Department of Neurosurgery, Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA.
Department of Pennsylvania State College of Medicine, Hershey, Pennsylvania, USA.
World Neurosurg. 2020 Dec;144:231-237. doi: 10.1016/j.wneu.2020.09.036. Epub 2020 Sep 19.
A dilated epidural venous plexus (DEVP) is a rare cause of radiculopathy, back pain, cauda equina syndrome, and other neurological symptoms. This vascular mass can be secondary to inferior vena cava obstruction, portal hypertension, vascular agenesis, and hypercoagulable states. Although rare, DEVP should be considered in the differential diagnosis for patients who present with lumbar radiculopathy.
We present 2 cases involving patients with lumbar DEVP as well as a literature review of the role of hypercoagulability, vascular anatomy, and inferior vena cava thrombosis in the development of DEVP. The first patient had a history of recurrent deep vein thrombosis, systemic lupus erythematosus, and antiphospholipid syndrome. The diagnosis of DEVP was determined after intraoperative biopsy. The patient reported symptom resolution at her 6-month postoperative appointment. The second patient developed DEVP associated with Klippel-Trenaunay syndrome. She presented with back pain and leg weakness, and DEVP was diagnosed via magnetic resonance imaging. A neurosurgeon is currently following the patient. We believe this is the first case of Klippel-Trenaunay syndrome associated with DEVP.
If a patient presents with an enhancing epidural lesion on magnetic resonance imaging and neurological symptoms, DEVP should be considered in the differential diagnosis. Additionally, a search for inferior vena cava thrombosis should be performed as well as risk factors for venous hypertension and hypercoagulable states.
硬膜外静脉丛扩张(DEVP)是神经根病、背痛、马尾综合征和其他神经症状的罕见原因。这种血管团块可能继发于下腔静脉阻塞、门静脉高压、血管发育不良和高凝状态。尽管罕见,但对于出现腰椎神经根病的患者,应考虑 DEVP 作为鉴别诊断。
我们介绍了 2 例涉及腰椎 DEVP 的病例,并对高凝状态、血管解剖和下腔静脉血栓形成在 DEVP 发展中的作用进行了文献复习。第一例患者有复发性深静脉血栓形成、系统性红斑狼疮和抗磷脂综合征病史。DEVP 的诊断是在术中活检后确定的。患者在术后 6 个月的随访中报告症状缓解。第二例患者发生与 Klippel-Trenaunay 综合征相关的 DEVP。她表现为背痛和下肢无力,通过磁共振成像诊断为 DEVP。神经外科医生正在对患者进行随访。我们认为这是首例与 DEVP 相关的 Klippel-Trenaunay 综合征。
如果患者磁共振成像上出现增强的硬膜外病变和神经症状,应考虑 DEVP 作为鉴别诊断。此外,应寻找下腔静脉血栓形成以及静脉高压和高凝状态的危险因素。