OPHTHALMIC FINDINGS IN LATE STAGE SJOGREN–LARSSON SYNDROME

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Abstract

Purpose:

To report spectral domain optical coherence tomography and fundus autofluorescence documentation of late stage macular findings associated with Sjogren–Larsson Syndrome in three adult siblings.

Methods:

Three adult siblings with Sjogren–Larsson Syndrome underwent ophthalmic examination and imaging.

Results:

Crystalline maculopathy and subretinal deposits, presumably lipofuscin accumulation, with macular atrophy were present in varying degrees in all three adult siblings.

Discussion:

In adults with Sjogren–Larsson Syndrome, crystalline retinopathy can progress to macular atrophy and the appearance of lipofuscin accumulation.

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