Management options of congenital pouch colon--a rare variant of anorectal malformation.

Abstract

Congenital pouch colon (CPC) was analysed for anatomical variations and surgical options. Records of CPC patients managed between 1999 and 2014 were studied. CPC was classified as complete (CCPC) and incomplete (ICPC) pouch. Of 400 cases of high anorectal malformations, 68 cases were CPC (17%). Male:female ratio was 2:1. Fistulous communication was colovesical, colocloacal, colovaginal and absent in 42, 15, 8 and 3 cases. ICPC and CCPC was 48 (70%) and 20 (30%). In neonatal period, proximal/end colostomy (31), ileostomy (6), pouch excision with abdominoperineal pull-through (18 cases) and coloplasty with end colostomy (7) were done. 6 presented as infants including 3 referred cases of CCPC with a window colostomy. Definitive surgery was completed in 56. Severe colonic dilatation after coloplasty was noted in 5, requiring excision of coloplasty segment in 2. Histopathology of excised pouch (45) showed muscle layer disorganization, widened sub mucosa, prominent vasculature and mature and immature ganglion cells, with no hypertrophy of nerve fibres. Diarrhoea and faecal incontinence (soiling) were more frequent in patients with CCPC versus ICPC. Six neonates with CCPC died. Six are awaiting definitive surgery. CPC had 8.8% neonatal mortality. Pouch excision and definitive procedure are feasible in neonates with CPC. Coloplasty in CCPC may result in postoperative colonic dilatation.