Central retinal artery occlusion in association with fibromuscular dysplasia

Abstract

A 14 year-old female, whose chief complaint was severe vision loss in the right eye for 2 days, presented to the Clinic of Ophthalmology of Fatih Sultan Mehmet Education and Research Hospital. The patient had been attending follow-up visits for 4 years, following a diagnosis of fibromuscular dysplasia by the Clinic of Pediatrics. The patient underwent a complete ophthalmo-logic, angiographic, hematologic, and systemic evaluation. Fundus fluorescein angiography was performed immediately, because of the cherry-red spot sign in the macula of the right eye. Fundus fluorescein angiography revealed evidence of marked stasis of the retinal arterial circulation in the right eye. Best corrected visual acuity was 20/400 in the right eye and 20/20 in the left eye.

Central Retinal Vein Occlusion Associated with Fibromuscular Dysplasia: A Case Report

“…FMD has been reported to correlate with rare instances of CRAO [ 3 , 4 ]. In at least two of the reported cases of CRAO in patients with FMD, renal involvement and hypertension were noted as contributory to the development of CRAO [ 4 ]. In such cases, management of hypertension is crucial to preventing pathology of retinal vasculature.…”

“…This hypertension may have compounded with the direct effects of FMD on retinal vasculature to contribute to the patient's presentation. FMD has been associated with vascular changes throughout the body including the retinal vascular beds [ 4 ]. The central retinal vein passes through a common adventitial sheath alongside the central retinal artery.…”

“…Involvement of the renal and carotid arteries can result in severe systemic hypertension therapy and carotid dissection, respectively [ 1 ]. While ocular manifestations of FMD are rare, central retinal artery occlusion (CRAO) has been reported [ 3 , 4 ]. We present a case of central retinal vein occlusion (CRVO) in a patient with FMD.…”

Paracentral Acute Middle Maculopathy Associated With Bilateral Optic Disk Swelling and Meningitis

“…Indeed, PAMM lesions have previously been noted in cases of both local and systemic inflammation such as birdshot chorioretinopathy 7 , postviral Purtscher-like retinopathy 5 , retinal vasculitis due to Behcet’s disease 8 and post-H1N1 vaccination 4 . Moreover, the internal carotid fibromuscular dysplasia found on CT angiography can predispose to retinal vessel occlusion 9 . However, to what extent this may contribute to the occurrence of PAMM has not been examined.…”

Central Retinal Artery Occlusion in a Young Female with Fibromuscular Dysplasia

“…CRAO as a complication of FMD is very rare. Only four cases of CRAO in FMD, (and one case of cilioretinal artery occlusion) have been reported in the literature [10,11,12,13,14] however none of them had embolus reorientation.…”