| Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies. | |
| | |
MedLine Citation:
|
PMID: 18370916 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
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. |
| | |
Authors:
|
Anne M Comi |
Related Documents
:
|
15105686 - Klippel-feil syndrome associated with persistent trigeminal artery. 9314616 - Klippel-trenaunay-weber syndrome. 1770536 - Transmission of proteus syndrome from father to son? 11040486 - Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive ... 7788106 - Vigabatrin in the management of generalized seizures in children. 2111636 - Mesangial immunoglobulin a deposits in minimal change nephrotic syndrome: a report of a... |
Publication Detail:
|
Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
|
Title: Lymphatic research and biology Volume: 5 ISSN: 1539-6851 ISO Abbreviation: Lymphat Res Biol Publication Date: 2007 |
Date Detail:
|
Created Date: 2008-03-28 Completed Date: 2008-05-20 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 101163587 Medline TA: Lymphat Res Biol Country: United States |
Other Details:
|
Languages: eng Pagination: 257-64 Citation Subset: IM |
Affiliation:
|
Neurology and Developmental Medicine, Kennedy Krieger Institute, Department of Neurology and Pediatrics, Johns Hopkins School of Medicine; Baltimore, MD 21205, USA. comi@kennedykrieger.org |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Brain
/
blood supply,
pathology Brain Diseases / etiology, pathology*, physiopathology Cerebrovascular Circulation Humans Sturge-Weber Syndrome / complications, physiopathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: The placenta theory and the origin of infantile hemangioma.
Next Document: Lymphatic drainage of calf melanoma skipping the superficial groin.