Prognosis of antenatally diagnosed vein of Galen aneurysmal malformations

In our experience of 168 consecutive cerebral arteriovenous shunts, all antenatally diagnosed lesions were vein of Galen aneurysmal malformations (VGAMs). This series consists of 18 cases of VGAMs detected by ultrasound during the third trimester of pregnancy. There were 12 normal vaginal deliveries, 5 deliveries by cesarean section, and 1 induced abortion. Sixteen newborns (94%) presented with systemic cardiac manifestations as the first clinical symptoms; 12/16 were managed effectively by digitalo-diuretic treatment, while 4 (25%) died shortly after birth form acute heart and/or multiorgan failure with extensive brain damage. Twelve babies underwent embolization via the arterial route in infancy (2 at 2 months of age). Total exclusion was obtained in 8 babies (67%, 3 with 6 months follow-up). Furthermore, 67% of the newborns managed by our team are neurologically normal (Denver and Brunet-Leizine tests). These results emphasize that the pessimism that follows antenatal discovery of these lesions and the previous assumption of a bad prognosis for VGAMs can nowadays be reviewed in the light of transarterial endovascular therapy applied according to a strict clinicoradiological protocol. Interventions in the neonatal period are rarely required. Generally, poorly timed mechanical therapy should be discouraged.     

Hydrodynamics in vein of Galen malformations

Forty-three patients with vein of Galen aneurysmal malformations (VGAM) referred to us for endovascular treatment between 1985 and 1990 and 335 additional cases published in the literature were reviewed with particular attention to the presence of ventricular enlargement and outcome after shunting. Hydrocephalus was the second most frequent symptom (46.8%); it is more frequent in infants (73%) than in children, adults (30%) or neonates (15%). Of the patients reported in the literature, 17.9% had undergone shunting. Within the shunted population there was an overall morbidity of 41% and a mortality of 10% (especially in the infant group). In our series 17 patients (39.5%) were shunted and a significant difference in the clinical outcome was noted between the shunted and the nonshunted group. Of the nonshunted patients, 66.6% were free of any neurological deficit or mental retardation and fewer than 5% presented with significant mental retardation. On the other hand, only 33.3% of the shunted patients had a favorable outcome and more than 15% developed significant mental retardation. Among the various causes of hydrocephalus in patients with VGAMs, such as obstruction of the aqueduct, subarachnoid hemorrhage, or ex vacuo hydrocephalus, high venous pressure may be of particular importance. In this article a physiopathological interpretation of the hydrodynamics in VGAMs is developed and a speculative explanation for CSF disorders related to ventricular shunting proposed. Treatment of hydrocephalus in VGAMs can be achieved through obliteration of the malformation or at least diminishing the venous pressure; surgical ventricular shunting does not have to be the first treatment of hydrodynamic disorders associated with VGAMs, especially in infants.     

Susceptibility weighted imaging in infants with staged embolization of vein of Galen aneurysmal malformations

Background and purpose

The vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation with a higher morbidity and mortality, especially in neonates. Ultrasound, CT and MR are usually used in diagnosis and treatment monitoring of these disorders. In this current study, we aim to examine utility of SWI in evaluation of treatment response in infants with VGAM.

Normal galenic drainage of the deep cerebral venous system in two cases of vein of Galen aneurysmal malformation

Introduction Vein of Galen aneurysmal malformations (VGAM) are assumed to be related to the persistence of the embryonic median prosencephalic vein of Markowski, which does not involute in cases of VGAM and becomes the venous collector of the shunt, characteristic of the malformation. The arterial feeders of VGAMs are all primitive meningeal arteries present during the embryonic period of the constitution of the malformation. It has also been assumed that the internal cerebral veins were absent in patients presenting with VGAM. There is no clear evidence indicating, however, that the deep venous structures cannot develop normally.Case reports We report on two cases of VGAM in which superselective retrograde transvenous catheterization and MRI demonstrated that normal internal cerebral veins were draining into the aneurysmal vein of Galen.Conclusions It is conceivable that, as in our patients, this drainage pattern is only angiographically detectable via selective retrograde transvenous drainage. The possibility of such normal deep galenic venous drainage must be considered in VGAM management, as it may imply occurrence of adverse effects when the malformation is occluded on the venous side.A commentary on this paper is available at     

Transarterial embolization of vein of Galen aneurysmal malformation after unsuccessful stereotactic radiosurgery

The authors present three cases of vein of Galen aneurysmal malformations (VGAMs) diagnosed in infancy and submitted by the referring teams for stereotactic radiosurgery as the initial therapy (therapeutic doses ranging between 20–25 Gy and 40–50 Gy to the peak dose). After the conventional follow-up of 18–24 months, no change could be detected in the angioarchitecture of the lesions. All three cases were then referred for endovascular treatment and underwent embolization by the transarterial route using liquid adhesives (N-butyl cyanoacrylate). This resulted in complete anatomical exclusion of the lesion. Regardless of the theoretical efficiency of radiosurgery in the management of brain arteriovenous malformations, the present authors believe that transarterial embolization remains the treatment of choice in VGAMs. It offers a high rate of morphological cure and the best chances for normal neurocognitive development. The time required by radiosurgery to achieve a significant result is too long for developing and maturing brain and may not prevent the negative effects of the lesion, mainly in regard to hemo- and hydrodynamic disorders (atrophy, subcortical calcifications, etc.) created by the VGAM, thus leading to irreversible mental retardation.     

Galen静脉动脉瘤样畸形一例并文献复习

目的 探讨Galen静脉动脉瘤样畸形的临床特征和血管内治疗。方法 报告1例Galen静脉动脉瘤样畸形病例，结合相关文献分析该病的分类、临床表现和影像特征，探讨其栓塞治疗。结果 Galen静脉动脉瘤样畸形是一种少见的血管性疾病，约占颅内血管畸形的1％。不同的类型影像特征和临床表现不同，确诊和治疗有赖于脑DSA，血管内栓塞治疗效果较好。结论 Galen静脉动脉瘤样畸形有较高的致残率和死亡率，血管内治疗是目前最好的治疗方法。     

Vein of Galen malformation

Since 1984, 43 patients with true vein of Galen ancurysmal malformations have been referred to us and managed according to our patient selection, technique, and follow-up guidelines. Thirty-four were embolized transarterially with bucrilate (isobutyl cyanoacrylate) or enbucrilate (N-butyl cyanoacrylate) embolization. No cutdown or hypotension during or after the embolization was used and no balloon catheter was employed. Forty-seven percent of the children had a completely occluded lesion which was confirmed when the child was at least 6 months of age at the follow-up angiographic examination; 52.9% were found to be completely normal or only to have mild cardiac failure that could be treated medically or moderate macrocephaly without neurological symptoms or mental retardation. In the embolized group 5.8% died as a result of the wrong treatment (1 case) or poor timing of embolization 3 days after ventricular shunting (1 case). The overall mortality (embolized and non-embolized groups) in the neonatal children was 27.7% with a total of 18.6% for all ages. Complete morphological exclusion of the arteriovenous malformation was accomplished in 41.9%; 74.4% of all children referred are now clinically normal or present moderate mental retardation which is diminishing. There was 3% neurological morbidity in the embolized group (only following the venous approach) in 78 sessions and more than 100 arteries embolized. These results compare favorably with surgical or other techniques of arterial embolization (balloon or particles), as well as transvenous (transtorcular or transfemora) embolization, where the morbidity and mortality are significantly higher and the late clinical evaluation is seldom satisfactory. We believe that presently there is no indication for surgery as a primary form of treatment if a properly trained interventional neuroradiological team is available.     

Galen静脉血栓临床和影像学表现

目的探讨Galen静脉血栓的病因、发病机制、临床、影像学表现及治疗。方法收集10例Galen静脉血栓患者的临床资料和影像学图像,结合Galen静脉解剖学及其血栓形成后病理学改变进行分析。结果 10例患者中,女性7例,男性3例,年龄16~49岁,平均年龄29.1±9.06岁,均急性起病,多以头痛、呕吐为首发症状,病情逐渐进展,可出现意识不清并伴有抽搐等症状。经治疗,6例治愈,2例好转,2例死亡。结论 Galen静脉血栓的病因多种;以急性颅内压增高表现为主,临床表现多样,多出现头痛、呕吐、抽搐及意识障碍等;Galen静脉血栓在急性期CT上呈高密度影,在亚急性期MRI上T_1WI、T_2WI均呈高信号有助于诊断;及时应用低分子肝素抗凝有望取得良好效果。     

Dilated vein of Galen in Kabuki syndrome

Kabuki syndrome (KS) comprises multiple congenital abnormalities and is characterized by a peculiar facial appearance, dermatoglyphic anomalies, mental retardation, skeletal abnormalities and postnatal growth retardation. We describe the case of a 23-month-old boy with the typical features of KS who had several malformations in the veins of the brain, which had not previously been described in patients with this syndrome. The MRI phlebogram of this patient showed that the vein of Galen was dilated and that it drained anomalously. The sinus rectus was abnormal and the longitudinal inferior venous sinus was absent. In view of this finding, together with the fact that structural brain abnormalities in KS are more frequent than in other congenital syndromes with multiple malformations, we propose that MRI be used in the diagnostic work-up of all patients with KS.     

Ethylenevinylalcohol copolymer (Onyx-18) used in endovascular treatment of vein of Galen malformation

Background Vein of Galen malformations (VGM) are rare congenital arteriovenous fistulas that usually present with heart failure in the neonate. Endovascular treatment options in the past have utilized coils, balloons, and acrylics. Case report We present, for the first time in the literature, a case of an infant with VGM treated initially with staged coil embolizations followed 1 year later by the transarterial and transvenous catheter based injection of Onyx-18 (ethylenevinylalcohol copolymer) in a single treatment session. The fistula was eliminated, and the infant’s cardiopulmonary symptoms were improved.     

Pathologic findings in a patient with a vein of Galen aneurysm treated by staged endovascular embolization

Vein of Galen aneurysms are uncommon malformations that can be treated with a combination of endovascular embolization and surgery. Since the relevant techniques are new and innovative, their pathologic sequelae are not yet well described. A patient treated with staged embolization over a 15-month period developed evidence of cerebral venous hypertension on angiography. At necropsy, we observed marked myointimal proliferation of the aneurysm wall, dura, and cerebral vessels in addition to severe widespread encephalomalacia, calcinosis, and rare mural thrombi. These vascular changes have not been previously described in cases of vein of Galen aneurysm, and may, in part, be a consequence of embolization therapy.     

The use of adenosine in the treatment of a high-flow vein of Galen malformation in an adult

The treatment of high-flow vein of Galen aneurysmal malformations (VGAM) remains a therapeutic challenge for the neurosurgeon and the neurointerventionalist, as it is associated with high morbidity and mortality rates despite recent advances in open cranial surgery and interventional neuroradiology. A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. Intravenous boluses of adenosine were injected as an attempt to slow down the arteriovenous shunting of a VGAM prior to endovascular treatment. Adenosine can be a very useful adjunct in patients with extremely high arteriovenous shunting. n-butyl cyanoacrylate (n-BCA) should be the embolic material of choice due to its quick polymerization and adhesive properties.     

Digitate intravascular appendages and aneurysm of the vein of Galen

Peculiar intravascular appendages were found in a case of aneurysm of the vein of Galen. Such appendages consisted of finger-like polypoid growths which protruded within the lumen of ectatic cerebral veins and arteries. They were solid, relatively large, and non-branching. Histologically, their structure was comparable to that of a vessel wall, but was concentrically laminated around the longitudinal axis. Some appendages were associated with thrombi. It is suggested that appendages of this kind probably represent secondary vascular malformations, that they are likely to develop in reaction to abnormal hemodynamic strains upon the vessel walls, and that they might be thrombogenic and, therefore, potentially beneficial for patients with arteriovenous shunts. Received: 26 July 1995 / Revised, accepted: 26 September 1995     

Surgical treatment of an aneurysm of the vein of Galen in a newborn with heart failure

Congestive heart failure is the usual complaint and cause of death in neonates with aneurysms of the great vein of Galen. Direct surgical obliteration of the feeding arteries cures the heart failure, but few such newborns survive the operation. The authors report a case of primary aneurysm of the great vein of Galen treated successfully by clipping the afferent arteries and reduction of the bulk of the aneurysm by bipolar coagulation. Five months after the operation the child's development is normal.     

Cerebral arteriovenous malformations in children: report on 62 cases

A series of 62 children with cerebral arteriovenous malformations admitted to our department in the course of 17 years (1975–1992) was reviewed in a retrospective study. In 54 cases hemorrhagic stroke was the first presenting symptom, followed by epilepsy in five cases. On admission 26 children presented with a neurological deficit, and 21 were admitted with a grade 3 status according to Botterell. Fifty-one malformations were supratentorial (41 hemispheric, 10 deep-seated) while 11 were infratentorial. According to Mori's criteria, 28 lesions were small, 19 medium, and 15 large. Fifty-two children were operated on, with total excision of the malformation achieved in 47 cases. In two children the malformation recurred. The evolution of neurological disorders has been studied with a mean follow-up of 8.5 years. Fifty patients had a satisfactory outcome on the Glasgow Outcome Scale. Four children died. These results were compared with those reported elsewhere in the pediatric literature.     

Limitations and technical considerations of endovascular treatment in neonates with high-flow arteriovenous shunts presenting with congestive heart failure: report of two cases

Case report We report two neonatal patients with high-flow intracranial or cervical arteriovenous (AV) shunts who presented with congestive heart failure and were successfully managed with endovascular treatment. One with vein of Galen malformation with body weight of only 2.0 kg is, to our knowledge, the smallest neonate successfully treated with endovascular treatment. The other neonate is, to our knowledge, the first vertebro-vertebral fistula patient presenting with heart failure and treated successfully with endovascular treatment.Conclusion Even in neonates, endovascular treatment is the procedure of choice for high-flow AV shunts presenting with progressive congestive heart failure resistant to medical management. We discuss limitations and technical considerations of endovascular treatments in small neonates with high-flow AV shunts and congestive heart failure.     

Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature

Objective Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI+S).Clinical presentation We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia.Conclusion A review of the literature reveals fewer than ten previous reports of familial CMI+S in the past 30 years. Although rare, the existence of familial cases of CMI+S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI+S.     

小脑脑桥角海绵状血管畸形三例并文献复习

目的 总结小脑脑桥角(CPA)区海绵状血管畸形(CMs)的临床表现、影像学特征及治疗特点.方法 2005年6月至2010年6月我科共收治CPA区CMs病例3例,进行报告.搜索MEDLINE上自1985年至2010年文献查到此类病例5例,进行文献复习.结果 文献复习的5例CMs临床表现为脑神经及小脑受累症状,但无脑干受压症状;影像见CMs均呈囊实性,与内听道及颅底硬膜无粘连;术中见CMs与周边结构有粘连,但可进行分离;手术治疗效果满意.本文报告的3例临床特征与之相符.结论 当CPA区见囊性病变与内听道及颅底硬膜无粘连时,应考虑到CMs的可能,显微外科手术切除多能获得良好的疗效.

Abstract：

Objective To investigate the clinical presentation, imaging features and treatment experience of cystic cavernous malformations ( CMs ) in the cerebellopontine angle ( CPA ) . Methods Three patients with the CMs of the CPA between 2005 and 2010 in our institute were summarised. Five patients from MEDLINE search of the English literature from 1985 to 2010 for CMs of the CPA were identified. We reviewed the characteristics of these 5 cases and our cases. Results Their presenting symptoms of 5 cases in the literature included headache, injury of cranial nerves in the CPA and cerebellar symptoms. But they did not show signs of brain stem compression. MRI and /or CT scans showed that the lesions in the eight cases of CMs all had solid and cystic components, and no adhesion with internal auditory canal and skull base dura mater was present in the CPA. Despite the fact that cystic CMs also involved cranial nerves, cerebellum, brainstem and arteries, they could be separated from these surrounding structures thanked to the presence of well - margined adhesion, which was contrary to solid CMs. We reported three cystic CMs of the CPA in our institute that was the same as the above. Conclusion Although cystic CMs of the CPA had no established imaging features, a diagnosis of CMs may be suspected when a cystic lesion is present in the CPA and does not involve internal acoustic meatus or dura matter of the skull base. Skillful microsurgical techniques will secure good outcomes for patients with cystic CMs in the CPA.     

Onyx胶治疗脑动静脉畸形相关动脉瘤二例及文献复习

目的 探讨Onyx胶治疗动静脉畸形(AVM)相关的供血动脉蒂动脉瘤中的应用价值.方法 结合文献复习,回顾性分析2例大脑后动脉远端AVM相关的大型供血动脉蒂动脉瘤病例资料.结果 根据病变血管构筑特征及临床特点,对1例病人采用Onyx胶结合弹簧圈的方法进行栓塞,而对另1例病人则采用单纯利用Onyx胶进行栓塞.结果 提示Onyx胶结合弹簧圈的方法能降低术中动脉瘤破裂的风险,并能达到致密栓塞.结论 Onyx胶结合弹簧圈的方法有助于治疗动静脉畸形(AVM)相关的大型供血动脉蒂动脉瘤.