Coats-like Vasculopathy in Inherited Retinal Disease

• DOI: 10.1016/j.ophtha.2023.07.027
• Publication Date: 2023-08-05T01:54:08Z
• Authors: Malena Daich Varela; Giovanni Marco Conti; Samantha Malka; Veronika Vaclavik; Omar A. Mahroo; Andrew R. Webster; Viet Tran; Michel Michaelides
• Keywords: Coats' disease

PurposeTo describe the largest, most phenotypically and genetically diverse cohort of patients with inherited retinal disease (IRD)-related Coats-like vasculopathy (CLV).DesignMulticentre retrospective study.Subjects67 IRD-related CLVMethodsReview clinical notes, ophthalmic imaging, molecular diagnosis from two international centres.Main outcome measuresVisual function, management response to treatment were evaluated correlated.ResultsThe prevalence CLV was 0.5%; 55% had isolated retinitis pigmentosa (RP), 21% early onset severe dystrophy, less frequent presentations syndromic RP, sector cone-rod achromatopsia, PAX6-related X-linked retinoschisis. The overall age 30.7 ± 16.9 years old (1 – 83). Twenty-one (31%) unilateral common features telangiectasia, exudates, exudative detachment (ERD) affecting inferior temporal retina. Macular oedema/schisis seen in 26% eyes ERD 63%. Fifty-four (81%) genetic testing, 40 whom molecularly solved.Sixty-six (58%) observed, 17 (15%) treated a single modality, 30 (27%) combined approach. Thirty-five 'good responders', 42 (37%) 'poor 22 (19%) low vision at baseline only 12 (11%) did not have longitudinal assessment. (62%) observed responded well, versus 14 (33%) eyes. Final best-corrected visual acuity (BCVA) significantly worse than (p 0.002); (60%) lost 15 ETDRS letters or more over follow up one both eyes, 21 progressed advanced stages impairment.ConclusionsIRD-related is rare, sporadic, mostly bilateral, no gender predominant, can occur types IRD any point disease, mean fourth decade life. Patients who decreased initial VA, ERD, changes quadrants, CRB1-retinopathy may be higher risk poor prognosis.