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: email@example.com).
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.