Cullen Sean, Alvarez Hortensia, Rodesch Georges, Lasjaunias Pierre
Department of Diagnostic and Therapeutic Neuroradiology, Bicetre Hospital, Kremlin-Bicetre, France.
Childs Nerv Syst. 2006 Sep;22(9):1103-10. doi: 10.1007/s00381-006-0075-x. Epub 2006 Apr 4.
A minority of patients with spinal arteriovenous malformations (SAVM) are symptomatic as neonates or infants. We analyzed the clinical and anatomic factors associated with this early presentation, and reviewed our experience in treating patients with these lesions.
A retrospective review of clinical records and imaging studies was performed for patients with SAVM who presented at less than 2 years of age and who were examined by the diagnostic and interventional neuroradiology service at our institution. Clinical, imaging and treatment data had been entered prospectively into a department database.
Thirteen patients were identified that had SAVMs that were either diagnosed or that became symptomatic before 2 years of age (nine boys, four girls). These represented 13% of the SAVM seen during the same period of time. The mean age at presentation was 6.9+/-6.4 months. Of the 13 patients, 11 had neurologic symptoms attributable to the spinal lesion. The presentation was nonhemorrhagic in ten patients and associated with hemorrhage in three. Ten lesions were fistulas [spinal cord arteriovenous fistulas (SCAVFs)] (77%); two were of the nidus type of malformation. There were syndromic associations in eight patients: hereditary hemorrhagic telangectasia (HHT) in six patients, all but one of these lesions were intradural high-flow perimedullary SCAVFs (46% of overall SAVM and 56% of SCAVF), and spinal arteriovenous metameric syndrome in two patients. One patient had Hirschprung's anomaly. Eight patients underwent endovascular treatment alone, one had surgery and embolization, and four were not treated. In all patients undergoing embolization, n-butyl cyanoacrylate liquid adhesive was used. Of those patients who underwent endovascular treatment, the lesion was completely obliterated in 7 and a 90% reduction in nidal size was achieved in the remaining 2. There was one treatment complication (infection) which resolved with medical therapy. No procedure-related permanent morbidity or mortality was seen. Follow-up data were available (mean 31.4 months) in ten patients (nine treated). All treated patients were either stable or improved, with none showing further deterioration following treatment.
Factors associated with early presentation in neonates and infants with spinal arteriovenous shunts include the presence of high-flow, solitary fistulas and HHT. Specifically, the presence of SCAVF in a child less than 2 years of age is highly suggestive of HHT. Despite the aggressive nature of these lesions, many are amenable to endovascular treatment, and this is associated with a favorable posttreatment course in most cases.
少数脊髓动静脉畸形（SAVM）患者在新生儿期或婴儿期即出现症状。我们分析了与这种早期表现相关的临床和解剖学因素，并回顾了我们治疗这些病变患者的经验。
对在我院诊断与介入神经放射科接受检查、年龄小于2岁的SAVM患者的临床记录和影像学研究进行回顾性分析。临床、影像学和治疗数据已前瞻性地录入科室数据库。
共确定13例患者，其SAVM在2岁前被诊断或出现症状（9例男孩，4例女孩）。这些患者占同期所见SAVM的13%。就诊时的平均年龄为6.9±6.4个月。13例患者中，11例有因脊髓病变导致的神经症状。其中10例表现为非出血性，3例与出血相关。有10个病变为瘘（脊髓动静脉瘘[SCAVF]）（77%）；2个为畸形团型畸形。8例患者有综合征相关性：6例为遗传性出血性毛细血管扩张症（HHT），除1例病变外，其余均为硬膜内高流量髓周SCAVF（占所有SAVM的46%，占SCAVF的56%），2例为脊髓动静脉节段性综合征。1例患者有先天性巨结肠畸形。8例患者仅接受了血管内治疗，1例接受了手术和栓塞，4例未接受治疗。所有接受栓塞治疗的患者均使用了正丁基氰基丙烯酸酯液体粘合剂。在接受血管内治疗的患者中，7例病变完全闭塞，其余2例畸形团大小缩小了90%。有1例治疗并发症（感染），经药物治疗后缓解。未观察到与手术相关的永久性致残或死亡情况。10例患者（9例接受治疗）有随访数据（平均31.4个月）。所有接受治疗的患者病情稳定或好转，治疗后均未出现进一步恶化。
新生儿和婴儿期脊髓动静脉分流早期表现相关的因素包括高流量、孤立性瘘和HHT的存在。具体而言，2岁以下儿童存在SCAVF高度提示HHT。尽管这些病变具有侵袭性，但许多病变适合血管内治疗，且在大多数情况下，这与良好的治疗后病程相关。
