Neonatal Sigmoid Colon Perforation: A Rare Occurrence in Low Anorectal Malformation and Review of the Literature.

Gastrointestinal perforation in neonates with anorectal malformation (ARM) is extremely uncommon. Delayed patient presentation is an important causative factor. A 2.5-kg neonate presented 72 hours after birth with abdominal distention and absent anal opening with meconium pearls. An abdominal X-ray revealed the presence of free gas. After adequate resuscitation patient underwent surgery. Closure of the sigmoid colon perforation with a proximal diverting loop colostomy with anoplasty was done. The literature reveals only two cases of sigmoid colon perforation with low ARM. Ours is the third case, in whom repair of the perforation and correction of the ARM was managed successfully at the same time.

Pyloric atresia--three cases and review of literature.

Pyloric atresia (PA) is a rare congenital anomaly that constitutes approximately 1% of all intestinal atresias, and its incidence is approximately 1 in 100,000 live births. PA may occur as an isolated condition or associated with other abnormalities, the most common being Junctional epidermolysis bullosa (JEB). Evidence suggests that PA-EB (Pyloric Atresia - Epidermolysis Bullosa) Syndrome is a distinct entity. In this report, we present three cases of pyloric atresia, one of which was associated with Junctional epidermolysis bullosa. The literature on the subject is also reviewed.

Should the ovary always be conserved in torsion? A tertiary care institute experience.

AIM: The aim of this study was to analyze our experience in conserving ovarian tissue in cases of ovarian torsion, irrespective of grade of necrosis at exploration. MATERIALS AND METHODS: All children with a diagnosis of ovarian torsion admitted to our hospital from January 2009 to January 2013 were included. Patients with underlying ovarian pathology were excluded. RESULTS: There were 13 torsions in 12 children (one bilateral). All underwent detorsion with or without evacuation of hematoma. Follow-up ultrasonography (USG) with color Doppler was done for all 13 ovaries, which showed an ovary with good vascularity and follicular development in 12 ovaries (92%). In 76% (10 of 13) of cases, intraoperatively, the ovary was judged to be moderately to severely ischemic/necrotic. Yet, follow-up sonograms showed the ovary with follicular development in all cases except one (7%). There were no major complications in our series. CONCLUSION: Simple detorsion, instead of traditionally advocated oophorectomy, was not accompanied by an increase in morbidity. On follow-up, almost all patients studied had functioning ovarian tissue despite the grave ischemia observed intraoperatively. Detorsion should be the procedure of choice for all cases of simple ovarian torsion in children.

Trocarless laparoscopic pyloromyotomy with conventional instruments: Our experience.

BACKGROUND: The incidence of hypertrophic pyloric stenosis is approximately 1-3 per 1,000 live births. Hypertrophic pyloric stenosis is seen more often in males, with a male-to female ratio of 4:1. Laparoscopic pyloromyotomy is becoming increasingly popular as the standard treatment for hypertrophic pyloric stenosis. MATERIALS AND METHODS: We describe our initial experience with laparoscopic pyloromyotomy in 16 infants using conventional laparoscopic instruments. Laparoscopic pyloromyotomy was performed through 5-mm umbilical port with 5mm 30 endoscope. Two 3-mm working instruments were inserted directly into the abdomen via separate lateral incisions. RESULTS: All patients were prospectively evaluated. The procedure was performed in 16 infants with a mean age of 36 days and mean weight of 3.1 kg. All procedures, except two, were completed laparoscopically with standard instruments. Average operating time was 28 mins, and average postoperative length of stay was 2.8 days. There were no major intraoperative and postoperative complications. CONCLUSION: Laparoscopic pyloromyotomy can be safely performed by using standard conventional laparoscopic trocarless instruments.

Meckel-Gruber syndrome: A rare and lethal anomaly with review of literature.

Meckel-Gruber syndrome is a rare autosomal recessive lethal malformation characterized by typical manifestations of occipital encephalocele, bilateral polycystic kidneys and post axial polydactyly. The worldwide incidence varies from 1 in 13,250 to 1 in 140,000 live births. Highest incidence was reported in Gujarati Indians. We report a rare case of Meckel-Gruber syndrome and review of literature.

Solid pseudo papillary tumor of the pancreas: An unusual tumor in children.

An 11-year-old girl with a pancreatic solid pseudo papillary tumor (SPT) is reported. Contrast enhanced computerized tomography (CECT) & Magnetic resonant imaging (MRI) abdomen revealed a well-defined inhomogenous mass arising from the retro-peritoneum in the left upper quadrant of the abdomen possibly from the tail of the pancreas. USG guided biopsy of tumor showed benign pseudo papillary tumor of pancreas. Complete excision of the tumor was carried out. Histopathology revealed pancreatic pseudo papillary tumor. Patient did well postoperatively & is on regular follow-up.

Midline mature teratoma inside a ruptured meningomyelocele of lumbosacral region, a case report.

Meningomyelocele (MMC) is very rarely associated with teratoma. Only few reports on the concurrence of a teratoma within a meningomyelocele have been published. The hypotheses of a possible common aetiology for this association include a single dysembryogenic process and neoplastic transformation of heterotopic primordial elements incorporated in the defect. We report an unusual case of midline mature teratoma which presented inside a ruptured lumbar meningomyelocele.