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Intestinal Neuronal Dysplasia in Children: Diagnostic Criteria and Immunohistochemical Markers.


Intestinal neuronal dysplasia is a complex entity that affects the enteric nervous system and manifests clinically with severe constipation in children. The diagnosis is established by the histopathological evaluation of rectal biopsies. However the diagnostic criteria, both qualitative and quantitative, have changed over time, hindering diagnostic practice. Objective: This study aims to evaluate the morphological criteria for the histological diagnosis of intestinal neuronal dysplasia in a series of patients with a previous diagnosis of this entity and to investigate the applicability of immunohistochemical expression of neural markers, like Hu and calretinin, on the diagnosis of intestinal neuronal dysplasia. Methods: A retrospective analysis will be performed on surgical specimens of the distal colon from 29 children, diagnosed with Intestinal neuronal dysplasia. The histopatholgical analysis, both qualitative and quantitative, will be conducted using sections processed for conventional histology (HE). The qualitative criteria will consider hyperplasia of the submucous plexus with hyperganglionosis and hypertrophy of the nerve trunks (Frankfurt 1990). The quantitative criteria will consider more than 20% giant ganglia in the submucosa, with more than eight neurons each on 25 ganglia, and child´s age over one year old (Meier-Ruge, 2006). The results of the histopathological analysis will be divided into 2 groups according to the age at surgery: children aged < 1 year and children aged > 1 year. The immunohistochemical expression of neural markers, Hu and calretinin, will be investigated in histological sections similar to those used for the conventional analysis (HE). We hope with this study to improve the accuracy of the histopathological diagnosis of intestinal neuronal dysplasia, which is a challenging issue in gastrointestinal pathology. (AU)