Chronic intestinal pseudo-obstruction: diagnosis and treatment.

Chronic intestinal pseudo-obstruction (CIP), a syndrome of disordered intestinal motility, is characterized by failure of the intestine to propel its contents through an unobstructed lumen. The symptoms and signs of CIP can be very similar to those of mechanical bowel obstruction. The two disease states differ in management, however; therefore, clinicians must differentiate the two. Abnormal propulsion can result from many causes. Cases can be classified as primary or secondary, and identifiable causes can be subdivided into those affecting neuronal control of motility and those directly affecting intestinal muscle function. Careful history, physical examination, and investigations help identify causative factors. The secondary forms of CIP are more common than the primary, and many cases are reversible by correction of the causative factor (e.g., drugs, metabolic abnormalities, or infection). Management of CIP depends on the cause of the disorder, the extent and location of intestine involved, and the severity of symptoms; general measures include dietary changes, prokinetic agents, and, rarely, surgical interventions. Some children with severe, refractory CIP who are wholly dependent on parenteral nutrition are candidates for consideration of intestinal transplantation.