Fujimura Miki, Kimura Naoto, Ezura Masayuki, Niizuma Kuniyasu, Uenohara Hiroshi, Tominaga Teiji
Department of Neurosurgery, Tohoku University Graduate School of Medicine; and.
J Neurosurg Pediatr. 2014 Jun;13(6):647-9. doi: 10.3171/2014.3.PEDS13610. Epub 2014 Apr 18.
The development of a de novo arteriovenous malformation (AVM) in patients with moyamoya disease is extremely rare. A 14-year-old girl developed an AVM in the right occipital lobe during the 4-year postoperative period following successful bilateral revascularization surgeries. She suffered a transient ischemic attack with hemodynamic compromise of the bilateral hemispheres at the age of 10 years. Results of an initial examination by 1.5-T MRI and MR angiography satisfied the diagnostic criteria of moyamoya disease but failed to detect any vascular malformation. Bilateral direct and indirect revascularization surgeries in the anterior circulation relieved her symptoms, and she underwent MRI and MR angiography follow-up every year after surgery. Serial T2-weighted MRI revealed the gradual appearance of flow voids in the right occipital lobe during the follow-up period. Magnetic resonance angiography ultimately indicated the development of an AVM 4 years after these surgeries when catheter angiography confirmed the diagnosis of an AVM in the right occipital lobe. The AVM remained asymptomatic, and the patient remained free of cerebrovascular events during the time she was observed by the authors. Acquired AVM in moyamoya disease is extremely rare, with only 3 pediatric cases including the present case being reported in the literature. The development of a de novo AVM in a postoperative patient with moyamoya disease appears to be unique, and this case may provide insight into the dynamic pathology of AVMs.
烟雾病患者新发动静脉畸形（AVM）极为罕见。一名14岁女孩在双侧血运重建手术成功后的4年随访期内，于右侧枕叶出现了一个AVM。她在10岁时曾因双侧半球血流动力学受损而发生短暂性脑缺血发作。初次1.5-T MRI及磁共振血管造影检查结果符合烟雾病诊断标准，但未检测到任何血管畸形。前循环的双侧直接和间接血运重建手术缓解了她的症状，术后她每年都接受MRI及磁共振血管造影随访。连续的T2加权MRI显示，随访期间右侧枕叶逐渐出现流空信号。磁共振血管造影最终提示，这些手术4年后出现了AVM，此时导管血管造影证实右侧枕叶存在AVM。该AVM一直无症状，在作者观察期间患者未发生脑血管事件。烟雾病患者获得性AVM极为罕见，文献中仅报道了包括本病例在内的3例儿科病例。烟雾病术后患者新发AVM的情况似乎很独特，该病例可能有助于深入了解AVM的动态病理学。
