Jain Narendra Kumar, Swaika Sweta, Thomas Bejoy, Kesavadas C, Kapilamoorthy T R
Department of Imaging Sciences & Interventional Radiology, Sree Chitra Tirunal Institute for Medical Science and Technology, Trivandrum, 695011, India,
Childs Nerv Syst. 2015 May;31(5):809-13. doi: 10.1007/s00381-014-2612-3. Epub 2015 Jan 9.
Vein of Galen malformations are rare congenital intracranial vascular malformations. The pathophysiological consequences usually manifest as high-output cardiac failure and neurological symptoms secondary to cerebral venous congestion and abnormal CSF flow. Management of these patients is complex. Indications and time to intervene are decided depending upon the age of child, clinical presentation, and multisystemic neonatal scores. Many of these children presenting early and who are not in cardiac failure are expectantly followed up. Spontaneous thrombosis of the vein of Galen malformation is a rare occurrence in these as such rare vascular malformations.
This review reports two cases of spontaneous thrombosis of the vein of Galen malformations with varying clinical as well as imaging outcomes. Possible pathophysiologic mechanisms are discussed with review of literature.
This report highlights the importance of vigilant clinical and imaging follow-up even after spontaneous thrombosis in the vein of Galen malformations.
大脑大静脉畸形是罕见的先天性颅内血管畸形。其病理生理后果通常表现为高输出量心力衰竭以及继发于脑静脉充血和脑脊液流动异常的神经症状。这些患者的管理很复杂。干预的指征和时机取决于患儿的年龄、临床表现和多系统新生儿评分。许多早期就诊且未出现心力衰竭的患儿会接受观察随访。大脑大静脉畸形自发血栓形成在这类罕见的血管畸形中较为罕见。
本综述报告了两例大脑大静脉畸形自发血栓形成的病例，其临床和影像学结果各异。通过文献回顾讨论了可能的病理生理机制。
本报告强调了即使在大脑大静脉畸形自发血栓形成后，进行密切临床和影像学随访的重要性。
