Unilateral optic atrophy preceding Coats disease in a girl with Parry-Romberg syndrome

PURPOSE. Parry-Romberg syndrome (PRS) is a rare disease of unknown etiology and pathogenesis, characterized by progressive hemifacial atrophy. Diverse ocular manifestations were reported in association with PRS, including enophthalmos, lid retraction, blepharoptosis, restrictive strabismus, ocular motor nerve dysfunction, Horner syndrome, reduced corneal sensitivity, band keratopathy, episcleritis, uveitis, neuroretinitis, and retinal vasculitis. METHODS. Descriptive case report. results. We report on the development of unilateral optic atrophy followed by ipsilateral Coats disease (CD) in a girl with PRS. Visual outcome was limited owing to the development of massive subretinal exudation and shallow retinal detachment in the posterior pole and inferior retina. CONCLUSIONS. Optic atrophy was not previously described in association with PRS. We describe the development of unilateral optic atrophy with subsequent CD, 5 years later, in a girl with PRS.