Raybaud C A, Hald J K, Strother C M, Choux M, Jiddane M
Neuroradiologie, CHU Timone, Marseille, France.
Neurochirurgie. 1987;33(4):302-14.
The angiographic and/or anatomic study of a series of 30 cases of Vein of Galen aneurysm (which is defined as an arterio-venous fistula within the wall of the vein of Galen itself, therefore as a purely extra cerebral lesion) permits the analysis of the afferent arteries, of the efferent veins, and of the aneurysmal sac itself. The examination of the afferent arteries confirms the data from the literature, indicating that most often it consists of the abnormal hypertrophy of otherwise normally organized arteries, including the dural arteries. In particular, the involvement of distal subcallosal branches of the anterior cerebral arteries as well as the arterio-arterial maze extending above the collicular plate, fit well the normal anatomy. The venous side of the malformation nevertheless presents with more unusual features; the most striking is the non-opacification of the straight sinus and/or part of the transverse sinuses in half of the cases; also, an aberrant falcine sinus is frequently observed, which actually represents the persistence of a normal channel in the fetal anatomy. A more complex pattern was observed in two cases in which the aneurysmal sac drained into the straight sinus via a loop made of a falcine sinus, the superior sagittal sinus and then backward through a second falcine sinus. Finally, from the systematization of the afferents arteries, the aneurysmal sac appears to belong both to the (prosencephalic) velum interpositum and to the ambient cistern. Such a vessel cannot be either the true vein of Galen or an internal cerebral vein; therefore it has to be a persistent fetal vein, the median prosencephalic vein, which drains the choroid plexuses of the lateral and third ventricles between the 7th and 12th weeks, and disappears normally to be replaced by the internal cerebral veins, when the intrinsic vascularization of the neural tube develops. The adult pattern of the brain artery being attained during the 8th week, the event that led to the arterio venous fistula should have occurred some time during the 3rd month. Unfortunately, no clue was found to orient to a specific cause for the anomaly. The frequently observed venous occlusions are actually not seen in neonates but rather in older children. This, and the fact that spontaneous thromboses within the malformative veins have been observed in vivo, make likely the conclusion that venous occlusions are a secondary phenomenon only.
对30例大脑大静脉瘤(定义为大脑大静脉壁内的动静脉瘘,因此是一种纯粹的脑外病变)进行血管造影和/或解剖学研究,有助于分析其输入动脉、输出静脉以及瘤囊本身。对输入动脉的检查证实了文献数据,表明其通常由原本正常结构的动脉异常肥大组成,包括硬脑膜动脉。特别是,大脑前动脉胼胝体下远端分支的累及以及延伸至丘板上方的动脉-动脉迷宫,与正常解剖结构相符。然而,畸形的静脉侧呈现出更不寻常的特征;最显著的是,半数病例中直窦和/或部分横窦不显影;此外,经常观察到异常的大脑镰窦,它实际上代表了胎儿解剖结构中正常通道的残留。在两例病例中观察到一种更复杂的模式,其中瘤囊通过由大脑镰窦、上矢状窦形成的环引流至直窦,然后向后通过第二个大脑镰窦。最后,从输入动脉的系统化来看,瘤囊似乎既属于(前脑)中间帆,也属于环池。这样的血管既不可能是真正的大脑大静脉,也不可能是大脑内静脉;因此它必定是一条持续存在的胎儿静脉,即正中前脑静脉,它在第7至12周期间引流侧脑室和第三脑室的脉络丛,当神经管的固有血管形成时,它通常会消失并被大脑内静脉取代。大脑动脉的成人模式在第8周时形成,导致动静脉瘘的事件应该发生在第3个月的某个时候。不幸的是,没有发现指向该异常特定原因的线索。经常观察到的静脉闭塞实际上在新生儿中未见,而在年龄较大的儿童中更为常见。这一点,以及在体内观察到畸形静脉内有自发血栓形成的事实,使得静脉闭塞只是一种继发现象这一结论很有可能成立。