Abstract
Purpose :
To evaluate the patterns and distribution of ocular angiogenic complications in patients with inherited retinal degenerations.
Methods :
A clinic-based database composed of patients clinically diagnosed as inherited retinal degenerations was reviewed and those complicated with angiogenic changes including choroidal neovascularization (CNV), non-CNV related macular edema (ME), exudative retinal detachment (ERD) and retinal vasculitis-like changes were further analyzed for fundus photography, spectrum domain optical coherence tomography (SD-OCT) and fluorescein angiography (FA).
Results :
Totally 207 cases clinically dignosed as multiple types of inherited retinal degenerations were included in our database, 47 patients were found to have angiogenic complications. CNV was shown predominantly in patients with Bietti crystalline dystrophy (BCD, 11 eyes, 8 out of 32 patients) and vitelliform macular dystrophy (VMD, 5 eyes, 4 out of 18 patients). Non-CNV related ME was presented in patients with BCD (24 eyes of 14 patients), VMD (7 eyes of 4 patients), retinitis pigmentosa (RP, 17 eyes, 12 out of 50 patients), and more rarely cone dystrophy (CD, 1 eye of 1 patient). Apart from attenuation of retinal vessels, novel vascular changes in the peripheral retina, including vasculitis-like damages, ERD and retinal neovascularization were also observed in patients with RP (7 eyes of 4 patients) and pigmented paravenous chorioretinal atrophy (PPCRA, 1 eye, 1 patient out of 6 patients).
Conclusions :
Various types of angiogenic pathology were displayed in a considerable number of retinal dystrophic diseases. CNV is a rather common complication among the patients with BCD and VMD, cautions should be taken for further therapeutic process. Occurrence of non-CNV related ME is more frequently seen among cases with BCD, VMD and RP, while appeared as a novel alteration in cases with CD. Other angiogenic complications such as retinal vasculitis, ERD and peripheral retinal neovascularization were also exhibited in individuals with RP as well as PPCRA, and corresponding medical or surgical treatments should be warranted to prevent further visual loss.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.