Congenital Pouch Colon with Unilateral Renal Agenesis and Monorchism

BACKGROUND Congenital pouch colon, also known as congenital short colon or "Pouch colon syndrome", is a rare condition that occurs in association with anorectal malformations; colon is either partially or completely replaced by pouch-like dilatation and communicates with the urogenital tract by means of a fistula. This anomaly is exclusively seen in Northern parts of India with only a few cases reported from elsewhere. CASE PRESENTATION A 1-day old neonate was presented with abdominal distension due to lack of passage of meconium. Clinical and radiological investigations revealed ano-rectal malformation. Incidental findings were left sided renal agenesis and right sided anorchia. Laparotomy revealed congenital pouch colon which was dealt accordingly. The baby is now healthy and awaiting further reconstructive surgery. CONCLUSION Although urogenital anomalies are not uncommon with congenital pouch colon, the finding of renal agenesis with unilateral anorchia is quite rare.