Quality of Life in Adults Operated On for Hirschsprung Disease in Childhood

Abstract

The aim of this study is to explore the long-term quality of life (QoL) in adults after surgery for Hirschsprung disease in childhood. Altogether 51 patients were operated for HD at our center during the period 1969 to 1989. In 2007, the validated instruments for assessing QoL, the SF-36 health survey and the Gastrointestinal Quality of Life Index (GIQLI), were sent to the 47 patients still alive at the time of the study. A retrospective review of the patient records was also done. Forty-two patients returned both questionnaires (89% response rate) at the median age of 28.5 (range 18-45) years. The median clinical follow-up time was 5.7 years (range 5 months-23 years). At the last clinical control, 4 (9%) patients had a terminal enterostomy, 12 (29%) had soiling, 5 (12%) had constipation, and 2 (5%) experienced recurrent enterocolitis. In contrast to males, the subscores for females were lower for general health and mental health than for an age- and sex-matched general population (P < 0.05). Patients having aganglionosis to the right colon had lower GIQLI scores than those with aganglionosis to the left colon (P < 0.05). In multivariate linear regression analysis, female sex was the only factor associated with lower GIQLI score (P < 0.05). The long-term QoL of adults operated for Hirschsprung disease in their youth is satisfactory. Female scores were lower for general and mental health, compared with the matched control group. The study indicates that the longer the aganglionic segment, the greater its impact on QoL in later life.