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Ophthalmic Images
December 10, 2015

Posterior Polar Annular Choroidal Dystrophy

Author Affiliations
  • 1Kellogg Eye Center, University of Michigan, Ann Arbor
JAMA Ophthalmol. 2015;133(12):e152148. doi:10.1001/jamaophthalmol.2015.2148

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.