Division of Interventional Neuroradiology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
Department of Pediatrics, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
World Neurosurg. 2020 Jul;139:567-576. doi: 10.1016/j.wneu.2020.04.086. Epub 2020 Apr 25.
Spinal vascular malformations are rarely multiple: fewer than 50 observations have been documented so far, with a maximum of 4 coexisting lesions per patient, always restricted to a single vertebral region (e.g., cervical or thoracic).
We describe the case of a 61-year-old woman with Cowden syndrome with 15 spinal arteriovenous fistulas (AVFs) at the cervical, thoracic, and lumbar levels and an adrenal AVF. She was initially referred for reevaluation of an upper cervical spinal epidural spinal arteriovenous fistula diagnosed 6 years earlier. Her history included breast carcinoma, a malignant salivary gland tumor, and removal of multiple ovarian, thyroid, and gastric hamartomas. Computed tomography and magnetic resonance imaging confirmed the presence of a prominent cervical vascular lesion. Spinal digital subtraction angiography revealed the presence of 15 additional vascular anomalies.
This multiplicity of AVFs appears to result from a combination of various factors including local regional hemodynamic changes, growth factor-mediated alterations involving notably vascular endothelial growth factor pathways, and the prothrombotic state associated with abnormalities in blood vessel structure.
脊髓血管畸形很少是多发的：迄今为止，仅有不到 50 例被记录，每个患者最多有 4 个共存病变，始终局限于单一的脊柱区域（例如颈椎或胸椎）。
我们描述了一例患有考登综合征的 61 岁女性，其在颈椎、胸椎和腰椎水平有 15 个脊髓动静脉瘘（AVF）和一个肾上腺 AVF。她最初因 6 年前诊断的上颈椎硬脊膜脊髓动静脉瘘而被转来重新评估。她的病史包括乳腺癌、恶性唾液腺肿瘤以及多发性卵巢、甲状腺和胃错构瘤的切除。计算机断层扫描和磁共振成像证实存在明显的颈椎血管病变。脊髓数字减影血管造影显示存在另外 15 个血管异常。
这种多发性 AVF 似乎是多种因素共同作用的结果，包括局部区域血液动力学变化、生长因子介导的改变，特别是涉及血管内皮生长因子途径的改变，以及与血管结构异常相关的血栓形成倾向。
