Fibroadipose Vascular Anomaly in the Upper Extremity: A Distinct Entity With Characteristic Clinical, Radiological, and Histopathological Findings
Fibroadipose Vascular Anomaly in the Upper Extremity: A Distinct Entity With Characteristic Clinical, Radiological, and Histopathological Findings
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DOI：
10.1016/j.jhsa.2019.05.008
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发表时间：
2020-01-01
影响因子：
1.9
通讯作者：
Upton, Joseph
中科院分区：
文献类型：
--
作者：
Cheung, Kevin;Taghinia, Amir H.;Upton, Joseph
目的：纤维脂肪血管异常（fafa）是一种肌肉内血管畸形，最近被描述为一种独特的临床实体。本文综述了上肢FAVA的临床、影像学和组织病理学特征。方法回顾性分析上肢faa病变病例系列。结果对19例上肢fafa患者进行回顾性分析。疼痛、僵硬、肿胀和屈曲挛缩是最常见的表现。除了一个局限于手部的病变外，所有病变都在10年内出现或发展为挛缩。10例患者接受手术切除。6例需要肌腱转移重建，3例需要自由功能肌肉转移。结论：上肢纤维脂肪性血管异常需要准确诊断，早期转诊到有经验的手外科多学科血管异常中心可能会受益。压缩服、心得安和硬化疗法似乎无效。手术切除集中在有症状的区域，并适当重建可能有利于挽救肢体功能受损。美国手部外科学会版权所有。版权所有。
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. Copyright (C) 2020 by the American Society for Surgery of the Hand. All rights reserved.