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Enfermedad de Hirschsprung - Overview

Nombres alternativos

Megacolon congénito

Definición:

Es una obstrucción del intestino grueso debido al movimiento muscular impropio en el intestino y es un trastorno congénito, es decir, que está presente al nacer.

Causas:

Las contracciones musculares intestinales, desencadenadas por los nervios que se encuentran entre las capas musculares, ayudan a que los materiales digeridos se movilicen a través del intestino, lo cual se denomina peristaltismo.

En la enfermedad de Hirschsprung, los nervios están ausentes de una parte del intestino. Las áreas carentes de dichos nervios no pueden empujar el material, causando un bloqueo. Los contenidos intestinales se acumulan detrás del bloqueo, haciendo que el intestino y el abdomen se hinchen.

La enfermedad de Hirschsprung causa aproximadamente el 25% de toda la obstrucción intestinal de los recién nacidos y ocurre cinco veces más en hombres que en mujeres. Esta enfermedad algunas veces está asociada con otros trastornos congénitos o hereditarios como el síndrome de Down.

  • Reviewed last on: 11/2/2009
  • Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Kessmann J. Hirschsprung's Disease: Diagnosis and Management. Am Fam Phys. 2006;74:1319-1322.

Wyllie R. Motility disorders and Hirschsprung disease. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 329.

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