A case of Hirschsprung's disease underwent surgery in adulthood

•Surgery in adult HD is difficult because of chronic fibrous changes of the rectum.•Surgeons should decide on the operative procedure and staged surgery.•Staged operations including the Duhamel-GIA method are a viable surgical option.

Adult Hirschsprung's disease is a rare motor disorder of the gut that is frequently misdiagnosed as refractory constipation. We describe a 30-year-old patient with adult Hirschsprung's disease with a history of chronic constipation requiring daily enema. Barium enema and rectal biopsy showed short segment-type Hirschsprung's disease with a grossly distended sigmoid colon with fecal retention. Staged operations, including the Duhamel-GIA method as a definitive surgery, completely resolved the patient's symptoms. There were no complications by the end of a 7-year follow-up. Adult Hirschsprung's disease should be considered in the differential diagnosis of cases where adult patients present with chronic constipation. Despite technical difficulty of the modified Duhamel-GIA procedure due to chronic fibrous changes of the rectum, it is a feasible surgical option for treating cases of adult Hirschsprung's disease.