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[血管发育异常与骨骼系统]

[Angiodysplasia and the skeletal system].

作者信息

Vollmar J, Vogt K

出版信息

Chirurg. 1976 Apr;47(4):205-13.

PMID:183938
Abstract
  1. Congenital angiodysplasias of the extremities are often associated with skeletal disorders, i.e., overgrowth (local giantism, hyperplasia) or reduced growth (hypoplasia). From a clinical point of view three types of combined lesions may be differentiated: (a) a secondary local giantism induced by hemodynamically active congenital a.v. fistulas, usually of intra- and extraosseous location (Weber-type); - (b) local giantism in combination with venous and/or lymphatic angiodysplasias; in this group skeletal overgrowth seems not to be caused by the vascular anomalies but represents more probably a coordinated "inborn error" of tissue composition and distribution (Klippel-Trenaunay-type); - (c) hemangiomatosis usually of venous (cavernous) type, affecting soft tissues as well as bones in association with skeletal hypoplasia. There is a retardation of bone growth by substitution and destruction of the epiphysial cartilages by the intraosseous hemangioma (Servelle-Martorelltype). In most cases (a-c) the use of special angiological investigations allows a clear diagnosis particularly concerning the pathogenesis of skeletal disorders. 2. In looking for an unequivocal terminology the clinical classification of these anomalies should be based on the leading clinical symptome, i.e., the giantism or the angiodysplasia. In this respect the vascular findings take on a predominant position. Paying regard to historical aspects the classification may be completed by the additional term "Klippel-Trenaunay" type, "Weber" type, "Servelle-Martorell" type. 3. An exact angiologic diagnosis gives further informations about prognosis and choice of treatment (group I: Early operative elimination or reduction of the a-v shuntvolume; group II and III: "wait and see!"; usually conservative treatment with compression bandages).
摘要
  1. 先天性肢体血管发育异常常与骨骼疾病相关,即过度生长(局部巨人症、增生)或生长受限(发育不全)。从临床角度来看,可区分出三种类型的合并病变:(a) 由血流动力学活跃的先天性动静脉瘘引起的继发性局部巨人症,通常位于骨内和骨外(韦伯型);-(b) 局部巨人症合并静脉和/或淋巴管血管发育异常;在这一组中,骨骼过度生长似乎不是由血管异常引起的,而更可能是组织组成和分布的协调性“先天性缺陷”(克-特综合征型);-(c) 通常为静脉(海绵状)型的血管瘤病,累及软组织和骨骼并伴有骨骼发育不全。骨内血管瘤取代并破坏骨骺软骨,导致骨生长迟缓(塞尔韦-马托雷尔型)。在大多数情况下(a - c),使用特殊的血管造影检查可做出明确诊断,尤其是关于骨骼疾病的发病机制。2. 在寻找明确的术语时,这些异常的临床分类应基于主要临床症状,即巨人症或血管发育异常。在这方面,血管检查结果占主导地位。考虑到历史因素,分类可通过附加术语“克-特综合征”型、“韦伯”型、“塞尔韦-马托雷尔”型来完善。3. 准确的血管造影诊断可提供有关预后和治疗选择的更多信息(第一组:早期手术消除或减少动静脉分流体积;第二组和第三组:“观察等待!”;通常采用压迫绷带进行保守治疗)。

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