Absence of Deep and Basal Veins Is Common and Clinically Relevant in Sturge-Weber Syndrome.

IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY
Pediatric neurology Pub Date : 2025-10-01 Epub Date: 2025-07-22 DOI:10.1016/j.pediatrneurol.2025.07.009
Fatimah M Albazron, E Mark Haacke, Ajay Kumar, Sagar Buch, Yang Xuan, Jeong-Won Jeong, Aimee F Luat, Michael E Behen, Nore Gjolaj, Csaba Juhász
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引用次数: 0

Abstract

Background: Common intracranial vascular abnormalities in Sturge-Weber syndrome (SWS) include leptomeningeal venous malformations (LVMs) and enlarged deep veins. A few small studies have reported absent deep veins in some patients. We used susceptibility-weighted imaging (SWI), a magnetic resonance imaging (MRI) sequence sensitive to detecting small veins, to evaluate deep cerebral veins and the basal vein of Rosenthal (BVR) and assess the radiological correlates and clinical impact of their absence.

Methods: Fifty young subjects, including 30 patients with unilateral SWS and 20 healthy controls, underwent 3T brain MRI prospectively. The presence or absence of the internal cerebral vein (ICV), its two main tributaries, and the BVR were evaluated on SWI in all 50 subjects and correlated with other brain abnormalities and clinical symptoms in the SWS group.

Results: Although deep veins and the BVR were identified bilaterally in all control subjects, absent veins were observed in 70% of patients with SWS: in the SWS-affected hemisphere, absent ICV in 15 (50%), thalamostriate vein in 11 (37%), septal vein in seven (23%), and BVR in nine (30%) patients. Absent contralateral veins were also observed. Absent veins were associated with enlarged and collateral veins. Absent BVR and ICV were associated with extensive LVM, brain atrophy, and worse motor functions (P < 0.05); absent BVR was also associated with stroke-like episodes.

Conclusions: Absence of deep and/or basal cerebral veins is common in SWS and is associated with venous vascular anomalies, parenchymal damage, and motor impairment. Absent BVR may also increase the risk for stroke-like episodes.

深静脉和基底静脉缺失在斯特奇-韦伯综合征中很常见,且与临床相关。
背景:Sturge-Weber综合征(SWS)常见的颅内血管异常包括小脑膜静脉畸形(lvm)和深静脉扩张。一些小的研究报告了一些病人没有深静脉。我们使用敏感性加权成像(SWI),一种对检测小静脉敏感的磁共振成像(MRI)序列,来评估脑深静脉和罗森塔尔基底静脉(BVR),并评估它们缺失的放射学相关性和临床影响。方法:50例青年单侧SWS患者30例,健康对照20例,行3T脑MRI前瞻性检查。对所有50名受试者的脑内静脉(ICV)及其两条主要支流的存在或缺失进行SWI评估,并与SWS组的其他脑异常和临床症状相关。结果:尽管在所有对照组中均发现了深静脉和BVR,但70%的SWS患者均发现了静脉缺失:在SWS影响的半球中,15例(50%)患者没有ICV, 11例(37%)患者没有丘脑纹静脉,7例(23%)患者没有间隔静脉,9例(30%)患者没有BVR。对侧静脉也未见。缺失静脉伴扩张静脉及侧支静脉。BVR和ICV缺失与广泛的LVM、脑萎缩和运动功能恶化相关(P < 0.05);BVR缺失也与卒中样发作相关。结论:脑深部静脉和/或脑基底静脉缺失在SWS中很常见,并与静脉血管异常、实质损伤和运动障碍有关。BVR缺失也可能增加卒中样发作的风险。
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来源期刊
Pediatric neurology
Pediatric neurology 医学-临床神经学
CiteScore
4.80
自引率
2.60%
发文量
176
审稿时长
78 days
期刊介绍: Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.
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