Clinical Characteristics and Treatment Outcomes in Unilateral Coats Disease: A Global Collaborative Study.

PURPOSE

To evaluate the clinical outcomes and prognostic factors in unilateral Coats disease in the era of anti-VEGF therapy.

DESIGN

Global, multicenter, retrospective case series.

SUBJECTS

Six hundred fifty-six eyes of 656 subjects with Coats disease were included in this study. Exclusion criteria were Coats disease secondary to retinitis pigmentosa as well as bilateral cases.

METHODS

Clinical data from patients with Coats disease were collected from 20 ophthalmic practices around the world. We compared early-stage (stage 1-2) and advanced-stage (stage 3-5) Coats disease in terms of clinical characteristics and treatment modalities.

MAIN OUTCOME MEASURES

Functional outcomes include achieving visual acuity (VA) of 0.3 logarithm of the minimum angle of resolution or better and VA improvement or stability. Anatomical failure was defined as the development of phthisis, chronic retinal detachment, massive fibrosis, or the requirement for enucleation.

RESULTS

Subjects with early-stage disease were significantly older, with a mean age of 17.4 ± 17.8 years, compared with 7.1 ± 7.1 years in the advanced-stage group (P < 0.001). There was a male predominance in both early and advanced stages (84.7%). Advanced disease was associated with a higher incidence of strabismus (20.2% vs. 6.7%, P < 0.001) and leukocoria (12.3% vs. 3.2%, P < 0.001). More subjects with early-stage disease received laser photocoagulation as monotherapy (44.7% vs. 21.1%, P < 0.001). Additionally, early-stage disease received more sessions of intravitreal anti-VEGF injections as adjunct therapy (4.4 ± 6.2 vs. 2.7 ± 2.1, P = 0.005). Factors associated with poorer functional outcomes included worse presenting VA, advanced disease stage, and the presence of a foveal nodule. Worse presenting VA and advanced disease stage were associated with lower likelihood of anatomical success, whereas combination therapy increased the odds of anatomical success.

CONCLUSIONS

Unilateral Coats disease predominantly affects males, regardless of disease stage. Identifying a foveal nodule is crucial for visual prognosis. Laser photocoagulation remains the primary treatment. Although anti-VEGF may prevent enucleation, its role in early-stage disease requires further clarification.

FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.