Fibroadipose Vascular Anomaly (Fava): A Newly Described and Under-Recognized Vascular Anomaly

Adam Farkas 1 Anthony Verstandig 1 Amos Peyser 2 Ehud Lebel 2 Gershon Zinger 2 Jack Applbaum 1
1Department of Radiology, Shaare Zedek Medical Center, Israel
2Department of Orthopedic Surgery, Shaare Zedek Medical Center, Israel

PURPOSE: To describe the clinical and imaging features of a newly described vascular anomaly.
Fibroadipose Vascular Anomaly (FAVA) is a newly described (2014) vascular anomaly comprosing dense fibrofatty tissue and slow-flow vascular malformations. FAVA is typically located in the extremities with the posterior calf compartment comprosing the majority of lesions. Severe pain and muscle contracture are typical presenting symptoms, while imaging demonstrates complex intramuscular lesions with fibrofatty overgrowth and phlebectasia (dilated veins). Diagnostic differentiation from more common vascular anomalies such as venous malformation is essential in order to tailor appropriate treatment and avoid unnecessary interventions.

MATERIALS AND METHODS: Retrospective review of 15 patients with confirmed or suspected FAVA seen in the vascular anomalies clinic between March 2014 and December 2017. Clinical, imaging, and pathological data, as well as treatments were reviewed.

RESULTS: 11 of 15 lesions were located in the lower extremity, with 6 centered in the posterior calf musculature. 3 lesions were located in the upper extremity and 1 in the chest wall. All patients presented with severe pain as their chief complaint, with 3 also reporting soft tissue swelling upon presentation. All patients had MRI as the primary imaging modality to diagnose FAVA, and 9 had additional percutaneous venography as imaging confirmation. 6 patients had histologic confirmation or histology supportive of a diagnosis of FAVA. 9 diagnoses were based on imaging alone. 2 patients had surgical excision, 1 had percutaneous cryotherapy, 7 had percutaneous sclerotherapy, and 1 had percutanous sclerotherapy + venous embolization. 4 patient are undergoing observation.

CONCLUSION: FAVA shares many clinical and diagnostic features with other vascular anomalies, particularly venous malformations. However, due to the fibrofatty tissue in FAVA, the mainstay of treatment is surgical or with thermal ablation. Proper diagnosis and specialist referral is essential with FAVA, in order to tailor effective treatment and avoid inappropriate interventions.

Adam Farkas
Adam Farkas