Ophthalmic changes in patients with hemifacial atrophy (Parry-Romberg syndrome)

Parry-Romberg syndrome (PRS) is a rare condition characterized by progressive, unilateral facial atrophy. We hypothesize that patients with this condition may have involvement of the ocular structures. Here, we report our ophthalmic, clinical and anatomical findings in an observational study of six patients with long-standing PRS.

Patients diagnosed with PRS were invited to participate in a clinic visit during which the following tests were administered and data recorded: best-corrected vision, refractive error, Ishihara color plates, Hertel exophthalmometry, gonioscopy, complete slit-lamp and dilated fundus examination, Intra Ocular Lens Master measurements and keratometry. Two-sample T tests were used to compare data between affected and unaffected eyes, as well as affected eyes and a normative population.

Six patients underwent complete eye examinations. The mean spherical equivalent of the affected eye was + 3.83 D, while that of the unaffected eye was + 0.13 D. The atrophic hemiface averaged 2.8 mm of enophthalmos on Hertel exophthalmometry. The axial length of the atrophic eye was 0.91 mm shorter than the unaffected eye. Compared to normative data, in patients with PRS, the difference between eyes was statistically different for each of the following variables: visual acuity, spherical equivalent, corneal diameter, axial length and flat and steep keratometry.

This is one of the first quantitative, exploratory studies with ophthalmic measurements in patients with PRS. Our results suggest the globe may demonstrate atrophic changes similar to other soft tissues in the face known to be affected by this condition.