Torsion of giant occipital encephalocoele in a 2-day-old infant: emergent surgery and outcome.

INTRODUCTION: Torsion of a giant occipital encephalocele leading to necrosis is an extremely rare complication found in neonates with only two such cases reported in literature previously. Infection and ulceration of the necrosed skin may lead to meningitis or sepsis. We present here a neonate with giant occipital encephalocele showing progressive necrosis during the first day of his life. CASE REPORT: A new-born baby, delivered vaginally, with no antenatal imaging was found to have a huge mass in the occipital region, which was covered by normal pink-purplish skin. During the first day of his life, the sac started becoming ulcerated accompanied with a rapid colour change in the skin, gradually turning darker and then black. The pedicle of the encephalocele was twisted with progressive necrosis of the encephalocele. MRI showed a giant encephalocele with single vein draining into the torcula and dysplastic occipital lobe herniating into the defect. The neonate was taken up for urgent excision and repair of the encephalocele. The encephalocele was excised 'in-total' with 'figure-of-8' repair of the meninges. One year after the operation, she appears to be well-developed without any neurological problems. CONCLUSION: Necrosis may have resulted from arterial or venous compromise caused by torsion of the pedicle during delivery or after birth. The high pressure inside the sac associated with the thin skin of the encephalocele may be another predisposing factor. In view of the risk of meningitis and rupture, immediate surgery with repair with minimal blood loss is indicated.

"Migratory Kerato osteomyelitis" - A refractory lesion of the mandible: A pathologist's and surgeon's dilemma!!

A 62 year old female patient presented with a chief complaint of non healing extraction socket in the lower left back teeth region. She underwent extraction of 37 at a private clinic following which an ulceroinfiltrative lesion developed at operated side. Multiple biopsies from the lesion were inconclusive. Computed tomography revealed an osteolytic lesion radiologically simulating carcinoma or a chondrosarcoma. Because of persistent debilitating symptoms the patient underwent Wide local excision (WLE) with left segmental mandibulectomy. Frozen sections were negative for malignancy. All margins were free from malignancy. Lymph nodes dissected showed reactive morphology. Ziehl-Neelsen, Periodic acid-Schiff and Gram stain were all negative. The lesion continued to spread even after successful surgical intervention with adequate surgical margin. The patient was eventually lost because of severe cardiac arrest during her last surgical intervention for mandibular arch reconstruction. As the lesion migrated from one side of the mandible to the other, kept showing clinical features of inflammation of the bone and bone marrow simulating features of osteomyelitis and the continued presence of keratin histologically, we contemplated whether we could give a nomenclature to the lesion as that of "Migratory Kerato osteomyelitis of the oral cavity." This could be one of case of a lifetime where multiple consultations with oral and general pathologists failed to reach a conclusive diagnosis!

Systemic mastocytosis associated with Hodgkin's lymphoma in a 4-year-old child.

Systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease (SM-AHN) represents a specific subtype of mastocytosis and is extremely rare in children. We describe a 4-year-old child with systemic mastocytosis associated with Hodgkin's lymphoma. The child had cutaneous mastocytosis and lymphadenopathy without other clinical features of SM, which was diagnosed only by bone marrow examination.

Thoracoscopy in stage 3 empyema thoracis in children - A safe and feasible alternative to thoracotomy.

BACKGROUND: Even though the role of thoracoscopy is well defined in Stage 2 empyema thoracis and is very popular, its role in the management of advanced empyema is still unclear. The technical difficulties and the potential complications are the principal reasons for the hesitancy in attempting video assisted thoracoscopic surgery (VATS) in advanced stages. METHODS: We prospectively studied the safety, feasibility and effectiveness of VATS for decortication in Stage 3 empyema. RESULTS: In the 61 cases that we attempted VATS over the last 7 years, we could complete the procedure in 45 patients (73.77%). Four children among them required re-do procedure later for persistent problems. Conversion to thoracotomy was needed in 16 patients (26.23%). The post-operative hospital stay of patients who underwent primary VATS decortication was significantly less when compared to patients requiring conversion (p < 0.0001). CONCLUSION: Thoracoscopy is a safe, feasible and effective option even in advanced empyema thoracis and should be offered in centers with adequate expertise and set up. LEVEL OF EVIDENCE: Level II.

Totally Implantable Venous Access Devices in Children Requiring Long-Term Chemotherapy: Analysis of Outcome in 122 Children from a Single Institution.

Children with malignancy require venous access that is reliable, safe and compliant on a long-term basis. There is little data available on utilization of totally implantable venous access devices (TIVAD) for long term chemotherapy in children in an Indian setting [1]. We report our long-term follow-up results of utilization of totally implantable venous access devices for long-term chemotherapy in children. This was a retrospective analysis of 122 children requiring long-term chemotherapy done between January 2008 and December 2013. Data collected included primary disease process, type of port, site of insertion, intraoperative events, early and late postoperative complications, and issues with utilization, maintenance and removal. 127 ports were placed in 122 children. The follow up ranged from 16 to 50 months. Internal jugular vein was accessed in 96.8 % of cases (123/127). Majority of children (61 %) had hematological malignancy. Early complications occurred in 5 children. Late complications occurred in 18 children which included port pocket infection in 3, port site skin issues in 5, catheter related issues in 3, venous thrombosis in 2 and catheter related bacteremia in 5 children respectively. Only 10 children have been lost to follow-up either due to death or discontinuation of treatment and rest are on follow up. Totally implantable venous access devices usage is safe and reliable for access needs in children for long-term chemotherapy. Their low complication and low cost maintenance should increase their utilization in children requiring long-term chemotherapy. Chemoport placement in children with hematological malignancy can be carried out safely without much impact on complication rates. Though management and compliance of children with malignancy has improved; critical analysis and standardization of port system care through prospective trials are necessary to reduce the morbidity and for cost analysis in these children.

Glandular diphallus with urethral duplication: Conventional technique for a rare congenital anomaly.

Diphallus is a rare anomaly and its association with urethral duplication is extremely rare. Numerous associated genitourinary and gastrointestinal anomalies have been reported with this condition. Challenges in the management are incorporation of the glans and the dominant urethra during reconstruction. We report the successful management of a case of glandular diphallus with complete urethral duplication retaining the dorsal urethra.

Quercetin does not alter the oral bioavailability of Atorvastatin in rats.

The study was undertaken to evaluate the effect of Quercetin on the pharmacokinetics of Atorvastatin Calcium. In-vivo Pharmacokinetic studies were performed on rats in a single dose study and multiple dose study. Rats were treated with Quercetin (10 mg/kg) and Atorvastatin Calcium (20 mg/kg) orally and blood samples were collected at (0) pretreatment and 0.5, 1, 1.5, 2, 2.5, 3, 4, 8, 12, 24 hours post treatment. Plasma concentrations of Atorvastatin were estimated by HPLC method. Quercetin treatment did not significantly alter the pharmacokinetic parameters of atorvastatin like AUC(0-24), AUC(0-α) , T(max), C(max) and T(½) in both single dose and multiple dose studies of Atorvastatin Calcium. Quercetin does not alter the oral bioavailability of Atorvastatin Calcium in rats.

Unicystic Ameloblastoma of Mandible Treated with an Innovative Approach: A Clinical Case Report.

Ameloblastoma is a true benign neoplasm with its origin from remnants of odontogenic epithelium. Unicystic ameloblastoma presents as a cystic lesion which clinically, radiographically, and macroscopically mimics a mandibular cyst, but microscopically exhibits ameloblastic epithelium lining part of the cyst cavity, with or without intraluminal growth and tumour infiltration into the fibrous connective tissue wall. An important and perplexing aspect associated with ameloblastoma is its management. We hereby present a case of unicystic ameloblastoma in a 63-year-old female and report an innovative technique of treating the case with split iliac crest graft.

Complications of chemoport in children with cancer: Experience of 54,100 catheter days from a tertiary cancer center of Southern India.

BACKGROUND: Chemoport is an essential part of the management of children with cancer and provides long-term venous access. There are few studies from resource poor countries reporting complications of chemoport. AIMS: This study was aimed at describing the complications of chemoport in patients with cancer. MATERIALS AND METHODS: This retrospective observational study analyzed 200 patients <15 years of age who underwent chemoport insertion. The medical records of these patients were reviewed for the patient characteristics, diagnosis, nature of port use, port-related complications and their management. RESULTS: A total of 209 ports were implanted in 200 patients and 24 ports were removed due to port-related complications. There were 122 boys and 78 girls whose ages ranged from 4 months to 13 years (median age 2.5 years). About72% of patients were <2 years old. The cumulative duration of catheterization was 54,100 days. Of 209 ports, there were 36 complications that led to the removal of 21 ports. Port-related infection was the most common infection observed in our study (0.66/1000 catheter days and 11.9%). Mechanical complications were seen in 9 patients. Venous thrombosis and skin necrosis occurred in one patient each. CONCLUSIONS: Use of chemoport is safe and is a boon for children with cancer in developing countries with incidence of complications similar to Western countries. Although use of chemoport is associated with complications, they are easily managed. With stringent catheter care by trained personnel, some complications can be prevented.

Triple A syndrome with ophthalmic manifestations in two siblings.

Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by adrenocorticotropic hormone resistant adrenal insufficiency, alacrima, achalasia of the esophageal cardia, progressive neurological degeneration and occasionally autonomic instability. We report the ophthalmic manifestations in 2 siblings from a consanguineous family with this syndrome. A routine ophthalmic examination showed absence of palpebral portion of lacrimal gland, dry eyes and sluggish pupillary reflexes in both eyes of these patients. Both of these patients had achalasia cardia while the boy additionally had increased serum cortisol levels. Topical ocular lubricants were prescribed for both of them and Heller's cardiomyotomy with fundus plication was done for achalasia. These cases would create an awareness of this rare condition in ophthalmology in addition to suggesting its management.

Cowper's syringocele in an adult.

Cowper's syringocele is cystic dilatation of the excretory bulbourethral gland duct. It is an uncommon condition and is usually reported in children. There are only ten previous case reports of late presentation in adulthood to the best of our knowledge. We present the imaging findings of such a case manifesting at 30 years of age.