Immunohistochemical study of enteric nervous system in hirschsprung's disease and intestinal neuronal dysplasia

Hirschsprung's disease (HD) is one of the most common motility disorders in pediatric age groups and it is very important that it be differentiated from other types of motility disorders, especially intestinal neuronal dysplasia B (IND B). Although many studies regarding the differences between the two disorders by immunohistochemical studies exist, there is as yet no consistent result. The purpose of this research was to study the immunohistochemical findings of enteric nervous system in these two motility disorders in comparison with colectomies without motility disorder.

Full wall thickness specimens of three groups of patients (HD, IND B and non motility disorders) were included in the study to be evaluated by immunohistochemistry (IHC). Markers were specific for neuronal cells and pace maker cells composed of PGP 9.5, c-kit, synaptophysin, S100 and CD56. The number of cells was evaluated in the muscularis properia, and myenteric plexus.

The number of all the IHC markers i.e. PGP9.5, c-kit, synaptophysin, S100 and CD56 was completely different in HD from the two other groups, while IND B was quite similar to control group.

Our finding suggests that there is a marked and significant difference between HD and IND B by IHC markers, which can be used as an additional test for the diagnosis of HD with more accuracy. Further multicenter studies with a greater number of cases would be necessary to find a cut-off point for every IHC marker to differentiate HD and IND B.