Congenital Pouch Colon.

Congenital pouch colon (CPC) is an unusual abnormality in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation. It is categorized into four subtypes (Types I-IV) based on the length of normal colon proximal to the colonic pouch. In males, the pouch usually terminates in a colovesical fistula just proximal to the bladder neck. In girls, the terminal fistula opens either into the urethra or in the vestibule, close to the urethral opening. Girls usually have a double vagina with a wide inter-vaginal bridge, a monocornuate uterus on each side, and urinary incontinence due to a widely open bladder neck. Associated major malformations are uncommon with CPC but sometimes, especially in reports from outside India, major abnormalities are present suggesting an early, severe error in embryogenesis. The more severe Types I/II CPC can usually be diagnosed by a large gas shadow or air-fluid level on X-Ray abdomen. For all subtypes of CPC, it is preferable to preserve a segment of the pouch by fashioning a narrow colonic tube for pull-through, the technique known as coloplasty or tubular colorraphy. Girls need additional management of the genitourinary abnormalities. Postoperatively, fecal continence levels are usually poor, especially with Types I/II CPC.