Pediatric Surgery International

, Volume 33, Issue 5, pp 517–521 | Cite as

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

  • Ankush GosainEmail author
  • Philip K. Frykman
  • Robert A. Cowles
  • John Horton
  • Marc Levitt
  • David H. Rothstein
  • Jacob C. Langer
  • Allan M. GoldsteinEmail author
  • On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group
Review Article



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.


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).


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.


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



Hirschsprung disease Hirschsprung’s disease Enterocolitis Hirschsprung-associated enterocolitis Hirschsprung’s-associated enterocolitis 


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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Ankush Gosain
    • 1
    • 2
    Email author
  • 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
    Email author
  • On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group
  1. 1.Division of Pediatric Surgery, Department of SurgeryUniversity of Tennessee Health Science CenterMemphisUSA
  2. 2.Children’s Foundation Research InstituteLe Bonheur Children’s HospitalMemphisUSA
  3. 3.Division of Pediatric Surgery and Departments of Surgery and Biomedical SciencesCedars-Sinai Medical CenterLos AngelesUSA
  4. 4.Section of Pediatric Surgery, Department of SurgeryYale University School of MedicineNew HavenUSA
  5. 5.Madigan Army Medical CenterTacomaUSA
  6. 6.Center for Colorectal and Pelvic ReconstructionNationwide Children’s HospitalColumbusUSA
  7. 7.Department of Pediatric SurgeryNationwide Children’s HospitalColumbusUSA
  8. 8.Department of Pediatric SurgeryWomen and Children’s Hospital of BuffaloBuffaloUSA
  9. 9.Division of General and Thoracic Surgery, Hospital for Sick Children, Department of SurgeryUniversity of TorontoTorontoCanada
  10. 10.Department of Pediatric Surgery, Massachusetts General HospitalHarvard Medical SchoolBostonUSA

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