PDF(Pubmed)
Abstract：
The vein of Galen aneurysmal malformation (VGAM) is a rare congenital arteriovenous fistula of the embryonic median prosencephalic vein of Markowski, resulting in its pathological dilation. If left untreated, it can lead to multiple severe complications in the neonatal period, among which obstructive hydrocephalus. We present a case report of a six-year-old male patient with severe status epilepticus and a clinical history of VGAM and obstructive hydrocephalus, diagnosed via an MRI and an MR-angiography. The hydrocephalus was treated via a ventriculostomy at the age of six months, while the VGAM underwent a partial transarterial endovascular embolization when the patient was four years old. The procedures were successful, and there were no significant post-operative complications observed. The epileptic seizures began at a later point and were successfully medicated with valproate. However, they resumed due to a lowering of the medication dosage by the patient\'s parents. The patient was given a new valproic acid regimen with an appropriate dosage, and his parents reported no further seizures. This case report emphasizes the use of appropriate prenatal and neonatal diagnostic methods for VGAM and explores the nature of the multi-procedural therapy approach towards the pathology and its complications in relation to a possibly idiopathic co-pathology, namely epilepsy.
摘要：
Galen动脉瘤畸形(VGAM)静脉是Markowski胚胎前脑正中静脉的罕见先天性动静脉瘘,导致其病理性扩张。如果不及时治疗，在新生儿期会导致多种严重的并发症,其中包括梗阻性脑积水。我们提供了一例6岁男性患者的病例报告，该患者患有严重的癫痫持续状态，并有VGAM和阻塞性脑积水的临床病史。通过MRI和MR血管造影诊断。脑积水在六个月大时通过脑室造口术治疗，而VGAM在患者4岁时接受了部分经动脉血管内栓塞。程序成功了,并且没有观察到明显的术后并发症。癫痫发作在稍后的时间点开始，并成功地用丙戊酸盐治疗。然而,由于患者父母降低了药物剂量，他们恢复了。患者给予适当剂量的新丙戊酸方案，他的父母报告没有进一步的癫痫发作。该病例报告强调对VGAM使用适当的产前和新生儿诊断方法，并探讨了与可能的特发性共病相关的病理及其并发症的多程序治疗方法的性质。即癫痫。
