血管内治疗儿童Galen静脉动脉瘤样畸形的初步观察

Primary observation of endovascular therapy for vein of Galen aneurysmal malformation in children

目的初步观察血管内治疗儿童Galen静脉动脉瘤样畸形(VGAM)的临床疗效。方法回顾性分析2005年1月至2021年11月上海交通大学医学院附属新华医院小儿神经外科采用血管内治疗的10例VGAM患儿(年龄≤3岁)的临床资料。单支供血动脉的VGAM予以弹簧圈联合Onyx胶栓塞治疗;对于瘘口较多、多支动脉供血的VGAM,给予分期栓塞治疗。术后3、6、12个月对患儿进行常规门诊随访。随访内容包括评估患儿的临床症状和丹佛发育筛查测验,复查头颅CT或MRI,行DSA检查评估VGAM的闭塞情况。将所有瘘口完全闭塞、无血流进入定义为完全栓塞。结果10例患儿术中DSA结果显示单支供血2例,多支供血8例;分别经1~3次血管内栓塞治疗,其中1次2例,2次3例,3次5例。术后所有患儿的VGAM均完全栓塞;均未出现颅内出血、感染、血栓形成等并发症。10例患儿术后的中位随访时间为34.5个月(2~116个月);复查DSA的时间为术后3~12个月。10例患儿术前存在脑积水,其中7例术后2个月内脑积水明显缓解,3例行脑室-腹腔分流术后脑积水缓解。至末次随访,10例患儿的临床症状均缓解;影像学检查显示VGAM的中位最大直径为0.3 cm(0~2.3 cm),均较前缩小(5例)或消失(5例),DSA显示瘘口均完全闭塞;丹佛发育筛查测验结果为正常8例,可疑2例。结论初步观察发现,血管内治疗可完全栓塞儿童VGAM,改善患儿的临床症状及生长发育情况,疗效较好。

Objective To observe the clinical effect of endovascular therapy for vein of Galen aneurysmal malformation(VGAM)in children.Methods The clinical data of 10 children with VGAM(aged≤3 years)treated with endovascular therapy in the Department of Pediatric Neurosurgery,Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from January 2005 to November 2021 were retrospectively analyzed.The VGAM of single supplying artery was treated with coil combined with Onyx glue embolization.For VGAM with multiple fistulas and multiple supplying arteries,staging embolization was performed.Routine outpatient follow-up was performed at 3,6,and 12 months after operation.Follow-up contents included assessment of clinical symptoms and Denver developmental screening test,head CT or MRI,and DSA to assess the occlusion situation of VGAM.Complete embolization was defined as complete occlusion of all fistulas without blood flow entry.Results DSA results of the 10 children showed that there were 2 cases with single blood supply and 8 cases with multiple blood supply.Endovascular embolization was performed for 1-3 times(1 time in 2 cases,2 times in 3 cases and 3 times in 5 cases).VGAM was completely embolized in all patients.There were no complications such as intracranial hemorrhage,infection and thrombosis.The median follow-up time of the 10 patients was 34.5 months(2-116 months).The time of DSA review was at 3-12 months post surgery.Ten children had hydrocephalus before surgery,among whom 7 had obvious hydrocephalus relief within 2 months after surgery,and 3 had hydrocephalus relief after ventriculoperitoneal shunt.By the last follow-up,the clinical symptoms of 10 children were relieved.Imaging examination showed that the median maximum diameter of VGAM was 0.3 cm(0-2.3 cm),which was reduced in size(5 cases)or disappeared(5 cases).DSA showed that the fistulas were completely occluded.The Denver developmental screening test results were normal in 8 cases and suspiciously abnormal in 2 cases.Conclusions Primary observation has shown that endovascular therapy can completely embolize VGAM,improve the clinical symptoms and growth and development of the children,which has a good effect.