Rare Case of a Kidney and Inferior Vena Cava Abnormalities with Extensive Lower Extremity Deep Vein Thrombosis in a Young Healthy Male

Abstract

Kidney and inferior vena cava (IVC) abnormalities with extensive deep vein thrombosis (DVT) is a very rare cause of DVT and has a diverse clinical presentation. Computed tomography (CT) angiography is the gold standard for diagnosis and treatment including thrombectomy, thrombolysis and systemic anticoagulation. We present a rare case of active young healthy male admitted with acute onset of right lower extremity pain and swelling who was found to have extensive DVT on doppler ultrasound. CT abdomen showed extensive clot burden involving right common femoral vein extending into internal and external iliac veins associated with IVC hypoplasia and hypoplastic left kidney. Patient underwent urgent thrombectomy, catheter directed thrombolysis and was discharged home in stable condition on oral anticoagulation.

Deep venous thrombosis in patients with atresia of the inferior vena cava and right kidney hypoplasia (KILT syndrome): Systematic review of the literature

“…16,22,28 Direct thrombolysis, mechanical thrombectomy, and balloon angioplasty were performed as the first-line therapy in three patients, followed by long-term anticoagulation. 29,33,40 Thrombosis was recurrent in nine patients (26.5% cases). In three patients, anticoagulation had already been stopped at the time of recurrence (after recurrence, long-term therapy was indicated in two patients, 13,30 and anticoagulation was not possible because of intracranial bleeding in others 26 ); for three patients, there were no data on anticoagulation treatment at the time of thrombosis.…”

“…All articles were published between 1998 and 2023. Sixteen articles were from Europe (four from the United Kingdom, 5,10,12,13 three from Italy, 14–16 three from Spain, 17–19 and one each from Belgium, 20 the Netherlands, 21 Switzerland, 22 Romania, 23 Slovakia, 24 and Poland 25 ), nine from North America (eight from the USA 26–33 and one from Canada 34 ), six from Asia (two from China 35,36 and one each from Japan, 37 Iran, 38 Jordan, 39 and the United Arab Emirates 40 ), and one from Australia. 41…”

“…7,46 Although less frequently reported, cases that were successfully treated with long-term DOAC have also been reported. 14,29,35,50 However, no studies have compared the two approaches. Progression or rethrombosis in patients on therapy with both VKA and DOAC has also been reported.…”

Trombosis venosa profunda y agenesia de vena cava inferior: presentación típica en una anomalía poco frecuente. Caso clínico

“…El retorno sanguíneo inadecuado causado por la alteración de la VCI produce hipertensión venosa, a pesar del desarrollo de un suministro colateral considerable, causando estasis venosa que puede conducir a trombosis 4,11,12 . La presencia de embolia pulmonar en pacientes con TVP y atresia de la VCI es rara 4,12 , probablemente porque la migración de trombos es impedida por la extensa red de circulación colateral compensatoria 4 . La ausencia de VCI también puede presentarse como insuficiencia venosa crónica y síndrome de congestión pélvica 1,2,4,6,8,11 .…”

“…La presencia de embolia pulmonar en pacientes con TVP y atresia de la VCI es rara 4,12 , probablemente porque la migración de trombos es impedida por la extensa red de circulación colateral compensatoria 4 . La ausencia de VCI también puede presentarse como insuficiencia venosa crónica y síndrome de congestión pélvica 1,2,4,6,8,11 . No obstante, la mayoría de los pacientes con agenesia de la IVC son asintomáticos, y el diagnóstico es incidental 11 .…”