Fibroadipose Vascular Anomaly of the Upper Extremity: Case Report of Extensive Resection and Secondary Tendon Transfer

Alomari and colleagues described in 2014 for the first time a distinct combination of vascular malformation, fibrofatty muscular infiltration and contracture which was termed fibroadipose vascular anomaly (FAVA) (J Pediatr Orthoped 34, 109-117 (2014). So far only few publications (J Pediatr Orthoped (2014) 34, 109-117; J Hand Surg (2020). 45, 68.e1, 68.e13; Ann Vasc Dis (2014) 7, 316-319; Pediatr Radiol 46, 1179-1186 (2016)) concerning this newly described disease have been published, covering only a limited number of cases. We present a case of a 19-year-old male patient suffering from a FAVA of the proximal forearm with a severe contracture of the infiltrated flexor musculature. Upon surgery, we observed infiltration of the ulnar nerve. We were able to successfully resect the vascular malformation. Secondary tendon transfer was performed after extensive resection of the flexor musculature.FAVA presents a differential diagnosis in patients with solid growth of the upper or lower extremity and contracture of the involved extremity. We conclude that patients suffering from FAVA of the upper extremity should be referred to a center specialized in oncologic extremity surgery and reconstructive hand and microsurgery.