Surgical treatment of blepharoptosis caused by chronic progressive external ophthalmoplegia.

Abstract

Chronic progressive external ophthalmoplegia (CPEO) is a neuromyopathic disorder characterized by progressive weakness of the extraocular and levator muscles, which causes blepharoptosis and impairment of ocular motility. Because of the risk of worsening of lagophthalmos and exposure keratitis due to an associated poor Bell phenomenon or weak orbicularis function, surgical treatment of the blepharoptosis caused by CPEO is problematic. We present our experience with a case of blepharoptosis in CPEO. A 61-year-old woman presented with slowly progressive bilateral blepharoptosis. A muscle biopsy of the rectus femoris revealed mitochondrial abnormalities, which satisfied the definition of the diagnosis of CPEO as mitochondrial encephalomyopathy. The lid opening was 2 mm, with maximal frontalis contraction, the levator function was zero, and the eyeball movement was severely limited. The blow movement was about 6 mm. Combination of modest blepharoplasty and frontalis suspension using a monofilament suture was performed. The postoperative result was satisfactory, and the patient's quality of life was markedly improved.