Pelvic congestion syndrome due to agenesis of the infrarenal inferior vena cava

Abstract

The inferior vena cava (IVC) is the main conduit of venous return to the right atrium from the lower extremities and abdominal organs. Agenesis of the IVC has an incidence of <1% in the general population [1], although it has been reported in the literature as occurring in up to 8.7% of the population [2]. Patients with absent IVC may present with symptoms of lower extremity venous insufficiency [6], idiopathic deep venous thrombosis [7], or pelvic congestion syndrome. To our knowledge there have only been a few cases reported in the literature of agenesis of the IVC associated with pelvic congestion syndrome [3,10,11]. We present another interesting case of pelvic congestion syndrome due to absent IVC.

The inferior vena cava: anatomical variants and acquired pathologies

“…IVC anatomical variants primarily result from abnormal regression or persistence of embryological veins [7]. Although most anomalies are asymptomatic incidental findings, they can cause lower extremity venous insufficiency, deep vein thrombosis, pelvic congestion syndrome and affect planning of vascular procedures [10][11][12][13][14][15][16][17]. The most common anomalies include duplicated IVC, left-sided IVC and interruption of the IVC.…”

“…It is a rarely described anomaly with unknown incidence [11,39,41,42]. Affected patients are at risk of developing lower extremity venous insufficiency, deep vein thrombosis, varicose veins and pelvic congestion syndrome [10][11][12][13][14][15]. In the absence of adequate flow through ascending lumbar veins and the azygos system, other collateral pathways can form involving abdominal wall, pelvic, gonadal and retroperitoneal vessels (Fig.…”

Extensive deep venous thrombosis in a young male with absent infrarenal inferior vena cava

“… 12 , 13 Anatomical drainage for patients with absent IVC predisposes to thrombosis, as distal venous shunting through collaterals results in venous stasis. 14 , 15 Many cases subsequently present with DVT, with common precipitants being major physical exertion, anabolic steroids, and immobilization. 16 , 17 …”

Deep Venous Thrombosis and a Very Rare Finding: Inferior Vena Cava Infra-renal Segment Agenesis

“…Patients with IVC anomalies can remain asymptomatic as long as there is functioning collateral venous drainage. However, when prothrombotic or venous insufficiency risk factors are present, symptoms may start to develop, usually with signs of venous insufficiency, pelvic congestion syndrome, DVT and compression syndromes from dilated collateral vascularization [4] .…”