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下肢毛细血管-静脉畸形

Capillary-venous malformation in the lower limb.

作者信息

Uihlein Lily C, Liang Marilyn G, Fishman Steven J, Alomari Ahmad I, Mulliken John B

机构信息

Dermatology Program, Boston Children's Hospital, Boston, Massachusetts.

出版信息

Pediatr Dermatol. 2013 Sep-Oct;30(5):541-8. doi: 10.1111/pde.12186. Epub 2013 Jul 7.

Abstract

Regional capillary malformation of a lower extremity is associated with the overgrowth of bone or soft tissue in several disorders, most commonly Klippel-Trenaunay syndrome and Parkes Weber syndrome. We have observed a subset of patients with a capillary malformation of the leg, minor growth disturbance, and prominent veins. The objective of the current study is to describe a series of patients with regional capillary malformation of the lower extremity in association with phlebectasia. This is a retrospective series of 17 patients diagnosed with capillary-venous malformation of the lower extremity. We excluded patients with clinical or radiographic evidence of lymphatic or arteriovenous malformation. Age, presentation, associated features, radiographic findings, and management were documented. In most patients the capillary malformation covered a large area without sharply demarcated borders. Four patients had one or more discrete, well-defined capillary stains involving less than 5% of the total surface area of the affected lower limb. Prominent veins were most common in the popliteal fossa and on the knee and dorsal foot. Approximately two-thirds of patients had a leg length discrepancy, with the affected leg being longer (n = 6) or shorter (n = 4); in many the affected leg was also slightly larger (n = 8) or smaller (n = 4) in girth. Radiographic imaging showed dilatation of superficial (n = 16), muscular (n = 9), and deep veins (n = 6). We characterize a subset of patients with regional capillary-venous malformation of the lower extremity with prominent veins and minor hypotrophy/hypertrophy that differs from Klippel-Trenaunay syndrome (capillary-lymphatic-venous malformation) but belongs at the minor end of the spectrum of vascular disorders with overgrowth.

摘要

下肢局部性毛细血管畸形与多种疾病中的骨骼或软组织过度生长相关,最常见的是Klippel-Trenaunay综合征和Parkes Weber综合征。我们观察到一部分患有腿部毛细血管畸形、轻度生长障碍和明显静脉的患者。本研究的目的是描述一系列伴有静脉扩张的下肢局部性毛细血管畸形患者。这是一项对17例被诊断为下肢毛细血管-静脉畸形患者的回顾性研究。我们排除了有淋巴管或动静脉畸形临床或影像学证据的患者。记录了患者的年龄、临床表现、相关特征、影像学表现及治疗情况。大多数患者的毛细血管畸形面积较大,边界不清。4例患者有一个或多个边界清晰的离散毛细血管斑,累及面积不到患侧下肢总面积的5%。明显的静脉最常见于腘窝、膝关节和足背。约三分之二的患者存在腿长差异,患侧腿较长(n = 6)或较短(n = 4);许多患者患侧腿的周径也略粗(n = 8)或略细(n = 4)。影像学检查显示浅静脉(n = 16)、肌静脉(n = 9)和深静脉(n = 6)扩张。我们描述了一组下肢局部性毛细血管-静脉畸形患者,其具有明显静脉且有轻度萎缩/肥大,不同于Klippel-Trenaunay综合征(毛细血管-淋巴管-静脉畸形),但属于伴有过度生长的血管疾病谱的轻度类型。

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