Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization.

Baird, Lissa C; Smith, Edward R; Ichord, Rebecca; Piccoli, David A; Bernard, Timothy J; Spinner, Nancy B; Scott, R Michael; Kamath, Binita M · J Pediatr · 2015

case_series · Level IV

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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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