A newer variant of congenital pouch colon with rectal agenesis: management strategy and review of the literature

Abstract

A 3-day-old male baby presented with clinico-radiological features of rectal atresia with colo-urinary fistula. There was no radiological evidence of congenital pouch colon (CPC), but per-operatively CPC of unusual anatomy was found. The CPC was intermediate between type I and type II of Narsimha's classification. The pouch had rudimentary appendix and proximal colovesical fistula. The distal end of CPC was free. In the first stage, diverting ileostomy was done. In follow-up, after 1 month because of rectourinary fistula baby developed hyperchloremic metabolic acidosis and uremic sepsis. First metabolic correction and control of sepsis were done. Thereafter, abdomino-posterior saggital approach was used for excision of colovesical fistula, appendectomy, coloplasty and colorectal anastmosis. The ileostomy was left undisturbed. The infant at present is waiting for the third stage ileostomy closure.

Figure 1

Perineum showing normal anus and perineum.

Figure 2

Cross-table x-ray with Hagar dilator in anal canal showing high anorectal malformation.

Figure 3

Peroperative photograph showing rudimentary appendix (left lower horizontal arrow), ileum opening in pouch (right horizontal arrow), proximal colovesical fistula (upward directed vertical arrow), urinary bladder (downward directed vertical arrow), distal blind ended pouch colon (left upper arrow).

Figure 4

Micturating cystourethrogram showing colovesical fistula. Antero-posterior view (a), lateral view (b).