Retinal Manifestations in Fibromuscular Dysplasia

Abstract

Fibromuscular dysplasia of the arteries (FMD) is a segmental angiopathy which may produce obstruction of the carotid, cerebral, renal, mesenteric, coronary or iliac arteries. Except for lesions related to arterial hypertension, retinal manifestations have not yet been reported. This paper describes the case of a 10-year-old boy with progressive deafness, a history of an unexplained stroke and progressive occlusions of the retinal arterioles in the fundus periphery. This resulted in retinal neovascularization and recurrent retinal and vitreous hemorrhages. Despite repeated photo- and cryocoagulation the eyes progressed to a tractional retinal detachment which was successfully treated by vitrectomy and scleral buckling. The diagnosis of FMD was made on the basis of a histopathological examination of a temporal artery biopsy. The child also presented an asymptomatic but severe aneurysmal dilatation of the aorta and CT scan and MRI showed dilated cerebral arteries. The father of our patient had died at the age of 27 years either from myocardial infarction or rupture of a dissecting aortic aneurysm. He was highly myopic and had lost one eye from retinal detachment. The younger brother of our patient also presents aneurysmal dilatation of the aorta and tortuous cerebral vessels. Ocular examination is still normal. The findings in this family are compatible with an autosomal dominant inheritance with variable expression.

Ocular ischemic manifestations in fibromuscular dysplasia

“…This chronic ischemic retinopathy includes arteriovenous anastomoses and neovascularization, which can produce complications such as retinal hemorrhages, massive vitreous hemorrhage or tractional retinal detachment. 19,20 Chronic retinal ischemia also produces thinning of the inner retinal layers, 20 as occurred in our patient 1 (Figure 2(c)). The optic nerve can also be affected by the lower blood supply, producing acute or chronic ischemic optic neuropathy, as happened in our three patients.…”

Central retinal artery occlusion in association with fibromuscular dysplasia

“…FMD of the retinal artery is rare in literature 5,13,14. We present a case of central artery occlusion in a girl who already had the diagnosis of FMD.…”

“…Hypertension, transient ischemic attack, headache, syncope, and subarachnoid hemorrhage are common clinical manifestations, whereas ocular manifestations are rarely mentioned 1,5. Clinical manifestations correlate with the affected site.…”

Retinal Vascular Occlusions and Macular Thinning in Fibromuscular Dysplasia

“…[6][7][8] In those cases, extrarenal vascular involvement was the primary cause of retinal pathology instead of being downstream sequelae of renovascular involvement. These cases represent acute involvement of the retinal vessels.…”