Unilateral orbital fibrosis with blepharoptosis and enophthalmos

A 59-year-old woman with congenital orbital fibrosis with ptosis and enophthalmos underwent surgery to widen the palpebral fissures, which resulted in an acceptable cosmetic appearance. This is a rare entity that has been classified as a subtype of congenital fibrosis of the extraocular muscles. However, after a review of literature we believe that congenital orbital fibrosis should be considered a separate entity: it is unilateral, diffusely involves the orbit (not just the extraocular muscles) and does not appear to have a hereditary predisposition.