Congenital Pouch Colon: Further Histopathological Perspectives

Abstract

Background: Congenital pouch colon is an uncommon anomaly worldwide and is usually associated with anorectal malformations. Imperforate anus with a large air fluid level on the abdominal x ray suggests the diagnosis. Most cases are diagnosed in neonates and an early management limit complications. Few studies have documented the histopathological features of congenital pouch colon. Case report: We present two cases with varied associated anomalies (Case 1 with rectovesical fistula, Case 2 with Mayer Rokitansky Kuster Hauser syndrome) and their histopathological features. Immunohistochemistry for calretinin showed paucity of ganglion cells and intrinsic fibers with occasional punctate positivity. The c-Kit immunostain documented fewer interstitial cells of Cajal. Cystitis glandularis with intestinal metaplasia (Case 1) and an additional muscle layer (Case 2) are described. Discussion/Conclusion: These novel histopathological features characterize the entity further and may be related to genesis of the pouch and its clinical manifestations.