Abstract No. 547 Interventional outcomes in the management of fibroadipose vascular anomaly at a tertiary care pediatric hospital

Abstract

Fibro-adipose vascular anomaly (FAVA) is a pediatric vascular malformation resulting in debilitating pain and reduced mobility in otherwise healthy children. Multidisciplinary assessment and treatment is vital for management, though there is minimal literature assessing postoperative outcomes or follow-up among FAVA patients. 22 patients with biopsy-proven FAVA between June 2013 to February 2020 were referred to our institution for evaluation. Lesions were classified by a typical (calf, arm) or atypical (foot, knee, thigh, gluteal, hand, thoracic, abdominal) location. Documentation of pre- and postprocedural pain at first follow-up visit, length of follow-up after intervention, number of repeat interventions, and development of an additional lesion was obtained. 16 patients (ages 3–15; mean, 10.3 years) with 20 separate lesions (8 typical location; 12 atypical location) pursued invasive treatment for FAVA to ameliorate their pain. Primary interventions performed included sclerotherapy (n = 15), cryoablation (n = 1), sclerotherapy and cryoablation (n = 1), or surgery (n = 3). Technical success for each intervention was 100%, and there were no major complications. Median initial follow-up time was 30 days. 2 patients were lost to follow-up. 10/14 (71%) of patients reported a decrease or absence of pain. 4/14 (28%) sclerotherapy patients required repeat intervention for pain and underwent repeat sclerotherapy (n = 2), cryoablation (n = 3), surgery (n = 1), or sirolimus therapy (n = 1). The two patients who were treated with repeat sclerotherapy required additional intervention and achieved pain relief after cryoablation and surgery, respectively. Median interval between repeat interventions was 484 days. 2/14 patients (14%) developed additional FAVA lesions that required treatment. Median interval for recurrent disease was 408 days. Virtually all patients achieved pain relief after initial intervention. A fraction of patients required reintervention within 18 months. Though a limited sample, cryoablation demonstrated 100% pain relief among the five patients who received cryoablation as either a primary or secondary treatment.