Long-term social outcome in children with moyamoya disease who have reached adulthood

Abstract

Object Although the reported surgical outcome is favorable, there is little information regarding the long-term quality of life in pediatric patients with moyamoya disease (MMD) when they grow to adulthood. The authors conducted a survey to provide details of social adaptation and satisfaction in adults who underwent revascularization surgery for MMD during childhood. Methods One hundred twenty-three patients with MMD who had undergone surgery during childhood and were older than 18 years of age were recruited for this study. The authors mailed self-administered questionnaires regarding each patient's education, employment, marriage, driver's license, physical condition, and satisfaction. Sixty-five patients (53%) responded. Results Compared with the general population, the patients showed a similar rate of attaining a higher education. Forty-nine (80%) of 61 patients who were not currently high school students had entered college or university. However, the presence of neurological deficits on preoperative examination was a negative predictor of entrance into a college or university, as well as employment. Some patients had difficulty in planning marriage because of physical handicaps, and the rate of acquiring a driver's license and actual driving were relatively low. Approximately 80% of the responders were satisfied with their treatment outcomes, but more than one-half reported subjectively assessed neurological problems. Conclusions In this study, pediatric patients with MMD showed favorable social adaptation in adulthood. The findings also indicated that early diagnosis and intervention before the establishment of neurological deficits are essential to children with MMD in becoming better socially adapted. Further, more thorough clinical examinations are required during follow-up in patients with MMD.