Enjolras O, Ciabrini D, Mazoyer E, Laurian C, Herbreteau D
Interdisciplinary Study Group for Vascular Anomalies, Hôpital Lariboisière, Paris, France.
J Am Acad Dermatol. 1997 Feb;36(2 Pt 1):219-25. doi: 10.1016/s0190-9622(97)70284-6.
Extensive pure venous malformations (VMs) involving the entire lower or upper limb and adjacent trunk form a particular group of rare vascular malformations.
Our purpose was to review 27 cases of extensive limb VMs and describe their characteristics and management.
Cases of extensive limb VMs were investigated, treated, and observed for a mean of 7 years.
Eleven cases involved the upper limbs and 16 involved the lower limbs. All involved skin and muscle. In 81% of cases in the lower limb there was also knee joint involvement that created severe functional impairment. Ultrasonography with Doppler (duplex scans), computed tomographic scans, and magnetic resonance imaging were helpful noninvasive diagnostic procedures in these patients, whereas arteriography and phlebography were less informative. Muscle involvement was present in 100% of patients and bone abnormalities in 63%. Leg length was either normal or there was slight limb undergrowth, except in three patients who had minor overgrowth of the affected limb. Coagulation profiles demonstrated localized intravascular coagulation in 88% of patients. The majority of patients had conservative management (elastic stockings). In a few, percutaneous sclerotherapy or partial excision of skin and muscle VMs was beneficial. Knee joint involvement required synovectomy and VM excision during childhood in 7 of 16 patients.
Extensive limb VMs are characterized by diffuse involvement of the skin, muscle, and joints, and by a specific localized intravascular coagulopathy with general consequences. This group of vascular malformations should be separated from the Klippel-Trenaunay and Parkes Weber syndromes.
广泛的单纯静脉畸形（VMs）累及整个下肢或上肢及相邻躯干，形成了一组特殊的罕见血管畸形。
我们的目的是回顾27例广泛的肢体VMs病例，并描述其特征及治疗方法。
对广泛的肢体VMs病例进行调查、治疗并平均观察7年。
11例累及上肢，16例累及下肢。均累及皮肤和肌肉。下肢81%的病例还累及膝关节，导致严重功能障碍。多普勒超声（双功扫描）、计算机断层扫描和磁共振成像对这些患者是有用的非侵入性诊断方法，而动脉造影和静脉造影提供的信息较少。100%的患者有肌肉受累，63%有骨骼异常。除3例患肢有轻度过度生长外，下肢长度正常或有轻度肢体发育不全。凝血检查显示88%的患者有局部血管内凝血。大多数患者采用保守治疗（穿弹力袜）。少数患者采用经皮硬化治疗或部分切除皮肤和肌肉VMs有益。16例患者中有7例膝关节受累，需要在儿童期进行滑膜切除和VMs切除。
广泛的肢体VMs的特点是皮肤、肌肉和关节弥漫性受累，以及一种具有全身性后果的特定局部血管内凝血病。这组血管畸形应与Klippel-Trenaunay综合征和Parkes Weber综合征相区分。
