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CRB1-Related Maculopathy With Cystoid Macular Edema

Yulia Wolfson, MD1; Carolyn D. Applegate, MGC2; Rupert W. Strauss, MD1,3; Ian C. Han, MD1; Hendrik P. Scholl, MD1
[+] Author Affiliations
1Department of Ophthalmology, Johns Hopkins University, Baltimore, Maryland
2Institute of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland
3Department of Ophthalmology, Medical University Graz, Graz, Austria
JAMA Ophthalmol. 2015;133(11):1357-1360. doi:10.1001/jamaophthalmol.2015.2814.
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This case series describes twin sisters with cystoid macular edema related to a CRB1 mutation.

Two identical twin sisters in their teens presented to the Inherited Retinal Degenerations Clinic at the Wilmer Eye Institute for evaluation of cystoid macular edema (CME), which had been treated intermittently with topical carbonic anhydrase inhibitors. Both girls were myopic from early childhood but denied any visual complaints such as night blindness or visual field constriction, and their family history was negative for retinal diseases or consanguinity.

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Figure 1.
Retinal Imaging and Visual Neurophysiology Testing

A, Color fundus photographs exhibit pigmentary macular abnormalities and edema. B, Fundus autofluorescence imaging demonstrates a perifoveal ring of relatively increased autofluorescence and a central petaloid pattern. C, Full-field electroretinography reveals moderately reduced cone responses with normal implicit times.

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Figure 2.
Longitudinal Changes on Spectral-Domain Optical Coherence Tomography in the Left Eye of Twin 2

Initial B-scan with best-corrected visual acuity of 20/50 (A) and last available B-scan 28 months later with best-corrected visual acuity of 20/40 (B), showing decreases in central subfield thickness (from 336 to 225 µm) and macular volume (from 7.85 to 6.93 mm3). Analogous findings were observed in both eyes of both twins during the same period. The green horizontal arrow on the infrared image (left image of each panel) indicates the location of the horizontal cross-sectional B-scan (right image of each panel). The red lines on the B-scan provide inner and outer segmentation boundaries of the retina and correspond to the internal limiting membrane (upper line) and Bruch membrane (lower line).

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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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