Development of interstitial cells of Cajal in a full-term infant without an enteric nervous system

The relationship between the development of the enteric nervous system and interstitial cells of Cajal (ICC) in the human small intestine was investig ated in a full-term infant who presented with intestinal pseudo-obstruction . Immunohistochemistry revealed absence of enteric nerves and ganglia but a bundant c-Kit immunoreactivity associated with Auerbach's plexus (ICC-AP). However, c-Kit immunoreactivity associated with the deep muscular plexus (I CC-DMP) and intermuscular ICC was absent. Electron microscopy showed ICC-AP with a normal ultrastructure; ICC-DMP were seen but were severely injured, suggesting degeneration. In vitro recording of intestinal muscle showed sl ow wave activity as well as response to cholinergic stimulation. Fluoroscop ic examination of the small bowel showed a variety of motor patterns, inclu ding rhythmic, propagating contractions. In conclusion, total absence of en teric nerves was associated with absence of normal ICC-DMP. However, a norm al musculature, including a network of ICC-AP, allowed for generation of rh ythmic, propagating contractile activity, suggesting the presence of functi onal motor activity.