许多研究表明,脊柱异常与Klippel-Trenaunay综合征(KTS)有关。迄今为止,没有大型的连续系列检查KTS患者脊髓神经血管畸形的患病率和亚型分布。
报告KTS人群脊髓神经血管表现的频谱和发生率。
这是一项横断面研究。包括国际血管异常研究协会定义的具有明确KTS标准的连续患者,这些患者在我们机构接受了脊髓神经影像学检查。所有研究均由神经放射科医师和高级放射科住院医师评估是否存在发育性静脉异常。海绵状畸形(CM),和动静脉分流(AVS)。
共116例明确的KTS患者接受脊髓神经影像学检查。共发现19例(16.4%)神经血管异常23例,包括4例多发异常患者。其中包括5例脊髓CMs患者(4.3%),椎旁或硬膜外静脉畸形14例(12.1%),和4例AVS患者(3.4%)。在AVS中,3个是硬膜外动静脉瘘,其中1个可能在硬膜外静脉畸形中从头形成。一种是圆锥髓质动静脉畸形。
我们的研究队列包括116名KTS患者,显示了广泛的脊髓神经血管异常,患病率相对较高。KTS的潜在表型描述应包括脊髓神经血管异常的可能性。
A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS.
To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population.
This was a cross-sectional
study. Consecutive patients with definite KTS as defined by International Society for the
Study of Vascular Anomalies criteria who underwent spinal neuroimaging at our institution were included. All studies were evaluated by a staff neuroradiologist and a senior radiology resident for the presence of developmental venous anomalies, cavernous malformations (CMs), and arteriovenous shunts (AVS).
A total of 116 patients with definite KTS who underwent spinal neuroimaging were included. A total of 23 neurovascular anomalies were found in 19 patients (16.4%), including 4 patients with multiple anomalies. These included 5 patients with spinal cord CMs (4.3%), 14 patients with a paraspinal or epidural venous malformation (12.1%), and 4 patients with an AVS (3.4%). Of the AVS, 3 were epidural arteriovenous fistulas, 1 of which likely formed de novo in an epidural venous malformation. One was a conus medullaris arteriovenous malformation.
Our
study cohort of 116 KTS patients demonstrated a wide spectrum of spinal neurovascular anomalies with a relatively high prevalence. Potential phenotypic descriptions of KTS should include the possibility for spinal neurovascular anomalies.