[Motility disorders of the small intestine]

Chronic intestinal pseudo-obstruction (CIPO) is the most severe form of intestinal motility disorder, which leads to chronic or intermittent symptoms and signs of (sub-)ileus despite the absence of an intestinal obstruction. Small bowel motility disturbances may occur as primary diseases or secondary to a large number of other diseases and disturbances including rheumatological diseases and neurotoxic drugs. Pathological alterations affect the nervous system, smooth muscles, and/or mesenchymal structures such as the interstitial cells of Cajal or glia cells. Clinical symptoms are unspecific so that the initially suspected diagnosis is almost always wrong. Thus, extensive and stepwise diagnostic procedures are required involving specialized centers in order to exclude intestinal obstruction, to search for complications and potential causes of the disease, to quantify the extension and severity of the motility disorder, and to clarify the pathomechanism if possible. General therapeutic goals include maintenance of adequate nutritional status, improvement of propulsive motility, amelioration of abdominal symptoms, and avoidance and/or therapy of complications. Some CIPO patients require permanent parenteral nutrition. If this causes intolerable complications, small bowel transplantation can be considered in suitable patients as ultima ratio.