A 'Gleam' in the Left Eye of a 5-Year-Old Boy

Mauricio E. Pons, MD; Maria Silvana Horenstein, MD

Disclosures

July 22, 2016

If untreated, Coats disease worsens progressively in most cases, with variable speed; therefore, early treatment is recommended. The disease does not respond to steroid or antibiotic treatment. Recommended treatments depend mainly on the stage of the disease; these include observation, laser photocoagulation, cryotherapy, retinal detachment repair with pars plana vitrectomy and/or scleral buckle, and enucleation.

Because treatment involves general anesthesia and frequent follow-up, discussions with a patient's parents (if the patient is a minor) regarding diagnosis, prognosis, and treatment goals are very important. Laser treatment has been used with a high success rate in cases with less exudation. Treatment is directed to areas of vascular leakage and nonperfusion, which decreases or eliminates further exudation and leads to resolution of the exudates and serous detachment. Fluorescein angiography is very useful in guiding laser treatment. Peripheral lesions are better treated with cryotherapy or a combination of laser and cryotherapy. Treatment may have to be repeated, because recurrences may follow.

In more advanced cases, drainage of the subretinal fluid may be necessary, with or without the use of a scleral buckle. Pars plana vitrectomy has been recently used to treat exudative and tractional retinal detachments.

Despite resolution of exudates, subretinal fibrosis and scarring may permanently impair vision. Because the disease is unilateral in most cases, patients may decide that this is not a major quality-of-life issue and use polycarbonate glasses for protection of the fellow eye, especially during sports activities.[2,6]

Associated complications of Coats disease include neovascular glaucoma (approximately 10% of patients), angle-closure glaucoma, anterior chamber cholesterolosis (3%), retinal/disc neovascularization, vitreous hemorrhage, secondary retinal vasoproliferative tumor, and intraretinal cysts.[6]

In this patient, enucleation was the preferred treatment because of the poor visual prognosis, advanced stage of the disease, presence of neovascular glaucoma, elevated intraocular pressure, closed anterior chamber angle, and strabismus. The eye was sent for pathologic examination, and the pathology report confirmed the clinical diagnosis.

During surgery, the eye was replaced with a hydroxylapatite implant. The four rectus muscles were attached to the implant. Six weeks later, the patient was fitted with a prosthetic, with excellent cosmetic results and good motility.

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