Rodesch Georges, Hurth Michel, Alvarez Hortensia, Tadié Marc, Lasjaunias Pierre
Service de Neuroradiologie Vasculaire Diagnostique et Thérapeutique, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
Neurosurgery. 2002 Aug;51(2):374-9; discussion 379-80.
Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances?
We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions.
All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease.
It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.
脊髓动静脉分流（SCAVS）目前根据其形态学特征进行分类。然而，某些分流无法完全归入通常描述的预定类别。能否根据最近在解剖学、生物学和遗传学方面的进展对这些分类进行重新评估？
我们回顾了1981年至1999年在比塞特医院接受治疗的155例SCAVS的临床和放射学档案。对病变的数量（单发或多发）、主要结构类型（病灶或瘘管）以及它们与相关节段性病变的可能联系进行了检查。
所有SCAVS均为动静脉畸形或瘘管，后者又分为微瘘管或大瘘管。所有SCAVS可分为三类，即遗传性病变（大瘘管和遗传性出血性毛细血管扩张症）、非遗传性病变（均为伴有节段性或脊髓节段性关联的多发病变）和单发病变（可能代表前一组中某一种的不完全表现）。在我们的系列SCAVS中，81%为单发病变，19%为多发病变；其中，59%为具有节段性特征的真正硬膜内分流。观察到10例科布综合征、3例克-特综合征和2例帕克思-韦伯综合征，均伴有脊髓病变。19%的SCAVS为瘘管；其中23%为大瘘管，83%与遗传性出血性毛细血管扩张症相关。
提出一种考虑到随时间演变且发生血管构筑变化的原发性畸形（病灶或瘘管）的分类似乎是合理的。认识到导致分流的最初因素（如遗传遗传性或遗传非遗传性）有助于对SCAVS进行不同的分类。
