Kevin Cheung1, Amir H Taghinia2, Ravi F Sood2, Ahmad I Alomari3, Samantha A Spencer4, Alyaa Al-Ibraheemi5, Harry P W Kozakewich5, Gulraiz Chaudry3, Arin K Greene2, John B Mulliken2, Cameron C Trenor6, Steven J Fishman7, Joseph Upton2. 1. Department of Plastic and Oral Surgery, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. Electronic address: kcheung@cheo.on.ca. 2. Department of Plastic and Oral Surgery, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. 3. Division of Vascular and Interventional Radiology, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. 4. Department of Orthopedic Surgery, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. 5. Department of Pathology, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. 6. Department of Hematology and Oncology, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA. 7. Department of Pediatric Surgery, Vascular Anomalies Center, Boston Children's Hospital, Harvard University, Boston, MA.
Abstract
PURPOSE: Fibroadipose vascular anomaly (FAVA) is an intramuscular vascular malformation that has been recently described as a distinct clinical entity. The clinical, radiological, and histopathological characteristics of FAVA in the upper extremity are reviewed. METHODS: This was a retrospective case series of upper-extremity FAVA lesions. RESULTS: We reviewed 19 patients with FAVA of the upper limb. Pain, stiffness, swelling, and flexion contractures were the most common presentations. Except for one lesion confined to the hand, all lesions either presented with or developed a contracture within 10 years. Ten patients underwent surgical debulking. Six required tendon transfer reconstruction and 3 necessitated a free functional muscle transfer. CONCLUSIONS: Fibroadipose vascular anomaly in the upper extremity requires an accurate diagnosis and may benefit from early referral to a multidisciplinary vascular anomaly center with experienced hand surgeons. Compression garments, propranolol, and sclerotherapy seem to be ineffective. Surgical resection focused on symptomatic regions with appropriate reconstruction may have benefit in salvage of limbs with compromised function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.
PURPOSE:Fibroadipose vascular anomaly (FAVA) is an intramuscular vascular malformation that has been recently described as a distinct clinical entity. The clinical, radiological, and histopathological characteristics of FAVA in the upper extremity are reviewed. METHODS: This was a retrospective case series of upper-extremity FAVA lesions. RESULTS: We reviewed 19 patients with FAVA of the upper limb. Pain, stiffness, swelling, and flexion contractures were the most common presentations. Except for one lesion confined to the hand, all lesions either presented with or developed a contracture within 10 years. Ten patients underwent surgical debulking. Six required tendon transfer reconstruction and 3 necessitated a free functional muscle transfer. CONCLUSIONS:Fibroadipose vascular anomaly in the upper extremity requires an accurate diagnosis and may benefit from early referral to a multidisciplinary vascular anomaly center with experienced hand surgeons. Compression garments, propranolol, and sclerotherapy seem to be ineffective. Surgical resection focused on symptomatic regions with appropriate reconstruction may have benefit in salvage of limbs with compromised function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.