OPTICAL COHERENCE TOMOGRAPHIC FINDINGS IN BIETTI'S CRYSTALLINE TAPETORETINAL DYSTROPHY

Purpose: To report the optical coherence tomography (OCT) findings of Bietti crystalline tapetoretinal dystrophy. Design: Observational case report. Methods: A subject with Bietti crystalline tapetoretinal dystrophy was evaluated with ophthalmoscopy, fluorescein angiography, and OCT (Stratus). Results: OCT showed thinning and hyporeflectivity of the outer nuclear layer in the macula due to photoreceptor degeneration. The retinal areas showing atrophy of the retinal pigment epithelium displayed greater penetration of the optical beam into the choroid and visualization of residual choroidal vessels. The hyperreflective band normally seen under the neurosensory retina was extremely wide when atrophy of the entire retinal pigment epithelium/choriocapillaris complex was found. Above this band, and within the neurosensory retina, some hyperreflective dot-like lesions were seen that could be related to crystals. Conclusions: In cases of Bietti crystalline tapetoretinal dystrophy, OCT shows changes secondary to fundus atrophy and more specific changes that can be related to the presence of crystals, thereby making OCT particularly useful for demonstrating macular atrophic abnormalities.