Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 May;33(5):517-521.
doi: 10.1007/s00383-017-4065-8. Epub 2017 Feb 2.

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

Ankush Gosain  1   2 , Philip K Frykman  3 , Robert A Cowles  4 , John Horton  5 , Marc Levitt  6   7 , David H Rothstein  8 , Jacob C Langer  9 , Allan M Goldstein  10 ; American Pediatric Surgical Association Hirschsprung Disease Interest Group
Affiliations
Review

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

Ankush Gosain et al. Pediatr Surg Int. 2017 May.

Abstract

Background: Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC.

Methods: The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review, and expert consensus were then used to summarize the current state of knowledge regarding diagnosis, management, and prevention of Hirschsprung-associated enterocolitis (HAEC).

Results: Guidelines for the diagnosis of HAEC and its clinical grade, utilizing clinical history, physical examination findings, and radiographic findings, are presented. Treatment guidelines, including patient disposition, diet, antibiotics, rectal irrigations and surgery, are presented.

Conclusions: Clear, standardized definitions of Hirschsprung-associated enterocolitis and its treatment are lacking in the literature. This guideline serves as a first step toward standardization of diagnosis and management.

Level of evidence: V.

Keywords: Enterocolitis; Hirschsprung disease; Hirschsprung-associated enterocolitis; Hirschsprung’s disease; Hirschsprung’s-associated enterocolitis.

PubMed Disclaimer

Figures

Figure 1
Figure 1. Guideline for the diagnosis of HAEC
Guideline for the diagnosis and grading of HAEC from grade I (possible HAEC) through grade III (severe HAEC) based on clinical history, physical examination, and radiographic findings. This figure is not intended as a scoring system, but rather a decision-support tool to ensure that all of the relevant history, examination and radiographic findings are considered. In general, presence of higher grade findings should prompt providers to assign the higher grade. (HAEC: Hirschsprung-associated enterocolitis, DRE: digital rectal examination).
Figure 2
Figure 2. Guideline for the management of HAEC
Guideline for the management of HAEC based on grade. Considerations for diet, antibiotics, rectal irrigations, and need for surgery are listed by grade. (PO: per os, NPO: nothing per os, ICU: intensive care unit, IV: intravenous, IVF: intravenous fluid).
See this image and copyright information in PMC

References

    1. Haricharan RN, Seo J-M, Kelly DR, et al. Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg. 2008;43:1115–1123. doi: 10.1016/j.jpedsurg.2008.02.039. - PubMed
    1. El-Sawaf M, Siddiqui S, Mahmoud M, et al. Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: A prospective, randomized, double-blind, placebo-controlled,multicenter trial. J Pediatr Surg. 2013;48:111–117. doi: 10.1016/j.jpedsurg.2012.10.028. - PubMed
    1. Ward NL, Pieretti A, Dowd SE, et al. Intestinal aganglionosis is associated with early and sustained disruption of the colonic microbiome. Neurogastroenterology & Motility. 2012;24:874–e400. doi: 10.1111/j.1365-2982.2012.01937.x. - PubMed
    1. Pierre JF, Barlow-Anacker AJ, Erickson CS, et al. Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung's disease. J Pediatr Surg. 2014;49:1242–1251. doi: 10.1016/j.jpedsurg.2014.01.060. - PMC - PubMed
    1. Yan Z, Poroyko V, Gu S, et al. Biochemical and Biophysical Research Communications. Biochemical and Biophysical Research Communications. 2014;445:269–274. doi: 10.1016/j.bbrc.2014.01.104. - PubMed

Substances

LinkOut - more resources