Intestinal neuronal disorders--a study of seven cases

Intestinal neuronal dysplasia (IND) has been reported as an innervation disorder that can present as isolated disease or may be associated with Hirschsprung's disease (HD). The interest in this disorder is growing as it mimics HD at clinical level but can be managed with a more conservative approach if an accurate diagnosis can be made. Many workers have tried to set up diagnostic criteria of this condition. But the importance of one criterion varied from one study to another. In our study we analysed seven cases of suspected innervation disorder that had undergone resection. A detailed histological study on these cases was performed and four of them were found to fulfill the diagnostic criteria of IND laid down by Kobayashi and his co-workers. These patients had hyperganglionosis, giant ganglia and ectopic ganglion cells in the lamina propria. In the other three cases some features were highly suggestive of the diagnosis of the IND and can be considered to be so if we follow other workers who have not given much importance to the simultaneous presence of all three criteria in a single case.