Characterization of Macular Neovascularization in Bietti's Crystalline Dystrophy Using Multimodal Imaging Modalities.

Abstract

The imaging data of 157 eyes in 79 patients with BCD were retrospectively reviewed. Twelve individuals (19 eyes) were found to have MNVs. Multimodal retinal imaging was used to evaluate the features of MNVs and the primary chorioretinopathy. The MNV lesions were shown as typical type 2 MNVs with subretinal hyperreflective material (SHRM), and usually detected along the borders of the RPE/choriocapillaris dropout. The active MNVs were noted in earlier stages of BCD, while the activity was seen reduced in advanced cases. On spectral domain optical coherence tomography, the outer retinal structures were demonstrated to be partially preserved above the SHRMs compared with the extensive atrophy contiguously. Fibrotic scaring of the MNVs was commonly observed and arteriolarization was usually shown within the scars. MNV was demonstrated to be a common complication secondary to BCD. The lesions were typical type 2 MNV of varied activities possibly associated with the degrees of the primary degeneration. Choriocapillaris hypoperfusion might participate in MNV development.