Mattassi R
Center for Diagnosis and Treatment of Vascular Malformations, Hospital Santa Corona, Garbagnate Milanese, Italy.
Semin Vasc Surg. 1993 Dec;6(4):233-44.
Congenital vascular-bone syndrome is an alteration in limb growth caused by congenital vascular malformations in childhood. A precise study of the anatomic and hemodynamic nature of the underlying defect is necessary, not only for diagnosis, but also for therapy. The use of old eponyms for diagnosis, such as "Klippel-Trenaunay," "Parkes-Weber," and "Servelle-Martorell" should be abandoned because they are meaningless and misleading. An anatomic/pathological categorization, according to the simple "Hamburg classification," is more practical for clinical purposes. Seven different types of vascular defects, all associated with a-v fistulas, are related with limb overgrowth. Four different types of vascular malformations may produce limb shortening. Complete diagnostic study should allow classifying the malformation into one of the groups listed and illustrated above. Precise diagnosis is useful because interventional therapy can correct limb length discrepancy if performed before epiphyseal closure.
先天性血管-骨综合征是一种由儿童期先天性血管畸形引起的肢体生长改变。不仅对于诊断，而且对于治疗而言，精确研究潜在缺陷的解剖学和血流动力学性质都是必要的。应摒弃使用诸如“克-特综合征”“帕克斯-韦伯综合征”和“塞尔维-马托雷尔综合征”等旧的诊断名称，因为它们毫无意义且具有误导性。根据简单的“汉堡分类法”进行解剖学/病理学分类，对于临床目的而言更为实用。七种不同类型的血管缺陷，均与动静脉瘘相关，与肢体过度生长有关。四种不同类型的血管畸形可能导致肢体缩短。完整的诊断研究应能将畸形归类为上述列出并说明的其中一组。精确的诊断很有用，因为如果在骨骺闭合之前进行介入治疗，可以纠正肢体长度差异。
