Purpose: The purpose of the study is to describe the clinical characteristics and surgical management of strabismus associated with chronic progressive external ophthalmoplegia. Methods: The authors present four patients with chronic progressive external ophthalmoplegia and strabismus requiring extraocular muscle surgery, with attention to presenting symptoms, patterns of misalignment, results of surgical and nonsurgical therapies, and associated ocular or systemic conditions or both. Results: Three patients reported diplopia before surgery. One patient presented with an esotropia, one with an exotropia, and two with hypertropia. Three patients required only one strabismus surgery, and one patient required multiple surgeries. Conclusions: Chronic progressive external ophthalmoplegia may have clinical characteristics similar to those of myasthenia gravis or thyroid ophthalmopathy. Patients with chronic progressive external ophthalmoplegia and strabismus frequently have diplopia in primary position and may benefit from extraocular muscle surgery to improve alignment. In addition, because these patients typically have poor motor fusion, prisms often are useful adjuncts to surgery.
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