RETINAL VASCULAR OCCLUSIONS AND MACULAR THINNING IN FIBROMUSCULAR DYSPLASIA Courtney L. Kraus, MD, Arsham Sheybani, MD, Ramin Schadlu, MD, Rajendra S. Apte, MD, PhD Purpose: To report retinal findings in a patient with fibromuscular dysplasia. Methods: A retrospective case report. Results: A 36-year-old female patient with fibromuscular dysplasia and a history of cerebrovascular events presented with painless vision loss in the left eye. Ophthalmologic evaluation demonstrated retinal vascular occlusive disease and secondary macular thinning. Conclusion: This case demonstrates that fibromuscular dysplasia can affect the retinal vasculature and lead to macular ischemia and vision loss. RETINAL CASES & BRIEF REPORTS 4:370–372, 2010 From the Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St. Louis, Missouri. F ibromuscular dysplasia is a systemic vascular disease attributed to an idiopathic, nonatheroscler- otic, and noninflammatory cause. 1 The musculature of arterial walls is involved in a segmental fashion. The etiology is unknown and diagnosis often follows demonstration of the characteristic angiographic findings of a ‘‘string-of-beads’’ pattern. Renovascular hypertension, transient ischemic attacks, and subarachnoid hemorrhage are all common clinical findings. 2 This is the first report of retinal thinning secondary to retinal arteriolar involvement with fibromuscular dysplasia (FMD). Case Reports A 36-year-old woman with a history of FMD and poorly controlled secondary hypertension presented with progressive, painless visual loss in the left eye. The patient had a history of diet- controlled diabetes mellitus and two strokes. Vascular surgery did not result in the desired improvement in systemic hypertension and renal function. On the day of the ophthalmologic examination, the patient had visual field loss. Visual acuity was 20/30 in the right eye and 20/200 in the left eye. There was no relative afferent pupillary defect. Confrontation testing confirmed the inferior field loss described by the patient. Ophthalmoscopic examination of the left eye demonstrated marked arteriolar constriction and corkscrew- shaped, tortuous retinal vasculature (Figure 1, A and B). Other findings included focal retinal arterial and venous obstructions and the formation of arteriovenous anastomoses (Figure 1C). Optical coherence tomography confirmed marked thinning of the inner third of the retina, primarily in the temporal aspect of the macula lutea (Figure ID). Ophthalmoscopic examination of her right eye did not show evidence of occlusive disease (Figure IE), and optical coherence tomography was normal. Discussion Fibromuscular dysplasia was initially described in 1938 by Leadbetter and Burkland 1 as a disease of the renal arteries. It is a nonatherosclerotic and non- inflammatory vascular disease classically associated with renovascular hypertension. 3 Extrarenal involve- ment of both small and medium arteries has been extensively documented, confirming FMD is a sys- temic angiopathy. Mettinger 4 reported the frequency of involved sites such as the renal artery, 58%; the cervicoencephalic, 35%; intraabdominal, 3%; iliac arteries, 2.5%; and other sites, 1.5%. Hypertension, stroke, headache, and syncope are common clinical features of FMD. In the ophthalmologic literature, hypertensive retino- pathy is the most frequently cited finding as sociated with FMD and one that improves with successful treat- ment of the renovascular hypertension. 5,6 Although The authors have no conflicts of interest to disclose. Reprint requests: Rajendra S. Apte, MD, PhD, 660 South Euclid Avenue, Box 8096. St. Louis, MO 63110; e-mail: apte@vision. wustl.edu 370