REVIEW   Free access

International Angiology 2018 April;37(2):127-42

DOI: 10.23736/S0392-9590.18.03961-5

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Vascular malformations: classification, diagnosis and treatment

Inês M. CARQUEJA ¹, Joel SOUSA ^{1, 2} ✉, Armando MANSILHA ^{1, 2}

¹ Faculty of Medicine of the University of Porto, Porto, Portugal; ² Department of Angiology and Vascular Surgery, Hospital de S. João, Porto, Portugal

INTRODUCTION: Vascular malformations are congenital lesions with complex clinical presentations and management. Their classification and treatment options have considerably changed throughout the years, with conflicting evidence in the literature. In this article, we aim to review the classification, diagnosis and treatment of the main vascular malformations.
EVIDENCE ACQUISITION: A thorough non-systematic review of the literature was conducted using PubMed/Medline. Seventy-nine articles were selected and included, according to their scientific relevance and relation with the subject.
EVIDENCE SYNTHESIS: Vascular malformations occur due to errors during vasculogenesis. They are present at birth, though they may not be apparent until later in life. These malformations are most frequently found in the head and neck, but can occur in any part of the body. They can be divided according to the predominant vessel type into arteriovenous, venous, capillary and lymphatic. Combined malformations occur when more than one type of vessel is present. Clinical presentation is variable and depends on the type of malformation, as well as location, size and relation with other structures. Symptoms such as ischemia, swelling, pain, thrombosis, deformity and functional impairment can be caused by these lesions. The diagnosis of vascular malformations is based in both clinical presentation and complementary imaging techniques, with special emphasis on magnetic resonance imaging. Depending on the malformation and clinical presentation, treatment may be medical or interventional, by means of either interventional techniques or surgery.
CONCLUSIONS: Vascular malformations are a complex group of pathologies, with different clinical presentations and treatment options, and therefore management by a multi-disciplinary team is essential. Their cure is often challenging and when not possible, treatment should aim at symptomatic control and improvement of patient’s quality of life.

KEY WORDS: Vascular malformations - Arteriovenous malformations - Lymphatic abnormalities - Lymphedema