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Pathophysiology of Hirschsprung’s Disease

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Hirschsprung's Disease and Allied Disorders

Abstract

Although the more striking histological feature in Hirschsprung’s disease is the absence of ganglion cells, it is unlikely that this is the only cause of the increased intestinal wall tone provoking a functional intestinal obstruction. In the first part of this chapter, we review the normal histological organization of the gut with the different cell types and their interaction accounting for intestinal motility. In the second part, we review the histopathological findings both in the aganglionic segment and in the proximal ganglionic segment in Hirschsprung’s disease which may account for the frequent discrepancy encountered between the length of the nonfunctional bowel and the degree of obstruction and also for the persistent obstructive symptoms after a pull-through operation for Hirschsprung’s disease.

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Authors and Affiliations

  1. National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin, Ireland

    Anne Marie O’Donnell, Sandra Montedonico & Prem Puri

  2. Hospital Carlos Van Buren, Escuela de Medicina, Universidad de Valparaíso, Valparaíso, Chile

    Sandra Montedonico

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  1. Anne Marie O’Donnell
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  2. Sandra Montedonico
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  3. Prem Puri
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Correspondence to Prem Puri .

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  1. National Children’s Research Centre, Our Lady’s Childrens Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

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O’Donnell, A.M., Montedonico, S., Puri, P. (2019). Pathophysiology of Hirschsprung’s Disease. In: Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-15647-3_9

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  • DOI: https://doi.org/10.1007/978-3-030-15647-3_9

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