Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis.
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.


METHODS
V.
Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, USA. agosain@uthsc.edu.|Children's Foundation Research Institute, Le Bonheur Children's Hospital, 50 North Dunlap, Suite 320R, Memphis, TN, 38,105, USA. agosain@uthsc.edu.
Ankush
http://orcid.org/0000-0002-0428-1503
A
Gosain
Division of Pediatric Surgery and Departments of Surgery and Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Philip K
PK
Frykman
Section of Pediatric Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.
Robert A
RA
Cowles
Madigan Army Medical Center, Tacoma, WA, USA.
John
J
Horton
Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, OH, USA.|Department of Pediatric Surgery, Nationwide Children's Hospital, Columbus, OH, USA.
Marc
M
Levitt
Department of Pediatric Surgery, Women and Children's Hospital of Buffalo, Buffalo, NY, USA.
David H
DH
Rothstein
Division of General and Thoracic Surgery, Hospital for Sick Children, Department of Surgery, University of Toronto, Toronto, Canada.
Jacob C
JC
Langer
Department of Pediatric Surgery, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Warren 1151, Boston, MA, 02114, USA. agoldstein@partners.org.
Allan M
AM
Goldstein
D000900:Anti-Bacterial Agents; D004760:Enterocolitis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications*; D006801:Humans; D007422:Intestines / Q000000981:diagnostic imaging; D008297:Male; D010808:Physical Examination; D017410:Practice Guidelines as Topic; D007507:Therapeutic Irrigation
D000900:Anti-Bacterial Agents
Enterocolitis; Hirschsprung disease; Hirschsprung-associated enterocolitis; Hirschsprung’s disease; Hirschsprung’s-associated enterocolitis