Paediatric gastrointestinal motility disorders

Normal gastrointestinal function requires an intact enteric musculature and enteric nervous system. There is a group of disorders in which abnormalities of either enteric nerves or muscle, or both, lead to clinical features of intestinal obstruction in the absence of physical obstruction - chronic intestinal pseudo-obstruction. These disorders may be congenital or acquired. The best known of the congenital enteric nervous system disorders is Hirschsprung disease where there is absence of nerve cells in the distal colon. Others include ganglioneuromatosis. Muscular disorders include degenerative leiomyopathy and mitochondrial disorders. The nervous or muscular system may also be affected secondarily in many systemic disorders. Surgical intervention is frequent in cases of chronic intestinal pseudo-obstruction and yields specimens for pathological examination. The pathological investigation of these specimens may yield only non-diagnostic features, but care is needed not to overlook those cases where a specific diagnosis may be made on the basis of the histopathology.