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An Extremely Rare Cause of Deep Venous Thrombosis: Inferior Vena Cava Agenesis FREE TO VIEW

Sukriti Kamboj, MD; Abhishek Mishra, MD; James Walsh, MD; Aref Agheli, MD
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Internal Medicine, Robert Packer Hospital/Guthrie, Sayre, PA

Chest. 2014;146(4_MeetingAbstracts):199A. doi:10.1378/chest.1994900
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Inferior vena cava agenesis (IVCA) is an extremely rare congenital anomaly. Proposed mechanisms include embryological dysgenesis, intrauterine or perinatal thrombosis. This case is a unique presentation of IVCA manifesting as bilateral lower limb deep venous thrombosis (DVT).

CASE PRESENTATION: 25 year old gentleman with no significant past medical history presented with abdominal pain, new onset bilateral lower limb painful swelling and worsening of chronic low back pain. Symptoms failed to improve with pain medications as an outpatient. Venous Duplex was obtained in view of lower limb swelling showed extensive bilateral lower limb DVT .CT scan as well as MRI of abdomen and pelvis showed IVCA with prominent collateralization and reconstitution of azygous and hemiazygous system. Of particular interest was finding of serpiginous retroperitoneal mass, made up of thrombosed collateral veins. Coagulation work up was unremarkable. He was treated with long term anticoagulation and elastic stockings with improvement in symptoms. Follow up imaging studies demonstrated resolution of thrombus.

DISCUSSION: IVCA is a rare congenital anomaly, which is found in approximately 5% of DVT patients who are younger than 30 years1.CT scan or MRI should be imaging of choice, as ultrasonography is usually unable to diagnose IVCA. Previous few case reports demonstrating association of DVT and IVCA had hypercoagulable state in common. This patient is a unique presentation of IVCA causing DVT in absence of hypercoagulable state.

CONCLUSIONS: If young patient presents with DVT without any provocative factor, there should be a high index of suspicion for IVCA. Long term oral anticoagulation and compression stockings are the mainstay of management although supportive data is lacking and it deserves further studies.

Reference #1: Tosetto A et al; Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep vein thrombosis. Lancet2001; 357: 1711.

Reference #2: Lambert M et al: Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vasc Med.2010 Dec;15(6):451-9.

Reference #3: Vasco et al; Deep venous thrombosis caused by congenital inferior vena cava agenesis and heterozygous factor V Leiden mutation - a case report. Int J Angiol. 2009 Autumn; 18(3): 147-149.

DISCLOSURE: The following authors have nothing to disclose: Sukriti Kamboj, Abhishek Mishra, James Walsh, Aref Agheli

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