Craniofacial fibrous dysplasia with ipsilateral keratoconus

Abstract

Fibrous dysplasia (FD) is a developmental dysplastic disorder of the bone, in which the normal matrix is replaced by fibroblastic proliferation. Keratoconus is usually an isolated sporadic disorder which may be associated with various ocular and systemic conditions. We report a case of ipsilateral keratoconus with craniofacial FD. Only a single such case has been reported in literature. An 18-year-old male patient presented to us with right eye (RE) proptosis and protrusion of the forehead and lateral skull associated with poor vision in the RE. He had earlier undergone conservative excision and recontouring for FD. Best-corrected visual acuity in the RE was counting fingers at 1 m and in the left eye was 20/20. Clinical signs of keratoconus were present in the RE. Corneal topography confirmed the diagnosis of keratoconus. The incidence of keratoconus in this case may be an independent finding or may be related to pressure on the globe due to expanding orbital walls.

Keywords

• corneal hydrops
• corneal topography
• fibrous dysplasia
• keratoconus