Long-term outcomes and the quality of life of Hirschsprung disease in adolescents who have reached 18 years or older--a 47-year single-institute experience.

Background And Aims: Hirschsprung disease is considered to be curable when performing definitive surgery. However, the postoperative bowel function is not always satisfactory. The long-term postoperative function of bowel evacuation and the quality of life of such patients are considered to be important. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17 years who had suffered from Hirschsprung disease in childhood.

Methods: From 1963 to 2009, 184 patients with Hirschsprung disease underwent definitive surgery, mostly Z-shaped anastomosis at Kyushu University Hospital. As a result, 146 (95.4%) of 153 of those patients survived and reached 17 years of age. Their present status and symptoms, anorectal functions, genitourinary functions, and social performance were evaluated during the clinical follow-up based on a questionnaire survey.

Results: In our series, the evacuation score was rated as "excellent" (score of 7-8/8) in 66.7%, "good" (5-6/8) in 19.0%, "fair" (3-4/8) in 11.9%, and "poor" (0-2/8) in 2.4%. Therefore, 85.7% were considered to have a satisfactory bowel function. However, only 21.4% had a completely normal score of 8/8. Incontinence occurred in 16.7%, and soiling was present in 19.0% of the questionnaire respondents. The genitourinary function was considered to be within the reference range, and urinary problems were minimal. Among the respondents, 45.2% were married, and 68.4% of those married had children. The educational and professional careers of the respondents were successful.

Conclusions: In general, the bowel function was satisfactory. However, the ratio of patients with completely normal bowel function was low. Because incontinence and soiling impair the quality of life, pediatric surgeons should therefore continue trying to achieve a complete bowel function after definitive surgery for the treatment of Hirschsprung disease.