Long-term outcome of Coats' disease: Implications for the classification of foveal vascular pathologies

Claudia Brockmann; Bert Müller; Shideh Schönfeld; Sibylle Winterhalter; Oliver Zeitz; Antonia M Joussen

doi:10.1111/aos.17554

Long-term outcome of Coats' disease: Implications for the classification of foveal vascular pathologies

Acta Ophthalmol. 2025 Jul 5. doi: 10.1111/aos.17554. Online ahead of print.

Authors

Claudia Brockmann^{1

2

3}, Bert Müller^{1

2

3}, Shideh Schönfeld^{1

2

3}, Sibylle Winterhalter^{1

2

3}, Oliver Zeitz^{1

2

3}, Antonia M Joussen^{1

2

3}

Affiliations

¹ Department of Ophthalmology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
² Corporate Member of Freie Universität Berlin, Humboldt-Universität Berlin, Berlin, Germany.
³ Berlin Institute of Health, Berlin, Germany.

PMID: 40616365
DOI: 10.1111/aos.17554

Abstract

Purpose: To investigate the long-term outcome in patients with Coats' disease regarding initial stages.

Methods: Within a retrospective, clinical, single centre study, patients with diagnosed Coats' disease treated from 1992 to 2019 (minimum follow-up of 12 months) were investigated. A total of, 75 out of 123 patients met the inclusion criteria.

Results: A total of, 77 eyes of 75 patients (80.5% male, 17.8 ± 18.6 years, median 10 years) were analysed. Mean follow-up time was 7.2 ± 5.5 years (range 1.1-21.1 years, median 6.3 years). Initial and final visual acuity was lower the higher the disease stage was. The need for surgical interventions was higher in more advanced stages. Notably, within stage 2B, significant differences in final visual acuity were observed depending on the presence of foveal exudation only (0.20 ± 0.29 logMAR) as compared to eyes with foveal vascular abnormalities (1.20 ± 0.55 logMAR, p < 0.001).

Conclusion: Based on our analyses, it is suggested to amend the well-defined and established classification system according to Shields by sub-dividing stage 2B (telangiectasia and exudation with foveal exudation) into 2B1 (telangiectasia and exudation with foveal exudation) and 2B2 (telangiectasia and exudation with foveal exudation and foveal Coats' typical vascular abnormalities), since both subgroups significantly differ with respect to prognosis of visual acuity.

Keywords: Coats' disease; classification system Shields; foveal vascular pathology; long‐term outcome.