TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Fibromuscular Dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease. It is a rare condition that occurs more frequently in middle-aged Caucasian women. Its prevalence is not well known. We present a case of FMD in a man with stroke secondary to dissection of the vertebral artery. Increased reporting of FMD cases in recent studies raises concern about under-diagnosis of the condition. CASE PRESENTATION: A 61-year-old Caucasian male with a past medical history of hypertension presented with dysarthria, extreme disequilibrium, and left-sided headache. The patient had normal strength and sensation in all four extremities and negative cerebellar testing on physical exam. He also had a productive cough upon presentation. Computed tomography (CT) head revealed a 3 cm acute non-hemorrhagic left cerebellar infarction. Computed tomography angiography (CTA) Head and Neck showed evidence of vertebral artery dissection on the left side, including the origin of the left posterior inferior cerebellar artery (PICA). The patient had a trial of cerebral angiogram for the vertebral artery dissection, which showed incidental evidence of FMD in the femoral arteries bilaterally. The patient also had vasospasm with the angiography that resolved post-injection of five milligrams of Verapamil intraarterial, and the procedure was discontinued. He was discharged on aspirin and statin, and anticoagulation starting in two weeks. DISCUSSION: There is no clear consensus on the prevalence of FMD in the general population [1]. However, many studies have assessed the prevalence in different patient populations to be around 3%-4% [2]. The United States Registry of FMD revealed the incidence of dissection of the carotid artery in the FMD patient population to be about 75% [1]. The incidence of dissection of the vertebral artery in patients diagnosed with FMD is much lower, about 17% [1]. The most common manifestations of cervical artery dissection (CeAD) are cerebral infarction and transient ischemic attack (TIA). In FMD patients with ischemic stroke or TIA secondary to CeAD, general guidelines for thrombolysis and thrombectomy should be followed. Most recent guidelines recommend treatment with either an anticoagulant or an antiplatelet agent for at least 3–6 months [3]. Endovascular or surgical repairs are mainly reserved for patients with recurrent ischemia despite initial antithrombotic therapy. CONCLUSIONS: FMD is an intriguing vascular disorder with unclear underlying etiology, challenging manifestations, and complex clinical diagnosis. Increased reporting of FMD cases in recent literature raises concern for underdiagnosis. Furthermore, in our case, FMD was incidentally discovered during a diagnostic procedure to evaluate the vertebral artery dissection. Therefore, we recommend further screening of any patient who presents with cephalo-cervical dissection or aneurysm for underlying FMD. REFERENCE #1: Olin, J.W., Froehlich, J., Gu, X., et al. 2012, "The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients", Circulation, vol. 125, no. 25, pp. 3182-3190. REFERENCE #2: Hendricks, N.J., Matsumoto, A.H., Angle, J.F., et al. 2014, "Is fibromuscular dysplasia underdiagnosed? A comparison of the prevalence of FMD seen in CORAL trial participants versus a single institution population of renal donor candidates", Vascular medicine (London, England), vol. 19, no. 5, pp. 363-367. REFERENCE #3: Powers, W.J., Rabinstein, A.A., Ackerson, et al. "2018 Guidelines for the Early Management of Patients With Acute Ischemic Stroke: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association", Stroke, vol. 49, no. 3, pp. e46-e110. DISCLOSURES: No relevant relationships by Elizabeth Mause, source=Web Response No relevant relationships by Mohammad Selim, source=Web Response
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