Cullen S, Krings T, Ozanne A, Alvarez H, Rodesch G, Lasjaunias Pierre
Department of Radiology and Neurosurgery, Brigham and Women's Hospital, Francis Street, Boston, MA 02115, USA.
Neuroimaging Clin N Am. 2007 May;17(2):207-21. doi: 10.1016/j.nic.2007.02.004.
Spinal arteriovenous shunts (SAVSs) are rarely diagnosed in infants and children, but they are important clinically because morbidity can be significant. Although these lesions do not form a distinct pathologic group separate from the SAVSs seen in older patients, experience with these malformations in the pediatric population has led to the identification of several important features that are characteristic of this group of SAVSs. Association with genetic abnormalities, heritable (hereditary hemorrhagic telangiectasia) and nonheritable somatic (spinal arteriovenous metameric syndrome or Cobb syndrome), is relatively common and likely underrecognized. Male predominance is more pronounced than in the adult population. Hemorrhagic presentation is more frequent than in adults, except in extremely young children. The natural history seems to be better than previously thought, with early rehemorrhage uncommon. Despite early presentation and severe symptoms, these lesions are frequently amenable to endovascular treatment, often with anatomic cure achieved and with improvement or stabilization of symptoms after partial targeted treatment.
脊髓动静脉分流（SAVS）在婴幼儿中很少被诊断出来，但临床上却很重要，因为其发病率可能很高。尽管这些病变与老年患者中所见的SAVS并没有形成一个独立的病理组，但儿科人群中这些畸形的治疗经验已导致识别出该组SAVS的几个重要特征。与遗传异常相关，包括遗传性（遗传性出血性毛细血管扩张症）和非遗传性体细胞性（脊髓动静脉节段性综合征或科布综合征），相对常见且可能未得到充分认识。男性优势比成人人群更为明显。除了极小的儿童外，出血表现比成人更频繁。其自然病史似乎比以前认为的要好，早期再出血并不常见。尽管早期出现且症状严重，但这些病变通常适合进行血管内治疗，通常能实现解剖学治愈，部分靶向治疗后症状会有所改善或稳定。
