Zollinger-Ellison Syndrome in a 12-year-old Child.

The syndrome described by Zollinger and Ellison in 1955 is a rare clinical entity which is even rarer in children. This report describes a 12-year-old boy who presented with refractory peptic ulcer disease which was finally diagnosed to be due to a gastrinoma and was successfully treated.

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.

Two-port laparoscopic management of a giant pseudocyst complicating ventriculoperitoneal shunt.

Insertion of a ventriculo-peritoneal (VP) shunt is the most common operative procedure for the treatment of hydrocephalus in children. Of the several causes of shunt malfunction, cerebrospinal fluid (CSF) pseudocyst is relatively uncommon. There are several modalities to treat a CSF pseudocyst. Laparotomy is required, at times, more than once. We managed a patient of CSF pseudocyst with two-port laparoscopy, by deroofing the psuedocyst and repositioning of the shunt. This minimally invasive technique avoids morbidity associated with laparotomy and aids in early recovery.

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.

Biliary fascioliasis: Management in a child using endoscopic retrograde cholangio pancreatography.

A 9-year-old boy presented with right upper abdominal pain and fever. The radiologic investigations revealed multiple cholangitic abscesses with cholangitis and worms in common bile duct. Endoscopic retrograde cholangio pancreatographic (ERCP) sphincterotomy, basketing, ballooning and extraction of Fasciola hepatica worms from the common bile duct were done.

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.

Minimal access surgery in newborns and small infants; five years experience.

AIMS AND OBJECTIVES: The aim of this study was to assess and present the outcome (initial experience and lessons learnt) of minimally invasive surgery for various indications in neonates and small infants (< 5 kg) at a single medical centre. MATERIALS AND METHODS: A retrospective analysis was performed on 65 patients (age day 2 to 10 months) managed with minimal access surgery (MAS) for various indications, between 2005 and 2010. We analyzed demographic information, procedures, complications, outcomes, and follow-up and overall feasibility of the procedure. RESULTS: No serious complications except one death in congenital diaphragmatic hernia (CDH) (due to other comorbidities) occurred. Intra operative hypercarbia and hypoxia were observed more frequently in thoracoscopic procedures. Intra operative hypothermia was not common and was well tolerated. Conversion to open procedure (n = 5), post operative ileus (n = 3), port site infection (n = 5) were other complications. CONCLUSION: MAS in neonates and small infants is a technically demanding but a feasible choice available. Some prior experience in older children is required for safe and effective outcome. Good quality optics, video equipments and instruments are required for safe and effective procedure. Intra operative measurement of oxygen saturation and temperature, and diligent post operative ICU care are mandatory for safe and successful outcome.

Traumatic diaphragmatic hernia: Management by video assisted thoracoscopic repair.

Report of the use of video assisted thoracic surgery for traumatic diaphragmatic hernia repair in two children.

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.

Role of hyperbaric oxygen therapy in severe head injury in children.

AIM: A brain injury results in a temporary or permanent impairment of cognitive, emotional, and/or physical function. Predicting the outcome of pediatric brain injury is difficult. Prognostic instruments are not precise enough to reliably predict individual patient's mortality and long-term functional status. The purpose of this article is to provide a guide to the strengths and limitations of the use of hyperbaric oxygen therapy (HBOT) in treating pediatric patients with severe brain injury. MATERIALS AND METHODS: We studied total 56 patients of head injury. Out of them 28 received HBOT. Only cases with severe head injury [Glasgow Coma Scale (GCS) < 8] with no other associated injury were included in the study group. After an initial period of resuscitation and conservative management (10-12 days), all were subjected to three sessions of HBOT at 1-week interval. This study group was compared with a control group of similar severity of head injury (GCS < 8). RESULTS: The study and control groups were compared in terms of duration of hospitalization, GCS, disability reduction,and social behavior. Patients who received HBOT were significantly better than the control group on all the parameters with decreased hospital stay, better GCS, and drastic reduction in disability. CONCLUSION: In children with traumatic brain injury, the addition of HBOT significantly improved outcome and quality of life and reduced the risk of complications.

Giant epignathus with midline mandibular cleft: Insights in embryology and management.

A congenital teratoid tumor arising and protruding through the mouth is classified as epignathus or fetus in fetu. On review of literature, we found various reports of midline mandibular and lower lip cleft associated with flexion contracture of neck, midline cervical cord, but there is only one report of association with midline dermoid. We present an unusual case of midline cleft of mandible with an epignathus. A 2.3-kg male child, delivered transvaginally in the 38(th) gestational week, was referred to us for management of a large irregular growth hanging outside the mouth. On examination, he had a wide median cleft of the mandible with tongue adherent to the "V"-shaped defect in the area of lower lip. A midline irregular mass of size 12 × 8 × 5 cm with variegated consistency was arising in the midline from the floor of the mouth between the tongue and lower lip. X-ray and computed tomography scan showed a rounded soft tissue mass arising from the alveolus with multiple calcifications within it along with a large triangular calcification and absence of hyoid bone. The mass was excised by mobilizing the tip of tongue. Staged repair was planned for the defect in the mandible. Unfortunately, the baby succumbed postoperatively to complex congenital heart disease. Histopathology was suggestive of epignathus. We discuss hereby the embryology and current management strategies of the problem.

An Unusual Presentation of Vallecular Cyst with near Fatal Respiratory Distress and Management Using Conventional Laparoscopic Instruments.

Vallecular cyst is a rare cause of upper airway obstruction in infants and children and presentation like acute stridor with near fatal respiratory distress is extremely rare. It is one of the rare causes of difficult intubation, during which cyst aspiration can improve the access. Vallecular cyst is commonly managed using microlaryngoscope and specialized instruments. We hereby report a method of endoscopic management of these cysts using conventional laparoscopic instruments.

Early experience with thoracoscopic repair of congenital diaphragmatic hernias in pediatric age group: results and lessons learned.

PURPOSE: To describe the surgical technique, initial results, and overview indications of thoracoscopic repair of congenital diaphragmatic hernia. PATIENTS AND METHODS: A retrospective review was undertaken of patients with CDH who underwent thoracoscopic repair from January 2006 to July 2010, in department of pediatric surgery in a tertiary care institute in India. Patients underwent surgery under general anesthesia. Reduction of the hernia contents was carried out using one trocar for telescope and two operating trocars. Pleural insufflation with carbon dioxide was maintained at a pressure of 6-10 mmHg. The hernia defect was repaired using nonabsorbable interrupted sutures. RESULTS: There were 17 patients, including 12 boys and 5 girls. Among 12 infants, there were 6 patients younger than 30 days. The other 5 patients were older than 1 year. The hernia was located in the left side in 14 patients and in the right side in 3 patients. The mean operative time was 110 min. Conversion was required in three patients. There were one recurrence and one postoperative death. CONCLUSIONS: Thoracoscopic repair is feasible and safe for children with CDH, including selective newborn. The technique causes minimal trauma, results in good respiratory function, and promotes early recovery.