成人大脑大静脉畸形

Kerolus Mena G, Tan Lee A, Lopes Demetrius K

Department of Neurosurgery, Rush University Medical Center, 1725 W. Harrison St. Ste 855, Chicago, IL 60612, USA.

Radiol Case Rep. 2017 Apr 15;12(3):585-589. doi: 10.1016/j.radcr.2017.03.012. eCollection 2017 Sep.

Vein of Galen malformations (VoGMs) are rare vascular malformations resulting from persistent shunting of primitive choroidal vessels into the median prosencephalic vein of Markowski. VoGMs are associated with poor clinical outcome with a reported 76.7% mortality if left untreated. We present an exceedingly rare case of a giant, untreated VoGM measuring 7.8 × 5.5 × 7 cm in a 42-year-old man. The embryologic origin, classification, clinical manifestations, and treatment options of VoGMs are discussed with a review of pertinent literature.

大脑大静脉畸形（VoGMs）是罕见的血管畸形，由原始脉络丛血管持续分流至马尔科夫斯基中脑前静脉所致。VoGMs与不良临床结局相关，据报道，若不治疗，死亡率达76.7%。我们报告了一例极为罕见的病例，一名42岁男性患有巨大的未经治疗的VoGM，尺寸为7.8×5.5×7厘米。本文结合相关文献综述，讨论了VoGMs的胚胎学起源、分类、临床表现及治疗选择。