Abstract

A number of motility disorders in children are associated with intestinal neuronal malformations and other anatomic defects. These disorders include Hirschsprung's disease, neuronal intestinal dysplasia, chronic intestinal pseudo-obstruction, and anorectal malformations. Hirschsprung's disease is now linked to specific genetic mutations, and laparoscopic techniques hold promise for less invasive surgical treatment. Neuronal intestinal dysplasia is now considered an important cause of postoperative bowel dysfunction in Hirschsprung's disease patients. Chronic intestinal pseudo-obstruction is characterized by recurrent bouts of abdominal distension, pain, and constipation. The surgical treatment of these patients is relegated to the placement of feeding and decompression tubes. For the past decade, posterior sagittal anorectoplasty has been the preferred approach in the repair of anorectal malformations. Long-term assessments of bowel function after the use of this procedure are now being published and show generally good outcome.

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