Bilateral Symmetric Autosomal Dominant Sector Chorioretinopathy with late Maculopathy: A Review Based on a Case with 48 Years Follow-up

Abstract

Purpose

To describe the long-term course of bilateral symmetric autosomal dominant sector chorioretinopathy in a 79-year-old man who was diagnosed at age 31.

Methods

Ophthalmic examinations including fundus photography, fluorescein and indocyanine angiography, adaptometry, and ocular electrophysiology were performed at intervals from 1962 to 2010.

Results and Conclusions

The patient experienced no visual symptoms during his entire working life, but acquired an exudative maculopathy in his left eye at age 67. Two years later, a central subretinal neovascular membrane affected the right eye. The latter responded only temporarily to photodynamic therapy (Visudyne), and from age 71 he had best-corrected visual acuities <0.1 in both eyes, with eccentric fixation. We are not aware of any report on late macular involvement in this disorder. Whether the maculopathy should be considered a late manifestation of the sector chorioretinopathy or a coincidental occurrence of age-related macular degeneration remains unsettled.