Department of Neurology, Hospital Kuala Lumpur, Jalan Pahang, 50586, Kuala Lumpur, Malaysia.
Department of Radiology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia.
BMC Neurol. 2021 Sep 21;21(1):366. doi: 10.1186/s12883-021-02394-3.
Spinal AVF (SAVF), a potentially treatable cause of myelopathy, remains a challenging diagnosis. Its rarity and non-specific imaging findings often result in misdiagnosis despite a high index of clinical suspicion. The classically described high T2 signal in the spinal cord or prominent vascular flow voids in the intradural space were not infrequently missed on initial imaging, only to be picked up at follow-up imaging after progression of symptoms. Additionally, small sized fistulas(< 1 mm) and SAVF involving less frequent locations like the craniocervical junction in a patient presenting with paraplegia further complicates the diagnosis. On rare occasions, acute atypical presentation following a surgery adds to the conundrum. Definite diagnosis with spinal angiography, the gold-standard modality requires the expertise of highly skilled interventionists which may otherwise lead to false negative findings. We describe four SAVF patients with unconventional presentations, highlighting less described clinical findings.
First was a 50-year-old man presented with spastic paraparesis and was found to have an AVF at the cervical region arising from the vertebral artery. Second, a 45-year-old man with acute paraplegia post-operatively, initially treated for a transverse myelitis before lumbar region AVF was detected. Thirdly, a 27-year-old man presented with subacute lower thoracic myelopathy and deteriorated after corticosteroid treatment. The last patient, who initially appeared to have conus medullaris/cauda equina syndrome had a SAVF at the mid thoracic level. Presentation varied with some exhibiting acute deterioration mimicking other spinal cord pathology such as inflammatory disorders. All patients eventually underwent endovascular treatment with successful embolization of SDAVF. None of them exhibited further neurological deterioration after embolization.
Successful treatment of SAVF is possible provided the diagnosis is made early, allowing timely intervention. Certain clues may aid the diagnosis. Firstly, arteriovenous fistula can be located distant to the clinical localization of myelopathy resulting in the unexpected longitudinally extensive spinal cord signal change. This clinical-radiological discrepancy can be a useful clue in diagnosing SAVF. Secondly, an acute myelopathic presentation immediately post-surgery may be related to SAVF. Other SAVF feature of note includes progressive myelopathy mimicking immune-mediated myelitis among young adults below 30 years of age refractory to immune therapy.
脊髓动静脉瘘（SAVF）是一种潜在可治疗的脊髓病病因，但诊断仍然具有挑战性。尽管临床怀疑指数很高，但由于其罕见性和非特异性的影像学表现，常常导致误诊。最初的影像学检查常未能发现脊髓内高 T2 信号或硬脊膜内明显的血管流空现象，只有在症状进展后进行随访影像学检查时才能发现。此外，在出现截瘫的患者中，较小的瘘管（<1mm）和较少见部位（如颅颈交界区）的 SAVF 进一步增加了诊断的复杂性。在极少数情况下，手术后出现急性非典型表现也增加了诊断的难度。金标准的脊髓血管造影术可以明确诊断，但需要高度熟练的介入专家的专业知识，否则可能导致假阴性结果。我们描述了 4 例 SAVF 患者的非典型表现，强调了较少描述的临床发现。
第一位患者是一位 50 岁男性，表现为痉挛性截瘫，发现颈段动静脉瘘起源于椎动脉。第二位患者是一位 45 岁男性，术后出现急性截瘫，最初被误诊为横贯性脊髓炎，后来才发现腰段动静脉瘘。第三位患者是一位 27 岁男性，表现为亚急性胸下部脊髓病，皮质类固醇治疗后病情恶化。最后一位患者，最初表现为圆锥/马尾综合征，在中胸段发现 SAVF。表现各异，有些患者表现为急性恶化，类似于其他脊髓病变，如炎症性疾病。所有患者最终均接受了血管内治疗，成功栓塞了 SDAVF。栓塞后，他们均未出现进一步的神经功能恶化。
只要及早诊断，及时干预，SAVF 的治疗是可行的。某些线索可能有助于诊断。首先，动静脉瘘可位于脊髓病的临床定位以外，导致意外的纵向广泛脊髓信号改变。这种临床-影像学的差异可能是诊断 SAVF 的有用线索。其次，手术后出现急性脊髓病表现可能与 SAVF 有关。其他值得注意的 SAVF 特征包括在 30 岁以下的年轻成人中表现为进行性脊髓病，类似于免疫介导的脊髓炎，对免疫治疗无效。
