Inferior Vena Cava Agenesis: A Rare Cause of Pelvic Congestion Syndrome

Abstract

Complete absence of Inferior Vena Cava (IVC) is a rare anomaly with a reported incidence of 0.0005% to 1%. This is often asymptomatic with incidental detection during cross-sectional imaging. It may also present with deep venous thrombosis, pulmonary thromboembolism or compressive symptoms in form of nerve root compression. Pelvic Congestion Syndrome (PCS) is an increasingly recognized entity with well laid out diagnostic criteria and evolving management protocols. Complete absence of IVC is a rare cause of pelvic congestion syndrome. We present a case of young female presenting with symptoms typical of pelvic venous congestion who was found to have complete absence of IVC as the underlying cause. She also had associated small left kidney with compensatory hypertrophy of the right kidney which is another rare association.

Keywords: Atrophic left kidney; Subcardinal vein; Supracardinal vein.

[Table/Fig-1]:

a) Axial section at the level of infra renal aorta and coronal reconstruction (white arrow); b) Shows non visualization of IVC with multiple tortuous vascular channels seen in the para aortic region (Black arrows). Few of these channels show thrombus within.

[Table/Fig-2]:

Axial section at the level of uterus shows multiple tortuous vessels replacing the right ovarian vein (black arrow), the left ovarian vein is dilated (white arrow) and both ovaries are prominent and cystic (stars). Tortuous vascular channels are also seen around the uterus.

[Table/Fig-3]:

Axial section at the level of kidneys shows the right renal vein communicating with the tortuous vascular channels; the left renal vein was also seen to communicate with these channels (not shown). Also, seen is atrophic left kidney with enlarged right kidney. IVC is not seen in this images (left to right).

[Table/Fig-4]:

Axial section at the level of aortic bifurcation shows absent common iliac veins. Dilated tortuous veins are seen adjacent to right common iliac artery (white arrow).The external Iliac veins are absent bilaterally.

[Table/Fig-5a,b]:

(a) Axial section at the level of superior margin of liver shows hepatic veins draining separately into the right atrium (black arrow). The prominent azygous vein can also be seen on the right side of aorta (white arrow). (b). Axial section at the level of aortic arch shows dilated azygous vein (white arrow) draining into the superior vena cava (star) Images (left to right).