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    Can You Guess January's Mystery Condition?

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    Make your diagnosis in the comments, and look for the answer in next month’s Blink.

    Fig. 1: central corneal epithelial bullae. Fig. 2: slit-lamp photograph with fluorescein staining. Fig. 3: slit-lamp photograph with fluorescein staining. Fig. 4: anterior segment OCT.

     

    Last Month’s Blink

    Bietti Crystalline Dystrophy

    Written by Ramya Gnanaraj, MD, Sankara Nethralaya Medical Research Foundation, Tamil Nadu, Chennai, India. Photo by Dr. Gnanaraj.

    Fig. 1: Slit-lamp exam of the anterior segment. Fig 2A, 2B: Fundus photos. Fig 3: Spectral-domain OCT.

    A 29-year-old primigravid woman who was 32 weeks pregnant present­ed to the retina clinic with reports of bilateral progressive decrease in VA and night vision over the past two years, with an acute increase in visual symptoms during pregnancy. She had no other significant medical or ocular history.

    On examination, the patient’s BCVA was 20/70 in the right eye and 20/50 in the left. In both eyes, slit-lamp exam of the anterior segment showed subepithelial yellow-white crystalline deposits (Fig. 1, arrow) in the peripheral cornea; IOP, anterior chamber, lens, and pupils were normal; and confronta­tion testing demonstrated peripheral visual field constriction.

    The optic discs were normal, but the posterior pole of both retinas showed yellow-white crys­talline deposits (Fig. 2). Spectral-domain OCT demonstrated hyperreflective crystals in the reti­nal pigment epithelium (RPE) and early RPE ab­normalities (Fig. 3). Full-field electroretinogram showed rod-cone abnormalities in both eyes.

    Bietti crystalline dystrophy is a rare autosomal recessive condition characterized by crystalline deposits in the cornea and degeneration of the retina, with progressive loss of choriocapillaris and RPE atrophy. There is no medical or surgical treatment for this condition. She was referred for genetic counseling and the care of low vision specialists.

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