Short and longterm prognosis

Data are accumulating to indicate that Hirschsprung's disease, a disorder once known exclusively to involve an aganglionic segment of distal colon, also affects motor function in other parts of the gut. The variability in manifestations could reflect the heterogeneity of basic genetic defects now recognized as being responsible for the phenotypic expression of Hirschsprung's disease. Abnormalities in esophageal motility are common, and duodenal motor dysfunction is present in 48% of patients.5

Miele et al have reported a systematic study of various aspects of gastrointestinal motor function in children with Hirschsprung's disease long after removal of the aganglionic colonic segment, observing gastrointestinal symptoms, including vomiting, distension and poor growth, long after surgery.33 Abnormalities in duodenal motor activity have also been observed in these children shortly after operation.34

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