Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration: A surgically treated case

Abstract

Absence of the inferior vena cava (IVC) is an uncommon congenital abnormality. Symptoms of lower extremity venous insufficiency resulting from this anatomic abnormality have been reported only once in the English literature, and no experience with surgical treatment of this condition has been published. We report the case of an otherwise healthy 41-year-old man with an 18-month history of severe venous insufficiency involving the right leg manifested by extensive ulceration that did not respond to aggressive conservative treatment. Duplex findings were not suggestive of venous obstruction or reflux, but venography documented no filling of the common iliac vein or inferior vena cava, and outflow was via collaterals to the azygous and hemiazygous systems. Computed tomography demonstrated complete absence of the inferior vena cava with azygous continuation. A prosthetic bypass from the external iliac to the intrathoracic azygous vein was performed with complete symptomatic relief after a 30-month follow-up period. Venous bypass surgery may play a role in treatment of this rare cause of venous insufficiency.

Agenesis of Inferior Vena Cava with Cavernous Transformation and Hypoplastic Infrarenal Part of IVC, Rare Congenital Vascular Anomalies: A Case Series

“…The reasons for the developmental failure are unclear. One hypothesis is embryonic dysontogenesis [1,11,12], but some authors suggest that it is the result of an intrauterineor perinatal thrombosis [10]. But in our case, patient did not presented with DVT despite of dysgenesis of IVC.…”

“…The hepatic segment drains directly into the right atrium, and the blood from the infrarenal IVC returns to the heart through the azygos and hemiazygos veins [8,10]. There is association with other cardiac and visceral anomalies, such as dextrocardia, atrial septal defect, atrioventricular canal, situsinversus, polysplenia,or asplenia [9,11,12]. …”

Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature

“…Ten patients with DVT, eight men and two women, whose mean age was 25 ± 4.5 [range: [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] years, were diagnosed as having IVCA ( Table 1). Five of them reported having a first-degree relative with a history of venous thrombosis.…”

Congenital anomalies of the IVC—embryological perspective and clinical relevance

“…Absence can be either complete or incomplete depending on the degree of involvement of the infrarenal IVC and the common iliac veins (Figure 10) (26). Absence of the infrarenal IVC may be asymptomatic or may present lower limb venous insufficiency, deep-vein thrombosis or varices (28). In cases of total absence of the IVC, blood returns usually through multiple collateral pathways, including the azygos/ hemiazygos system, emptying into the SVC.…”