Coats disease in 351 eyes: Analysis of features and outcomes over 45 years (by decade) at a single center

Abstract

Purpose: To assess features and outcomes of Coats disease over 5-decades. Methods: Retrospective review of Coats disease patients at a single center. Features and outcomes were compared based on decade of presentation. Results: There were 351 patients with Coats disease. The presenting median age (6 years), male sex (84%), and unilaterality (100%) did not change per decade. Coats disease classification did not change per decade with Stage 1 (1%), Stage 2 (21%), Stage 3 (68%), Stage 4 (6%), and Stage 5 (1%). Clinical features that changed per decade (1970s vs. 1980s vs. 1990s vs. 2000s vs. 2010s) included 1980s features of more eyes with exudation in all 4 quadrants (22% vs. 58% vs. 44% vs. 33% vs. 27, P = 0.01) and total exudative retinal detachment (33% vs. 53% vs. 39% vs. 27% vs. 21%, P < 0.001). Imaging features that changed per decade included 2010s greater fluorescein angiographic extent of retinal non-perfusion in mean clock hours (4 vs. 4 vs. 3 vs. 5 vs. 6, P = 0.003), and 1980s greater mean height of retinal detachment ultrasonographically (5 vs. 12 vs. 5 vs. 5 vs. 4 mm, P < 0.001). Treatment features that changed per decade included 1980s greater primary enucleation (11% vs. 16% vs. 3% vs. 4% vs. 1%, P = 0.001), and 2010s greater use of laser photocoagulation (55% vs. 33% vs. 38% vs. 40% vs. 72%, P < 0.001), sub-Tenon corticosteroid (0% vs. 4% vs. 5% vs. 8% vs. 29%, P < 0.001), and intravitreal anti-VEGF) (0% vs. 4% vs. 2% vs. 13% vs. 18%, P = 0.003). Outcomes that changed per decade included 2010s findings of more complete resolution of subretinal fluid (64% vs. 59% vs. 38% vs. 58% vs. 72%, P = 0.01) and less need for primary/secondary enucleation (17% vs. 27% vs. 14% vs. 13% vs. 6%, P = 0.04). Conclusion: Eyes with Coats disease in the 1980s demonstrated more advanced findings, often requiring enucleation. Over the decades, greater use of laser photocoagulation and injections has led to improved disease resolution with greater globe salvage.

Coats disease: An overview of classification, management and outcomes

“…Coats disease is typically unilateral (95--100%). [152526] True cases of bilateral Coats disease are rare, with the other eye being asymptomatic and showing subtle peripheral telangiectatic vessels. Male predominance and unilaterality are consistent features in the literature with no specific explanation.…”

“…Male predominance and unilaterality are consistent features in the literature with no specific explanation. [252627282930] In a recent large analysis on 351 consecutive cases of Coats disease from a single center, 295 (84%) were male and 351 (100%) were unilateral. [26] Using strict definition of Coats disease, this condition is sporadic and nonhereditary with no associated systemic abnormalities or racial predilection.…”

Advanced Coats’ disease treated with intravitreal brolucizumab combined with laser photocoagulation

“…Coats' disease, first described in 1908, 1 is characterized by peripheral vascular ectasia, aneurysms, and exudation. 2 Historically, the main treatment included repetitive laser photocoagulation to areas of nonperfusion and telangiectasias. 3 Recent studies have demonstrated upregulation of VEGF in patients with Coats’ disease, 4 and this has prompted interest in intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapy as an adjuvant to laser photocoagulation.…”

Commentary: Coats disease: Our experience in India

“…The present study by Shield et al has beautifully illustrated the changing pattern of clinical features, progression, management options, and outcomes over the past 45 years. [1] When compared with western literature, bilateral involvement is more commonly reported in Indian studies. A study by Rishi et al has showed severe visual impairment and extensive four-quadrant involvement at initial presentation.…”

“…The clinical differentiation between retinoblastoma and Coats disease has been extensively highlighted in the present study. [1] The findings of ultrasonography, computed tomography scan, and contrast-enhanced magnetic resonance imaging should also be corroborated. If dilemma still persists, cases can undergo enucleation (in cases with no vision potential) or should be kept under close follow-up, which is difficult in Indian settings.…”