A Case of Best Disease Accompanied by Pachychoroid Neovasculopathy

The aim of this case presentation is to describe ocular findings of a 22-year-old patient with Best vitelliform macular dystrophy accompanied by pachychoroid neovasculopathy. Color fundus photography, fundus autofluorescence (FAF), optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) images were reviewed. Fundoscopic examination showed bilateral yellowish vitelliform-like submacular deposits. FAF revealed these deposits as hyperautofluorescent spots. OCT showed flat irregular pigment epithelial detachments corresponding to these submacular deposits. OCT showed choroidal thickening and dilatation of the large outer oval choroidal vessels. Fundus fluorescein angiography could not be performed because the patient was pregnant. En face OCTA images of the choriocapillaris illustrated the choroidal neovascular network. In this case report, we describe for the first time the coexistence of Best vitelliform macular dystrophy and pachychoroid neovasculopathy with OCTA images enabling visualization of the neovascular network in a patient with contraindication for fluorescein angiography.