Older age at diagnosis of Hirschsprung disease decreases
risk of postoperative enterocolitis, but resection of
additional ganglionated bowel does not
Ramanath N. Haricharan
, Jeong-Meen Seo
, David R. Kelly
Elizabeth C. Mroczek-Musulman
, Charles J. Aprahamian
, Traci L. Morgan
Keith E. Georgeson
, Carroll M. Harmon
, Jacqueline M. Saito
, Douglas C. Barnhart
Division of Pediatric Surgery, University of Alabama at Birmingham, Birmingham, AL 35233, USA
Division of Pediatric Surgery, Sungkyunkwan University School of Medicine, Seoul 135-710, South Korea
Department of Pathology and Laboratory Medicine, The Children's Hospital of Alabama, Birmingham,
AL 35233, USA
Received 5 February 2008; accepted 9 February 2008
Purpose: This study was conducted to determine the effect of age at diagnosis and length of
ganglionated bowel resected on postoperative Hirschsprung-associated enterocolitis (HAEC).
Methods: Children who underwent endorectal pull-through (ERPT) between January 1993 and
December 2004 were retrospectively reviewed. t Test, analysis of variance, Kaplan-Meier, and Cox's
proportional hazards analyses were performed.
Results: Fifty-two children with Hirschsprung disease (median age, 25 days; range, 2 days-16 years)
were included. Nineteen (37%) had admissions for HAEC. Proportional hazards regression showed that
HAEC admissions decreased by 30% with each doubling of age at diagnosis (P = .03) and increased
9-fold when postoperative stricture was present (P b .01), after controlling for type of ERPT, trisomy
21, transition zone level, and preoperative enterocolitis. Thirty-six children, with age at initial
operation less than 6 months, were grouped based on length of ganglionated bowel excised (A [5 cm]
and B [N5 cm]). No significant difference in the number of HAEC admissions during initial 2 years
post-ERPT was seen between groups A (n = 18) and B (n = 18). The study had a power of 0.8 to
detect a difference of 1 admission over 2 years.
Conclusions: Children diagnosed with Hirschsprung disease at younger ages are at a greater risk for
postoperative enterocolitis. Excising a longer margin of ganglionated bowel (N5 cm) does not seem to
be beneficial in decreasing HAEC admissions.
© 2008 Elsevier Inc. All rights reserved.
Hirschsprung associated enterocolitis (HAEC) remains a
serious and potentially life-threatening complication. First
described in detail by Bill and Chapman  in 1962, HAEC
can occur either preoperatively or after a definitive procedure.
Presented at the 59th Annual Meeting of the Section on Surgery,
American Academy of Pediatrics, San Francisco, CA, October 25-27, 2007.
Corresponding author. Tel.: +1 205 939 9688; fax: +1 205 975 4972.
E-mail address: email@example.com (D.C. Barnhart).
0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved.
Journal of Pediatric Surgery (2008) 43, 1115–1123