Jornal Vascular Brasileiro
https://www.jvascbras.org/article/doi/10.1590/1677-5449.202201262
Jornal Vascular Brasileiro
Case Report

A rare cause of deep vein thrombosis: inferior vena cava agenesis

Agenesia da veia cava inferior: uma causa rara de trombose venosa profunda

Pablo Del Canto Peruyera; Manuel Javier Vallina-Victorero Vázquez

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Abstract

Abstract: Inferior vena cava agenesis is a rare condition and is often misdiagnosed. This anomaly is asymptomatic in the majority of cases and is usually diagnosed during imaging tests carried out for other purposes. The most frequent manifestation is deep vein thrombosis (DVT) in lower limbs and anticoagulation therapy is the most frequent treatment option. Other techniques such as thrombolysis and venous bypass are also described. We report two cases diagnosed at our institution during the last year, both of which presented with an episode of DVT. We opted for indefinite anticoagulation therapy and both patients remain asymptomatic, after 1 year of surveillance in the first case and 6 months in the second, with no new episodes of DVT. Although it is not a life-threatening anomaly, it is important to make an appropriate diagnosis and provide treatment to improve the symptoms and quality of life of these patients.

Keywords

inferior vena cava, agenesis, deep venous thrombosis, diagnosis, treatment, vascular congenital anomalies

Resumo

Resumo: A agenesia da veia cava inferior é uma condição rara e, muitas vezes, mal diagnosticada. Essa anomalia é, na maioria dos casos, assintomática, sendo geralmente diagnosticada durante exames de imagem realizados com outras finalidades. A manifestação mais comum é trombose venosa profunda (TVP) em membros inferiores, e a terapia anticoagulante é a opção de tratamento mais frequente, embora outras técnicas, como trombólise e derivações venosas, também tenham sido descritas. Relatamos dois casos diagnosticados na nossa Instituição no último ano, ambos com episódio de TVP. O tratamento consistiu em anticoagulação por tempo indeterminado e, após 1 ano de acompanhamento no primeiro caso e 6 meses no segundo, ambos os pacientes permanecem assintomáticos, sem novos episódios de TVP. Embora não seja uma anomalia com risco de vida, é importante realizar diagnóstico e tratamento adequados para melhorar os sintomas e a qualidade de vida desses pacientes.
 

Palavras-chave

veia cava inferior, agenesia, trombose venosa profunda, diagnóstico, tratamento, anomalias vasculares congênitas

References

1 Campbell M, Deuchar DC. Absent inferior vena cava, symmetrical liver, splenic agenesis,and situs inversus, and their embryology. Br Heart J. 1967;29(2):268-75. http://dx.doi.org/10.1136/hrt.29.2.268. PMid:5336120.

2 Dean SM, Tytle TL. Acute right lower extremity iliofemoral deep venous thrombosis secondary to an anomalous inferior vena cava: a report of two cases. Vasc Med. 2006;11(3):165-9. http://dx.doi.org/10.1177/1358863x06074829. PMid:17288122.

3 Gayer G, Luboshitz J, Hertz M, et al. Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein trombosis. AJR Am J Roentgenol. 2003;180(3):729-32. PMid:12591684.

4 Kapetanakis S, Papadopoulos C, Galani P, Dimitrakopoulou G, Fiska A. Anomalies of the inferior vena cava: a report of two cases and a short review of the literatura. Folia Morphol. 2010;69(3):123-7. PMid:21154280.

5 Shah NL, Shanley CJ, Prince MR, et al. Deep venous thrombosis complicating a congenital absence of the inferior vena cava. Surgery. 1996;120(5):891-6. http://dx.doi.org/10.1016/S0039-6060(96)80100-0. PMid:8909527.

6 Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000;20(3):639-52. http://dx.doi.org/10.1148/radiographics.20.3.g00ma09639. PMid:10835118.

7 Malaki M, Willis AP, Jones RG. Congenital anomalies of the inferior vena cava. Clin Radiol. 2012;67(2):165-71. http://dx.doi.org/10.1016/j.crad.2011.08.006. PMid:22070941.

8 Ramanathan T, Hughes TM, Richardson AJ. Perinatal inferior vena cava thrombosis and absence of the infrarenal inferior vena cava. J Vasc Surg. 2001;33(5):1097-9. http://dx.doi.org/10.1067/mva.2001.114205. PMid:11331855.

9 Wax JR, Pinette MG, Fife J, Blackstone J, Cartin A. Absent infrarenal inferior vena cava, an unusual cause of pelvic varices. J Ultrasound Med. 2007;26(5):699-701. http://dx.doi.org/10.7863/jum.2007.26.5.699. PMid:17460016.

10 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. http://dx.doi.org/10.1016/S0140-6736(00)04010-1. PMid:11273066.

11 Lambert M, Marboeuf P, Midulla M, et al. Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vasc Med. 2010;15(6):451-9. http://dx.doi.org/10.1177/1358863X10391355. PMid:21183652.

12 Nanda S, Bhatt SP, Turki MA. Inferior vena cava anomalies - a common cause of DVT and PE commonly not diagnosed. Am J Med Sci. 2008;335(5):409-10. http://dx.doi.org/10.1097/MAJ.0b013e318155748d. PMid:18480663.

13 Tanabalan C, Papatsoris AG, Masood J. Absent inferior vena cava with resulting bladder varices - a rare cause of frank hematuria. Urol J. 2012;9(2):456. PMid:22641487.

14 Yugueros X, Alvarez B, Fernández E, Boqué M, Matas M. Compressive symptoms due to thrombosed or hypertrophic collateral circulation in infrarenal inferior vena cava agenesis. Ann Vasc Surg. 2013;27(2):238.e9-13. http://dx.doi.org/10.1016/j.avsg.2012.06.014. PMid:23380553.

15 Kamerath J, Morgan WE. Absent inferior vena cava resulting in exercise-induced epidural venous plexus congestion and lower extremity numbness. Spine. 2010;35(18):E921-4. http://dx.doi.org/10.1097/BRS.0b013e3181e83d6e. PMid:21381233.

16 Balzer KM, Pillny M, Luther B, Grabitz K, Sandmann W. Spontaneous rupture of collateral venous aneurysm in a patient with agenesis of the inferior vena cava: a case report. J Vasc Surg. 2002;36(5):1053-7. http://dx.doi.org/10.1067/mva.2002.128939. PMid:12422117.

17 Dougherty MJ, Calligaro KD, DeLaurentis DA. Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration: a surgically treated case. J Vasc Surg. 1996;23(1):141-6. http://dx.doi.org/10.1016/S0741-5214(05)80044-8. PMid:8558729.

18 Evanchuk DM, Von Gehr A, Zehnder JL. Superficial venous thrombosis associated with congenital absence of the inferior vena cava and previous episode of deep venous thrombosis. Am J Hematol. 2008;83(3):250-2. http://dx.doi.org/10.1002/ajh.21089. PMid:17918250.

19 Schneider JG, Eynatten MV, Dugi A, Duex M, Nawroth PP. Recurrent deep venous thrombosis caused by congenital interruption of the inferior vena cava and heterozygotus factor V Leiden mutation. J Intern Med. 2002;252(3):276-80. http://dx.doi.org/10.1046/j.1365-2796.2002.01034.x. PMid:12270010.

20 Körber T, Petzsch M, Placke J, Ismer B, Schulze C. Acute thrombosis of pelvic and leg veins in agenesis of the renal segment of the inferior vena cava. Z Kardiol. 2001;90(1):52-7. http://dx.doi.org/10.1007/s003920170212. PMid:11220087.
 


Submitted date:
02/16/2023

Accepted date:
05/09/2023

Sociedade Brasileira de Angiologia e Cirurgia Vascular (SBACV)"> Sociedade Brasileira de Angiologia e Cirurgia Vascular (SBACV)">
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