Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization

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

doi:10.1016/j.jpeds.2014.10.067

Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization

J Pediatr. 2015 Feb;166(2):470-3. doi: 10.1016/j.jpeds.2014.10.067. Epub 2014 Oct 30.

Authors

Lissa C Baird¹, Edward R Smith², Rebecca Ichord³, David A Piccoli⁴, Timothy J Bernard⁵, Nancy B Spinner⁶, R Michael Scott², Binita M Kamath⁷

Affiliations

¹ Department of Neurological Surgery, Oregon Health & Science University, Portland, OR; Doernbecher Children's Hospital, Portland, OR.
² Department of Neurological Surgery, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
³ Departments of Neurology and Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA; Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA.
⁴ 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.
⁵ Children's Hospital Colorado, Aurora, CO; University of Colorado, Aurora, CO.
⁶ Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA; Division of Genomic Diagnostics, The Children's Hospital of Philadelphia, Philadelphia, PA.
⁷ 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.

PMID: 25465847
DOI: 10.1016/j.jpeds.2014.10.067

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.

Publication types

Evaluation Study

MeSH terms

Alagille Syndrome / complications*
Cerebral Revascularization*
Child, Preschool
Female
Humans
Infant
Male
Moyamoya Disease / etiology*
Moyamoya Disease / surgery*
Retrospective Studies
Treatment Outcome