Progressive hemi-facial atrophy and keratoconus.

Progressive hemifacial atrophy (Parry-Romberg syndrome, PHA) is characterised by slowly progressive atrophy, usually involving one side of the face, and may be associated with ocular manifestations which include enophthalmos, restrictive strabismus and hypotony. We report a case of keratoconus seen in a patient with PHA after obtaining approval from our Institutional Review Board. We are unaware of previous reports in the literature describing an association between the two. A 40-year-old female with a known diagnosis of leftsided PHA was referred to the Cornea Services for progressive blurring of vision in both eyes. On examination, her visual acuity in the right eye (RE) was 6/120 correctable to 6/18 and in the left eye (LE) was 6/60 correctable to 6/ 30. She had left-sided facial hemiatrophy with an enophthalmos (Fig. 1) of 5 mm. Her ocular movements were full and she was orthophoric. Intra-ocular pressure was 7 mmHg in the RE and 5 mmHg in the LE. Anterior segment examination revealed Vogt striae, Fleischer’s ring and distorted mires on Placido’s disc in both eyes. Posterior segment examination was normal. A corneal topography was done which confirmed the diagnosis of keratoconus (Figs. 2). Her manifest refraction in the RE was -4.00/ -7.00 x 170 and in the LE was +3.00/-11.00 x 180. Progressive Hemi-facial Atrophy and Keratoconus