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.