Coats' disease: trends and long-term treatment outcomes in a tertiary referral centre.

Abstract

Background: The long-term treatment outcomes in Coat's disease - particularly in the era of newer pharmacotherapies such as anti-vascular endothelial growth factor (VEGF) agents and depot steroids - are poorly understood.

Aim: To describe the clinical features and treatment outcomes of 148 eyes with Coats' disease assessed in a referral centre over 30 years.

Materials and methods: We conducted a retrospective chart review of patients diagnosed with Coats' disease between 1 June 1987 and 31 July 2017. The demographic, clinical and treatment data were collected and long-term functional and anatomical outcomes were analysed based on the treatment either with conventional therapy (cryo/laser) or along with adjuvants like intravitreal steroids or anti-VEGFs.

Results: The mean age at presentation was 15.22 years (median 11). Familial exudative vitreoretinopathy was the most common referral diagnosis, 76/148 (51.5%), followed by Coats' disease, 37/148 (25%), and retinoblastoma, 35/148 (23.6%). Stage 3B was most common at presentation (31.8%), followed by 2B (22.3%) and 2A (16.9%). A total of 107 patients were treated either with conventional therapy or in combination with adjuvants. The mean follow-up period was 24.95 months. The visual acuity improved from baseline logMAR 2.17 (Snellen-20/2958) to logMAR 1.88 (Snellen-20/1517) at final follow-up (p = 0.004). The improvement in visual acuity was better when the presenting BCVA was <1 logMAR (Snellen 20/200), p = 0.004. No statistically significant change in BCVA was noted between conventional and adjuvant groups, p = 0.5. However, the final anatomical outcome was good in 78/99 (78.8%) in the conventional group and 45/49 (91.8%) in the adjuvant group, respectively (p = 0.046).

Conclusion: In this series of patients with Coats' disease over three decades, the use of intravitreal steroids or anti-VEGFs as adjuvants resulted in better anatomical outcomes. A better baseline visual acuity, lower stage of the disease, and older age at presentation were found to be the factors leading to favourable visual outcomes.

Summary: In the current series of 148 eyes with Coats' disease, adjuvant treatment with intravitreal steroids or anti-VEGFs resulted in better outcomes as compared with conventional cryotherapy or laser photocoagulation alone. Patients with Coats' disease who had presented with better visual acuity at baseline, lower stage of the disease and older age had better final visual outcomes.