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Established and emerging concepts in Hirschsprung’s-associated enterocolitis

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Abstract

Hirschsprung’s disease (HSCR) is a common cause of neonatal bowel obstruction and the approach to diagnosis and surgical treatment is well defined and accepted. Hirschsprung’s-associated enterocolitis (HAEC) remains a frequent cause of pre-operative and post-operative morbidity and mortality, with unchanged treatment guidelines over multiple decades. Recent advances in our understanding of the genetics underlying HSCR have allowed the development of animal models, some of which recapitulate the HAEC phenotype. These animal models, along with recent translational studies, have implicated multiple facets of mucosal immunity and microbiome dysbiosis in the development of HAEC. Here, we will review the established epidemiology, modes of diagnosis and treatment of HAEC. Furthermore, we will explore emerging concepts in the pathogenesis of this disease; including animal models, alterations in mucosal immunity, dysbiosis of the intestinal microbiome, specific genetic susceptibility, and novel treatment modalities.

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Acknowledgments

This work was supported by the National Institutes of Health (K08DK098271) and the American College of Surgeons (George H.A. Clowes Award) to AG.

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Authors and Affiliations

  1. Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Sciences Center, Memphis, TN, USA

    Ankush Gosain

  2. Children’s Foundation Research Institute, Le Bonheur Children’s Hospital, Memphis, TN, USA

    Ankush Gosain

  3. Pediatric Surgery, University of Tennessee Le Bonheur Pediatric Specialists, 49 North Dunlap, Suite 266, Memphis, TN, 38105, USA

    Ankush Gosain

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Correspondence to Ankush Gosain.

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Gosain, A. Established and emerging concepts in Hirschsprung’s-associated enterocolitis. Pediatr Surg Int 32, 313–320 (2016). https://doi.org/10.1007/s00383-016-3862-9

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