Congenital pouch colon: Case report and literature review

Abstract

Background: congenital pouch colon (CPC) is an extremely rare Congenital gastrointestinal pathology, in which the normal colon is either partially or completely replaced by a pouch-like dilatation communicating with the urogenital tract through a fistula. That's divided into 2 types: Complete CPC and Incomplete CPC. Worldwide and middle east Arabian ethnicity except for Indians, show high scarcity regarding the incidence. Herein, we report a case of incomplete congenital pouch colon syndrome with glandular hypospadias and cardiac anomalies that are considered to be the 2nd documented case in the middle east and the first in Westbank.

Case presentation: A 1-day-old newborn boy with prenatal history of abdominal cyst in 2nd trimester US, presented hours after birth with abdominal distention (Fig- 1), in addition to the imperforate anus. The abdominal x-ray showed many dilated bowel loops and gasless soft tissue density with calcifications on the right side (Fig- 2). Exploratory laparotomy was done and showed a pouch-like colon that later on was treated via 2 stages of operation.

Clinical discussion: early identification of CPC and differentiation from colon dilatation due to anorectal malformation is essential for the patient's welfare. CPC is more common in males, usually noticed in the neonatal period with abdominal distention, absence of anus, and intestinal obstruction. CPC is managed surgically depending on its type.

Conclusion: congenital pouch colon is a rare but important differential diagnosis of abdominal distention, which should always be at the back of the surgeon's mind especially when anorectal malformation is present.

Keywords: Anorectal malformation; Coloplasty; Congenital pouch colon.

Fig. 1

1-day old baby with markedly distended abdomen with right sided abdominal bulge with ill-defined mobile mass.

Fig. 2

Abdominal radiograph showing dilated bowel loops on left with soft tissue density occupying the right abdomen. black arrows show linear calcification laterally.

Fig. 3

Operative view of incomplete congenital pouch colon.

Fig. 4a

Colostogram showing contrast in urethra.

Fig. 4b

Retrograde ascending urethrogram showing recto-bladder neck fistula, black arrow shows contrast in the rectum, White arrow pointing toward the fistula that connects the rectum to the bladder's neck.