OBJECT: In moyamoya disease, despite its progressive nature of the occlusive lesions in pediatric patients, the prevalence of the progression in adult patients is undetermined. Furthermore, the optimal timing of the revascularization surgery for progressive cases is controversial. To address these issues, we retrospectively investigate four cases with the adult-onset moyamoya disease manifesting as progression before revascularization surgery. METHODS: From March 2004 to May 2007, 49 patients with adult-onset moyamoya disease aged from 19 to 62 years old (mean 40.5) underwent superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 63 hemispheres. All patients were strictly followed up by magnetic resonance (MR) imaging/angiography postoperatively. Twenty-seven hemispheres of 15 adult patients without surgery were also followed up at outpatient service during the same period. If the patients manifest as the progression of the steno-occlusive lesion on the hemisphere without surgery, they undergo revascularization surgery after the confirmation of hemodynamic compromise. RESULTS: During this period, 47 hemispheres including those of outpatient cases were conservatively followed up after initial diagnosis. Among them, six hemispheres (12.8%) of four patients had been proven to show apparent progression of steno-occlusive lesion and were subjected to revascularization surgery. Postoperative courses were uneventful in all four cases, and no patient suffered cerebrovascular event on the operated hemisphere after surgery. CONCLUSION: Adult-onset moyamoya disease, either bilateral or unilateral, has a substantial risk for progression, and careful follow-up is necessary for asymptomatic hemisphere. Once the patient manifests as the progression of cerebrovascular occlusive lesions or ischemic symptoms, we recommend revascularization surgery after the confirmation of the hemodynamic compromise.
OBJECT: In moyamoya disease, despite its progressive nature of the occlusive lesions in pediatric patients, the prevalence of the progression in adult patients is undetermined. Furthermore, the optimal timing of the revascularization surgery for progressive cases is controversial. To address these issues, we retrospectively investigate four cases with the adult-onset moyamoya disease manifesting as progression before revascularization surgery. METHODS: From March 2004 to May 2007, 49 patients with adult-onset moyamoya disease aged from 19 to 62 years old (mean 40.5) underwent superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 63 hemispheres. All patients were strictly followed up by magnetic resonance (MR) imaging/angiography postoperatively. Twenty-seven hemispheres of 15 adult patients without surgery were also followed up at outpatient service during the same period. If the patients manifest as the progression of the steno-occlusive lesion on the hemisphere without surgery, they undergo revascularization surgery after the confirmation of hemodynamic compromise. RESULTS: During this period, 47 hemispheres including those of outpatient cases were conservatively followed up after initial diagnosis. Among them, six hemispheres (12.8%) of four patients had been proven to show apparent progression of steno-occlusive lesion and were subjected to revascularization surgery. Postoperative courses were uneventful in all four cases, and no patient suffered cerebrovascular event on the operated hemisphere after surgery. CONCLUSION: Adult-onset moyamoya disease, either bilateral or unilateral, has a substantial risk for progression, and careful follow-up is necessary for asymptomatic hemisphere. Once the patient manifests as the progression of cerebrovascular occlusive lesions or ischemic symptoms, we recommend revascularization surgery after the confirmation of the hemodynamic compromise.