[Hirschsprung disease in the adult. Report of a case]

To describe a case of Hirschsprung's disease (HD) in an adult patient.

HD is diagnosed in the newborn in 80 to 90% of cases. HD is rare in the adult, and usually affects an ultrashort segment of the bowel.

A 49-year-old woman with a history of constipation since birth is involved. She was submitted to an abdominal laparotomy because of fecal impaction. A colostomy was performed. The diagnosis of HD affecting, the descending colon was established with manometry and histopathology. She underwent surgery, and a proctectomy and left hemicolectomy with colo-anal anastomosis was performed.

Adult HD is an infrequently diagnosed entity that must be suspected in a patient with chronic, intractable constipation from infancy, evidence of megacolon, and typical manometric and histopathologic features. The short and ultrashort-segment disease are more often found in older children and adults. The differential diagnosis includes idiopathic megarectum, functional constipation, and colonic pseudo-obstruction.