– Authors: Malena Daich Varela et al.
– Journal: Ophthalmology, 2023

Main Points:
– Describing the largest cohort of patients with inherited retinal disease (IRD)-related Coats-like vasculopathy (CLV).
– Multicentre retrospective cohort study.

– ۶۷ patients with IRD-related CLV.
– Data from two international centers.
– Review of clinical notes, ophthalmic imaging, and molecular diagnosis.

– IRD-related CLV prevalence: 0.5%.
– ۵۵% had isolated retinitis pigmentosa (RP).
– ۲۱% had early onset severe retinal dystrophy.
– Other presentations included syndromic RP, sector RP, cone-rod dystrophy, achromatopsia, PAX6-related dystrophy, and X-linked retinoschisis.

**Age at Diagnosis:**
– Mean age of CLV diagnosis: 30.7 ± ۱۶.۹ years (ranged from 1 to 83 years).

**Clinical Features:**
– Telangiectasia, exudates, and exudative retinal detachment (ERD) were common.
– Macular edema/schisis in 26% of eyes.
– ERD in 63% of eyes.

**Genetic Testing:**
– ۸۱% of patients had genetic testing.
– ۴۰ patients had a molecular diagnosis.

**Management:**
– ۵۸% of eyes were observed.
– ۱۵% of eyes were treated with a single modality.
– ۲۷% of eyes had a combined approach.

**Response to Treatment:**
– ۳۱% of eyes were ‘good responders’.
– ۳۷% of eyes were ‘poor responders’.
– ۱۹% with low vision only observed.
– ۱۱% lacked longitudinal assessment.
– Better response in observed eyes (62%) compared to treated eyes (33%).

**Visual Acuity:**
– Final best-corrected visual acuity (BCVA) worse than baseline.
– ۶۰% of patients lost 15 ETDRS letters or more during follow-up.
– ۳۱% progressed to more advanced visual impairment stages.

**Conclusions:**
– IRD-related CLV is rare, mostly bilateral, with no gender predominance.
– Occurs in various IRD types at any disease stage.
– Mean onset in the fourth decade of life.
– Poor prognosis associated with decreased initial VA, ERD, CLV changes in multiple retinal quadrants, and CRB1-retinopathy.