Hirschsprung’s disease in adults: Clinical and therapeutic features

Hirschsprung’s disease (HD) is rare in adults and it is thus often undiagnosed or misdiagnosed. Through this series of 12 patients we try to study the clinical characteristics of this pathology, to define its diagnostic clues and to assess the different therapeutic approaches. Definitive diagnosis is established on histology of specimens from the rectum and colon. The disease involved the rectum and the sigmoid colon in 2 patients and was confined to the rectum, in the 10 others. Treatment was in all cases surgical consisting of recto-colic resection associated with coloanal anastomosis and a protective right lateral ileostomy. We conclude that Hirschsprung’s disease is rare in adults but by no means exceptional. It should be considered in young adults with a history of chronic constipation. Diagnosis is first of all clinical. When barium enema appearances are pathognomonic we needn’t resort to histology to confirm the diagnosis. Anorectal manometry does not usually show RAIR. Current primary treatment of HD diagnosed in adults consists mainly of surgical resection.