Risk factors for enucleation in Coats disease

The largest cohort study thus far found that the extent of retinal detachment and severity of aneurysms, in addition to iris neovascularization, are significant predictors of enucleation.

How the eye of a patient with Coats disease looks like. Note the conjunctival hyperemia, leukocoria, corneal edema, and posterior synechiae. (Source via CC by 2.0, Fernandes BF et al., 2007)

Coats disease is an infrequent nonhereditary retinal disease that usually presents in pediatric patients (median age: 5 years, male predisposition with incidence 76%) with vision loss, strabismus or leukocoria. On examination, the retina has telangiectatic vessels, micro- and macroaneurysms, and intraretinal and subretinal exudation.

Because it is a vascular disease, treatment methods include anti-VEGF or corticosteroid injections, laser photocoagulation, surgery, and enucleation for advanced cases that have painful glaucoma or phthisis bulbi.

In a previous series of 150 eyes, enucleation had to be done in 16% of cases due to neovascular glaucoma or advanced Coats disease. This retrospective study of 351 patients (351 eyes) aimed to analyze the clinical risk factors that could predict the need for enucleation.

Results showed that in addition to iris neovascularization, the extent of retinal detachment and the angiographic severity of light bulb aneurysms predicted enucleation. Other findings that were seen in the 32 eyes (12%) that had to be enucleated were total retinal detachment, more telangiectatic vessels, and subretinal fluid.

Limitations of the study include incomplete data imaging in a quarter of patients, as well as the bias towards the presence of more advanced disease due to referrals to rule out retinoblastoma. Nevertheless, this is the largest cohort of patients to date. The study recommended detailed notes of retinal findings in the clinical examination for patient evaluation, as well as optimization of medical and surgical treatment, to prevent disease progression.

Udyaver, S., Dalvin, L., Lim, L., Mazloumi, M., Atalay, H., & Khoo, C. et al. (2019). Predictors of enucleation in Coats disease: analysis of 259 eyes of 259 patients at a single center. Journal of American Association for Pediatric Ophthalmology and Strabismus. doi: 10.1016/j.jaapos.2019.05.015

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