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Kongenitale Innervationsstörungen des Kolon

Ein diagnostischer Leitfaden

Congenital disorders of the colonic innervation

A diagnostic guide

  • Schwerpunkt: Kinderpathologie
  • Published:
Der Pathologe Aims and scope Submit manuscript

Zusammenfassung

Der Morbus Hirschsprung (MH, Aganglionose) ist die wichtigste Form der kongenitalen Innervationsstörungen des Darms mit Indikation zu einer chirurgischen Intervention. Die Hypoganglionose im Übergangssegment stellt die bedeutendste Entität von Krankheitswert dar. Die präoperative Klärung der Länge des innervationsgestörten Segments ist nach wie vor eine diagnostische Herausforderung für den Kliniker und Pathologen. Enzymhistochemische Untersuchungen stellen die Methode der Wahl dar, bei deren Anwendung jedoch bestimmte Einschränkungen zu beachten sind.

Andere Dysganglionosen, insbesondere die so genannte neuronale intestinale Dysplasie (NID), lassen sich wegen einer zu großen Überlappung mit alterskorrelierten Normwerten nicht zweifelsfrei als Entität definieren. Die einzige Ausnahme bildet die als Teilsymptom einer genetisch bedingten Erkrankung auftretende Ganglioneuromatose.

Abstract

Hirschsprung’s disease (HD, aganglionosis) is the most important form of congenital disturbance of intestinal innervation, requiring surgical intervention. Furthermore, hypoganglionosis of the transitional zone forms the most significant factor in morbidity. Pre-operative definition of the length of neuronally disturbed segment is still a diagnostic challenge for both clinical physician and pathologist. Enzyme histochemical studies form the method of choice, but certain limitations in their use must be observed.

Other dysganglionoses, particularly the so-called “Intestinal Neuronal Dysplasia” (IND) cannot—because of an excessive overlapping with age-correlated normal values—unequivocally be defined as an entity on its own. The only exception to this, is the ganglionic neuromatosis, which arises as part of a genetic illness.

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  1. Abteilung für Kinderpathologie, Klinikum der Johannes-Gutenberg-Universität Mainz

    W. Coerdt, H. Müntefering, E. Rastorguev & V. Gerein

  2. Abteilung für Kinderpathologie, Klinikum der Johannes-Gutenberg-Universität Mainz, Langenbeckstraße 1, 55101, Mainz

    W. Coerdt

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  1. W. Coerdt
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  4. V. Gerein
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Correspondence to W. Coerdt.

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Coerdt, W., Müntefering, H., Rastorguev, E. et al. Kongenitale Innervationsstörungen des Kolon. Pathologe 25, 292–298 (2004). https://doi.org/10.1007/s00292-004-0704-7

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  • DOI: https://doi.org/10.1007/s00292-004-0704-7

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