Format

Send to

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

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

Author information

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.

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.

PMID:
25465847
DOI:
10.1016/j.jpeds.2014.10.067
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center