2016•Journal of Pediatric Surgery Case ReportsOpen access

A case of Hirschsprung's disease underwent surgery in adulthood

Richi Nakatake, Yoshinori Hamada, Hirokazu Miki, Takeshi Shirai, Yusuke Nakamura, Hiroshi Hamada, Morihiko Ishizaki, Masanori Kon

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Abstract

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.

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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.

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Available abstract

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.

Key concepts: Medicine, Hirschsprung's disease, Megacolon, Barium enema, Constipation, Chronic constipation, Surgery, Enema

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