Study about Hirschsprung’s Disease in Adults: An Approach to Clinical and Therapeutic Features

Introduction: Hirschsprung’s disease (HD) is definitely a childhood disease as it is the most frequent cause of bowel obstruction in the newborn and in the child whereas it is rare in adults and it is thus often undiagnosed or misdiagnosed.

Objective: Through this series of 12 cases of HD we try to study the clinical characteristics of this pathology, to define its diagnostic clues and to assess the different therapeutic approaches.

Materials and Methods: This is a series of 12 patients referred to our department by the emergency department of HABIB THAMEUR hospital in Tunis for acute bowel obstruction.

Ten of them had selectively a lateral discharge colostomy.
One patient had a peritoneal lavage and the Hartman colostomy for a stercoral (fecal) peritonitis following perforation of the sigmoid colon.
One patient had a peritoneal lavage and the Bouilly Wolkman double stomy for a stercoral peritonitis following perforation of the left colon.
Anorectal manometry was performed in 25% of cases only. It revealed in each patient the absence of recto-anal inhibitory reflex (RAIR).
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. Histology of the operative specimens confirmed the diagnosis of HD.

Conclusion: 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.