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Hirschsprung Disease

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Pediatric Neurogastroenterology

Abstract

Hirschsprung disease is a life-threatening birth defect defined by the absence of enteric nervous system (ENS) cells in the distal bowel. Since the enteric nervous system controls bowel motility and influences blood flow, immune system and epithelial function, bowel that lacks an ENS does not work well. Symptoms include intractable constipation, abdominal distension, growth failure, bilious vomiting, and enterocolitis (explosive and often bloody diarrhea) that predisposes to sepsis. There is remarkable variability in symptoms among affected children. Here we summarize and simplify the complex literature about Hirschsprung disease.

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  1. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Robert O. Heuckeroth

  2. Division of Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Robert O. Heuckeroth

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  1. Division of Gastroenterology, Department of Pediatrics, Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Montreal, QC, Canada

    Christophe Faure

  2. Department of Gastroenterology, Hepatology and Liver Transplant, Queensland Children’s Hospital School of Medicine University of Queensland, Brisbane, QLD, Australia

    Nikhil Thapar

  3. Division of Pediatric Gastroenterology, Nationwide Children’s Hospital, Columbus, OH, USA

    Carlo Di Lorenzo

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Heuckeroth, R.O. (2022). Hirschsprung Disease. In: Faure, C., Thapar, N., Di Lorenzo, C. (eds) Pediatric Neurogastroenterology. Springer, Cham. https://doi.org/10.1007/978-3-031-15229-0_26

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  • DOI: https://doi.org/10.1007/978-3-031-15229-0_26

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-15228-3

  • Online ISBN: 978-3-031-15229-0

  • eBook Packages: MedicineMedicine (R0)

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