A Rare case of Type 4 Congenital Pouch colon without fistula with external anal marking in a female subject

Document Type: Case Report


1 Assistant Professor, Pediatric Surgery department, CNBC, New-delhi, India

2 DNB Resident, Pediatric surgery department, CNBC, New Delhi, India

3 Assistant Professor, Pathology department, CNBC, New-delhi, India


CPC is a rare reginal variant as per international Krickenbeck classification, in which colon (partly or
complete) is replaced by a pouch like dilatation, opening into urogenital tract via a large fistula. Theassociation of CPC without a fistula with ARM is very rare.
Type I: Absent large colon, with ileum opening directly into pouch.
Type II: The presence of short segment of caecum which then opens into pouch.
Type III: The presence of good length of colon between ileum and pouch.
Type IV: The presence of large colon with only sigmoid and rectum converted into pouch
A 2-day-old female baby presented with radiological features of pouch colon, which intra-operatively revealed type 4 CPC without fistula. The vestibule had two normal openings, with an external anal marking in perineum with puckering of skin but without any caliber. She underwent excision of pouch with end stoma and awaiting abdomino-perineal pull through at 6 months of age. To best of our knowledge, there is no case reported of CPC without fistula with external anal marking and puckering.