Interstitial Cells Of Cajal In The Of Hirschsprung Normal Colon In The Distribution And Clinical Significance

Objective: Hirschsprung's Disease(HD) is a common congenital disease characterized by aganglionic, lack of the　normal　bowel movement. Numerous abnormalities of innervation have been described in HD, but the cause of dysmotility of the bowel in HD remains unclear. The interstitial cells of Cajal (ICC) are regarded as the pacemaker cells of the gut musculature. They can generate slow waves spontaneously and play an important role in the control of gut motility. Their ontogeny remains unclear, but the role of the tyrosine kinase receptor c-kit in their development has been recognized. This study examined the density of interstitial cells of Cajal in human colon and in Hirschsprung's Disease to clarify the pathophysiology of HD.Method: The distribution of interstitial cells of Cajal was evaluated by immunohistochemiscal method using an anti-c-kit antibody in 38 HD Patients and 70 normal controls. The control colorectal tissues were obtained from surgical resection performed for carcinoma of the colon or rectum, and previous bowel motility of those patients had been proved to be normal. Tissue was defined as normal when the section was not invaded by the tumor.Results: The pattern of distribution of ICC in the normal intestine was the same for the right, transverse, left colon and the rectum. The density of ICC was significantly greater at the myenteric plexus than at either longitudinal or circular muscle layers(P<0.01). The distribution of ICC in the proximal, ganglionic portion of HD appeared similar to that observed in the normal colon(P>0.05). In the aganglionic segment, the density of ICC was marked reduced in comparison with normal control or ganglionic segment of HD specimen(P<0.01).Conclusion: 1. The pattern of distribution of ICC in the normal intestine was the same in the right, transverse, left colon and the rectum. The higher density of ICC was found in the myenteric plexus of the normal colon, which is the proposed region of pace making area. 2. The significant lack of ICC was found in aganglionic bowel segments ,which might be another cause of gut dysmotivity of patients with HD.