Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Interstitial cells of Cajal (ICCs ) are pacemaker cells which are densely distributed throughout the whole gastrointestinal tract . ICCs have important functions in neurotransmission , generation of slow waves and regulation of mechanical activities in the gastrointestinal tract , especially for the coordinated gastrointestinal peristalsis . Therefore , a loss of ICCs could result in gastrointestinal motor dysfunction . In recent years c-kit labeling has been widely used to study pathological changes of ICCs in gastrointestinal motility disorders . Paediatric gastrointestinal motility disorders such as hypertrophic pyloric stenosis , Hirschsprung 's disease , total colonic aganglionosis , hypoganglionosis , intestinal neuronal dysplasia , internal anal sphincter achalasia , megacystis microcolon intestinal hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICCs networks . This review describes the distribution of ICCs in the normal gastrointestinal tract and its altered distribution in intestinal motility disorders of childhood .