Coats’ disease stabilized in 15 of 16 eyes, and 11 of 16 eyes experienced complete resolution, following intravitreal ranibizumab as an adjunct to ablation therapy, according to new research published in Clinical Ophthalmology.

The application of an anti-vascular endothelial growth factor (anti-VEGF) agent with ablation treatment reportedly achieved favorable outcomes; however, prior reports have cautioned that the combination is associated with vitreoretinal (VR) fibrosis and tractional retinal detachment (TRD). The researchers sought to provide more data to assist physicians in the treatment of the rare condition, which can lead to neovascular glaucoma (NVG) and phthisis bulbi with a blind eye that may require enucleation.

They reviewed the records of patients diagnosed with exudative Coats’ disease with stage 2 (hard exudates), 3 (exudative retinal detachment [ERD]), or 4 (ERD with NVG) at a single private eye center in Egypt from April 2016 until November 2020.


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The researchers analyzed 16 eyes of 16 patients (median age at presentation was 5 years). Researchers noted Parry-Romberg syndrome in 1 patient. Stage 2 disease was seen in 4 patients, of whom two had extrafoveal exudation and two had foveal exudation. Twelve of the 16 patients had stage 3 disease at the time of presentation, at which time only five of them were younger than 4 years.

All patients underwent off-label intravitreal injection of the anti-VEGF agent ranibizumab with a dose of 0.5 mg/.05 mL and a median of 1 injection, and 12 patients underwent ablation therapy simultaneously. Fourteen patients underwent cryotherapy while 4 patients underwent laser therapy. The eyes of 2 patients were treated with both ablation modalities. Re-treatment was required in 4 patients who had no response or partial resolution.

Retinal fibrosis developed in 15 cases after intervention but was subretinal and stable in 12 patients, while 3 participants developed VR fibrosis. None progressed to TRD. One of the 3 patients that underwent external SRF drainage experienced an iatrogenic break that required further intervention via plans plana vitrectomy, which involves ERD transforming to rhegmatogenous detachment with a higher risk of VR fibrous proliferation.

At the follow-up (median of 16 months, range 6 to 48 months), the disease had stabilized in 15 patients and progressed to stage 5 (end-stage with non-salvageable globe) in 1 patient. Complete resolution was seen in 11 eyes.

Complete resolution was defined in stage 2 as a disappearance of telangiectasia (evaluated clinically and confirmed by fundus fluorescein angiography imaging) and progressive reduction in exudate load, and in stage 3 as complete resolution of surgical subretinal fluid (SRF) with flattening of the retina. Partial resolution in stage 3 was defined as a reduction of SRF or intraretinal macular edema.

“We recommend for external SRF drainage to be preserved for eyes where ablation therapy is not feasible,” according to the researchers. 

Limitations of the study included its single-centered, non-comparative retrospective nature and a relatively small sample size.

Reference

Nowara M, Fouad YA, Aziz IA, et al. Experience with Intravitreal ranibizumab as an adjunct to ablation therapy in eyes with exudative Coats’ disease. Clinical Ophthalmol. 2021:15 367–373. doi:10.2147/OPTH.S293030.