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多灶性和节段性脊髓动静脉畸形。19例病例回顾。

Multifocal and metameric spinal cord arteriovenous malformations. Review of 19 cases.

作者信息

Matsumaru Y, Pongpech S, Laothamas J, Alvarez H, Rodesch G, Lasjaunias P

机构信息

Service de Neuroradiologie Vasculaire diagnostique et thérapeutique, CHU de Bicêtre; Le Krernlin-Bicêtre, France -

出版信息

Interv Neuroradiol. 1999 Mar 30;5(1):27-34. doi: 10.1177/159101999900500105. Epub 2001 May 15.

Abstract

We describe 19 (16.0%) multiple vascular malformations (AVMs) in 119 spinal cord arteriovenous malformations (SCAVMs). The associated lesions were eight vertebral vascular malformations, two cutaneous, four limbs, four radicular AVMs, three bifocal SCAVMs; one patient had a bifocal cord lesion associated with vertebral and limb localisations. Various syndromic associations were seen: nine Cobb, two Klippel-Trenaunay-Weber, one Parkes Weber. An additional subgroup of unclassified associations is constituted by seven cases with bifocal intradural uni or multimetameric lesions. In our SCAVMs series, the incidence ofmultipie vascular lesions is high, in particular multifocal intradural malformations. Metameric distribution is the most frequent type of multiplicity. Identification of the myelomeric level involved in SCAVM allows segmental link between various lesions of mesodermal or neural crest origin to be discussed.

摘要

我们在119例脊髓动静脉畸形(SCAVM)中发现了19例(16.0%)多发性血管畸形(AVM)。相关病变包括8例椎体血管畸形、2例皮肤病变、4例肢体病变、4例神经根性AVM、3例双灶性SCAVM;1例患者有与椎体和肢体定位相关的双灶性脊髓病变。可见多种综合征关联:9例Cobb综合征、2例Klippel-Trenaunay-Weber综合征、1例Parkes Weber综合征。另外一个未分类关联的亚组由7例双灶性硬膜内单节段或多节段病变组成。在我们的SCAVM系列中,多发性血管病变发生率较高,尤其是多灶性硬膜内畸形。节段性分布是最常见的多发类型。确定SCAVM所累及的脊髓节段水平有助于探讨中胚层或神经嵴起源的各种病变之间的节段联系。

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