Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is relatively rare but one of the most important causes of severe neonatal hypoglycemia. Recognition of this entity becomes important due to the fact that the hypoglycemia is so severe and frequent that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening event if not recognized and treated effectively in time. Near-total pancreatectomy may be required for patients with intractable hypoglycemia despite medical treatment; however, that may result in diabetes mellitus or recurrent postoperative hypoglycemia. This review aims to consolidate the traditional concepts and current information related to the pathogenesis and management of PHHI.

Aim : In children suffering from severe hepatosplenic schistosomiasis, the surgical protocol includes splenectomy and autoimplantation of spleen morsels in the major omentum, which has the potential of reducing the overwhelming postsplenectomy infection (OPSI). The purpose of the study was to evaluate the remaining splenosis, with the mean postoperative followup of 12.1 ± 5.6 years; to compare the efficacy of the 2 evaluation tools. Materials and Methods : Nineteen patients underwent, when they were children, portal decompression and autoimplantation of spleen. After a mean age of 23.4 ± 5.3 years, they were investigated regarding the number of infections and OPSI. They had undergone hepatosplenic scintigraphy with labeled colloidal SnTc ^99m and abdominal ultrasound. It was considered efficient splenosis when the patients presented with 5 or more spleen nodules. The evaluation was performed by 3 observers. Results : None of the patients had increased infection rate or developed OPSI. Sixteen (84.2%) presented efficient splenosis. Conclusions : The remaining postoperative splenosis was considered efficient in the majority of patients in the long-term followup; and nuclear medicine was considered the gold standard for splenosis evaluation.

Aims: To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages. Materials and Methods : A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed. Results: Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis. Conclusions: In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.

Aim: Our experience of 4 cases of urethral duplication is reported here. Materials and Methods: A retrospective chart review. Results: The age at presentation varied from newborn to 10 years. The clinical presentation ranged from prepubic sinus to diphallus urethra. There were 2 each incomplete duplication with only external openings (Type IA) and complete duplication of Effmann Type IIA2. All underwent complete excision of accessory urethra and corrections of associated anomalies. Conclusions: Urethral duplications have a varied presentation. At follow up, all are asymptomatic with good cosmetic result.

Aim : To report the surgical complications of Ascaris lumbricoides infestation in children. Materials and Methods : This is a retrospective study and cases of intestinal ascariasis managed conservatively were excluded. Results : Sixteen children presented with Ascariasis sequelae, which included ileal volvulus (n=5), perforations (n=4), intussusception (n=1), biliary ascariasis (n-1) and impacted multiple worm boluses (n=5). Plain abdominal radiographs showed pneumoperitoneum (3), cigar bundle appearance (3) and multiple air and fluid levels (13). Sonography showed floating worms with free fluid (2), sluggish peristalsis and moderate free fluid (7) and intestinal worm bolus (11). The surgical procedures included milking of worms (in all), bowel resection (6), closure of perforation (3) and manual reduction of intussusception (1). Biliary ascariasis was managed conservatively and the progress monitored with sonography. There were 3 deaths all of whom had intestinal volvulus, bowel necrosis and toxemia. Conclusion : Sonography can be helpful in diagnosing the presence of worms, its complications and in evaluating response to treatment. Early surgical intervention in those with worm bolus, peritonism, and volvulus may salvage bowel and reduce mortality.

Aim: To evaluate our results of definitive repair of anorectal malformations in patients with delayed presentation, during and beyond adolescence. Material and Methods: It is a retrospective analysis of all adolescent patients presenting for the first time for definitive repairs and innate patients - colostomy performed during the neonatal period, but who had lost to follow-up. It includes 15 patients (2 male and 13 female) aged from 13 to 32 years. Three well-decompressed female patients were managed by primary anterior sagittal anorectoplasty (ASARP). Twelve patients underwent staged procedures. Five patients (two male and three female) underwent posterior sagittal anorectoplasty (PSARP). The oldest male patient underwent abdominal-PSARP. Results: All of them attained socially acceptable fecal continence at follow-up of 1-4½ years. They are satisfied with the functional and cosmetic outcome of repair of their anomalies. Conclusions: Prospects of fecal continence are good when definitive repair of anorectal malformations is done by an expert, even in the adolescent age group and beyond.

Extrarenal calyces are one of the rare renal anomalies associated with the collecting system. Their association with renal ectopia or horse shoe kidney is known. But, here in, we are reporting an intraoperative surprise, where extrarenal calyces were associated with pelviureteric junction obstruction and routine preoperative imaging did not show any light on the diagnosis.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.

A rare case of Amyand's hernia in an infant who presented with chronic discharging scrotal sinus is reported.

Report of incidentally detected sewing needles on plain abdominal radiographs in two patients without any prior history of ingestion or of being pierced: one in the liver and the other in the peritoneum encased by omentum. These case reports point out a rare mode of entry of needles into the abdominal cavity by penetration from outside.

A child with isolated renal mucormycosis who was successfully treated with systemic anti-fungal therapy and nephrectomy is reported.

Two uncommon cases of locally invasive pulmonary inflammatory myofibroblastic tumor are reported. Diagnosis was established by a prior thoracotomy and incisional biopsy. Complete excision was curative and both children remain asymptomatic at last follow up.

A 2-year-old boy with heterotaxy syndrome with associated polysplenia, ventricular septal defect, and malrotation of gut with volvulus is presented. There was delay in diagnosis due to the unusual plain abdominal radiography findings. At laparotomy, the midgut was also found to be enclosed in a thin transparent cocoon.