髓周动静脉瘘（PMAVF）一例及文献复习

目的:探讨髓周动静脉瘘(perimedullary arteriovenous fistulas,PMAVF)的临床、影像学特征以及治疗方案的选择,以提高对该病的诊断水平。方法:报道1例PMAVF患者的临床诊治经过,并结合相关文献对PMAVF的临床、影像学特征和治疗方法进行总结。结果:1例表现为进行性下肢麻木伴无力的患者,并逐渐出现大小便失禁,胸腰段MRI示第9胸椎水平附近椎管内髓周血管明显迂曲扩张。行脊髓血管造影检查示右侧胸9肋间动脉发出的脊髓前动脉在胸12椎体水平通过分支供应的异常引流静脉走形于脊柱右侧。予Onyx胶栓塞引流静脉及瘘口,术后一年肌力恢复至4级左右。结论:PMAVF早期临床表现多不具有特异性,后逐渐出现进展性脊髓病变表现。脊髓MRI对提示PMAVF诊断具有重要作用,脊髓血管CTA和ceMRA有助术前判断供血动脉和瘘口位置。脊髓血管造影仍是诊断该病的金标准。手术切除和血管内治疗是治疗该病的主要方法。

Perimedullary Arteriovenous Fistulas: Case Report and Literature Review

ABSTRACT Objective: To increase the awareness of perimedullary arteriovenous fistulas (PMAVF) by investigating the clinical and imaging characteristics as well as treatment options of the disease. Methods: The diagnosis and treatment of a patient with PMAVF were reported. A summary of the clinical and imaging characteristics as well as treatment modalities of the disease was made based on literatures. Results: One patient presented with progressive lower limb numbness accompanied by weakness, and gradually developed incontinence. MRI of thoracic and lumbar segments showed obvious tortuosity and dilatation of perimedullary artery near the ninth thoracic vertebrae level. The examination of spinal angiography(DSA) showed that the anterior spinal artery from the right thoracic 9 intercostal artery at the level of the thoracic 12 vertebra supplied an abnormal drainage venous mass at the right side of the spine. The patient underwent curative embolization of draining veins and fistula with Onyx?glue embolization. Muscle strengths of the lower limbs recovered to scale 4 one year post-operation. Conclusion: Most of the early clinical manifestations of PMAVF are not specific and then progressive myelopathy gradually appears. Spinal MRI plays an important role in the diagnosis of PMAVF. CT angiography and contrast-enhanced MR angiography (ceMRA) of the spinal cord are helpful to determine the location of fistula before operation. Digital subtraction angiography (DSA) is still the gold standard for the diagnosis of the disease. Surgical resection and endovascular therapy are two main modalities to treat this disease.