Format

Send to

Choose Destination

See 1 citation found using an alternative search:

See comment in PubMed Commons below
Lymphat Res Biol. 2007;5(4):257-64. doi: 10.1089/lrb.2007.1016.

Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies.

Author information

  • 1Neurology and Developmental Medicine, Kennedy Krieger Institute, Department of Neurology and Pediatrics, Johns Hopkins School of Medicine; Baltimore, MD 21205, USA. comi@kennedykrieger.org

Abstract

Sturge-Weber syndrome (SWS) is defined by the association of a facial capillary malformation (port-wine stain), with a vascular malformation of the eye, and/or vascular malformation of the brain (leptomeningeal angioma). Variants exist where only one of these three structures is involved with the vascular malformation. SWS occurs sporadically and is congenital. Port-wine stains occur in 3 per 1000 live births. No good population-based data exist for how many people have Sturge-Weber syndrome, however, estimates range between one in 20-50,000 live births. This review summarizes literature regarding the main features and pathophysiology of Sturge-Weber syndrome, however the cause of this syndrome remains obscure. Recent advances in neuroimaging have provided important insights into the progression of neurologic injury that occurs as a result of impaired blood flow. Important limitations exist, however, as currently the early diagnosis and exclusion of Sturge-Weber syndrome is impaired by the poor sensitivity of imaging in the newborn period and early infancy. Several important controversies complicate our ability to care for these patients and include the questions of ideal timing of surgery, whether seizures themselves contribute to the neurologic injury, and what the role of low-dose aspirin should be. This review will summarize several recent advances in our understanding of the mechanisms of brain injury in SWS, new measures for quantifying the neurologic involvement and new approaches and controversies in the management of the neurologic complications.

PMID:
18370916
DOI:
10.1089/lrb.2007.1016
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Mary Ann Liebert, Inc.
    Loading ...
    Support Center