Diagnosis and management of chronic intestinal pseudo-obstruction syndromes in infancy and childhood.

Chronic intestinal pseudo-obstruction is a rare disorder characterized by ineffective intestinal propulsion in the absence of mechanical factors. It depends on isolated or associated disturbances of intrinsic and extrinsic innervation of the smooth muscle, neurotransmitters and hormones. In children the most common causes are postoperative paralytic ileus, pneumonia or pancreatitis, occurring usually in the first year of life. There is also hereditary transmission, comprising the familial visceral myopathy (hollow visceral myopathy) and the familial visceral neuropathy. The pseudo-obstruction may be associated with congenital anomalies (digestive or not) in 10 to 30% of the cases, mainly malrotation, pyloric stenosis and bladder atony. Diagnostic difficulties may lead to exploratory laparotomy. A precise diagnosis requires judicious interpretation of radiologic, manometric, radioisotopic and hystologic findings. Medical treatment includes drugs acting on gastrointestinal motility as well as hydroelectric and nutritional support, besides treatment or prevention of infections. It is possible that in the future these patients may be treated by surgical implantation of electrodes promoting gastrointestinal myoelectrical stimulation.