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01-05-2017 | Review Article

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

Authors: Ankush Gosain, Philip K. Frykman, Robert A. Cowles, John Horton, Marc Levitt, David H. Rothstein, Jacob C. Langer, Allan M. Goldstein, On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group

Published in: Pediatric Surgery International | Issue 5/2017

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

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Title: Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis
Authors: Ankush Gosain
Philip K. Frykman
Robert A. Cowles
John Horton
Marc Levitt
David H. Rothstein
Jacob C. Langer
Allan M. Goldstein
On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group
Publication date: 01-05-2017
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Surgery International / Issue 5/2017
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-017-4065-8

At a glance: The STEP trials

Springer Medicine

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

Abstract

Background

Methods

Results

Conclusions

Level of evidence

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

Level of evidence

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