Document Detail


Klippel-Trenaunay-type syndrome: an eponym for various expressions of the same entity.
MedLine Citation:
PMID:  8721902     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Klippel-Trenaunay (KT) or as it is also called Klippel-Trenaunay-Weber (KTW) syndrome is a rare congenital phacomatosis of uncertain aetiology and variable expression. The classical syndrome is a triad of congenital mesodermal abnormalities characterized by cutaneous angiomatous nevus commonly called port-wine stain, venous varicosities and hypertrophy of soft tissue and/or overgrowth of bone of one or more limbs. Clinically, a diversity of phenotypes with subjacent malformations may be encountered all having in common abnormalities of the mesoblastic sheets that include angioblastic, lymphoblastic and osteoblastic lineages. Each of them may give rise to malformations that may exist alone or in an unlimited diversity of associations. We present three widely different clinical expressions of this syndrome and we support the proposal to group all these malformations under the eponym of Klippel-Trenaunay-Type (KTT) syndrome.
Authors:
B S Atiyeh; R S Musharrafieh
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of medicine     Volume:  26     ISSN:  0025-7850     ISO Abbreviation:  J Med     Publication Date:  1995  
Date Detail:
Created Date:  1996-12-10     Completed Date:  1996-12-10     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7505566     Medline TA:  J Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  253-60     Citation Subset:  IM    
Affiliation:
Division Plastic and Reconstructive Surgery, American University of Beirut-Medical Center, Beirut, Lebanon.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Female
Humans
Hypertrophy
Klippel-Trenaunay-Weber Syndrome / diagnosis*,  pathology
Leg
Magnetic Resonance Imaging
Male
Muscles / pathology
Terminology as Topic*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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