Agenesis of the Intrahepatic Inferior Vena Cava: A Case Report and Literature Review

Abstract

Anomalies of the inferior vena cava (IVC) are an uncommon finding in the general population. A wide range of IVC anomalies has been described in the literature, the majority of which lack clinical significance.Agenesis of the IVC (AIVC) is a rare anomaly of the IVC in the general population. This anomaly may involve either complete agenesis of the IVC or agenesis of a segment of the IVC. Agenesis of the suprarenal segment is the most commonly occurring variant, while agenesis of the infrarenal and hepatic segments is less common. Here we report a case of agenesis of the intrahepatic segment of the IVC.

Infrarenal inferior vena cava agenesis presenting as acute abdomen and hydronephrosis – case report

“…The latter is divided based on location into intrahepatic, suprarenal, renal, and infrarenal segment agenesis. Only 6% of all cases are renal or infrarenal variants [1] , [2] , [3] , 5 , 7] . Some authors suggest that agenesis of the infrarenal IVC segment develops because of intrauterine thrombosis rather than a congenital malformation, but there is still no clear evidence for either theory [2] , [3] , [4] , 8 , 9] .…”

“…Unfortunately, it is often challenging to visualize VCI due to overlaying structures and bowel gas. Therefore, CT or MRI angiography is done because these modalities have much higher sensitivity and can easily show the exact anatomy of the collateral network [3 , 8 , 9 , 13 , 19 , 21] . In addition, genetic testing for thrombophilia and searching for other causes of hypercoagulability, such as prolonged immobilization, recent trauma, surgery, or vascular catheterization, are highly recommended [8] .…”

“…Administration of anticoagulant medications, compression stockings, lower extremity elevation, and thrombolysis in selected cases is the most common treatment strategy, but since the condition is rare, there is still debate about the therapeutic strategy [8 , 9 , 14 , 15 , 20 , 21] . While many studies suggest lifelong anticoagulation [3 , 21] , others suggest against it. Riera-Mestre et al.…”

“…Its embryonic development is complex and consists of several interconnected phases between the sixth and eighth gestational weeks: formation, regression, and anastomosis of 4 pairs of veins (supracardinal and subcardinal veins, posterior cardinal vein, and vitelline [omphalomesenteric] veins). Understandably, many different malformations can occur, such as duplication, malposition, or agenesis, most of which have no clinical significance [1] , [2] , [3] , [4] . Their incidence in the general population ranges from 0.05% to 8.7% [5] .…”