Background: German pathologists have developed a consensus for histological features of intestinal neuronal dysplasia.
Methods: A blind reevaluation of ganglionic suction rectal biopsies from infants and children who initially presented with symptoms of intestinal dysmotility was made.
Results: 84 of 411 specimens had sufficient depth of submucosa for adequate assessment. Questionnaires or clinical interviews were employed 3-5 years after biopsy in these 84 patients to assess the relationship between histological changes and persistent symptomology. Eighteen children were lost to follow-up, 4 others had Hirschsprung's disease the study biopsy specimen having been taken from the pulled-through bowel after surgical resection of the aganglionic segment. The remaining 62 patients were divided into three groups. There were six patients in group A (both obligatory criteria) and 28 in group B (nonessential, or just one of the obligatory criteria), and 28 in group C (normal appearances). On follow-up, two of the 28 (7%) in group B, and six of the 28 (21%) in group C had persistent dysmotility symptoms.
Conclusions: Histological criteria of the consensus of German Pathologists for intestinal neuronal dysplasia was unhelpful in predicting the clinical outcome and therefore, should not influence clinical management. As one of the obligatory criteria, hyperplasia of the submucosal plexus was significantly more common in neonates (< 4 weeks), it is concluded that this is an age-related variation.