Purpose: To describe a case of familial Paget disease of bone associated with bilateral enophthalmos and mild exposure keratopathy.
Methods: Case report and literature review.
Results: A 71-year-old woman with familial Paget disease of bone had a long history of "receding eyes," headaches, and moderately dry eyes. She had bilateral enophthalmos with mild exposure keratopathy. Radiologic and endocrinologic investigation confirmed Paget disease of bone affecting the skull.
Conclusions: Enophthalmos in association with Paget disease of bone is rare and is likely to result from differential expansion of the cranium compared with the orbital bones.