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Intestinale neuronale Dysplasie Typ B

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Intestinal neuronal dysplasia type B: how do we understand it today?

  • Schwerpunkt: Gastrintestinale Motilitätsstörungen
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Zusammenfassung

Die intestinale neuronale Dysplasie Typ B (IND B) stellt eine Anomalie des Plexus submucosus dar, die sich in einer sichtbaren Vermehrung von Riesenganglien äußert und mit einer typischerweise milden chronischen Obstipationssymptomatik einhergehen kann. Die Diagnose einer IND B ist eine morphologische Diagnose und beruht auf einem gesteigerten Anteil von Riesenganglien im Plexus submucosus in Abhängigkeit vom Alter des Patienten. Nach gegenwärtigem Erkenntnisstand kommen Riesenganglien bis zum Ende des 1. Lebensjahres physiologisch vermehrt vor, sodass die Diagnose einer IND B erst nach Ablauf des 1. Lebensjahres gestellt werden sollte. Die IND B kann isoliert vorkommen und findet sich gehäuft in Assoziation mit dem M. Hirschsprung. Häufig bessert sich die Obstipationssymptomatik bei Patienten mit IND B bis zum 4. Lebensjahr unter konservativer Therapie spontan. Bei persistierender schwerer Obstipation kann selten eine chirurgische Resektion notwendig werden. Die Pathogenese der IND B ist nicht geklärt und die Ätiologie unbekannt. Weitere Studien bei Patienten und Tiermodellen sind daher dringend angesagt.

Abstract

Intestinal neuronal dysplasia type B (IND B) is currently considered to be a subtle malformation of the submucosal plexus, leading to an increased proportion of over-sized ganglia and potentially accompanied by a mild, chronic gastrointestinal motility disturbance. The diagnosis of IND B is morphologically based and involves the demonstration of an increased proportion of giant ganglia in the submucous plexus related to the patient’s age. Giant ganglia are physiologically frequent in the neonatal period. Therefore, IND B should not be diagnosed prior to 1 year of age. Morphological features of IND B may occur as an isolated finding or may be observed proximal to an aganglionic segment. IND B and constipation may resolve spontaneously up to the age of 4 years. Treatment of IND B is usually conservative, surgical resection is currently deemed necessary only in a minority of patients. The pathogenesis of IND B is still incompletely understood and the etiology unknown. Future research on the basis of standardized diagnostic conditions is expected to result in a better understanding of this disease, and to reveal the cause of aberrant ganglion development.

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  1. Institut für Pathologie, Universitätsspital Basel, Schönbeinstraße 40, 4031, Basel, Schweiz

    E. Bruder &  W.A. Meier-Ruge

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  1. E. Bruder
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  2. W.A. Meier-Ruge
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Correspondence to W.A. Meier-Ruge.

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Bruder, E., Meier-Ruge, W. Intestinale neuronale Dysplasie Typ B. Pathologe 28, 137–142 (2007). https://doi.org/10.1007/s00292-007-0894-x

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