Mendoza-Ayús Santiago David, El Naamani Kareem, Atallah Elias, Sizdahkhani Saman, Kaul Anand, Mouchtouris Nikolaos, Tjoumakaris Stavropoula, Gooch Michael R, Rosenwasser Robert H, Jabbour Pascal
Department of Neurosurgery, Thomas Jefferson University and Jefferson Hospital for Neuroscience, Philadelphia , Pennsylvania , USA.
Oper Neurosurg. 2024 Dec 1;27(6):765-771. doi: 10.1227/ons.0000000000001195. Epub 2024 Apr 30.
Cognard type V fistula (CVF) is a rare type of dural arteriovenous fistula characterized by spinal perimedullary venous drainage. Owing to the lack of pathognomonic findings, misdiagnosis is common. Patients often undergo multiple spinal angiograms negative for spinal vascular malformations. Digital subtraction angiography is the gold standard diagnostic tool. The preferred treatment option is endovascular management with embolization through a transarterial, transvenous, or combined approach. Other options include open surgery, stereotactic radiosurgery, or a combination of both.
The patient from case # 1 presented with progressive weakness and hypoesthesia in the bilateral lower extremities, with urinary and bowel incontinence. The DSA identified a CVF fed by the meningohypophyseal trunk and a draining perimedullary vein. Embolization with 0.1 ccs of Onyx-18 was performed with complete fistula occlusion. The patient from case # 2 developed bilateral lower extremity weakness, diffuse numbness, and urinary incontinence. The DSA showed a CVF fed by tributaries from the ascending pharyngeal artery and posterior meningeal artery branches of the V3 segment, draining into a perimedullary vein. Embolization with 0.3 cc of Onyx-18 was performed with 100% occlusion of the fistula. A 1-year follow-up angiogram confirmed complete fistula occlusion. Both patients consented to the procedure.
Even if a patient only presents symptoms of myelopathy, CVF should be considered. Herein, we present 2 cases of CVF with direct drainage into the perimedullary veins which presented exclusively with myelopathy syndrome and describe treatment with trasarterial embolization with Onyx.
科尼亚德V型瘘(CVF)是一种罕见的硬脑膜动静脉瘘,其特征为脊髓髓周静脉引流。由于缺乏特异性表现,误诊很常见。患者常接受多次脊髓血管造影,结果均显示脊髓血管畸形阴性。数字减影血管造影是金标准诊断工具。首选的治疗方案是通过经动脉、经静脉或联合方法进行栓塞的血管内治疗。其他选择包括开放手术、立体定向放射外科手术或两者结合。
病例1的患者表现为双侧下肢进行性无力和感觉减退,伴有大小便失禁。数字减影血管造影显示由脑膜垂体干供血、髓周静脉引流的CVF。用0.1毫升的Onyx - 18进行栓塞,瘘管完全闭塞。病例2的患者出现双侧下肢无力、弥漫性麻木和尿失禁。数字减影血管造影显示由咽升动脉分支和V3段脑膜后动脉分支供血、流入髓周静脉的CVF。用0.3毫升的Onyx - 18进行栓塞,瘘管100%闭塞。1年的随访血管造影证实瘘管完全闭塞。两名患者均同意进行该手术。
即使患者仅表现出脊髓病症状,也应考虑CVF。在此,我们报告2例直接引流至髓周静脉的CVF,其仅表现为脊髓病综合征,并描述了用Onyx进行经动脉栓塞的治疗方法。