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Updated: Sep 20, 2025
Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment
Ruth A Lewit1, Korah P Kuruvilla1, Ming Fu1
1Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Sciences Center, 50 N. Dunlap Street, Suite 320, Memphis, TN 38105, USA; Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.
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.
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