Journal of Vascular Surgery
Volume 44, Issue 2 , Page 416, August 2006

Congenital absence of the inferior vena cava: A rare risk factor for idiopathic deep-vein thrombosis

  • Roger W. Simon, MD

      Affiliations

    • Angiology Division, Institut of Diagnostic Radiology, University Hospital Zurich, Zurich, Switzerland
    • ,
  • Beatrice R. Amann-Vesti, MD

      Affiliations

    • Angiology Division, Institut of Diagnostic Radiology, University Hospital Zurich, Zurich, Switzerland
    • ,
  • Thomas Pfammatter, MD

      Affiliations

    • Department of Radiology, Institut of Diagnostic Radiology, University Hospital Zurich, Zurich, Switzerland
    • ,
  • Renate Koppensteiner, MD

      Affiliations

    • Angiology Division, Institut of Diagnostic Radiology, University Hospital Zurich, Zurich, Switzerland

Article Outline

 

A 39-year-old man in good health presented to our hospital with acute swelling of the left leg. No risk factors for deep venous thrombosis (DVT) were present, in particular, no immobilization, surgery, known thrombophilia, or family history. Venous duplex ultrasonography demonstrated complete thrombosis of the left iliac and femoral veins, and the inferior vena cava was not detectable. Abdominal magnetic resonance angiography (Cover) confirmed the absence of the infrarenal portion of the vena cava. The renal veins (R) drained into dilated lumbar veins (L), and only at the intrahepatic level was the diameter of the vena cava inferior normal (A). The hemiazygos vein (H) was dilated as a compensatory mechanism. and as anatomically expected, the thoracic dilated hemiazygos system drained into the dilated azygos vein (Az) (B). No congenital defects of the heart or abdominal organs were seen.

Screening for thrombophilia was performed without any pathologic findings. The patient was initially treated with low-molecular-weight heparin, followed by warfarin for 1 year and compression therapy.

The diagram (C) shows the different pathways of venous drainage from the lower half of the body in absence of the inferior vena cava: through the (1) superficial epigastric vein-thoracoabdominal vein-subclavian vein, the (2) inferior epigastric vein-superior epigastric vein-subclavian vein, and the (3) ascending lumbar vein-azygos vein-superior vena cava.

In >80% of patients presenting with DVT, a risk factor can be identified.1 An absent or hypoplastic infrarenal vena cava is a rare risk factor for DVT in young adults. The drainage of the lower limbs through the azygos vein might be insufficient, causing stasis and eventually thrombosis.2 Venous claudication might be present. The prevalence of congenital absence of the vena cava inferior is estimated at 0.5% of the general population.3

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Appendix. Supplementary Data 

Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jvs.2005.05.004.

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Electronic annex 

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References 

  1. Chee YL , Culligan DJ , Watson HG . Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young . Br J Haematol . 2001;114:878–880
  2. Ruggeri M , Tosetto A , Castaman G , Rodeghiero F . Congenital absence of the inferior vena cava (a rare risk factor for idiopathic deep-vein thrombosis) . Lancet . 2001;357:441
  3. Schneider JG , Eynatten MV , Dugi KA , Duex M , Nawroth PP . Recurrent deep venous thrombosis caused by congenital interruption of the inferior vena cava and heterozygous factor V Leiden mutation . J Intern Med . 2002;252:276–280

PII: S0741-5214(05)00751-2

doi:10.1016/j.jvs.2005.05.004

Journal of Vascular Surgery
Volume 44, Issue 2 , Page 416, August 2006

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