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Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization
Lissa C Baird1, Edward R Smith2, Rebecca Ichord3
1Department of Neurological Surgery, Oregon Health & Science University, Portland, OR; Doernbecher Children's Hospital, Portland, OR.
Abstract:
Vasculopathy is well-described in Alagille syndrome (ALGS); however, few data exist regarding neurosurgical interventions. We report 5 children with ALGS with moyamoya who underwent revascularization surgery. Postsurgical complications included 1 stroke and 1 death from thalamic hemorrhage. Global function improved in survivors. Revascularization is reasonably safe in patients with ALGS and may improve neurologic outcomes.
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