Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment

Abstract

Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald Hirschsprung and is a potentially deadly complication of Hirschsprung Disease. HAEC is classically characterized by abdominal distension, fever, and diarrhea, although there can be a variety of other associated symptoms, including colicky abdominal pain, lethargy, and the passage of blood-stained stools. HAEC occurs both pre-operatively and post-operatively, is the presenting symptom of HSCR in up to 25% of infants and varies in overall incidence from 20 to 60%. This article reviews our current understanding of HAEC pathogenesis, diagnosis, and treatment with discussion of areas of ongoing research, controversy, and future investigation.