Evaluation and management of the lateral marginal vein in Klippel-Trénaunay and other PIK3CA-related overgrowth syndromes

The lateral marginal vein is an anomalous clinical entity found in association with Klippel-Trénaunay and other PIK3CA-related overgrowth syndromes. Although it is reported to affect <20% of patients with Klippel-Trénaunay syndrome, this venous anomaly has been associated with significant morbidi...

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Bibliographic Details
Published inJournal of vascular surgery. Venous and lymphatic disorders (New York, NY) Vol. 8; no. 3; pp. 482 - 493
Main Authors Fereydooni, Arash, Nassiri, Naiem
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.05.2020
Subjects
Ablation Techniques - adverse effects
Class I Phosphatidylinositol 3-Kinases - genetics
Embolization, Therapeutic - adverse effects
Endovascular Procedures - adverse effects
Genetic Predisposition to Disease
Humans
Klippel-Trenaunay-Weber Syndrome - diagnostic imaging
Klippel-Trenaunay-Weber Syndrome - genetics
Klippel-Trenaunay-Weber Syndrome - therapy
Klippel-Trénaunay syndrome
Lateral marginal vein
Mutation
Persistent embryonic vein
Phenotype
PIK3CA-related overgrowth syndromes
Treatment Outcome
Veins - abnormalities
Veins - diagnostic imaging
Veins - surgery
Venous Insufficiency - diagnostic imaging
Venous Insufficiency - genetics
Venous Insufficiency - therapy
Persistent embryonic vein
PIK3CA-related overgrowth syndromes
Lateral marginal vein
Klippel-Trénaunay syndrome
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Summary:The lateral marginal vein is an anomalous clinical entity found in association with Klippel-Trénaunay and other PIK3CA-related overgrowth syndromes. Although it is reported to affect <20% of patients with Klippel-Trénaunay syndrome, this venous anomaly has been associated with significant morbidity and mortality attributable to venous hypertension and potentially lethal thromboembolic events. Limited literature exists on the diagnosis and management of this rare anomaly, with most of the reports focusing on retrospective clinical experience at a few centers of excellence. Despite these limitations, a systematic approach to diagnosis and treatment of this anomaly is warranted and expounded on herein. When plausible, clinical recommendations based on best available literature are made.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
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ISSN:2213-333X
2213-3348
DOI:10.1016/j.jvsv.2019.12.003