Abstract

Congenital malformations of the inferior vena cava (IVC) are rare and underreported. They can be a risk factor for deep venous thrombosis (DVT) as a result of inadequate venous drainage of the lower extremities through collateral circulation. The significant number of cases reported in the literature highlights their importance, warranting investigating their existence in younger individuals with idiopathic DVT of the lower extremities and pelvic veins. In this systematic review, we depict the typical presentation of IVC malformations, their management, and the management of their associated DVT.

Highlights

  • The inferior vena cava (IVC) congenital variants include agenesis, interruption with azygous or hemiazygous continuation, web formation, hypoplasia, left-sided location, and duplication; these conditions may be identified solely or in conjunction with one another

  • Evidence has shown that patients with agenesis of inferior vena cava (AIVC) are prone to develop deep vein thrombosis (DVT) of the lower extremities at a younger age [6]

  • We identified 188 patients with IVC malformation presenting with deep venous thrombosis (DVT)

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Summary

Introduction

The inferior vena cava (IVC) congenital variants include agenesis, interruption with azygous or hemiazygous continuation, web formation, hypoplasia, left-sided location, and duplication; these conditions may be identified solely or in conjunction with one another. Their projected prevalence in the general population is approximately 4% [1]; IVC anomalies are usually asymptomatic and are incidentally diagnosed during investigations for other medical conditions. Evidence has shown that patients with agenesis of inferior vena cava (AIVC) are prone to develop deep vein thrombosis (DVT) of the lower extremities at a younger age [6] This warrants investigating IVC malformation as an etiologic factor in young patients diagnosed with idiopathic DVT

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