Kebede Tesfaye, Hawaz Yohannes, Assefa Getachew
Ethiop Med J. 2013 Jan;51(1):77-83.
Arteriovenous malformations are uncommonly occurring congenital conditions and great vein of Galen malformations are rare form of arteriovenous malformations. Aneurysmal dilatation of the median prosencephalic vein of Markowski is responsible for the clinical presentation of patients and most children manifest the disease during infancy. We present three cases of great vein of Galen malformations, presenting to Tikur Anbessa hospital, one with thrombosis and communicating hydrocephalus; the other two with obstructive hydrocephalus and no thrombosis. All patients have markedly dilated intracranial veins with vascular enhancement pattern in venous phase of brain CT scan. Physicians should be aware of different radiological presentations of vein of Galen malformations and different clinical presentation of these cases. Very rarely the vein of Galen malformation can have thromboses and obliteration of itself The clinicians should also know that CT scan and MRI are the corner stones for the diagnosis of vein of Galen malformations but more fetuses are diagnosed with VGM in the uterus by ultrasound during ANC.
动静脉畸形是先天性疾病,较少见,大脑大静脉畸形是动静脉畸形的一种罕见形式。马尔科夫斯基中脑前静脉的动脉瘤样扩张是患者临床表现的原因,大多数儿童在婴儿期发病。我们报告了三例在蒂库尔·安贝萨医院就诊的大脑大静脉畸形病例,其中一例伴有血栓形成和交通性脑积水;另外两例伴有梗阻性脑积水且无血栓形成。所有患者在脑部CT扫描静脉期均有颅内静脉明显扩张及血管强化表现。医生应了解大脑大静脉畸形的不同影像学表现以及这些病例的不同临床表现。大脑大静脉畸形极少会发生血栓形成并自行闭塞。临床医生还应知道,CT扫描和MRI是诊断大脑大静脉畸形的基石,但在产前检查期间,更多的胎儿是通过超声在子宫内被诊断为大脑大静脉畸形。