The curious case of the disappearing IVC: A case report and review of the aetiology of Inferior Vena Cava Agenesis

Abstract

We report the case of a previously well 18-year-old male who presented to the Emergency Department with lower limb pain. An ultrasound demonstrated extensive left sided deep vein thrombosis and computed tomography demonstrated inferior vena cava agenesis, leading to the diagnosis of inferior vena cava agenesis associated deep vein thrombosis. The aetiology of inferior vena cava agenesis is explored in depth.

Inferior vena cava anomalies and variations: imaging and rare clinical findings

“…Today, the most frequently encountered and published anomalies include retroaortic left renal vein, left IVC, double IVC, circumaortic left renal vein, interruption of IVC with azygos and hemiazygos continuation, absence of the infrarenal IVC, and circumcaval ureter [ 2 , 8 , 12 ]. In general, the prevalence of IVC anomalies is reported as 0.5 % in the world [ 13 ]. In the present pictorial essay we summarize imaging findings of ten patients with frequent and infrequent IVC anomalies including absence of infrarenal IVC, left IVC, double IVC, interruption of IVC with CA, left retroaortic vein, and accessory continuous hemiazygos vein.…”

The Typical Presentation Spectrum of Deep Vein Thrombosis Associated with Inferior Vena Cava Malformations

“…Despite its advantage of being a rapid noninvasive imaging modality, MRI imaging is now replacing CT as the optimal investigative tool avoiding radiation and giving more accurate delineation of thrombus as well as any IVC anomaly. MRI is also used to follow up patients to determine morphological changes in the thrombus following therapy [ 17 ]. Only 56% of patients in our case series underwent MRI; however, in the case reports from 2014 onwards, 79% underwent an MRI underlining the tendency mentioned above.…”

Deep Vein Thrombosis Provoked by Inferior Vena Cava Agenesis

“…The exact aetiology of the IVCA remains unclear, but it is believed that thrombosis of the IVC in the perinatal period can lead to agenesis [ 6 , 7 ]. Anomalies of the IVC are sometimes associated with other congenital anomalies, especially of the spleen and liver, as they have concurrent development with the IVC embryologically [ 1 ].…”

“…Giving the rarity of the disease, there is no evidence based approach management [ 14 ]. Treatment is directed towards prevention of thrombosis formation or recurrence; life-long vitamin K antagonist is required for patients with IVCA in addition to elastic stocking and leg elevation to relieve venous stasis [ 1 , 2 , 8 , 15 ]. Surgical correction can be an option for management depending on the severity of the venous stasis symptoms and response to medical and conservative treatment [ 11 , 14 , 16 , 17 ].…”

“…Inferior vena cava agenesis (IVCA) is a rare congenital anomaly. IVCA can be asymptomatic or can present with vague, nonspecific symptoms such as abdominal or lower back pain [ 1 ]. Most of the patients with IVCA can also present with DVT [ 2 ].…”