Congenital pouch colon: is it really a rare pathology?

Congenital pouch colon is a condition of a shortened and pouch-like dilated colon and it is usually associated with an anorectal malformation. The pathogenesis and embryology of congenital pouch colon are not well understood, but dietary, environmental factors and familial inheritance may be contributing factors in this pathology. Most of the cases in the literature have been reported from India. This increased regional incidence may be attributed to the lack of awareness of this pathology or its mislabeling rather than regional distribution. Congenital pouch colon is classified into four types based on the length of the abnormal colon. A variable dilatation of the rectum and sigmoid is always present in anorectal malformation. However, there is no clear definition of a limit for the dilatation of the rectum and sigmoid observed in anorectal malformation. Furthermore, many surgeons do not routinely take a biopsy from a dilated rectum or sigmoid during a colostomy procedure in anorectal malformation cases. For these reasons, type IV congenital pouch colon can be easily underdiagnosed. Surgical treatment options in type IV congenital pouch colon include resection of the affected sites of the colon or excisional tapering coloplasty. In the undiagnosed cases, congenital pouch colon results in severe constipation and overflow incontinence. We herein report two additional new cases of type IV congenital pouch colon.