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Morbus Hirschsprung und neuronale intestinale Dysplasie

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Kinderchirurgie

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Als Morbus Hirschsprung im engeren Sinn bezeichnet man eine, erstmals 1886 von Harald Hirschsprung (Hirschsprung 1887) beschriebene Erkrankung, bei der wie Titel 1901 (Tittel 1901) entdeckte, die Nervenzellen im Darm fehlen. Die Folgen sind subileus- oder lleuserscheinungen bis hin zur toxischen Peritonitis. Das Krankheitsbild umfasst aber inzwischen auch andere Fehlbildungsformen des enteralen Nervensystems wie die Hypoganglionose, bei der die Ganglien hypoplastisch sind und ihre Zellzahl vermindert ist oder die Hyperganglionose, bei der die Anzahl der Nervenzellen innerhalb der Darmwandganglien erhöht ist. Betrifft diese Fehlbildung nur den Plexus submucosus, spricht man nach (Meier-Ruge 91971) von einer Intestinalen neuronalen Dysplasie (IND). Es kann jedoch auch — insbesondere im Tiermodell — zu IND-ähnlichen Veränderungen des Plexus myentericus kommen. Weiterhin gibt es Reifestörungen der Darmwandplexus, Fehlbildungen des enteralen Bindegewebes (Desmose), Erkrankung der interstitiellen Cajal-Zellen, die Motilitätsstörungen zu Folge haben, und weitere Dysmorphien, die entweder keine funktionelle Bedeutung haben wie die Ektopie von Ganglien in die Ringmuskulatur, oder morphologische Veränderungen, deren funktionelle Bedeutung noch nicht eindeutig geklärt ist wie ein herabgesetzter Parasympathikotonus des Plexus myentericus.

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    Prof. Dr. med. A.M. Holschneider

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Holschneider, A. (2009). Morbus Hirschsprung und neuronale intestinale Dysplasie. In: Kinderchirurgie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-89032-4_29

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  • DOI: https://doi.org/10.1007/978-3-540-89032-4_29

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