Objective:To investigate the role of interstitial cells of cajal(ICC) in the pathogenesis of HD through observing the distribution of the cells in normal colon and HD's different segments.Methods:The aganglionic、transitional and dilated segments were collected in operations from 25 children with Hirschsprung’s disease.Another 6 normal segmengts were also collected.Each sections was stained by c-Kit immunohistochemistry.The distribution of the interstitial cells of Cajal was observed by light microscope.Results:The ICC in aganglionic segments of colon from Hirchsprung’s disease were decreased significantly and statistically than that in dilated and normal colon.Moreover,whole mount preparations showed that,not only the number of ICC-IM was decreased down,but also the branches of ICC-IM and the cellular network were also broken down.Conclusion:The pathogenesis of HD is related to the abnormal distribution of ICC,which lead to or aggravate the bowel spasm in HD.

Objective KG1This study is to evaluate defectography in postoperative defecation function of Hirschsprung′s disease (HD). KG2MethodsKG1 Between 1979 and 1993, 30 HD cases were treated operatively and followed-up by defectography. KG2ResultsKG1 Thirty cases were classified into 3 groups, according to the standard quantitative clinical scoring systems with the stooling score from 0 to 14. There were 4 cases (13%) graded as excellent (maximum score of 14) with normal bowel habit, 21 cases (70%) as good (score between 10～13) with minor continence problems, 5 cases (16 7%) as fair (score between 5～9) with marked limitations in social life. Anorectal manometry study showed that the anal resting pressure and voluntary sphincter force (maximal queeze pressure minus resting pressure) in fair group were significantly lower than that in control group( P