Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome

S. Keisin Wang, Natalie A. Drucker, Alok K. Gupta, Francis E. Marshalleck, Michael Dalsing

Research output: Contribution to journalReview article

9 Citations (Scopus)

Abstract

Objective A dearth of information exists in the literature regarding current practice in the management of Klippel-Trénaunay syndrome (KTS), a rare condition. We review and describe the etiology, diagnosis, and treatment of KTS. Methods Relevant data were synthesized from a Medline review using a combination of the keyterms “Klippel” and “Trénaunay.” The majority of hits described singular case reports and were subsequently excluded. The remaining papers were then reviewed and included on the basis of the quality of evidence and the authors' discretion. Conclusions KTS is characterized by a clinical triad of extremity varicosities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is clinically supplemented with magnetic resonance imaging and computed tomography. Although this syndrome is associated with significant comorbidities, such as pain, edema, ulcerations, and pruritus, it is rarely the cause of death. The backbone of treatment is nonoperative in nature but should be supplemented with minimally invasive, endovascular, and rarely open surgical procedures for refractory cases.

Original languageEnglish (US)
Pages (from-to)587-595
Number of pages9
JournalJournal of Vascular Surgery: Venous and Lymphatic Disorders
Volume5
Issue number4
DOIs
StatePublished - Jul 1 2017

Fingerprint

Vascular Malformations
Practice Management
Pruritus
Hypertrophy
Comorbidity
Cause of Death
Edema
Extremities
Tomography
Magnetic Resonance Imaging
Bone and Bones
Pain
Skin
Therapeutics

ASJC Scopus subject areas

  • Surgery
  • Cardiology and Cardiovascular Medicine

Cite this

Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome. / Wang, S. Keisin; Drucker, Natalie A.; Gupta, Alok K.; Marshalleck, Francis E.; Dalsing, Michael.

In: Journal of Vascular Surgery: Venous and Lymphatic Disorders, Vol. 5, No. 4, 01.07.2017, p. 587-595.

Research output: Contribution to journalReview article

Wang, S. Keisin ; Drucker, Natalie A. ; Gupta, Alok K. ; Marshalleck, Francis E. ; Dalsing, Michael. / Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome. In: Journal of Vascular Surgery: Venous and Lymphatic Disorders. 2017 ; Vol. 5, No. 4. pp. 587-595.
@article{e317887ceafc4a05956a534e077e11e2,
title = "Diagnosis and management of the venous malformations of Klippel-Tr{\'e}naunay syndrome",
abstract = "Objective A dearth of information exists in the literature regarding current practice in the management of Klippel-Tr{\'e}naunay syndrome (KTS), a rare condition. We review and describe the etiology, diagnosis, and treatment of KTS. Methods Relevant data were synthesized from a Medline review using a combination of the keyterms “Klippel” and “Tr{\'e}naunay.” The majority of hits described singular case reports and were subsequently excluded. The remaining papers were then reviewed and included on the basis of the quality of evidence and the authors' discretion. Conclusions KTS is characterized by a clinical triad of extremity varicosities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is clinically supplemented with magnetic resonance imaging and computed tomography. Although this syndrome is associated with significant comorbidities, such as pain, edema, ulcerations, and pruritus, it is rarely the cause of death. The backbone of treatment is nonoperative in nature but should be supplemented with minimally invasive, endovascular, and rarely open surgical procedures for refractory cases.",
author = "Wang, {S. Keisin} and Drucker, {Natalie A.} and Gupta, {Alok K.} and Marshalleck, {Francis E.} and Michael Dalsing",
year = "2017",
month = "7",
day = "1",
doi = "10.1016/j.jvsv.2016.10.084",
language = "English (US)",
volume = "5",
pages = "587--595",
journal = "Journal of Vascular Surgery: Venous and Lymphatic Disorders",
issn = "2213-333X",
publisher = "Elsevier Inc.",
number = "4",

}

TY - JOUR

T1 - Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome

AU - Wang, S. Keisin

AU - Drucker, Natalie A.

AU - Gupta, Alok K.

AU - Marshalleck, Francis E.

AU - Dalsing, Michael

PY - 2017/7/1

Y1 - 2017/7/1

N2 - Objective A dearth of information exists in the literature regarding current practice in the management of Klippel-Trénaunay syndrome (KTS), a rare condition. We review and describe the etiology, diagnosis, and treatment of KTS. Methods Relevant data were synthesized from a Medline review using a combination of the keyterms “Klippel” and “Trénaunay.” The majority of hits described singular case reports and were subsequently excluded. The remaining papers were then reviewed and included on the basis of the quality of evidence and the authors' discretion. Conclusions KTS is characterized by a clinical triad of extremity varicosities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is clinically supplemented with magnetic resonance imaging and computed tomography. Although this syndrome is associated with significant comorbidities, such as pain, edema, ulcerations, and pruritus, it is rarely the cause of death. The backbone of treatment is nonoperative in nature but should be supplemented with minimally invasive, endovascular, and rarely open surgical procedures for refractory cases.

AB - Objective A dearth of information exists in the literature regarding current practice in the management of Klippel-Trénaunay syndrome (KTS), a rare condition. We review and describe the etiology, diagnosis, and treatment of KTS. Methods Relevant data were synthesized from a Medline review using a combination of the keyterms “Klippel” and “Trénaunay.” The majority of hits described singular case reports and were subsequently excluded. The remaining papers were then reviewed and included on the basis of the quality of evidence and the authors' discretion. Conclusions KTS is characterized by a clinical triad of extremity varicosities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is clinically supplemented with magnetic resonance imaging and computed tomography. Although this syndrome is associated with significant comorbidities, such as pain, edema, ulcerations, and pruritus, it is rarely the cause of death. The backbone of treatment is nonoperative in nature but should be supplemented with minimally invasive, endovascular, and rarely open surgical procedures for refractory cases.

UR - http://www.scopus.com/inward/record.url?scp=85020773263&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85020773263&partnerID=8YFLogxK

U2 - 10.1016/j.jvsv.2016.10.084

DO - 10.1016/j.jvsv.2016.10.084

M3 - Review article

C2 - 28624001

AN - SCOPUS:85020773263

VL - 5

SP - 587

EP - 595

JO - Journal of Vascular Surgery: Venous and Lymphatic Disorders

JF - Journal of Vascular Surgery: Venous and Lymphatic Disorders

SN - 2213-333X

IS - 4

ER -