Neurosurg Focus
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Moyamoya disease is a chronic cerebrovascular occlusive disorder that results in severe morbidity and death. There is much controversy surrounding the optimal treatment for adult patients with the disorder. There have been no randomized trials to assess the efficacy of any single surgical treatment, and existing case series suffer from inadequate power, selection bias, and inherent differences in patient characteristics. In this article the authors review the literature concerning the optimal surgical treatment of adult patients with moyamoya disease.
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Moyamoya disease (MMD) is a progressive, occlusive disease of the distal internal carotid arteries associated with secondary stenosis of the circle of Willis. Symptoms include ischemic infarcts in children and hemorrhages in adults. Bypass of the stenotic vessel(s) is the primary surgical treatment modality for MMD. ⋯ Indirect techniques rely on the approximation of vascularized tissue to the cerebral cortex to promote neoangiogenesis. This tissue may be in the form of muscle, pericranium, dura, or even omentum. This review highlights the surgical options available for the treatment of MMD.
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Many children with sickle cell anemia (SCA) also have clinical and radiographic findings of an arteriopathy suggestive of moyamoya syndrome. These patients may continue to experience strokes despite optimal medical management. The authors wished to define features of moyamoya syndrome associated with SCA and determine the results of surgical revascularization in these patients at early and late follow-up. ⋯ The clinical and radiographic features of moyamoya syndrome associated with SCA appear comparable to primary moyamoya disease. Successful treatment of these patients requires multidisciplinary care involving hematologists, anesthesiologists, and neurosurgeons. Operative treatment of moyamoya syndrome using pial synangiosis appears to be safe and confers long-lasting protection against further stroke in this population, and provides an alternative for failure of optimal medical therapy in patients. This study underscores the potential merit of screening patients with SCA for moyamoya syndrome.