Fibro-Adipose Vascular Anomaly: Clinical-Radiologic-Pathologic Features of a Newly Delineated Disorder of the Extremity
Fibro-Adipose Vascular Anomaly: Clinical-Radiologic-Pathologic Features of a Newly Delineated Disorder of the Extremity
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DOI：
10.1097/bpo.0b013e3182a1f0b8
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发表时间：
2014-01-01
影响因子：
1.7
通讯作者：
Kozakewich, Harry P.
中科院分区：
文献类型：
--
作者：
Alomari, Ahmad I.;Spencer, Samantha A.;Kozakewich, Harry P.
背景：四肢血管异常的诊断和处理可能具有挑战性，因为这些疾病并不常见，临床上可能会重叠。本文的目的是描述纤维脂肪血管异常的临床、放射学和组织病理学特征。纤维脂肪血管异常是一种以前未被发现的肢体疾病。方法：回顾分析四肢肌内致密纤维脂肪组织和慢流血管畸形患者的临床、影像、手术和组织病理学资料。结果：16例四肢纤维脂肪血管异常患者(男3例，女13例)符合临床、放射学和组织病理学纳入标准。发病年龄从出生时到28岁不等。病变部位：小腿10例，前臂/腕3例，大腿3例。14名患者表现为剧烈疼痛。在小腿损伤的患者中，有7人的踝关节背屈受限。在影像上，复杂的肌肉内病变取代了肌肉纤维，出现纤维脂肪过度生长和静脉扩张(静脉扩张)。筋膜外部分包括脂肪过度生长、静脉扩张和偶尔的淋巴管畸形。组织病理学特征包括致密的纤维组织、脂肪和萎缩的骨骼肌内的淋巴浆细胞聚集体。脂肪组织也渗入病变周围的骨骼肌。有大的、不规则的、有时过度肌肉化的静脉通道和较小的簇状通道。其他发现包括机化血栓、淋巴成分和致密的纤维组织包围的神经。结论：临床、放射学和组织病理学特征构成了一个独特的实体，包括肌肉的纤维脂肪渗透、伴有疼痛的异常静脉扩张和患肢痉挛。临床和放射学结果允许诊断为具有重大治疗意义的蚕豆。证据级别：III级。
Background:The diagnosis and management of vascular anomalies of the extremities can be challenging as these disorders are uncommon and may clinically overlap. The aim of this paper is to describe the clinical, radiologic, and histopathologic features of fibro-adipose vascular anomaly (FAVA), a previously unrecognized disorder of the limb.Methods:The clinical, imaging, operative, and histopathologic data from patients with a unique intramuscular lesion of the extremities comprising dense fibrofatty tissue and slow-flow vascular malformations were retrospectively reviewed.Results:Sixteen patients diagnosed with FAVA of the extremity (3 male and 13 female individuals) met the clinical, radiologic, and histopathologic inclusion criteria. The age at presentation ranged from the time of birth to 28 years. The locations of the lesions were: calf (n=10), forearm/wrist (n=3), and thigh (n=3). Fourteen patients presented with severe pain. Seven of the patients with calf lesions had limited ankle dorsiflexion. On imaging, the complex intramuscular lesions replaced muscle fibers with fibrofatty overgrowth and phlebectasia (dilation of the veins). The extrafascial component comprised fatty overgrowth, phlebectasia, and an occasional lymphatic malformation. The histopathologic features comprised dense fibrous tissue, fat, and lymphoplasmacytic aggregates within atrophied skeletal muscle. Adipose tissue also infiltrated skeletal muscle at the periphery of the lesion. There were large, irregular, and sometimes excessively muscularized venous channels and smaller, clustered channels. Other findings include organizing thrombi, a lymphatic component, and dense fibrous tissue-encircled nerves.Conclusions:The constellation of clinical, radiologic, and histopathologic features constitutes a distinct entity comprising fibrofatty infiltration of muscle, unusual phlebectasia with pain, and contracture of the affected extremity. The clinical and radiologic findings permit the diagnosis of FAVA with major therapeutic implications.Level of Evidence:Level III.