Optical coherence tomography in a case of Bietti's crystalline dystrophy

Purpose: To demonstrate the cross-sectional morphology in crystalline retinopathy. Methods: A 24-year-old woman with Bietti's crystalline dystrophy (BCD) and bilaterally decreased vision and nyctalopia was examined by optical coherence tomography (OCT). Results: Fundus examination demonstrated numerous reflective, yellow-white crystalline deposits scattered throughout the posterior pole and midperipheral retina. Optical coherence tomography disclosed an abnormally level of high reflectivity in red to orange colours throughout the entire neuroretina, retinal pigment epithelium (RPE) and choroid. The RPE-choriocapillaris complex was thickened and hyper-reflective, corresponding with dense crystalline deposits. Conclusion: The observed uncommon hyper-reflectivity in BCD relates to the deposition of numerous infiltrates. In vivo investigations by OCT confirmed histological studies that a crystalline retinopathy corresponds with numerous infiltrates in the retina, RPE and choroid. The RPE and choroid have intensive hyper-reflection and may be the primary location of the disease.