Mizutani Katsuhiro, Consoli Arturo, Maria Federico Di, Condette Auliac Stéphanie, Boulin Anne, Coskun Oguzhan, Gratieux Julie, Rodesch Georges
1Department of Diagnostic and Interventional Neuroradiology, Hôpital Foch, Suresnes, Hauts-de-Seine, France.
2Department of Neurosurgery, Keio University School of Medicine, Shinjuku, Tokyo; and.
J Neurosurg Spine. 2021 Apr 2;34(6):920-930. doi: 10.3171/2020.9.SPINE201258. Print 2021 Jun 1.
Few classifications of intradural spinal arteriovenous shunts (ID-SAVSs) have considered their anatomical localization in relation to their phenotype and angioarchitectonics. The authors propose another vision of ID-SAVSs allowing a reappraised classification based on analysis of the anatomical disposition, angioarchitecture, and histogenetic location of these vascular malformations.
The radiological and clinical records of 210 patients with ID-SAVSs were retrospectively reviewed, considering their localization, vascular architectonics, and correlation with the 5 histogenetic units of the spinal cord. Among these, 183 files with complete data allowed precise analysis of the ID-SAVSs.
Among these 183 files (162 and 21 cases with single and multiple lesions, respectively), different entities were identified: 13 pial macro arteriovenous fistulas (MAVFs), 92 pial micro arteriovenous fistulas (mAVFs), 33 superficial pial niduses, and 69 intramedullary niduses. Thirteen sulcal shunts (either fistulas or niduses) were considered subtypes of pial lesions. Among the 21 multiple cases, 11 were monomyelomeric while 10 were multimyelomeric. Pial lesions, either fistulas or niduses, were dominantly vascularized by pial arteries (anterior or posterior depending on the localization of the shunt) and occasionally (except for MAVFs) by transmedullary arteries. Pial niduses occasionally extended into the funiculus by recruiting intrinsic veins or by extension of the nidus itself inside the white matter. Intramedullary niduses were always vascularized by both centrifugal and centripetal feeders, respectively, from sulcal arteries (SAs) and pial arteries. Sulcal lesions are pial lesions located within the ventral median sulcus and vascularized by SAs and veins. Single or multiple ID-SAVSs can be part of various syndromes such as hereditary hemorrhagic telangiectasia, Parkes-Weber, RASA1, CLOVES, and spinal arteriovenous metameric syndromes. Histogenetic analyses revealed a specific distribution of each ID-SAVS in the 5 histogenetic units of the spinal cord: intramedullary niduses were found almost equally from cervical to thoracic units, while MAVFs and mAVFs were mostly found from thoracic to postcrural ones. Pial niduses showed intermediate features between intramedullary and fistulous lesions and were mostly distributed from brachial to crural segments.
Precise analysis of the anatomical disposition of ID-SAVSs in relation to functional histogenetic units allows a better understanding of these lesions and improved therapeutic management.
很少有硬脊膜内脊髓动静脉分流（ID-SAVS）的分类考虑到其解剖定位与表型和血管构筑学的关系。作者提出了另一种对ID-SAVS的见解，允许基于对这些血管畸形的解剖位置、血管构筑和组织发生位置的分析进行重新评估的分类。
对210例ID-SAVS患者的放射学和临床记录进行回顾性分析，考虑其定位、血管构筑以及与脊髓5个组织发生单位的相关性。其中，183份具有完整数据的文件允许对ID-SAVS进行精确分析。
在这183份文件中（分别有162例和21例单发和多发病变），识别出了不同的类型：13例软膜大动静脉瘘（MAVF）、92例软膜微动静脉瘘（mAVF）、33例浅表软膜病灶和69例髓内病灶。13例沟分流（瘘或病灶）被认为是软膜病变的亚型。在21例多发病例中，11例为单节段性，10例为多节段性。软膜病变，无论是瘘还是病灶，主要由软膜动脉供血（根据分流的位置为前或后），偶尔（MAVF除外）由经髓动脉供血。软膜病灶偶尔通过募集固有静脉或病灶本身在白质内的延伸而延伸至脊髓索。髓内病灶总是分别由来自沟动脉（SA）和软膜动脉的离心和向心供血支供血。沟病变是位于腹侧正中沟内并由SA和静脉供血的软膜病变。单发或多发的ID-SAVS可以是各种综合征的一部分，如遗传性出血性毛细血管扩张症、帕克斯-韦伯综合征、RASA1综合征、CLOVES综合征和脊髓动静脉节段性综合征。组织发生学分析显示，每种ID-SAVS在脊髓的5个组织发生单位中有特定的分布：髓内病灶在颈段到胸段的分布几乎相等，而MAVF和mAVF大多在胸段到骶尾部发现。软膜病灶表现出髓内和瘘性病变之间的中间特征，主要分布在臂段到骶段。
对ID-SAVS与功能性组织发生单位相关的解剖位置进行精确分析，有助于更好地理解这些病变并改善治疗管理。
