Share this article on:

Inflammatory Bowel Disease Manifesting After Surgical Treatment for Hirschsprung Disease

Levin, David N.*; Marcon, Margaret A.; Rintala, Risto J.; Jacobson, Dustin*; Langer, Jacob C.*

Journal of Pediatric Gastroenterology and Nutrition: September 2012 - Volume 55 - Issue 3 - p 272–277
doi: 10.1097/MPG.0b013e31824f617a

ABSTRACT Eight children developed chronic inflammatory bowel disease (IBD) 4 to 21 years after surgery for Hirschsprung disease. Three had trisomy 21 and 6 experienced chronic or recurrent enterocolitis. Four had a family history of IBD. Clinical presentation included chronic diarrhea, hematochezia, abscess, and fistula formation. Three required surgery for fistula, stricture, and small bowel obstruction and the other 5 were managed medically. Recognition of this condition may be important in the long-term follow-up of children with Hirschsprung disease, and patients who have carried a diagnosis of chronic enterocolitis may warrant further investigation looking for evidence of IBD.

*Division of General and Thoracic Surgery

Division of Gastroenterology, Hospital for Sick Children, Toronto, Canada

Department of Paediatric Surgery, Hospital for Children and Adolescents, University of Helskinki, Helsinki, Finland.

Address correspondence and reprint requests to Jacob C. Langer, MD, Robert M. Filler Chair, Paediatric General and Thoracic Surgery, Rm 1526, Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8 Canada (e-mail:

Received 16 February, 2011

Accepted 29 September, 2011

D.L. was supported in part by a Comprehensive Research Experience for Medical Students bursary, University of Toronto, and in part by the Robert M. Filler Chair in Pediatric General and Thoracic Surgery, Hospital for Sick Children, Toronto.

The authors report no conflicts of interest.

Copyright 2012 by ESPGHAN and NASPGHAN