An unusual case of deep venous thrombosis in a young patient: congenital absence of the infrarenal portion of the inferior vena cava

Abstract

Deep venous thrombosis (DVT) is a commonly encountered diagnosis in clinical practice with a variety of well-established risk factors. Congenital absence of the inferior vena cava (IVC) is an extremely rare but established risk factor for DVT. Patients who develop DVTs are at high risk of long-term complications, including DVT recurrence and post-thrombotic syndrome. Here we report a rare case of a 27-year-old female who presented with an extensive DVT of the right lower extremity secondary to complete absence of the infrarenal portion of the IVC, confirmed on computed tomography. There is little consensus regarding the appropriate management of this patient population, and a brief review of the current evidence follows.

Extensive deep venous thrombosis in a young male with absent infrarenal inferior vena cava

“…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. 9, 18, 19 Thrombus burden can extend into the pelvis, leading to abdominal and lumbar pain; subsequent clot extension into the LLs may result in oedema, venous insufficiency, and eventually ulceration 15,20 .…”

“… 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 …”

Inferior vena cava-syndrome

“…As has been shown by Lin et al monophasic waveforms in the common femoral veins or the external iliac veins are reliable indicators of proximal venous obstruction [40], as in case of endoluminal obstruction or external compression respiratory variation of the Doppler waveform is dampened, resulting in a monophasic constant flow curve (see Figure 1). If this flow pattern is present in both femoral veins involvement of both iliac veins and/or the IVC is signified [41]. Furthermore, reversed flow in the superficial epigastric veins indicate for iliaco-caval occlusion or compression [42].…”

“…Furthermore, reversed flow in the superficial epigastric veins indicate for iliaco-caval occlusion or compression [42]. Thus, a monophasic flow-pattern in both femoral veins and/or a reversed flow in the superficial epigastric veins inevidently indicates a closer examination of the proximal venous tracts [40][41][42].…”

“…If good ultrasonographic views can be obtained the exact diagnosis (IVC-thrombosis, stenosis, tumor compression, IVC-anomaly) may be established by DUS alone [41]. Contrast-enhanced ultrasound (CEUS) is useful for the detection of IVC-thrombosis and in differentiating bland thrombus from tumor invasion [43].…”