| ObjectiverThe incidence rate of Hirschsprung's disease is1/5000. Although! the study about pathogenesis of Hirschsprung'sdisease have been hundreds years, there are many problems aboutpathogenesis of Hirschsprung's disease. The research is toinvestigating the role of ICC (the interstitial cell of Cajal) on thepathogenesis of Hirschsprung's disease by observing thedistribution of ICC in HD.Methods: The distribution of the ICC was studied usingspecific antibodies in the aganglionic, transitional and dilatedsegments of colon from 26 children with Hirschsprung's disease,which was collection in operation, and c-kit immunohisto--chemistry was progressed by SP.Results : The density of the interstitial cells from the dilatedsegment to the transitional segment and aganglionicsegment decreased gradually. ICC is close to myenteric nerveplexus and The distribution of ICC is surrounding or insetting thenerve plexus of dilated segment to the transitional segmentand aganglionic segment.In aganglionic segments of HD, the interstitial cells werescarce.There is not ICC in the nerve plexus.Conclusions : Our study may reveal the relation between theabnormal distribution of ICC and the pathogenesis of HD. Wesuspect that:l.The defect of Embryo matrix damage the migration of neuralcrest cell and the differentiation and maturation of ICC2.Existence of ENS needs rich and full differentiation network ofICC.3. The migration and full development of ganglion cell need ICC.Our data may explain the abnormal spontaneous electricalactivity in aganglionic segments of HD.We can conclude that theabnormal distribution of ICC may cause or enhance occurrence of HD.
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