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Symptomatic Venous Aneurysm of the Internal Jugular Vein Presenting with Small Thrombotic Inclusions in von Willebrand Disease Type I

1Medical University of Graz, Department of Internal Medicine, Division of Vascular Medicine, Graz, Austria, 2Medical University of Graz, Department of Radiology, Division of General Radiology, Graz, Austria, 3Medical University of Graz, Department of Radiology, Division of Neuroradiology, Vascular and Interventional Radiology, Graz, Austria, 4Medical University of Graz, Department of Surgery, Division of Vascular Surgery, Graz, Austria

Abstract Number: PB1542

Meeting: ISTH 2020 Congress

Theme: Platelet Disorders and von Willebrand Disease » VWF and von Willebrand Factor Disorders - Clinical Conditions

Background: Venous aneurysms present a rare condition, and only a few case reports exist about venous aneurysms located in the area of the jugular veins. Even if venous aneurysms are classified benign, they may cause severe complications such as local thrombosis, rupturing and pulmonary embolism.

Aims: This case reports a venous aneurysm of the right internal jugular vein (IJV) showing small thrombotic inclusions in a 42 year-old woman with bleeding tendency due to von Willebrand disease (vWD) type I.

Methods: The patient presented to our vascular medicine outpatient clinic at the Medical University of Graz because of a painful swelling in the right neck area with an increase in size within the past few months. A previously performed CT-scan assumed venous thrombosis of the right jugular veins. Further diagnostic workup was performed by color-encoded duplex sonography and MRI.

Results: Color-encoded duplex sonography revealed a hypoechogenic lesion with small intraluminal hyperechogenic spots connected to the IJV. The MRI showed a T2-hyperintense lesion with intraluminal inhomogeneity, consistent with a venous aneurysm. Anticoagulation with Edoxaban 60 mg o.d. was preexisting because of the suspicion of jugular vein thrombosis. The patient reported a significant decrease of symptoms with this therapy, but also common episodes of nose bleeding, which may result from a combination of a bleeding tendency due to vWD with intake of oral anticoagulation. Surgical as well as endovascular treatment for the aneurysm were supplied, but both options were refused because the patient did not feel capable of these procedures with regard to a severe panic disorder.

Conclusions: In spite of lack for evidence-based recommendations, we decided for a resumption of anticoagulation with Edoxaban in a reduced dose of 30 mg o.d. and for strict observance of bleeding complications for this rare case of IJV aneurysm with thrombotic lesions in concomitant vWD type I.

To cite this abstract in AMA style:

Guetl K, Reiter C, Portugaller RH, Tiesenhausen K, Gressenberger P, Muster V, Brodmann M, Gary T. Symptomatic Venous Aneurysm of the Internal Jugular Vein Presenting with Small Thrombotic Inclusions in von Willebrand Disease Type I [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/symptomatic-venous-aneurysm-of-the-internal-jugular-vein-presenting-with-small-thrombotic-inclusions-in-von-willebrand-disease-type-i/. Accessed October 1, 2023.

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