Awad I A, Robinson J R, Mohanty S, Estes M L
Neurovascular Surgery Program, Yale University School of Medicine, Cleveland, Ohio.
Neurosurgery. 1993 Aug;33(2):179-88; discussion 188. doi: 10.1227/00006123-199308000-00001.
The clinical relevance of any scheme for classification of vascular malformations of the brain remains controversial. Widely accepted pathologic classifications include discrete venous, arteriovenous, capillary, and cavernous malformations. Of 280 cases of possible vascular malformations evaluated by a single cerebrovascular service during a 5-year period, 14 were instances of mixed vascular malformations including definite features of more than one pathologically discrete type of malformation within the same lesion. There were six instances of mixed cavernous and venous malformations in the same lesion; in all instances, the cavernous malformation accounted for the clinical presentation. There were three cases of mixed venous and arteriovenous malformations (arterialized venous malformations), presenting with the typical histoarchitectural appearance of a venous malformation, but with arteriovenous shunting; all cases were symptomatic, two with hemorrhage and one with focal neurological symptoms. There were five cases of predominantly cavernous malformations with features of arteriovenous malformation or capillary telangiectasia in the same lesion. These five cases presented clinically as angiographically occult lesions indistinguishable from a cavernous malformation. Lesions including a venous malformation were recognizable preoperatively because of characteristic imaging features of the venous malformation. Other mixed vascular malformations were indistinguishable on diagnostic studies from pure cavernous malformations. Of the 14 mixed vascular malformations, 11 included a cavernous malformation that was usually responsible for the symptomatic presentation. In the other three cases, manifestations of clinical lesions were due to arteriovenous shunting within a venous malformation. We conclude that mixed vascular malformations of the brain are rare entities with distinct clinical, radiological, and pathological profiles. Their identification generates several hypotheses about common pathogenesis or causation-evolution among different types of lesions.
任何脑血管畸形分类方案的临床相关性仍存在争议。广泛接受的病理分类包括离散性静脉畸形、动静脉畸形、毛细血管畸形和海绵状畸形。在某单一脑血管服务机构5年期间评估的280例可能的血管畸形病例中，有14例为混合性血管畸形，即在同一病变内包含不止一种病理上离散类型畸形的明确特征。同一病变内有6例海绵状畸形与静脉畸形混合的情况；在所有这些病例中，海绵状畸形是临床表现的原因。有3例静脉畸形与动静脉畸形混合（动脉化静脉畸形），呈现出静脉畸形典型的组织架构外观，但存在动静脉分流；所有病例均有症状，2例出血，1例有局灶性神经症状。有5例主要为海绵状畸形的病例，在同一病变内具有动静脉畸形或毛细血管扩张症的特征。这5例临床上表现为血管造影隐匿性病变，与海绵状畸形难以区分。包括静脉畸形的病变术前可因其静脉畸形的特征性影像学表现而被识别。其他混合性血管畸形在诊断研究中与单纯海绵状畸形难以区分。在这14例混合性血管畸形中，11例包含海绵状畸形，通常是症状表现的原因。在其他3例中，临床病变的表现是由于静脉畸形内的动静脉分流。我们得出结论，脑混合性血管畸形是罕见的实体，具有独特的临床、放射学和病理学特征。它们的识别引发了关于不同类型病变之间共同发病机制或因果演变的几种假设。
