title: Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization.
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.
authors.affiliation: Department of Neurological Surgery, Oregon Health & Science University, Portland, OR; Doernbecher Children's Hospital, Portland, OR.
authors.forename: Lissa C
authors.initials: LC
authors.lastname: Baird
authors.affiliation: Department of Neurological Surgery, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Edward R
authors.initials: ER
authors.lastname: Smith
authors.affiliation: Departments of Neurology and Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA; Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Ichord
authors.affiliation: Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA; Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: David A
authors.initials: DA
authors.lastname: Piccoli
authors.affiliation: Children's Hospital Colorado, Aurora, CO; University of Colorado, Aurora, CO.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Bernard
authors.affiliation: Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA; Division of Genomic Diagnostics, The Children's Hospital of Philadelphia, Philadelphia, PA.
authors.forename: Nancy B
authors.initials: NB
authors.lastname: Spinner
authors.affiliation: Department of Neurological Surgery, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: R Michael
authors.initials: RM
authors.lastname: Scott
authors.affiliation: Division of Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada; University of Toronto, Toronto, Ontario, Canada. Electronic address: binita.kamath@sickkids.ca.
authors.forename: Binita M
authors.initials: BM
authors.lastname: Kamath
mesh_terms: D016738:Alagille Syndrome / Q000150:complications*; D002548:Cerebral Revascularization; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009072:Moyamoya Disease / Q000209:etiology* / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome