[Venous aneurysms associated with lymphatic malformations]

To analyze and describe the association between the development of venous aneurysms of the mayor vessels in patients with lymphatic malformations.

Retrospective review of patients diagnosed with both VA and LM from 1993 to 2014 and a descriptive analysis of clinical course was performed.

We found 6 patients, 50% females, who had LM and VA confirmed with imaging techniques. All were diagnosed between 20 weeks- 12 years of age. LM were found within the thoracic cavity (n= 4), intrathoracical-cervical area (n= 1) and lower extremities (n= 1). In most cases the dilated veins were near the LM and thus, the mediastinal vessels were most commonly affected (superior vena cava-innominated (n= 1), venous jugulosubclavian confluence (n= 2), superior vena cava (n= 2) and popliteal vein (n= 1)). A total of 4 patients required surgical treatment of the LM with complete excision in 2 of the cases. Patients with a prenatal diagnosis of lymphatic malformation were most likely to present venous aneurysms at birth (n= 2), however the remaining patients (without prenatal diagnosis) developed them later on (average 6 years). Unlike lower extremity aneurysms, none of these aneurysms grew or required antiplatelet therapy; local thrombosis developed in one which resulted in pulmonary thromboembolism and one developed mitral valve insufficiency (regurgitation?) which required valve replacement.

VA is extremely rare, and there is not therapeutic algorithm, therefore treatment should be individualized.