Park Sangil, Choi Hye-Yeon
Department of Neurology, Kyung Hee University College of Medicine, Kyung Hee University Hospital at Gangdong, Seoul, Republic of Korea.
Medicine (Baltimore). 2020 Jul 10;99(28):e21108. doi: 10.1097/MD.0000000000021108.
The natural history of fibromuscular dysplasia (FMD) is unclear. Furthermore, the correlation between radiologic findings and clinical significance has not been documented. Previously, the development of new vascular symptoms was reported in a small number of patients, but some of these symptoms were from other vascular causes. New arterial lesions were rarely observed during follow-up in the previous reports.
A 40-year-old man was admitted due to dysarthria and left-sided weakness. He had developed flank pain due to bilateral renal infarction about 10 months earlier. He had no known risk factors for atherosclerosis. Initial neurological examination revealed a mild weakness and central facial palsy on the left side.
Diffusion-weighted magnetic resonance imaging revealed a small acute infarction in the right insular cortex. Magnetic resonance angiography and digital subtraction angiography showed a severe stenosis with post-dilatation in the right internal carotid artery (ICA). There was a focal ectatic lesion in the left ICA. On the previous abdominal computed tomography angiography (CTA), there were arterial lesions suggestive of dissection in the bilateral renal arteries and a rod-shaped ectasia in the left common iliac artery (CIA). The pathological diagnosis was mixed-type FMD involving the intima and media.
The patient was prescribed antiplatelet agents for prevention of further ischemic events and followed up regularly.
Seven years after the initial renal infarction, the patient developed abdominal pain radiating to the back. Abdominal CTA revealed that an aortic dissection had developed in the infrarenal aorta, which was shown as normal previously. The ectasia in the left CIA and left ICA showed no interval changes during follow-up.
We present a patient who developed spontaneous symptomatic dissection of the bilateral renal arteries, right ICA, and abdominal aorta during 7 years of follow-up, which were caused by pathologically confirmed FMD. Besides the symptomatic multifocal dissection, the patient showed an asymptomatic multifocal ectasia on cerebral and abdominal angiographies that had not changed over 7 years.
纤维肌发育不良(FMD)的自然病史尚不清楚。此外,放射学表现与临床意义之间的相关性尚未得到证实。此前,少数患者报告出现了新的血管症状,但其中一些症状是由其他血管原因引起的。在既往报告的随访中,很少观察到新的动脉病变。
一名40岁男性因构音障碍和左侧肢体无力入院。约10个月前,他因双侧肾梗死出现侧腹痛。他没有已知的动脉粥样硬化危险因素。初始神经系统检查显示左侧轻度无力和中枢性面瘫。
弥散加权磁共振成像显示右侧岛叶皮质有一个小的急性梗死灶。磁共振血管造影和数字减影血管造影显示右侧颈内动脉(ICA)严重狭窄并扩张后改变。左侧ICA有一个局灶性扩张性病变。在之前的腹部计算机断层血管造影(CTA)中,双侧肾动脉有提示夹层的动脉病变,左侧髂总动脉(CIA)有棒状扩张。病理诊断为累及内膜和中膜的混合型FMD。
患者服用抗血小板药物以预防进一步的缺血事件,并定期随访。
首次肾梗死7年后,患者出现放射至背部的腹痛。腹部CTA显示肾下腹主动脉发生了主动脉夹层,之前显示正常。左侧CIA和左侧ICA的扩张在随访期间无变化。
我们报告了一名患者,在7年的随访中发生了双侧肾动脉、右侧ICA和腹主动脉的自发性症状性夹层,病理证实为FMD所致。除了有症状的多灶性夹层外,患者在脑和腹部血管造影上还显示有无症状的多灶性扩张,7年未变。