Van Name Jonathan
Internal Medicine, University of Florida College of Medicine, Gainesville, USA.
Cureus. 2025 Jan 28;17(1):e78127. doi: 10.7759/cureus.78127. eCollection 2025 Jan.
Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease of medium-sized arteries that causes abnormal cellular growth in arterial walls and most commonly affects young to middle-aged women (20-50 years of age). While FMD often involves the renal arteries, it can affect any arterial bed. FMD has a characteristic angiographic appearance of a "string of beads." However, rarely patients may present with an FMD-like clinical phenotype without characteristic angiographic FMD evidence. An FMD phenotype consists of a specific constellation of arterially-induced symptomatology (e.g., pulsatile tinnitus in cerebrovascular FMD) in the setting of acute arterial disturbances typically found in FMD (e.g., arterial dissection). This case report discusses a cerebrovascular FMD clinical phenotype manifesting as a distal spontaneous coronary artery dissection (SCAD) in a middle-aged man with chronic migraines, pulsatile tinnitus, and no prior cardiac history. In this case, a patient presented to the emergency room with elevated high-sensitivity troponin levels and anginal chest pain thought to be secondary to a non-ST-elevated myocardial infarction (NSTEMI). A left heart catheterization revealed non-obstructive atherosclerosis and a spontaneous coronary artery dissection in the distribution of the distal left anterior descending artery. To assess potentially involved arterial beds, further work-up consisting of a bilateral carotid and renal artery duplex demonstrated significant carotid arterial tortuosity, a finding consistent with cerebrovascular FMD. Per consultation with neurology and cardiovascular surgical services, a computed tomography angiography (CTA) aorta and CTA head and neck were obtained, which demonstrated subtle irregularities and tortuosities concerning for FMD but without characteristic findings. Further medical optimization was initiated for blood pressure and migraine symptomatology control. After completion of the diagnostic FMD work-up, the patient was discharged with monthly cardiovascular and neurological follow-up. This case report illustrates the important multifactorial practice of history-taking, laboratory evidence evaluation, and diagnostic imaging interpretation to ascertain the correct diagnosis in an atypical presentation of an uncommon disease spectrum.
纤维肌发育不良（FMD）是一种发生于中等大小动脉的非动脉粥样硬化、非炎症性血管疾病，可导致动脉壁细胞异常生长，最常影响年轻至中年女性（20 - 50岁）。虽然FMD常累及肾动脉，但它可影响任何动脉床。FMD在血管造影上具有特征性的“串珠样”表现。然而，很少有患者可能表现出类似FMD的临床表型，但没有特征性的血管造影FMD证据。FMD表型由一组特定的动脉性诱发症状（如脑血管FMD中的搏动性耳鸣）组成，通常出现在FMD中常见的急性动脉紊乱（如动脉夹层）的情况下。本病例报告讨论了一名患有慢性偏头痛、搏动性耳鸣且无既往心脏病史的中年男性，其脑血管FMD临床表型表现为远端自发性冠状动脉夹层（SCAD）。在该病例中，一名患者因高敏肌钙蛋白水平升高和心绞痛样胸痛就诊于急诊室，最初认为是继发于非ST段抬高型心肌梗死（NSTEMI）。左心导管检查显示非阻塞性动脉粥样硬化以及左前降支远端分布区的自发性冠状动脉夹层。为评估可能受累的动脉床，进一步的检查包括双侧颈动脉和肾动脉双功超声，结果显示颈动脉明显迂曲，这一发现与脑血管FMD一致。经与神经科和心血管外科会诊，进行了主动脉计算机断层血管造影（CTA）以及头颈部CTA检查，结果显示有一些细微的不规则和迂曲，怀疑与FMD有关，但无特征性表现。针对血压和偏头痛症状控制启动了进一步的药物优化治疗。在完成FMD诊断性检查后，患者出院，每月进行心血管和神经科随访。本病例报告说明了在罕见疾病谱的非典型表现中，通过多因素的病史采集、实验室证据评估和诊断性影像学解读来确定正确诊断的重要性。
