[Intestinal neuronal dysplasia type B: how do we understand it today?].
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.
Bruder
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Institut für Pathologie, Universitätsspital Basel, Schönbeinstrasse 40, 4031 Basel, Switzerland. elisabeth.bruder@unibas.ch
Meier-Ruge
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D003248:Constipation / Q000473:pathology / Q000503:physiopathology; D017615:Enteric Nervous System / Q000473:pathology; D005724:Ganglia / Q000473:pathology; D005769:Gastrointestinal Motility / Q000502:physiology*; D041981:Gastrointestinal Tract / Q000473:pathology*; D006627:Hirschsprung Disease / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007410:Intestinal Diseases / Q000473:pathology* / Q000503:physiopathology