DIFFERENT CLINICAL PRESENTATIONS OF A RARE VENOUS MALFORMATION-INFERIOR VENA CAVA AGENESIS

Authors

  • Marta Machado Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Nuno Coelho Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Pedro Maximiano Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Carolina Semião Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • João Peixoto Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Luís Fernandes Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Pedro Brandão Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal
  • Alexandra Canedo Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Vila Nova de Gaia / Espinho, Porto, Portugal

DOI:

https://doi.org/10.48750/acv.461

Keywords:

Inferior Vena Cava Agenesis, Deep Vein Thrombosis, Vascular anomalies

Abstract

INTRODUCTION: Inferior vena cava agenesis is one of the most uncommon anomalies of this vessel, with an estimated prevalence of 0.0005–1% in the general population. However, around 5% of the patients younger than 30 years with a diagnosis of deep vein thrombosis have this anomaly.

METHODS AND MATERIAL: Report of two clinical cases of inferior vena cava agenesis with different clinical presentations.

CLINICAL CASE 1: A 40-year-old man was admitted with a 3 days history of unilateral lower limb swelling and pain, gradually progressing to the inability to walk. On physical examination he had swelling, bruising and tenderness involving leg and thigh and prominent engorged superficial abdominal collateral veins.
Venous Doppler Ultrasound showed left deep venous thrombosis extending from popliteal vein to common iliac vein. A computed tomography angiogram showed agenesis of the infrarenal vena cava and patent renal veins draining in to the azygous system and hemiazygous systems.

The patient as discharged with rivaroxaban and compression stockings. At 2 months follow up he was asymptomatic and still anticoagulated.

CLINICAL CASE 2: A 35 year- old woman, with a previous history of recurrent lower limb varicose veins surgery and left internal malleolar ulcer at 30 years presented at medical department 3 years later with complains of ulcer recurrence. A computed tomography angiogram revealed an absent infrahepatic vena cava, prominently dilated azygos and hemiazygos veins with enlarged retroperitoneal collaterals.

The patient initiated dressing care with oxide zinc and oral rivaroxaban.

DISCUSSION/CONCLUSION: The majority of cases remain asymptomatic. However, when symptomatic, the majority present as proximal DVT involving the iliac and femoral veins. CT or MRI should be the imagiological methods used to diagnose this anomaly. No clear consensus has been reached on therapeutic strategy, other than long-term antico- agulation and elastic stockings.

Downloads

Download data is not yet available.

References

Man L.; Hendricks N.; Maitlan H. IVC agenesis: a rare cause of deep vein thrombosis. J Thromb Thrombolysis 2015 Oct.

Gil R., Pérez A., Arias J., Pascual F., and Romero E. Agenesis of the inferior vena cava associated with lower extremities and pelvic venous thrombosis. J Vasc Surg 2006; 44:1114-6.

Pomeranz C., Cullen D., Bellah R. Deep venous thrombosis in a child with inferior vena cava and renal anomalies: KILT syndrome. Pediatric Radiology; 11 January 2018

Lambert M., Marboeuf P., Midulla M., Trillot N., Beregi J., Mounier-Vehier C., Hatron P. and Jude B. Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vascular medicine 15(6) 451–459, 2010.

Singh K., Poliquin J., Syversten G., Kohler D. A rare cause of venous thrombosis: Congenital absence (agenesis) of the inferior vena cava. int J angiol 2010;19(3):e110-e112.

Aranda J., Cerda C., Mussali S., Flores J. Inferior Vena Cava Agenesis: An Unusual Cause of Deep Vein Thrombosis and Pulmonary Embolism in Young Adult Patients. EJVES Short Reports, 2018 39 12e15.

O’Connor D., O’Brien N., Khani T., Sheehan S. Superficial and Deep Vein Thrombosis Associated With Congenital Absence of the Infrahepatic Inferior Vena Cava in a Young Male Patient. Dublin, Ireland Ann Vasc Surg 2011; 25: 697.e1-697.e4

Yang C, Trad HS, Mendonça SM, Trad CS. Congenital inferior vena cava anomalies: a review of findings at multidetector computed tomography and magnetic resonance imaging. Radiol Bras. 2013 Jul/Ago;46(4):227–233

Protti G, Elia F, Bosco F, Aprà F. An eminent absence: agenesis of inferior vena cava underlying bilateral iliac vein thrombosis. EJCRIM 2020

Sitwala P., Ladia V., Brahmbhatt P., Jain V., Bajaj K. Inferior Vena Cava Anomaly: A Risk for Deep Vein Thrombosis. North American Journal of Medical Sciences. Nov 2014, Volume 6, Issue 11.

Downloads

Published

2022-03-02

How to Cite

1.
Machado M, Coelho N, Maximiano P, Semião C, Peixoto J, Fernandes L, Brandão P, Canedo A. DIFFERENT CLINICAL PRESENTATIONS OF A RARE VENOUS MALFORMATION-INFERIOR VENA CAVA AGENESIS. Angiol Cir Vasc [Internet]. 2022 Mar. 2 [cited 2022 May 19];17(4):325-9. Available from: https://acvjournal.com/index.php/acv/article/view/461

Issue

Section

Clinical Case