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

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

Research output: Contribution to journalReview article

14 Scopus citations

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 2017

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ASJC Scopus subject areas

  • Surgery
  • Cardiology and Cardiovascular Medicine

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