Inherited retinal disease (IRD)- related Coats-like vasculopathy (CLV) is rare, sporadic, and principally bilateral, according to results published in Ophthalmology. Michel Michaelides, MD, and colleagues conducted a multicenter retrospective cohort study to describe patients (N=67) with IRD-related CLV. The prevalence of IRD-related CLV was 0.5%. More than one-half (55%) of patients had isolated retinitis pigmentosa, while 21% had early onset severe retinal dystrophy. The overall age at the time of CLV diagnosis was 30.7 (range, 1-83). The most common retinal features were telangiectasia, exudates, and exudative retinal detachment (ERD) affecting the inferior and temporal retina; ERD was observed in 63% of patients. Final best corrected visual acuity was significantly worse than baseline; 60% of patients lost 15 ETDRS letters or more over follow-up in one or both eyes, and 31% progressed to more advanced stages of visual impairment. “Patients with IRD-related CLV who have decreased initial [visual acuity], ERD, CLV changes affecting two or more retinal quadrants, and CRB1 retinopathy may be at higher risk of a poor prognosis,” Dr. Michaelides and colleagues wrote.
