Neuronal intestinal dysplasia - Modern diagnosis and therapy - report of 23 patients

Findings in 23 children with neuronal intestinal dysplasia (NID) are presented. Twelve children had Hirschsprung's disease that masked the existence of proximal NID. In all cases the diagnosis was established by histological-histochemical examination. Amelioration of symptoms by conservative treatment alone was achieved in 4 children; in 13 cases colostomy was necessary. In 6 patients colon resection was performed: 2 suffered from both colitis and obstruction; the other 4 were treated for obstruction, multiple perforations, persistant adynamia of the left colon, and fistulation. In 9 patients the colostomy was closed. Four of 6 children who did not have sonographic confirmation of motility had complications due to colon dysmotility after closure of their normally functioning colostomy. Therapeutic measures are based exclusively on clinical and functional parameters. The clinical picture dictates the emergency measures (colostomy, colon resection, and colectomy). Normal colostomy function is not a sufficient criterion of normal colon motility. Functional sonography of the colon is included in the planning of further therapy. Indications, timing, and extent of colon resection can be reliably determined using the sonographic method.