abnormal posturing was also exhibited with the arms held in wing beating position (arms abducted with elbows at level of the shoulders and arms flexed with hand pronated and positioned just under the nose). In addition, in the wing beating position, there was slight flexion of the right wrist. No abnormal posturing was demonstrated in supination. These signs were most obvious with writing, but there was no abnormal posturing evident with the patient holding a writing instrument. However, immediately upon placing the writing instrument onto the paper and initiating writing, a greater extent of flexion of the metacarpophalangeal joints and wrist was evident. With continued writing, the thumb also extended and writing was characterized as having a jerky quality and was illegible. (). No mirrored movements of the contralateral limb were evident.\nThe consensus treatment plan from the patient care conference was to pursue DBS with surgical targeting of the VoA/VoP complex based upon literature review. Although there is limited data on thalamic stimulation, immediate tremor suppression intraoperatively has been reported. For these reasons, we initially planned on placing a Boston Scientific (Valencia, California) 2201 electrode with 8 vertically and evenly spaced contacts into the thalamus. The intention was that the contacts would span the VoA/VoP. The surgical approach has been previously described []. Two microelectrodes (AlphaOmega NeuroProbe) were simultaneously descended to target at 12.46 mm (X), 1.23 mm (Y), and 8.45 mm (Z) (tract 2) and 2 mm anterior from this tract (tract 1).\nThe first trajectory had minimal microelectrode recording (MER) consistent with thalamic cells. However, intraoperative electrophysiology was most consistent with STN recordings beginning at 2.3 mm above the intended target. Consistent with the motor territory of the STN, kinesthetic responses were evident from 1.6 mm above to –2.3 mm below radiographic derived target. Using the MER electrode that has a collar 3 mm from the tip of the electrode and can be used for stimulation, we tested macrostimulation of the STN at 1.5 mm above the intended target. STN macrostimulation provided an improvement in dystonic posturing and in writing tested by performing Archimedes’ spirals at 0.5 mA. The patient reported paresthesia in the right arm at 1mA that became intolerable and spread into his face as the stimulation intensity increased.\nThe second trajectory performed simultaneously with the first trajectory, which spanned the targeted brain areas showed kinesthetic responses, specifically active range of motion, during motor testing at 9.4mm, 5.3 mm, 2.3 mm, above intended target. This location was most consistent with the VoP. Macrosimulation at 3 mm and 5.5 mm above target provided less benefit as tested in posturing and in writing as well as greater stimulation adverse effects compared with track one macrostimulation . Given the beneficial effects of STN stimulation, the DBS electrode Boston Scientific 2202 with horizontal directional contacts of the second and third level was implanted with the tip at –3.5 mm below the intended target. Despite the clinical benefits, the patient experienced low threshold for stimulation induced adverse effects at 0.5 mA that was not overcome with various parameter adjustments and use of directional contacts. Consequently, the DBS electrode was removed and a third tract using MER was implanted.\nThe third track was implanted at 1.7 mm posterior and 1.7 mm medial of tract 2. Thalamic activity was recorded from 10 mm to 0.8 mm above radiographic target. Kinesthetic responses of passive range of motion of the upper limb were obtained from 4.7 mm to 0.8 mm above radiographic target (). No clear kinesthetic active range of motion of the upper limb was obtained in this tract consistent with VIM. Given the uncertainty of VoP compared with VIM stimulation, macrostimulation was applied at 7 mm, 4 mm and 2.3 mm above radiographic derived target. Greatest benefit was obtained at 4 mm compared with all macrostimulation tests of all tracts at 1.5 mA (). Furthermore, no stimulation adverse effects were obtained. Given that less benefit was obtained with more dorsal macrostimulation, the Boston Scientific 2202–45 electrode was implanted with the tip positioned at 0 mm of the intended target. Intraoperative testing of the DBS electrode demonstrated superior clinical benefit with only transient paresthesia up to 2 mA ().\nOne week post lead implantation, a Boston Scientific IPG battery (Vercise; Valencia, California) was placed in the left pectoral area and cervical extension wires were connected. Programming was initiated one month post lead implantation. and was initially programmed every one to two weeks for the first three programming sessions at University of Colorado Hospital. After the preliminary three programming appointments, his care was transferred to the Veterans Affairs (VA) Hospital where he is seen every four months. To assess efficacy, motor scales were collected in the form of Archimedes spirals, Writer’s Cramp Rating Scale (WCRS), and handwriting samples such as writing his name and the word “sunny” which caused him to continuously write without taking the pen off the paper to elicit dystonic posturing with sustained activity []. Although not validated, we did perform the WCRS that has been used in previous studies and provides an assessment of severity with higher scores indicative of worse performance []. There was a noticeable improvement in the WCRS motor scores from baseline, 18 score, at intraoperative programming, 5 score, and at four weeks post-operative, 4 score. Traditional monopolar review was performed at each level by increasing amplitude in increments of 0.5 mA with other parameters held constant at 60 µs and of 130 Hz. Upon demonstrating greatest benefit with ring mode stimulation of contacts 5–6–7–, monopolar review was then conducted of these individual contacts, which demonstrated monopolar stimulation of 6(–) as well as 8(–) provided marked benefit (). At 10 months postop, the patient is no longer taking medication for dystonia and his programming settings are: case (+), 8(–), 1 mA, 20 µs, 80 Hz. In a prior programming session, the patient was discharged to test monopolar stimulation of 6– and 8– with separate patient programs. The patient determined that 8– provided slightly greater benefit in tremor control compared with 6–. Testing was performed initially at 60 µs and 130 Hz. Subsequently, a range of frequencies (60 to 225 Hz) were tested. Based upon these findings, 80 Hz provided greatest benefit without inducing adverse effects. Thereafter, further benefit was obtained by increasing the amplitude but at the cost of worsening ataxia and paresthesia. Therefore, pulse width was reduced and the patient was discharged with and gradually increased stimulation to 2mA with his patient programmer. |
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