Faculty of Medicine, Universidad de los Andes, Cra 1 N° 18A - 12, Bogotá, Colombia.
Department of Radiology, Lahey Hospital and Medical Center, 41 Mall Road, Burlington, MA, USA.
Childs Nerv Syst. 2021 Oct;37(10):3271-3276. doi: 10.1007/s00381-021-05072-4. Epub 2021 Feb 17.
The vein of Galen malformation is caused by an abnormal shunting between choroidal arteries and the median prosencephalic vein during embryological development, leading to increased blood flow to the deep cerebral veins, intracranial damage, and systemic repercussions. Idiopathic spontaneous thrombosis of a vein of Galen malformation is rare, and its association with acute sinusitis has not been reported in the literature. We present the case of a girl with a postnatal diagnosis of a vein of Galen malformation at the age of 16 months, with secondary pulmonary hypertension that was adequately controlled with spironolactone. At 3 years old, while expecting elective endovascular treatment, the patient developed spontaneous thrombosis of the vein of Galen malformation, concomitant to an acute sinusitis episode, with complete resolution of the vascular malformation and secondary pulmonary hypertension. The patient continued with normal neurological development over a 5-year follow-up. We discuss the main pathophysiologic mechanisms that can explain spontaneous thrombosis of VOGMs and the patient's outcome. Awareness of different mechanisms that can lead to spontaneous thrombosis can help in the decision-making process and prompt targeted approaches to individual patients with a vein of Galen malformation.
Galen 静脉畸形是由于胚胎发育过程中脉络膜动脉和正中前脑静脉之间异常分流引起的,导致深部脑静脉血流增加、颅内损伤和全身反应。Galen 静脉畸形自发性血栓形成较为罕见,且其与急性鼻窦炎的关联在文献中尚未报道。我们报告了一例 16 个月大的婴儿,出生后诊断为 Galen 静脉畸形,继发肺动脉高压,用螺内酯充分控制。3 岁时,在期待选择性血管内治疗时,患者并发急性鼻窦炎发作,出现 Galen 静脉畸形自发性血栓形成,血管畸形和继发性肺动脉高压完全缓解。患者在 5 年的随访中继续正常神经发育。我们讨论了可以解释 VOGM 自发性血栓形成的主要病理生理机制和患者的结局。了解不同的机制可以导致自发性血栓形成,有助于决策过程,并为 Galen 静脉畸形患者提供有针对性的治疗方法。