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Hirschsprung's disease: Clinical and experimental observations

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Abstract

Hirschsprung's disease (HD) is a relatively common cause of intestinal obstruction in the newborn. It is characterized by an absence of ganglion cells in the distal bowel beginning at the internal sphincter and extending proximally for varying distances. The etiology of HD-associated enterocolitis remains a complex issue. This study has provided further support for a possible infectious etiology of enterocolitis complicating HD.

Résumé

La maladie de Hirschprung est rare (une sur 5000 naissances), atteint surtout les garçons, mais constitue une cause fréquente d'occlusion chez le nouveau-né. Elle se caractérise par l'absence de cellules ganglionnaires dans l'intestin distal d'extension proximale variable. Dans 75% des cas, elle n'intéresse que le rectum et colon sigmoïde. Le diagnostic repose sur la clinique, les données de la manométrie anorectale et surtout l'examen anatompathologique, qui seul confirme la zone aganglionnaire. Le traitement est une reconstruction colorectale, soit traditionnellement en deux-temps, soit, de plus en plus souvent, en un seul temps opératoire. L'association d'une entérocolite est une complication grave et nécessite, en urgence, une réanimation adaptée et souvent une colostomie.

Resumen

La enfermedad de Hirschsprung es una causa relativamente común de obstrucción intestinal en el recién nacido. Se caracteriza por ausencia de las células ganglionares en el colon distal a partir del esfínter interno hasta distancias variables en sentido proximal. La aganglionosis aparece confinada al rectosigmoide en 75% de los pacientes, al sigmoide, ángulo esplénico o colon transverso en 17%, y a la totalidad del colon con un corto segmento del ileon en 8% de los pacientes. La aganglionosis intestinal total con ausencia de células ganglionares desde el duodeno hasta el recto constituye la forma más rara dse enfermedad de Hirschsprung. Se estima la incidencia de la enfermedad de Hirschsprung en 1 en 1.500 nacimientos vivos. Spouge y Baird estudiaron la incidencia en la prpovincia de British Columbia y reportan una tasa de 1 en 4.417. La entidad es más común en los niños, con una relación masculino: femenino de 4∶1, pero esta mayor prevalencia en el sexo masculino es menos evidente en los pacientes con largos segmentos afectados, donde la relación de sexos es 1.5∶2.1. En este artículo se revisa la etiología, patofisiología, patología, cuadros clínicos, métodos de diagnóstico y manejo. La enterocolitis se mantiene como la complicación más grave de la enfermedad de Hirschsprung y se asocia con alta morbilidad y mortalidad.

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Correspondence to Prem Puri M.D..

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Puri, P. Hirschsprung's disease: Clinical and experimental observations. World J. Surg. 17, 374–384 (1993). https://doi.org/10.1007/BF01658706

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Keywords

  • Experimental Observation
  • Ganglion Cell
  • Intestinal Obstruction
  • Como
  • Estima