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Klippel‐Trenaunay syndrome

Klippel‐Trenaunay syndrome <h5>INTRODUCTION</h5> Klippel‐Trenaunay syndrome is an important vascular disorder that is often misunderstood in some quarters. Here I define Klippel‐Trenaunay syndrome, distinguish vascular malformations from vascular tumors, and challenge four commonly held conceptions about Klippel‐Trenaunay syndrome. <h5>KLIPPEL‐TRENAUNAY SYNDROME DEFINED</h5> Klippel‐Trenaunay syndrome 1 consists of 1) combined vascular malformations of the capillary, venous, and lymphatic types, 2 2) varicosities of unusual distribution, in particular the lateral venous anomaly (Fig. 1 ) observed during infancy or childhood, and 3) limb enlargement. Males and females are equally affected. The lower limb is involved in almost 95% of patients, the upper limb accounting for about 5% of patients. Approximately 15% have combined upper and lower limb involvement. Uncommonly, patients may have trunk involvement only [Young, 1988 ; Servelle, 1985 ; Lindenauer, 1965 ; Samuel and Spitz, 1995 ]. Well over 1,500 cases have been recorded [Lindenauer, 1965; Samuel and Spitz, 1995 ; Servelle, 1985 ; Young, 1988 ] (J.B. Mulliken, personal communication, 1999). Servelle [ 1985 ] alone documented 768 operated patients. 1 Diagram of lateral venous anomaly. From Young [ 1988 ]. Varicosities in Klippel‐Trenaunay syndrome differ from commonly occurring varicose veins in two ways. The distribution is different, being more extensive, http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Medical Genetics Part A Wiley

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References (48)

Publisher
Wiley
Copyright
Copyright © 2000 Wiley Subscription Services, Inc., A Wiley Company
ISSN
1552-4825
eISSN
1552-4833
DOI
10.1002/1096-8628(20000731)93:3<171::AID-AJMG1>3.0.CO;2-K
Publisher site
See Article on Publisher Site

Abstract

<h5>INTRODUCTION</h5> Klippel‐Trenaunay syndrome is an important vascular disorder that is often misunderstood in some quarters. Here I define Klippel‐Trenaunay syndrome, distinguish vascular malformations from vascular tumors, and challenge four commonly held conceptions about Klippel‐Trenaunay syndrome. <h5>KLIPPEL‐TRENAUNAY SYNDROME DEFINED</h5> Klippel‐Trenaunay syndrome 1 consists of 1) combined vascular malformations of the capillary, venous, and lymphatic types, 2 2) varicosities of unusual distribution, in particular the lateral venous anomaly (Fig. 1 ) observed during infancy or childhood, and 3) limb enlargement. Males and females are equally affected. The lower limb is involved in almost 95% of patients, the upper limb accounting for about 5% of patients. Approximately 15% have combined upper and lower limb involvement. Uncommonly, patients may have trunk involvement only [Young, 1988 ; Servelle, 1985 ; Lindenauer, 1965 ; Samuel and Spitz, 1995 ]. Well over 1,500 cases have been recorded [Lindenauer, 1965; Samuel and Spitz, 1995 ; Servelle, 1985 ; Young, 1988 ] (J.B. Mulliken, personal communication, 1999). Servelle [ 1985 ] alone documented 768 operated patients. 1 Diagram of lateral venous anomaly. From Young [ 1988 ]. Varicosities in Klippel‐Trenaunay syndrome differ from commonly occurring varicose veins in two ways. The distribution is different, being more extensive,

Journal

American Journal of Medical Genetics Part AWiley

Published: Jul 31, 2002

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