Congenital pouch colon (CPC), a condition associated with anorectal agenesis has been reported mainly from the Indian subcontinent though there have been few case reports from other areas. The pouch differs from a normal colon structurally, histologically and functionally. The management involves a diversion colostomy at birth with or without the excision of pouch followed by pull through. This review article attempts to compile all the available literature on pouch colon along with the authors' experience and discuss the relevant issues for proper diagnosis and management. A changing trend has been seen in the most common type of CPC seen over the years from complete congenital pouch colon that accounted for more than 70% of cases earlier to incomplete pouch colon that is more commonly seen now.

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Aim : The aim of this study was to analyze the clinical and management aspect of congenital pouch colon. Materials and Methods : This retrospective study was carried out on 40 cases of congenital pouch colon managed in the department of paediatric surgery from 01, January 2000 to 31, December 2005. Results : The incidence of congenital pouch colon (CPC) in the present study was 11.290 % (40 of 354) of all anorectal malformations (ARM) and 19.60% (40 of 204) of high ARM. Of these 40 cases of CPC included in the study, 82.5% were male and 17.5% were female. The average age of presentation to hospital was 3.36 days with a range of 1-11 days; of these 25% patients were admitted with poor general condition. Preoperative diagnosis of CPC was possible in 75% of cases in our study. 60% ( n =24) of cases had incomplete pouch colon (Type III and IV) while 40% ( n =16) had complete pouch colon (Type I and II). Right transverse colostomy was done in 16 patients and colostomy at descending colon just proximal to pouch in two patients for incomplete (Type III and IV) CPC as a primary initial procedure. Ileostomy was done in 14 patients and window colostomy in three patients for complete (Type I and II) CPC. As definitive procedures, pouch excision and abdomino-perineal pull-through of colon was done in 17 patients, coloplasty and abdomino-perineal pull-through of coloplasty colon was done in four patients and pouch excision and abdomino-perineal pull-through of terminal ileum in four patients in our series. None of our patients underwent primary single stage procedure. Conclusion: (1) CPC comprises 11.290 % of all ARM and 19.60% of high ARM. (2) Right transverse colostomy was opted for incomplete pouch colon, while Ileostomy and window colostomy were opted for complete pouch colon as initial surgical procedures. (3) Staged surgical procedures for management of CPC were well tolerated in our series.

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Accidental corrosive ingestion is not a rare occurrence in children because of easy access to strong household cleansers. Gastric injury as a predominant finding following acid ingestion in pediatric age group is less widely known with a few cases being reported in Indian literature. We report two such cases of gastric outlet obstruction following accidental ingestion of dilute acid. Both children have typical clinical presentation just 4-6 weeks after the ingestion. Upper GI endoscopy and barium meal studies confirmed the diagnosis. Early surgical intervention resulted in a satisfactory recovery. Both patients are doing well on follow up.

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Over a period of 5-year (May 2000 to April 2005) 29 patients of congenital pouch colon (CPC) were managed by single pediatric surgeon in the Department of Pediatric Surgery of a tertiary hospital. Of these, 11 were girls (M: F: 1.6:1). Detailed anatomy could be studied in nine patients, were included in this study. Age of presentation in female subjects ranged 1 day to 8 years. On examination, eight of the nine patients had single perineal opening suggesting a very high incidence of association of cloaca in female subjects with CPC. Four had short urogenital sinus with colonic pouch opening in the posterior wall of urinary bladder. In other two patients, CPC terminated in short cloaca. Anomalies of mullerian structures such as uterus didelphus and septate vagina ware commonly encountered. Proximal diversion with or without pouch excision was done as the initial preliminary treatment for all those patients who presented in early life. Of the nine girls, two died after the preliminary surgery. Only five patients have undergone definitive surgery. Definitive surgery included abdomino-perineal pull-through of proximal normal colon ( n =2), tubularization of pouch with abdomino- posterior sagittal- pull through ( n =2) and abdomino-posterior sagittal urethra-vaginoanorectoplasty with pull through of tapered pouch colon in one patient. Two of these patients had concomitant bowel vaginoplasty. Three patients with tubularized pouches had constipation and spurious diarrhea. However, good pseudo-continence of bowel was achieved on regular bowel washes. The cosmetic appearance of perineum in all these three patients was acceptable. The patients in whom pouch excision was done had diarrhea and severe perineal excoriation resistant to conservative management.

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Pediatric wandering spleen is a clinical rarity. Generally it remains asymptomatic, but may present as a painless migratory lump in the left hypochondrium. Rarely it may present as acute abdomen after undergoing torsion over its pedicle leading to infarction and gangrene. Available treatment options include splenectomy or splenopexy. Splenectomy at times is associated with post-operative infections. Splenopexy may result in recurrent torsion.

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Pyloric duplication is the rarest duplication in gastrointestinal tract. Only seven cases have been reported so far in the English literature. In most cases pre-operative diagnosis was not made. We present such a case in a 9-day-old baby, presenting with non-bilious vomiting since birth. The diagnosis was made pre-operatively by ultrasonography, which is a good investigation for pre-operative diagnosis. The case was operated successfully without opening the pyloric canal.

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Children with segmental dilatation of the colon suffer severe constipation and are clinically indistinguishable from Hirschsprung disease. Segmental dilatation of colon is rare in neonates. In this report, a neonate with unusual combination of segmental dilatation of the colon and high anorectal malformation is presented.

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Congenital pouch colon is considered to be a malformation seen only in north india. we carried out a prospective study of congenital pouch colon from 1991 to 2005 to determine what interventions are most suited for the short and long term management of this compex malformation. anatomical details, procedures and continence outcomes were recorded. patients were managed in a private and public hospital by a single surgeon. 17 patients could be enrolled in the study. we could reconstruct 16/17 patients. with aggressive bowel management, 9/14 patients could achieve continence.

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A Prune Belly syndrome with VATER/VACTERL association is an extremely rare. They are either stillborn or die within few days of life, only few such cases have been reported in literature. We are presenting here a male neonate of Prune Belly syndrome associated with incomplete VACTERL with brief review of literature.

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An association of coronal hypospadias with meatal stenosis and anterior urethral valves is described in this report. A combination of double obstruction in anterior urethra may overshadow the expression of proximal one.

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Congenital diverticulum of the anterior urethra in male is uncommon and typically occurs at the penoscrotal junction. This is a report of a large primary diverticulum in the anterior urethra in a 4-year-old male child who was successfully treated with diverticulectomy and urethroplasty.

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Klippel-Trenauny syndrome is a disturbance in the development of the mesodermal and ectodermal tissues occurring in utero, which is characterized by vascular nevi, varicose veins, soft tissue, and occasionally, bone hyperplasia. Our patient is a 6-year-old boy with presentation of left lower extremity over growth, abdominal mass, abdominal pain, bilateral buttock mass, rectal bleeding, and skin hemangiomatosis. The major problems of this case were involvement of the levator ani, external anal sphincter, and encasement of the sciatic nerves within the buttock mass. We concluded that the use of muscle and nerve stimulator for detection and saving sphincters and the nerves in these cases could improve the results of surgical resection.

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A 7-months-old male child presented with severe lower gastrointestinal bleeding. His radionuclide scan showed ectopic gastric mucosa in Meckel's diverticulum as well as in distal ileum. On exploration a 5 cm indurated bleeding ulcer found in ileum but no ulcer in Meckel's diverticulum or adjacent ileum. Histopathological examination of the specimen confirmed the diagnosis of ectopic gastric mucosa in ileum causing bleeding ulcer. Resection of the segment with end-to-end anastomosis cured the child. Such a synchronous lesion with Meckel's diverticulum has the potential to be missed, unless careful evaluation is done.

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The aim of this study is, could we transform demucosed colon into the small bowel? To find the answer, this study was investigated the surface changes of demucosed colon segment, interposed into the small bowel fashioned as a blind loop. This study was performed in five healthy young stray dogs. A blind loop was fashioned, using a 5 cm long demucosed colonic segment and a 20 cm long ileal segment. Ileal and colonic continuity were restored by primary anastomosis. Four weeks later, blind loop was resected and examined histologically under light microscopy. Histological findings revealed that demucosed colonic segment interposed into the small bowel fashioned as a blind loop, was covered with small bowel epithelium.

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