Congenital pouch colon is a form of anorectal malformation, rarely reported outside north India. Hallmarks of this
malformation are a short colon containing a large distal pouch with a fistula connecting to the urinary system. Herein, the
authors report the case of a Thai male neonate with a congenital pouch colon type II who was initially misdiagnosed as a
common imperforate anus. As a result, urinary tract infection and metabolic acidosis developed after a colostomy. A definitive
surgery consisting of a tabularized coloplasty and an abdominoperineal pull-through was performed at one month of age.
After closure of the colostomy, the child experienced transient loose stool with perineal excoriation for about three months and
then gradually improved. At three years of age, the patient had normal bowel movements and adequate sensation, and a
contrast enema showed a normal sized neorectum. An anal endosonogram revealed good localization of the rectum. A rectal
manometry showed spontaneous rectal contraction and a complete rectoanal inhibitory reflex. The present case provides
evidence suggesting that preservation of the native pouch colon is not contraindicated in this type of congenital pouch colon
syndrome.

Keywords: Congenital pouch colon, Pouch colon syndrome, Rectal manometry