From rare anomaly to clinical reality; managing venous thrombosis in the setting of congenital inferior vena cava agenesis
DOI:
https://doi.org/10.18203/2349-2902.isj20243557Keywords:
Inferior vena cava, Congenital agenesis, Deep vein thrombosisAbstract
A congenital absence of the inferior vena cava (IVC) is a rare anomaly known to be linked with idiopathic deep venous thrombosis (DVT). This anomaly is more commonly observed in individuals with other congenital cardiac anomalies, with an incidence ranging from 0.6% to 2%. Here we are reporting a rare case of missing IVC presented with extensive bilateral lower extremity and iliac veins DVT. The symptoms responded well to therapeutic doses of heparin. In this article, we will review some other cases of IVC agenesis and different management including medical, interventional, and surgical approaches.
References
Iqbal J, Nagaraju E. Congenital absence of inferior vena cava and thrombosis: a case report. J Med Case Rep. 2008;2:46.
Ker J. Rare congenital anomaly of the inferior vena cava. Int J Cardiol Heart Vasc. 2015;9:63-4.
Ghandour A, Partovi S, Karuppasamy K, Rajiah P. Congenital anomalies of the IVC- embryological perspective and clinical relevance. Cardiovasc Diagn Ther. 2016;6(6):482-92.
Singh K, Poliquin J, Syversten G, Kohler DO. A rare cause of venous thrombosis: Congenital absence (agenesis) of the inferior vena cava. Int J Angiol. 2010;19(3):110-2.
Kannappan M, Sakthi Velavan S. Inferior Vena Cava Agenesis: An Underrated Cause of Deep Venous Thrombosis. Cureus. 2023;15(1):33667.
Koc Z, Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings. Eur J Radiol. 2007;62(2):257-66.
Ueda J, Hara K, Kobayashi Y, Ohue S, Uchida H. Anomaly of the inferior vena cava observed by CT. Comput Radiol. 1983;7(3):145-154.
Anderson FA Jr, Wheeler HB, Goldberg RJ, et al. A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study. Arch Intern Med. 1991;151(5):933-8.
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(9254):441.
García-Fuster MJ, Forner MJ, Flor-Lorente B, Soler J, Campos S. Anomalías de la vena cava y trombosis venosa profunda inferior vena cava malformations and deep venous thrombosis. Rev Esp Cardiol. 2006;59(2):171-5.
Ker J. Rare congenital anomaly of the inferior vena cava. Int J Cardiol Heart Vasc.2015;9:63-4.
Kannappan M, Sakthi Velavan S. Inferior Vena Cava Agenesis: An Underrated Cause of Deep Venous Thrombosis. Cureus. 2023;15(1):33667.
Ertuğrul İ, Doğan V, Örün UA, Karademir S. A rare association: inferior vena cava anomalies and congenital heart diseases. Turk Kardiyol Dern Ars. 2015;43(8):717-9.
Alexiou VG, Ntanika A, Pappas G, Vassiliou A, Palialexis K, Geroulakos G. Conservative treatment vs thrombus removal for Iliofemoral vein thrombosis in patients with congenital abnormalities of the inferior vena cava: a case report and systematic review of the literature. J Thromb Thrombolysis. 2022;54(2):230-54.
Chew RR, Lim AH, Toh D. Congenital absence of inferior vena cava: an under recognised cause of unprovoked venous thromboembolism. QJM. 2018;111(2):117-8.
Hamidian Jahromi A, Coulter AH, Bass P 3rd, Zhang WW, Tan TW. Pharmacomechanical thrombectomy and catheter-directed thrombolysis of acute lower extremity deep venous thrombosis in a 9-year-old boy with inferior vena cava atresia. Vasc Med. 2015;20(2):139-42.
Aldağ M, Çiloğlu U. Combined pharmacomechanical thrombectomy with selective catheter-directed thrombolysis in patients with acute proximal deep vein thrombosis. Turk Gogus Kalp Damar Cerrahisi Derg. 2022;30(2):176-83.
Grieff AN, Shafritz R, Beckerman WE. Extravascular reconstruction of a congenitally absent inferior vena cava. J Vasc Surg Cases Innov Tech. 2020;6(4):681-5.
Ganguli S, Kalva S, Oklu R. Efficacy of lower-extremity venous thrombolysis in the setting of congenital absence or atresia of the inferior vena cava. Cardiovasc Intervent Radiol. 2012;35(5):1053-8.
Parsa P, Lane JS 3rd, Barleben AR, Owens EL, Bandyk D. Congenital agenesis of inferior vena cava: a rare cause of unprovoked deep venous thrombosis. Ann Vasc Surg. 2015;29(5):15-8.
Ali B, Rana MA, Langsfeld M, Marek J. A rare cause of claudication treated with IVC reconstruction: a case report. International journal of surgery case reports. 2015;14:69-71.
