Outcome of Ptosis Surgery in Patients with Chronic Progressive External Ophthalmoplegia

Objectives: To evaluate the clinical features and the outcome of ptosis surgery in patients with chronic progressive external ophthalmoplegia (CPEO). Materials and Methods: The demographic features, surgical approaches, anatomic and functional outcomes, and complications were reviewed in patients with CPEO who had undergone ptosis surgery by a single surgeon between the years 2005 and 2013. The patients were asked to evaluate their postoperative result as either worse, no change, good, or very good. Results: Seven men and 5 women with an average age of 50 +/- 14.08 years (range: 28-72) were included in the study. Ragged red fibers (RRF) were identified in 5 out of 9 patients' levator and orbicularis muscle biopsy specimens. Average levator function was 5.4 +/- 2.6 mm (range: 2-9). Frontalis suspension surgery with silicone rod was performed in 8 patients; two of those 8 patients had lower lid elevation with hard palate graft prior to ptosis surgery. The remaining 4 patients had levator resection. Postoperative margin reflex distance -1 was between +1 and +3 in all patients. One patient had punctate keratopathy following surgery, which responded rapidly to intensive use of lubricants. Head position was improved in all patients; postoperative result was rated 'very good'. Conclusion: Eyelid elevation must be tailored to result in sufficient interpalpebral area so as to allow for normal visual function and avoid exposure keratopathy. Lower eyelid elevation with hard palate graft may help to achieve this goal by displacing the interpalpebral area superiorly without introducing additional risk for corneal exposure.