大脑大静脉畸形

1、Galen大脑大静脉动脉瘤样畸形多见于新生儿及婴儿，婴幼儿及儿童约占90，成人约占10。正常大脑大静脉位于松果体后方，由双侧大脑内静脉内联合形成，它向后流入大脑镰及小脑幕连接处的直窦。Galen大脑大静脉动脉瘤样畸形定义是指位于中间帆池的胚胎残余前脑内侧静脉与动脉发生直接交通，形成的动脉瘤样畸形。没有正常的大脑大静脉和直窦缺如，畸形通过大脑镰窦向上矢状窦回流，该静脉不汇集正常脑组织的静脉回流，动静瘘口可为单个或多个。Galen大脑大静脉动脉瘤样畸形可分为真性Galen大脑大静脉动脉瘤样畸形和假性Galen大脑大静脉动脉瘤样扩张，前者与血管胚胎异常发育有关，根据瘘口的位置分为脉络膜型和壁型。后者

2、主要是中脑AVM或脑膜AVF与大脑大静脉相连，静脉流出道狭窄或闭塞导致该静脉病理性扩张。先天性动静脉短路使动脉血流通过动静脉瘘口直接大脑大静脉，使其呈球形或橄榄形扩张，血流引流至直窦，导致直窦扩张，压迫四叠体及导水管，使之移位造成脑积水。颈内动脉、椎动脉及基底动脉常扩大及迂曲，常是双侧大脑后动脉分之与静脉交通，单侧者罕见。进入大脑大静脉的动脉可扩张或在大脑大静脉旁形成网状结构。在分流部位可见动脉结构逐渐向静脉结构依位，血管壁内弹力板及肌层逐渐变薄及消失。Galen大脑大静脉动脉瘤样畸形可压迫大脑导水管，导致颅高压症状引起脑脊液循环和吸收障碍产生梗阻性脑积水。由于 出现“盗血”现象易使周围脑组织

3、缺血，造成脑梗死，同时“盗血”使大量动脉血未经正常脑组织循环直接经动静脉瘘口流入脑静脉窦，从而使心脏负荷及博出量增大，心脏输出量为正常的3-4倍，造成充血性心力衰竭。这种病人常见头颅增大，头皮血管扩张，可闻及颅内杂音及头皮血管杂音，肝脾可肿大1。其次常伴有其他血管异常，如脑分流血管发育不良。心脏可有移位、中隔缺损、动脉导管未闭、全身广泛性血管瘤疾病等系统畸形。 Galen大脑大静脉动脉瘤样畸形可分为新生儿、婴儿、儿童和成人四个年龄组。新生儿组主要表现为严重心衰和颅骨杂音；婴儿组表现为轻度心衰，颅骨增大，颅骨杂音；儿童及成人组表现为头痛、嗜睡、脑积水、抽搐、智力下降。Galen大脑大静脉动脉瘤样

4、畸形的诊断主要依靠脑血管造影、CT扫描、核磁共振扫描（MRI及MRA）、经颅多普勒超声等影像学检查。脑血管造影可了解血管畸形的供血动脉、引流静脉及盗血情况，显示动静脉瘘口部位及类型，造影的同时还能进行颅内动静脉畸形的栓塞治疗；核磁共振扫描（MRI及MRA），不需注射造影剂，能显示脑的正常和异常血管、畸形血管与脑功能区的关系，以及可以显示高流量动静脉畸形盗血后脑组织的缺血改变；CT扫描对出血范围、血肿大小，血栓形成的梗塞灶、脑室内出血、脑积水有较高诊断价值，注入造影剂后可较好显示病灶轮廓。经颅多普勒超声可帮助确定血流方向和动静脉畸形血管结构类型；区别动静脉畸形的流入和流出血管；深部动静脉畸形的定

5、位；动态监测动静脉畸形输入动脉的阻断效果和血液动力学改变。 Galen大脑大静脉动脉瘤样畸形是特殊位置的动静脉畸形，死亡率及致残率高，常伴有心功能不全，盗血严重。此病治疗关键是栓塞动静脉瘘口，避免损伤引流静脉。目前此病治疗方法分为手术和非手术治疗两种，手术治疗包括：1、供血动脉结扎术。2、动静脉畸形切除术。3、血管内栓塞。4、立体定向夹闭供血动脉。非手术疗法有放射治疗。血管内栓塞对Galen大脑大静脉动脉瘤样畸形疗效肯定，且安全性高，为临床广泛应用。临床表现： 甲1日龄，足月，女性阴道分娩的产钳/生通过延长。 Pregnancy was complicated by in utero diag

6、nosis of cranial AV malformation. 妊娠合并子宫畸形的诊断颅视听。 At birth, the infant was floppy and cyanotic requiring stimulation and PPV. 出生时，婴儿是软盘和需要刺激和PPV的发绀。 He has cardiac anomalies including, CPAPUR, ASK, and PDA. 他，ASK的心脏异常包括CPAPUR，与PDA。 He has had several episodes of upper extremity flexing, lower extrem

7、ity extension and right eye deviation. 他有几次上肢屈曲，下肢延长和右眼的偏差。 Radiographic Findings: X线表现： Figure 1: Grayscale ultrasound showing a sonolucent posterior third ventricular mass. 图1：灰阶超声显示一sonolucent后第三左室质量。 Figure 2: Color Doppler image showing turbulent flow within the mass. 图2：彩色多普勒超声图像显示质量湍流内。 Figur

8、e 3A : Sagittal T1 weighted MR imaging showing hypointensity of the varix resulting form a loss of phase coherence of mobile protons. 图3a：矢状T1加权低信号质子磁共振成像显示静脉曲张引起的流动损失的形式相一致。 Figure 3B: Axial T2 weighted image showing the varix with collateral vessels. 图3b：轴流T2加权图像显示血管静脉曲张的抵押品。 Figure 4: MRA depicts

9、 arterial feeders to better advantage. 图4：动脉MRA的描绘，以更好地利用馈线。 Figure 5: MRV depicts venous collaterals. 图5：静脉侧支循环的MRV描绘。 Figure 6A 图6a Figure 6B 图6b Figure 6A&B: Diffusion weighted images and ADC maps show ischemic changes from venous steel. 图6a和B：扩散加权像和ADC图显示静脉钢缺血性变迁。 Diagnosis: Vein of Galen Ma

10、lformation 诊断： 静脉畸形的盖伦 Discussion: 讨论： Classification: Several classifications based on the angioarchitecture and anatomy have been developed the most widely used scheme is from Yasagil's experience 1. 分类：有几个分类解剖基础上，血管构筑，并已开发出了使用最广泛的计划的经验是从Yasagil的1。 In his classification there are four subtypes

11、 of vein of Galen malformations. 在分类有四种亚型盖伦静脉畸形的。 · Type I: Lesions are direct arteriovenous shunts to the medial prosencephalic vein supplied by choroidal, pericallosal, and superior cerebellar arteries. I型：小脑上动脉病变的直接动静脉分流到静脉，内侧prosencephalic提供的脉络膜，pericallosal。 · Type II: Lesions have si

12、milar shunts supplied by transmesencephalic and transdiencephalic perforators. II型：病变有transdiencephalic穿类似的分流和提供的transmesencephalic。 · Type III: Lesions are combinations of types I and II. III型：病变类型组合是第一和第二。 · Type IV: Lesions consists of an arteriovenous shunt distant from the vein of Gal

13、en but drains into it, producing dilatation. IV型：病变由一动静脉分流盖伦从遥远的静脉，但水渠，生产扩张。 Another useful and practical classification has arisen from the endovascular group at Bicetre Hospital in Paris 2. 另一个有用的和实际的分类出现了巴黎2从腔内组比塞特尔医院为例。 They have classified anomalous arteriovenous shunts involving the vein of Ga

14、len into two groups: (a) true vein of Galen aneurysmal malformations (VGAMs) and (b) vein of Galen dilatations that occur secondary to high-flow parenchymal AVMs draining into this vessel. 他们分为异常组：静脉的分流，涉及到两个盖伦静脉（a）真实静脉动脉瘤样畸形的盖伦（VGAMs）和（b）静脉盖伦伸缩发生继发于高流量动静脉畸形血管实质引流这项工作。 Two subtypes of VGAMs have bee

15、n identified on the basis of their angioarchitecture: (a) a mural form and (b) a choroidal form. 两个VGAMs亚型有：确定对他们的基础上构筑学（一）一壁画的形式和（b）一脉络膜形式。 The so-called choroidal form is the most common type. 所谓脉络膜形式是最常见的类型。 Choroidal AVMs classically demonstrate an abundance of bilateral arterial supply from the

16、 choroidal arteries, pericallosal arteries and subependymal branches of the thalamoperforating vessels. 经典展示室管膜下脉络膜动静脉畸形血管分支的thalamoperforating有余双边动脉供应的动脉和脉络膜动脉，pericallosal。 These vascular connections are extracerebral, subarachnoid and communicate with the median pros encephalic vein. 这些血管连接脑外，蛛网膜

17、下腔和专业人员交流与颅内静脉中位数。 Mural-type AVMs represent approximately one-third of VGAMs, They receive uni- or bilateral supply from the collicular and posterior choroidal vessels and drain into a persistent median prosencephalic vein, Outlet obstruction is common. 壁画型动静脉畸形约占1 - VGAMs三分之一，他们收到一个持久的中位数prosencep

18、halic静脉单向或双边供应从丘，后脉络膜血管和排入，出口阻塞是常见的。 Etiology : Vein of Galen aneurysmal malformations probably represent an arteriovenous fistula (AVF) in the wall of a persistent embryonic vascular channel called the median prosencephalic vein 3, 4. 病因 ：静脉畸形瘤的盖伦可能代表了一个持续的胚胎血管动静脉瘘的通道（瘘管）在墙上称为中位数prosencephalic静脉3，4

19、。 This dorsal vein lies in the roof of the diencephalons and drains the developing choroids plexus 5. 这背静脉位于间脑的屋顶和排水渠的发展脉络丛5。 In turn the median prosencephalic vein drains into a primitive accessory sinus called the falcine sinus. 反过来水渠中位数prosencephalic静脉窦成原始的falcine配件窦调用。 By week 10 of fetal develo

20、pment the median prosencephalic vein regresses as the definitive internal cerebral veins appear. 通过发展10周胎儿静脉后退中位数prosencephalic作为最终大脑内静脉出现。 A caudal remnant remains as the vein of Galen 6. 阿尾残余仍然作为静脉盖伦6。 If the median prosencephalic vein does not regress, a fistulous connection with the primitive ch

21、oroidal arteries may persist. 如果静脉中位数prosencephalic不倒退，一脉络膜动脉瘘管连接与原始的可能依然存在。 A VGAM is the result. 阿VGAM是结果。 Imaging: On imaging studies these malformations appear as large masses in the posterior incisural region, sometimes extending rostrally and anteriorly displacing the third ventricle. 影像：在这些畸形

22、的影像学显示为incisural地区大型群众在后，有时会延长rostrally和前方取代第三脑室。 Ultrasound: Transcranial B-mode ultrasound shows a sonolucent posterior third ventricular mass ( Fig. 1 ) and obstructive hydrocephalus. 超声：经颅B型超声显示sonolucent后第三左室质量（ 图1）和梗阻性脑积水。 It is important to demonstrate continuity with the straight sinus or a

23、persistent falcine sinus. 重要的是要表现出连续性直窦窦或持续falcine。 Color flow imaging ( Fig. 2 ) often discloses turbulent bi-directional flow within the enlarged vein of Galen 7. 彩色血流成像（ 图2）经常披露动荡的双向流量之内7扩大静脉盖伦。 Computed Tomography: An iso- or hyperdense midline mass posterior to the third ventricle is seen on no

24、nenhanced studies. 电脑断层扫描：通过ISO -中线或高密度大规模的第三脑室后部是看到平扫研究。 Mixed attenuation may be seen if the varix is partially thrombosed. 混合衰减可以看出，如果静脉曲张部分血栓形成。 Patent VGAMs enhance strongly following contrast administration. 大力加强专利VGAMs以下对比管理。 Mass effects with hydrocephalus and secondary encephalomalacic chan

25、ges are common 8. encephalomalacic变化与质量的影响是常见的脑积水和中学8。 Magnetic Resonance Imaging: On MR the varix will be hypointense resulting form a loss of phase coherence of mobile protons ( Fig. 3 ). 磁共振成像：论议员的静脉曲张会导致形成一个低信号）损失3阶段（ 图一致性移动质子。 Areas of acute thrombosis will be isointense to brain on short TR/TE

26、 sequences and hypointense on T2 weighted spin echo or gradient echo sequences, whereas subacute thrombus will have a high intensity on both short TR and long TR spin echo sequences. 急性血栓形成区等信号，以低信号将大脑短章/ TE的序列，在T2加权自旋回波或梯度回波序列，而亚急性血栓将有章自旋高强度的TR和长短期回声序列。 Thrombosis of varying age sometimes lines the

27、 wall of the varix. 血栓形成不同年龄有时行静脉曲张的墙壁。 MRA ( Fig. 4 ) and MRV ( Fig. 5 ) can depict the arterial feeders and delineate venous flow patterns. 磁共振血管成像（ 图4）和MRV（ 图5）可以描绘动脉馈线和划定静脉血流模式。 T2 and diffusion weighted sequences ( Fig. 6 ) show ischemic white matter changes secondary to venous steel. T2和扩散物质变化

28、的加权序列（ 图6）显示继发静脉缺血性白钢。 Cerebral Angiography: Arterial supply to VGAMs is usually via enlarged choroidal and thalamoperforating arteries. 脑血管造影：动脉供应VGAMs通常是通过扩大和thalamoperforating脉络膜动脉。 In the fistula group the posterior choroidal arteries are the dominant supply, followed by anterior choroidal and t

29、halamoperforating and anterior cerebral branches. 在瘘组后脉络膜动脉是最主要的供应，其次是前分行脉络膜和thalamoperforating和前脑。 In the nidus type the thalamoperforating vessels are the common arterial feeders 9. 在病灶类型的thalamoperforating船只的共同动脉馈线9。 Venous drainage patterns include aneurismal dilatation of the vein of Galen (ven

30、ous varix) with or without stenosis. 静脉引流模式包括无狭窄或动脉瘤，静脉扩张的盖伦与（静脉静脉曲张）。 Drainage into an accessory or inferior falcine sinus is also common 10. 窦渠成配件或劣质falcine也很普遍10。 The falcine sinus is a primitive channel that connects the vein of Galen into the superior sagittal sinus coursing posterior superiorl

31、y within the falx cerebri. 该falcine窦是一种原始的通道，连接大脑内静脉窃喜的镰窦后上方的盖伦到上矢状。 Complications : Complications of VGAMs include obstructive hydrocephalus, atrophy of adjacent structures due to compression, venous thrombosis and hemorrhage. 并发症 ：VGAMs并发症包括阻塞性脑积水，出血萎缩血栓形成和邻近建筑物，由于压缩，静脉。 Periventricular leukomalac

32、ia, cortical laminar necrosis and atrophy secondary to ischemia and steal phenomenon or high output congestive heart failure are common 5. 脑室周围白质软化，皮质层萎缩坏死继发于缺血现象，窃取或高输出充血性心力衰竭是常见的5。 Treatment: The current recommendations for treatment of vein of Galen malformations include transarterial or transven

33、ous embolization techniques. 治疗：盖伦静脉畸形的治疗目前的建议包括经静脉栓塞予，或技术。 Close coordination among the obstetricians, neurosurgeons, neonatologists and the endovascular team is essential to optimize prenatal planning. 关闭队之间的协调产科医生，神经外科，neonatologists和血管内必须优化产前规划。 References: 参考文献： 1. Yargasil MG. Microneurosurger

34、y . Yargasil镁。 显微神经外科 。 New York: Thieme 1988. 纽约：姆1988年。 2. Garcia-Monaco R, Lasjuanias P, Berenstein A. Therapeutic management of vein of Galen aneurysmal malformations. Interventional Neuroradiology: Endovascular Therapy of the Central Nervous System : New York: Raven, 1992:113-127. 加西亚，摩纳哥俄，Lasj

35、uanias磷，Berenstein答：治疗动脉瘤的静脉畸形管理的盖伦。 介入神经放射学：血管内系统治疗的中枢神经 ：纽约：乌鸦，1992:113-127。 3. Lasjuanias P. Vascular Diseases in Neonates, Infants and Children . Lasjuanias体育血管疾病在新生儿，婴儿和儿童 。 New York: Springer Verlag 1997. 纽约：斯普林格出版社1997年。 4. Tomsick TA, Ernst RJ, Twe JM, et al. Tomsick助教，恩斯特的RJ，您现在JM，等。 Adult

36、choroidal vein of Galen malformation. AJNR 1995; 16: 861-865. 成人盖伦脉络膜静脉畸形。AJNR 1995，16：861-865。 5. Brunelle F. Arteriovenous malformation of the vein of Galen in children. Pediatr Radiology 1997; 27:501-513. 动静脉畸形的儿童布鲁内尔楼在盖伦静脉。Pediatr放射学杂志 1997; 27:501-513。 6. Horowitz MB, Jungreis CA, Quisling RG, Pollock I. vein of Galen aneurysms: a review and current perspective. AJNR 1994; 15:1486-1496. 霍罗威茨兆，Jungreis核证机关，盖伦瘤吉斯林的RG，波洛克一脉：一检讨和当前观点。AJNR 1994;