The bowel function and quality of life of Hirschsprung disease patients who have reached 18 years of age or older - the long-term outcomes after undergoing the transabdominal soave procedure

Abstract

Background/purpose: Hirschsprung disease (HD) is considered curable, but the postoperative bowel function is not always satisfactory. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17years of age who had undergone definitive operations in childhood.

Method: From 1984 to 2016, 110 patients with HD underwent definitive surgery at our institute. Sixty-three patients who had undergone the transabdominal Soave procedure reached 18years of age. Their present status and symptoms, anorectal function, genitourinary function, and social performance were evaluated during the clinical follow-up via a questionnaire survey.

Result: The mean age of the questionnaire respondents was 25.0 (19-37) years. The bowel function was mostly good. However, 56% of patients had abdominal pain more than once a week. Regarding evacuation symptoms, incontinence and soiling occurred in 18.7%. Among the respondents 33.3% were married, and 60% of those who were married had children. The respondents had achieved success in their education and professional careers.

Conclusion: The bowel function of most patients was satisfactory, although some had chronic abdominal symptoms. Pediatric surgeons should continue trying to achieve complete bowel function after definitive surgery of HD.

Type of study: Prospective Cohort Study.

Level of evidence: Level III.

Keywords: Adulthood; Bowel function; Hirschsprung disease; Long-term outcome; Quality of life; Soave procedure.