A Case of Undiagnosed Coats Disease Rears Its Head

An 18-year-old Hispanic man presented with complaints of decreased vision in his right eye with onset 3 to 4 weeks ago. This was his first eye examination, and his ocular history was unremarkable. His health history included obesity but was otherwise unremarkable. He did not report taking any medications or drugs, and he reported no medication or environmental allergies. He also reported no trauma, surgery, or injury to the eye. Family ocular and medical history was also noncontributory.

CLINICAL EVALUATION

On examination, UCVA was counting fingers at 3 feet OD, with no improvement with pinhole, and 20/20 OS. BCVA was 20/400 OD and 20/20 OS. Examination of extraocular muscles and confrontation visual fields were unremarkable. Pupil testing showed a 2+ afferent pupillary defect in the right eye. IOP was 16 mm Hg OU.

Anterior segment examination revealed a leukocoria in the right eye upon retroillumination but was otherwise unremarkable. On dilated fundus examination, massive exudation from the 4 to 12 clock hours and sectoral retinal detachment were observed in the right eye (Figure). Examination of the left eye was unremarkable.

DIAGNOSIS

I diagnosed the patient with exudative retinopathy (suspect for Coats disease) in the right eye and referred him immediately to ophthalmology (retina). He eventually received cryotherapy in the right eye with 35 spots. After cryotherapy he exhibited improvement in peripheral fluid, but the exudates were persistent. UCVA remained counting fingers at 5 feet OD.

DISCUSSION

Coats disease is an idiopathic retinal telangiectasia with onset in early childhood.¹ The disease is typically progressive in nature, leading to an increase in subretinal exudation and resultant retinal detachment. It has unilateral involvement in most cases. The majority of patients (75%) are male; there is no racial predilection.¹

Most patients present with Coats disease in the first decade of life. In our patient’s case, the time course of his disease was unclear, but his presentation suggested a degree of chronicity (see Coats Disease Signs and Symptoms).

TREATMENT

Management of patients with Coats disease includes observation for mild, non–vision-threatening cases. For more serious cases, options include laser ablation, anti–vascular endothelial growth factor therapy, intravitreal triamcinolone, cryotherapy (as in our case) for extensive exudation, vitreoretinal surgery for retinal detachment, and enucleation of painful eyes with neovascular glaucoma.¹

SCREEN VISION EARLY AND OFTEN

This patient’s case is a prime example of the importance of regular eye exams, especially in young children. If Coats disease in this patient had been caught earlier, the loss of vision in his right eye could have been prevented. He continues to be closely followed by retina specialists.