PREDICTING THE CLINICAL-RESPONSE TO CISAPRIDE IN CHILDREN WITH CHRONIC INTESTINAL PSEUDOOBSTRUCTION

We assessed upper gastrointestinal anatomy and function with contrast radiology and antroduodenal manometry in 51 children with chronic inte stinal pseudo-obstruction (CIP) prior to entering these patients into an open-label outpatient trial of cisapride. The diagnosis of CIP was based on characteristic symptoms requiring special nutritional support (parenteral in 30, tube feeding in 12) or interfering with daily acti vities (documented by diary in nine). At a time the subjects were not acutely ill, antroduodenal pressures were recorded for >4 h fasting an d >1 h after a complex liquid meal. Results were categorized by the mo st prominent manometric abnormality as myopathy (n = 6), absent migrat ing motor complex (MMC) (n = 27), failure to induce fed pattern (n = 7 ), MMC plus discrete abnormalities (n = 7), and postprandial duodenal hypomotility (n = 4). Patients in the first two categories did not hav e effective MMCs, but those in the last three categories did. Compared to children without MMCs, those with MMCs rarely required parenteral nutrition (p < 0.001). All children were treated with oral cisapride 0 .2 mg/kg/dose t.i.d., and evaluated every 2 months for up to 1 yr. Of 49 evaluable subjects, the final global assessment was unchanged in 25 , fair (improved symptom score) in 17, or excellent (change from TPN t o tube feeding or tube feeding to oral feeding) in seven. Children wit h MMCs (13 of 18) responded more often to cisapride than those without MMCs (11 of 3 1), p < 0.02. All four subjects with postprandial duode nal hypomotility had excellent responses. Children with normal diamete r bowel responded more often than those with dilated bowel, p < 0.004. To summarize, in children with CIP, absence of the MMC was associated with need for greater intensity of nutritional support and decreased response rate to cisapride. The response to cisapride was highly varia ble within the study group, but often could be predicted by the presen ce or absence of bowel dilation and MMCs.