Document Detail

Persistent fetal vasculature and spontaneous hyphema in a patient with Klippel-Trénaunay-Weber syndrome.
MedLine Citation:
PMID:  20451865     Owner:  NLM     Status:  MEDLINE    
Klippel-Trénaunay-Weber syndrome is characterized by the triad of capillary malformations (usually port-wine stains), varicose veins or venous malformations of unusual distribution, and soft-tissue or bony hypertrophy of an extremity. The syndrome can be diagnosed on the basis of 2 of these 3 features. In the atypical form of the syndrome, capillary malformations may be absent. Recognition is possible during infancy or early childhood, and evaluation and treatment is important to minimize morbidity. We describe the case of an 11-year-old girl who presented with hyphema after a Valsalva maneuver and was found to have persistent fetal vasculature in the affected eye. She had systemic features of Klippel-Trénaunay-Weber syndrome. Magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance venography scans of the brain and orbits were normal. Magnetic resonance imaging scan of the left hypertrophied lower limb revealed venous malformations leading to soft-tissue hypertrophy. To our knowledge, this is the first case of persistent fetal vasculature and hyphema in a patient with Klippel-Trénaunay-Weber syndrome.
Luna Dhir; Anthony G Quinn
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus     Volume:  14     ISSN:  1528-3933     ISO Abbreviation:  J AAPOS     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-05-10     Completed Date:  2010-08-04     Revised Date:  2010-09-01    
Medline Journal Info:
Nlm Unique ID:  9710011     Medline TA:  J AAPOS     Country:  United States    
Other Details:
Languages:  eng     Pagination:  190-2     Citation Subset:  IM    
Copyright Information:
Copyright 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.
West of England Eye Unit, Royal Devon & Exeter Hospital, Exeter, United Kingdom.
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MeSH Terms
Hyphema / diagnosis,  etiology*
Klippel-Trenaunay-Weber Syndrome / complications*,  diagnosis
Leg / pathology
Magnetic Resonance Imaging
Persistent Hyperplastic Primary Vitreous / diagnosis,  etiology*
Comment In:
J AAPOS. 2010 Aug;14(4):376; author reply 376-7   [PMID:  20736130 ]

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