Unilateral Congenital Third Nerve Palsy Associated with Osteogenesis Imperfecta

Purpose: Congenital third nerve palsy was generally thought to exist in isolation without associated abnormalities. In this report, we present a case of congenital third nerve palsy in osteogenesis imperfecta.Methods: A 6-month-old girl with osteogenesis imperfceta presented with ptosis and large incomitant exotropia in left eye (LE) at birth. Ocular examination revealed total third nerve palsy in LE. Brain CT scan was normal. She underwent surgery for strabismus at the age of two years and surgery for ptosis after four months.Results: Ocular alignment has greatly improved after recess-resect procedure on the horizontal rectus muscles in LE. She achieved a good functional outcome after ptosis surgery.Conclusion: Congenital third nerve palsy may associate with systemic disease. To our knowledge, this represents the first report on demonstrating this association in osteogenesis imperfecta