34例下肢法瓦病临床分析

Retrospective clinical analysis of 34 cases with fibro-adipose vascular anomaly on the lower extremities

目的明确一种新的脉管性疾病亚型的命名——法瓦病（fibro-adipose vascular anomaly，FAVA），探讨其诊断及治疗方法，降低误诊率，提高治愈率。方法2011年1月至2015年12月，单中心收治34例下肢FAYA病例，根据临床资料分析其临床特点，总结其影像学及病理学诊断要点。所有患者均采取手术治疗，完整切除病变。结果34例患者总误诊率为76．5％（26／34），术后随访1-3年，平均19个月，肢体功能正常，病情无复发，手术治愈率100％。结论FAVA多被误诊为普通的血管瘤或血管畸形，正确命名该病有利于临床诊断与治疗，手术切除病变是较好的治疗方法之一。

Objective To define a new subtype of vascular anomaly, named fibro-adipose vascular anomaly（FAVA）and to discuss the methods of diagnosis and treatment in order to decrease the misdiagnosis rate and improve the recovery rate. Methods From Jan. 2011 to Dec. 2015, 34 cases with FAVA on the lower extremities received surgical treatment in our center. The clinical data were collected to summarize the imaging and pathological characteristics for diagnosis. Results The misdiagnosis rate was 76.5% （26/34） in all these 34 patients. The cure rate was 100% after operation. The patients were followed-up for 1 -3 years（ average, 19 months） with normal function and no recurrence. Conclusions FAVA is usually misdiagnosed as hemangioma or vascular malformation. The disease should be well defined to help the diagnosis and treatment. Surgical excision is one of the optional treatments.