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Ophthalmic Images |

Posterior Polar Annular Choroidal Dystrophy Online Only

Devon H. Ghodasra, MD1; Cagri Besirli, MD, PhD1
[+] Author Affiliations
1Kellogg Eye Center, University of Michigan, Ann Arbor
JAMA Ophthalmol. 2015;133(12):e152148. doi:10.1001/jamaophthalmol.2015.2148.
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This case report describes a young boy with a history of a ruptured left globe and a lack of light perception in the left eye.

A young boy with a history of a ruptured left globe presented for evaluation of retinal atrophy in the right eye. He had no ocular concerns, and there was no family history of eye disease. His visual acuity was 20/40 OD by Snellen testing, and he had no light perception OS. He had a normal anterior segment in the right eye and enophthalmia and phthisis in the left eye. The findings on dilated fundus examination of the patient’s right eye are shown in the Figure. An ultrasonogram showed complex retinal detachment in the patient’s left eye. Electroretinographic rod, combined rod-cone, and cone responses of the right eye were highly reduced. The 32-Hz flicker response in the right eye showed a highly reduced amplitude and delayed peak implicit time. The findings on ophthalmologic examination were consistent with posterior polar annular choroidal dystrophy. Fundus examination findings of the patient’s mother showed no abnormalities in either eye. At a 6-month follow up appointment, the patient’s visual acuity and appearance of his right fundus were unchanged.

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Figure.

A, Fundus photograph of the patient’s right eye showing atrophy of the retinal pigment epithelium and choroid along the temporal arcades with preservation of the macula. B, Fluorescein angiography demonstrating normal filling of retinal vessels with window defects in areas of chorioretinal degeneration.

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