Johnston I H, Whittle I R, Besser M, Morgan M K
Neurosurgery. 1987 May;20(5):747-58. doi: 10.1227/00006123-198705000-00013.
The vein of Galen malformation is a midline arteriovenous fistula with aneurysmal dilatation of the vein of Galen. The clinical details of diagnosis and treatment in 13 patients with such lesions together with a review of 232 cases collected from the literature are presented in this report. There were 132 males, 77 females, and 36 cases in which the sex was not stated. Eighty patients presented as neonates, 82 were 1 to 12 months old, 39 were 1 to 5 years old, 22 were 6 to 20 years old, and 22 were over the age of 20. The most common presenting symptoms were congestive cardiac failure (110 cases), raised intracranial pressure secondary to hydrocephalus (94 cases), cranial bruit (57 cases), focal neurological deficit (37), seizures (26 cases), and hemorrhage (25 cases). The most characteristic vascular supply to the midline fistula involved multiple bilateral vessels, although bilateral posterior cerebral and unilateral posterior cerebral supply was relatively common. The overall figures for treatment and outcome showed that 91 patients (37.1%) were treated by direct operation and 29 patients (11.3%) were treated by other forms of operation, predominantly shunting or remote vessel ligation. Forty-six patients (18.8%) were treated by medical means (digoxin, diuretics, and ventilatory support). In 79 patients (22.2%), there was no treatment or no details of treatment were available. There was an overall series mortality of 55.6% (no details were available in 33 cases) and a 37.4% mortality for surgically treated cases. After operation, there was a 46.3% incidence of significant morbidity in surviving patients. Neonatal patients fared worst, with an overall mortality of 64 of 70 cases (91.4%) where details were available. The outcome was equally bad for surgically and conservatively treated cases. Operation in the 1- to 12-month age group was more successful, but still carried a mortality of 31.7%, with a significant morbidity in approximately half of the surviving patients. Over the age of 1 year, the surgically treated patients had a 25.6% mortality and a 42.3% major morbidity in survivors. Consideration is given to some of the ways in which these figures may be improved, in particular a staged approach during the neonatal period, with the use of selective embolization or occlusion of vessels to reduce the volume of the arteriovenous shunt until the patient is older and better able to tolerate major operation.
大脑大静脉畸形是一种中线动静脉瘘，伴有大脑大静脉的动脉瘤样扩张。本报告介绍了13例此类病变患者的诊断和治疗临床细节，并对从文献中收集的232例病例进行了综述。其中男性132例，女性77例，36例未提及性别。80例患者为新生儿，82例年龄在1至12个月，39例年龄在1至5岁，22例年龄在6至20岁，22例年龄超过20岁。最常见的临床表现为充血性心力衰竭（110例）、脑积水继发颅内压升高（94例）、颅部杂音（57例）、局灶性神经功能缺损（37例）、癫痫发作（26例）和出血（25例）。中线瘘最具特征性的血管供应涉及多支双侧血管，尽管双侧大脑后动脉供血和单侧大脑后动脉供血相对常见。治疗和预后的总体数据显示，91例患者（37.1%）接受了直接手术治疗，29例患者（11.3%）接受了其他形式的手术治疗，主要是分流术或远端血管结扎术。46例患者（18.8%）接受了药物治疗（地高辛、利尿剂和通气支持）。79例患者（22.2%）未接受治疗或没有治疗细节。总体系列死亡率为55.6%（33例无详细信息），手术治疗病例的死亡率为37.4%。术后，存活患者中有46.3%发生严重并发症。新生儿患者情况最差，在70例有详细信息的病例中，总体死亡率为64例（91.4%）。手术治疗和保守治疗病例的预后同样不佳。1至12个月龄组的手术更为成功，但死亡率仍为31.7%，约一半的存活患者有严重并发症。1岁以上患者，手术治疗后的死亡率为25.6%，存活者中有42.3%发生严重并发症。文中考虑了一些可能改善这些数据的方法，特别是在新生儿期采用分期方法，使用选择性栓塞或血管闭塞来减少动静脉分流的体积，直到患者年龄更大且更能耐受大手术。
