Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman.

PURPOSE: To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. OBSERVATIONS: Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. CONCLUSIONS AND IMPORTANCE: Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology.

Early retinoic acid deprivation in developing zebrafish results in microphthalmia.

Vitamin A deficiency causes impaired vision and blindness in millions of children around the world. Previous studies in zebrafish have demonstrated that retinoic acid (RA), the acid form of vitamin A, plays a vital role in early eye development. The objective of this study was to describe the effects of early RA deficiency by treating zebrafish with diethylaminobenzaldehyde (DEAB), a potent inhibitor of the enzyme retinaldehyde dehydrogenase (RALDH) that converts retinal to RA. Zebrafish embryos were treated for 2 h beginning at 9 h postfertilization. Gross morphology and retinal development were examined at regular intervals for 5 days after treatment. The optokinetic reflex (OKR) test, visual background adaptation (VBA) test, and the electroretinogram (ERG) were performed to assess visual function and behavior. Early treatment of zebrafish embryos with 100 µM DEAB (9 h) resulted in reduced eye size, and this microphthalmia persisted through larval development. Retinal histology revealed that DEAB eyes had significant developmental abnormalities but had relatively normal retinal lamination by 5.5 days postfertilization. However, the fish showed neither an OKR nor a VBA response. Further, the retina did not respond to light as measured by the ERG. We conclude that early deficiency of RA during eye development causes microphthalmia as well as other visual defects, and that timing of the RA deficiency is critical to the developmental outcome.

Pilot Study for OCT Guided Design and Fit of a Prosthetic Device for Treatment of Corneal Disease.

Purpose. To assess optical coherence tomography (OCT) for guiding design and fit of a prosthetic device for corneal disease. Methods. A prototype time domain OCT scanner was used to image the anterior segment of patients fitted with large diameter (18.5-20 mm) prosthetic devices for corneal disease. OCT images were processed and analyzed to characterize corneal diameter, corneal sagittal height, scleral sagittal height, scleral toricity, and alignment of device. Within-subject variance of OCT-measured parameters was evaluated. OCT-measured parameters were compared with device parameters for each eye fitted. OCT image correspondence with ocular alignment and clinical fit was assessed. Results. Six eyes in 5 patients were studied. OCT measurement of corneal diameter (coefficient of variation, CV = 0.76%), cornea sagittal height (CV = 2.06%), and scleral sagittal height (CV = 3.39%) is highly repeatable within each subject. OCT image-derived measurements reveal strong correlation between corneal sagittal height and device corneal height (r = 0.975) and modest correlation between scleral and on-eye device toricity (r = 0.581). Qualitative assessment of a fitted device on OCT montages reveals correspondence with slit lamp images and clinical assessment of fit. Conclusions. OCT imaging of the anterior segment is suitable for custom design and fit of large diameter (18.5-20 mm) prosthetic devices used in the treatment of corneal disease.