Adult Hirschsprung's disease. Clinical features and functional outcome after surgery

To assess the clinical features and long-term functional outcome after surgery, eight patients operated on for adult Hirschsprung's disease during the 20-year period, 1968-1988, were re-examined. There were six men and two women with a mean age of 40 +/- 3 years at the time of survey. The mean age of the patients at the time of diagnosis was 23.8 years (range, 16 to 41 years). Results were compared with eight consecutive patients operated on for Hirschsprung's disease during the neonatal period. The Duhamel operation was performed initially on seven patients in the adult group and on all patients in the neonatal group. Anterior resection was performed on one adult patient initially but this was converted subsequently to Soave's pull-through operation. None of the patients had constipation after definitive surgery. The mean defecation frequency was two in both groups. Three patients in the adult group and two patients in the neonatal group were completely continent, others had occasional soiling. The mean anal sphincter pressures were lower in the neonatal group than in the adult group and in each group lower than in normal controls. Adult Hirschsprung's disease must always be suspected in the context of chronic constipation. Functional results after surgery in adult patients are similar to those achieved in children.