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. 2023 Jan 18;10(1):003721.
doi: 10.12890/2023_003744. eCollection 2023.

Inferior Vena Cava Atresia: A Rare Vascular Malformation

Telma Alves  1 , Diogo Alves Leal  1 , Ricardo Rodrigues  1 , Carolina Terra  2 , Sandra Santos  1 , Joana Cascais Costa  1 , Fátima Silva  1
Affiliations

Inferior Vena Cava Atresia: A Rare Vascular Malformation

Telma Alves et al. Eur J Case Rep Intern Med. .

Abstract

Inferior vena cava (IVC) atresia is a rare congenital vascular malformation. We describe the case of a 20-year-old woman with IVC atresia who presented with a 3-month history of fatigue, oedema of the lower limbs and episodes of lipothymia. Transthoracic echocardiography and cardiac catheterization were performed, revealing interruption of the IVC with circulation through the azygos and hemiazygos system. An abdominal and pelvic computerized tomography (CT) scan confirmed the findings, demonstrating the absence of the IVC below the renal veins. Blood tests did not reveal any relevant results. These findings are consistent with the diagnosis of IVC atresia, a rare condition with no standard treatment. As a surgical approach was not possible, pharmacological measures were implemented for primary prevention of possible thrombotic events.

Learning points: Inferior vena cava atresia is a rare vascular malformation.The clinical presentation is non-specific: most diagnoses are made after a thrombotic event or as an incidental finding on imaging.Consensus on definitive treatment is lacking, but the increased thrombotic risk warrants primary prevention of thrombosis.

Keywords: Inferior vena cava; collateral circulation; vascular malformations.

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Conflict of interest statement

Conflicts of Interests: The authors declare there are no competing interests.

Figures

Figure 1
Figure 1
Abdominal venous collateral circulation
Figure 2
Figure 2
Thoraco-abdomino-pelvic computed tomography (CT) in the portal venous phase. The inferior vena cava (IVC) is not identified in its normal position, lateral to the abdominal aorta (circle). Multiple collateral vessels are observed in the abdominal wall (star)
Figure 3
Figure 3
Thoracic CT images in the portal venous phase show that abdominal venous drainage occurs through the azygos and hemiazygos veins (circle). The thoraco-epigastric veins are engorged up to their confluence with the axillary veins (star)
Figure 4
Figure 4
Sagittal CT images show a short segment, mainly intrahepatic, of the IVC. It begins after the confluence of the renal veins, where it has a filiform calibre (orange arrow), later receiving collateral vessels up to an intrahepatic portion (blue arrow), which has the usual calibre and course, draining to the right atrium
See this image and copyright information in PMC

References

    1. Mabud TS, Sailer AM, Swee JKY, Tamboli M, Arendt VA, Jeon GS, et al. Inferior vena cava atresia: characterisation of risk factors, treatment, and outcomes. Cardiovasc Intervent Radiol. 2020 Jan;43(1):37–45. - PubMed
    1. Koc Z, Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings. Eur J Radiol. 2007 May;62(2):257–266. - PubMed
    1. Liu Y, Guo D, Li J, Zhang X, He J, Huang M, et al. Radiological features of azygos and hemiazygos continuation of inferior vena cava: a case report. Medicine (Baltimore) 2018 Apr;97(17):e0546. - PMC - PubMed
    1. Skeik N, Wickstrom KK, Schumacher CW, Sullivan TM. Infrahepatic inferior vena cava agenesis with bilateral renal vein thrombosis. Ann Vasc Surg. 2013 Oct;27(7):973e19–23. - PubMed
    1. Hung ML, Kwon D, Sudheendra D. Endovascular IVC reconstruction in an 18 year old patient with subtotal IVC atresia. EJVES Vasc Forum. 2021 Jun 10;52:5–10. - PMC - PubMed

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