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Axial 2D FIESTA shows dilated parametrial veins up to 2 cm (arrow). On T2-weighted magnetic resonance images, pelvic varices appear as multiple hyperintense dilated tubular structures around the uterus, ovaries, and pelvic sidewall.
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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...
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Unilateral absence of pulmonary artery is a rare developmental anomaly. Infrahepatic inferior vena cava interruption is a well-recognized but uncommon developmental anomaly. Presence of both these anomalies in a single individual is extremely rare. A 58-year-old man with a history of recurrent lower extremity deep vein thrombosis and venous insuffi...
Citations
... Pelvic congestion syndrome is a chronic pelvic pain event caused by increased congestion in the pelvic area due to impaired venous drainage as a result of insufficiency of the pelvic veins (3). A rare cause of this syndrome is IVC agenesis (3,16). IVC agenesis should be considered in patients presenting with pelvic pain. ...
Inferior Vena cava (IVC) agenesis is a rare congenital anomaly, which may cause significant consequences with regard to morbidity and mortality. In this case report, a patient diagnosed with IVC agenesis which was seen intraabdominal intensive venous collateral during surgery for cholecystectomy is presented.
... Extensive collateral circulation develops to bypass the interruption, of which 4 pathways are the most significant: the paravertebral plexus draining into the vena cava superior (VCS) through the azygos-hemiazygos system, the hemorrhoidal plexus draining into the portal vein, the gonadal system draining into suprarenal VCI, and the superficial system draining into subclavian veins or VCS through anterior abdominal wall veins [10][11][12] . Besides the unusual iliocaval collateral, the paravertebral and abdominal wall collaterals were the primary pathways in our case. ...
The inferior vena cava agenesis (IVCA) is a rare and often asymptomatic malformation due to the abundant development of the collateral circulation. However, it is frequently found in young people and carries a significant risk of deep venous thrombosis (DVT). It is estimated that about 5% of patients under 30 years of age presenting with DVT have this condition. We report a case of a previously healthy 23-year-old patient presenting with signs of acute abdomen and hydronephrosis due to the thrombophlebitis of an unusual iliocaval venous collateral, which developed secondary to IVCA. After treatment, the iliocaval collateral and hydronephrosis completely regressed on a 1-year follow-up. To our knowledge, this is the first such case reported in the literature.
... 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 Patients with this condition are generally male, under 40 years of age, and without other identifiable risk factors for thrombosis, presenting with DVT. ...
A previously well, independent 20-year-old man presented with a 4-day history of progressive left lower limb pain with associated phlegmasia cerulea dolens. Duplex venous ultrasound and CT venogram revealed extensive deep vein thrombus from the left popliteal vein to abnormal venous vasculature proximally. Notably, no infrarenal inferior vena cava was detected, with distal venous return channeled through lumbar and visceral collateral channels into the azygous system. Treatment included systemic anticoagulation, catheter-directed thrombolysis, and prolonged therapeutic anticoagulation. In the absence of other risk factors, anatomical abnormalities should be considered in young, well patients presenting with lower limb venous thrombosis.
... 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. 8) [13]. ...
The inferior vena cava (IVC) is the largest vein in the body, draining blood from the abdomen, pelvis and lower extremities. This pictorial review summarises normal anatomy and embryological development of the IVC. In addition, we highlight a wide range of anatomical variants, acquired pathologies and a common pitfall in imaging of the IVC. This information is essential for clinical decision making and to reduce misdiagnosis.
... The majority of symptomatic patients complain of disabling venous claudication or skin changes caused by chronic elevation of peripheral venous pressure. Some patients may experience lumbago or pelvic congestion symptoms, caused by venous hypertension in paraspinal or pelvic veins [1]. ...
Background
It is unclear whether cardiopulmonary exercise intolerance in patients with chronic obstruction of the inferior vena cava (IVC) is reversible following endovascular IVC reconstruction.
Methods
In 17 patients (mean age 45 ± 15 years, 71% men) with post-thrombotic syndrome due to IVC obstruction and preserved left ventricular ejection fraction (mean 58 ± 3%), we performed cardiopulmonary exercise testing before and 3 months after IVC reconstruction (mean 4.1 ± 1.5 implanted stents). The median time from latest episode of deep vein thrombosis to intervention was 150 (interquartile range 102–820) days.
Results
At baseline, 12 (71%) patients reported New York Heart Association (NYHA) class II or III symptoms, 76% did not achieve >85% of predicted oxygen uptake at peak exercise (mean 61.8 ± 13.7%). After IVC reconstruction, the following changes were observed at anaerobic threshold: work rate increased by 14.6 W, 95%CI (−0.7; 30.0), oxygen uptake increased by 1.8 ml/kg, 95%CI (0.3; 3.3). Oxygen pulse increased by 1.95 ml per beat, 95%CI (1.12; 2.78), corresponding to a mean relative increase of 22.5%, 95%CI (12.4; 32.7) (p < 0.001). The following changes were observed at peak exercise: work rate increased by 48.1 W, 95%CI (27.8; 68.4), oxygen uptake increased by 6.4 ml/kg, 95%CI (3.8; 9.1). Oxygen pulse increased by 2.68 ml per beat, 95%CI (1.60; 3.76), corresponding to a mean relative increase of 29.4%, 95%CI (17.7; 41.2) (p < 0.001). At follow-up, 5 (29%) patients remained in NYHA class II.
Conclusions
In patients with chronic IVC obstruction, cardiopulmonary exercise intolerance as a result of impaired cardiac filling is at least partially reversible following endovascular IVC reconstruction.
Study registration
URL: https://clinicaltrials.gov. Unique identifier: NCT02433054.
... There has also been reports of surgical interventions such as the venous thrombectomy or even the prosthetic replacement of the IVC, with successful results. The surgical approach is only recommended for those patients with rapid deterioration of the chronic venous insufficiency, or those that present DVT and concomitant collapse of the collateral venous drainage.1,3,6,16 ...
Abnormalities of the inferior vena cava (IVC) are rare. Its embryological development occurs between the sixth and eighth week of gestation, and depends on the persistence or regression of 3 pairs of veins: the posterior cardinal veins, the sub cardinal veins and the supra cardinal veins. The type of congenital alteration depends the moment that embryogenesis is altered. The most frequent clinical presentation is deep vein thrombosis, which occurs mainly in young men. Other clinical presentations are pelvic venous congestion and chronic venous insufficiency. The diagnosis requires a high diagnostic suspicion, and it is mainly through contrast-enhanced computed tomography of the abdomen and pelvis. The treatment will depend on the type of malformation and its presentation.
... 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] . Conservative therapy with anticoagulation, compression stockings and management of complications seem to be the mainstay of treatment, but invasive surgical interventions with an endovascular approach or even prosthetic replacement of the IVC have also been reported, with successful results [1,5] . ...
Bilateral deep vein thrombosis (DVT) should prompt investigation for pro-thrombotic conditions and the exclusion of vascular compromise due to intrinsic and extrinsic factors. The authors present the case of a 47-year-old man admitted with bilateral DVT and diagnosed with inferior vena cava (IVC) infra-renal segment agenesis, and discuss the management of this rare condition.
LEARNING POINTS
Bilateral deep vein thrombosis in young and otherwise healthy individuals should prompt investigation of the aetiology and risk factors in case life-long treatment and anticoagulation therapy is required.
Although rare, anatomical variations and congenital malformations should be considered even in adult patients.
Rare medical cases should prompt a multidisciplinary approach to investigation and treatment, as prognosis will depend on both therapeutic procedures and anticipation of complications.
Pelvic venous disorders are inter-related pathologic conditions caused by reflux and obstruction in the pelvic veins. It can present a spectrum of clinical features based on the route of transmission of venous hypertension to either distal or caudal venous reservoirs. Imaging can help to visualize pelvic vascular and visceral structures to rule out other gynecologic, gastrointestinal, and urologic diseases. Endovascular treatment, owing to its low invasive nature and high success rate, has become the mainstay in the management of pelvic venous disorders. This article reviews the pathophysiology, clinical presentations, and diagnostic and therapeutic approaches to pelvic venous disorders.
Pelvic venous disorders are a group of interrelated conditions characterized by venous incompetence. These conditions often manifest with nonspecific symptoms that overlap with many gynecological, gastrointestinal, and urologic diseases. Clinical diagnosis can be difficult, and imaging can play a vital role in differentiating etiology. Sonographic evaluation is often the first step in evaluating these symptoms. Special attention to possible underlying pelvic venous disorders can reveal characteristic findings, support diagnosis, and guide treatment. Here we review pelvic congestion syndrome, nutcracker syndrome, May-Thurner syndrome, and other venous disorders, with a specific focus on sonographic findings and considerations.
Abnormalities of the inferior vena cava are rare. Its embryological development occurs between the sixth and eighth week of gestation and depends on the persistence or regression of three pairs of veins: the posterior cardinal veins, the subcardinal veins and the supracardinal veins. The type of congenital alteration depends on the moment that embryogenesis is altered. The most frequent clinical presentation is deep vein thrombosis, which occurs mainly in young men. We report a 16-year-old male presenting with edema of the left leg. No risk factor for thrombosis was recorded. A Doppler ultrasound confirmed the presence of a deep femoro-popliteal vein thrombosis. An abdominal CAT scan showed an agenesis of the supra and infra-adrenal inferior vena cava, with multiple collaterals. The study for thrombophilia was negative. The patient was treated with vitamin K antagonists with a good clinical response.
