Central retinal artery occlusion in association with fibromuscular dysplasia
A 14-year-old girl with a previous diagnosis of FMD was referred to the clinic of ophthalmology with the complaint of severe blurring in the right eye for 2 days. She had a diagnosis of FMD for 4 years. According to her medical history, obtained from the patient file belonging to the Department of Pediatrics, she had unusually high systemic blood pressure (180/110 mmHg), recorded at a regular visit. After realizing decreased flow in her left renal artery by Doppler ultrasonog-raphy, she received the angiography procedure. The final report of the procedure was that band-like narrowing in the left renal artery gives rise to thought fibromuscular dysplasia with no pathologic finding in vertebral arteries. Two months after establishing the diagnosis of FMD (which was four years ago), she received balloon angioplasty and started captopril to control her systemic blood pressure.\nOcular examination revealed afferent pupillary defect in the right eye. Best corrected visual acuity (BCVA) was 20/400 in the right eye and 20/20 in the left eye. The examination of anterior segment and ocular pressure were bilaterally within normal limits. Because of the cherry-red spot sign in the macula of the right eye, the patient immediately underwent stereoscopic color fundus photography and fluorescein fundus angiography. Fundus examination revealed that the left eye was normal () but that the right eye contained ischemic whitening with a cherry-red spot area (). Fluorescein fundus angiography demonstrated abnormal retinal arterial and choroidal dye filling in the early phase in the right eye. CRAO was diagnosed from the characteristic appearance ( and ).\nThere were no systemic risk factors such as diabetes mel-litus, hyperlipidemia, or smoking. Hematologic evaluation was also performed and all coagulation parameters were within normal limits. The patient then underwent carotid evaluation and echocardiographic study to determine the source of possible embolism. For carotid evaluation, carotid angiography was performed. Echocardiographic and carotid angiographic findings were within normal limits and there was no source of embolism such as valve or wall lesions. There was no tenderness in the face over the temporal artery. Erythrocyte sedimentation rate, C-reactive protein, and platelet count were all within normal limits.\nThe systemic blood pressure of the patient was 140/95 mmHg without antihypertensive drugs. She reported that she was taking her systemic antihypertensive drugs irregularly. At the latest visit (6 months ago), the patient’s right BCVA decreased to hand motion.