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JAMA Ophthalmology Clinical Challenge
March 2014

Pigmented Ocular Fundus Lesions in a 6-Year-Old Girl

Author Affiliations
  • 1Bascom Palmer Eye Institute, University of Miami, Miami, Florida

Copyright 2014 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Ophthalmol. 2014;132(3):351-352. doi:10.1001/jamaophthalmol.2013.8157

A 6-year-old girl was incidentally discovered to have fundus lesions after presenting with head trauma that resolved without permanent intracranial pathology. She had no significant medical history or prior ocular problems. Best-corrected visual acuity was 20/20 OD and 20/25 OS, with eccentric fixation and trace exophoria in the left eye. Pupils were equally round and reactive. Anterior segment examination revealed an enlarged right corneal nerve. The left macula had a fibrotic-appearing epiretinal membrane (Figure 1A). There were 2 small, flat, pigmented lesions along the arcades of the right fundus (Figure 1B), likely atypical congenital hypertrophy of the retinal pigment epithelium (CHRPE) lesions, or pigmented ocular fundus lesions (POFLs). Observation was elected. On follow-up, optical coherence tomography showed focal macular thickening in the left eye, most likely a retinal hamartoma (Figure 1C), and intraretinal abnormalities between the retinal pigment epithelium (RPE) and photoreceptor layer in the right eye, consistent with POFLs and likely representing combined retina-RPE hamartomas (Figure 1D). B-scan ultrasonography and biometric A-scan showed bilaterally clear vitreous without retinal detachment. Fluorescein angiography showed hyperfluorescence around the macula lesion without leakage, consistent with staining (Figure 1E), in the left eye. There was no leakage or hyperfluorescence of the POFLs in the right eye (Figure 1F).

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