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[脊髓硬脊膜动静脉畸形]

[Spinal dural arteriovenous malformations].

作者信息

Reith Wolfgang, Udelhoven Anne, Kettner Michael

机构信息

Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, Kirrbergerstraße 1, 66421, Homburg, Deutschland.

出版信息

Radiologie (Heidelb). 2025 Sep 12. doi: 10.1007/s00117-025-01509-y.

Abstract

Vascular malformations of the spinal cord are a rare and complex clinical entity and can lead to severe morbidity with progressive spinal cord symptoms if not treated properly. In early stages the disease is characterized by slowly progressive, nonspecific symptoms, such as gait disturbance, paresthesia, diffuse sensory symptoms and radicular pain. In the late stages, bowel and bladder incontinence, erectile dysfunction, and urinary retention may develop. In recent decades the understanding and treatment of spinal vascular malformations have improved with the evolution of diagnostic tools and treatment modalities; however, it is still difficult to treat these cases because of the complexity of the pathology. All spinal dural arteriovenous fistulas (sdAVF) are most likely acquired; however, the exact etiology remains unknown. They typically present as a progressive myelopathy and have a poor prognosis if left untreated. Delayed diagnosis and intervention are associated with an increased risk of neurological deficits and a reduced likelihood of recovery. The general aim of treatment is disconnection of the arteriovenous shunt, which can be achieved by either open surgery or endovascular techniques. The surgical treatment of sdAVFs should be regarded as a combination of techniques with the shared goal of disconnecting the fistula.

摘要

脊髓血管畸形是一种罕见且复杂的临床病症,如果治疗不当,可导致严重的发病情况,并伴有进行性脊髓症状。在疾病早期,其特征为缓慢进展的非特异性症状,如步态障碍、感觉异常、弥漫性感觉症状和神经根性疼痛。在晚期,可能会出现大小便失禁、勃起功能障碍和尿潴留。近几十年来,随着诊断工具和治疗方式的发展,对脊髓血管畸形的认识和治疗有了改善;然而,由于病理情况复杂,治疗这些病例仍然困难。所有脊髓硬脊膜动静脉瘘(sdAVF)很可能是后天获得性的;然而,确切病因仍不清楚。它们通常表现为进行性脊髓病,如果不治疗,预后很差。延迟诊断和干预与神经功能缺损风险增加及恢复可能性降低相关。治疗的总体目标是切断动静脉分流,这可通过开放手术或血管内技术实现。sdAVF的手术治疗应被视为多种技术的组合,其共同目标是切断瘘管。

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