Surgical results and pathological findings in the oculopharyngeal dystrophy syndrome

The oculopharyngeal dystrophy syndrome is an autosomal dominant disorder commonly seen in patients of French-Canadian descent. The syndrome is characterized by progressive ptosis and dysphagia, with onset in midlife. In this series 26 patients (47 eyelids) underwent surgery to correct the ptosis. The aponeurotic advancement technique, performed in 28 of the lids, resulted in an average elevation of 2.5 mm (mean follow-up interval 8.6 months). The combined aponeurosis-Müller's muscle advancement technique, performed in 17 lids, resulted in an average elevation of 3.5 mm (mean follow-up period 8.0 months). Müller's muscle as well as the levator muscle was found to be involved in the dystrophic process.