Management of myogenic ptosis in chronic progressive external ophtalmoplegia

Introduction Patients with mitochondrial myopathies, of which chronic progressive external ophthalmoplegia (CPEO) is the most common, can present with ptosis and proximal limb weakness.1 Ptosis associated with CPEO can provide challenging management problems. This form of ptosis may have poor levator function. Additional features must be considered in determining the surgical treatment. For example, these patients may have limited upgaze and a poor Bell’s phenomenon, poor orbicularis function, dry eye, and resultant poor corneal protective mechanisms.2 This would put them at greater risk for exposure keratopathy after ptosis repair. In this article, we describe our experience with a patient with CPEO who had pre-operative moderate levator function and good orbicularis function that after performing levator resection developed severe exposure keratopathy. A 20-year-old man was referred to Farabi Eye Hospital, Tehran, Iran with bilateral lagophthalmos. He had a Hutchinson myopatic face similar to the CPEO facial pattern. He had lid swelling. Visual acuity was 2/10 in both eyes. Slit lamp examination revealed corneal edema, epithelial defects bilaterally, and signs of corneal exposure keratopathy (Figure 1A). The patient had been undergone bilateral levator resection 2 weeks before in a city at North of Iran. His pre-operative visual acuity was 10/10 and bilateral ptosis was present with levator function measuring 8mm in both eyes. The histological report showed ragged red fibers compatible with CPEO. To avoid more severe complications from corneal exposure, tarsorrhaphy was performed on both eyes. After 3 weeks and when corneal signs subsided, one of the tarsorrhaphied eyes was opened, but after 10 days, he represented with signs of exposure keratopathy and again underwent tarsorrhaphy. Tarsorrhaphy and opening of the sutured lids was repeated 4 times in a period of 5 months, and then due to corneal haziness and vascularization the surgery was revised. Both upper eyelids were so tight and without any movement so that after opening of tarsorrhaphy, upper lid retracted and there was not any option unless repeating tarsorrhaphy. In both eyes after opening of the previous incision, the levator was recessed. Lower lid recession with posterior lamellar scleral spacer graft was performed on both eyes for narrowing the vertical palpebral fissure. After this operation, he had mild ptosis with marginal reflex distance measuring 2 mm, and lower lid elevation, so that the vertical palpebral fissure measured 5 mm. Slit lamp examination showed corneal haziness and vascularization in both eyes. Acuity decreased in the right eye to 1/10 and left eye to 4/10. No worsening of signs and symptoms were Iranian Journal of Neurology