Upper esophageal sphincter myotomy in oculopharyngeal muscular dystrophy: long-term clinical results.

From 1980 to 1995, 53 patients with oculopharyngeal muscular dystrophy (OPMD) underwent an upper esophageal sphincter (UES) myotomy for the control of marked dysphagia. From this number, a group of 21 patients had been evaluated for preoperative and postoperative symptoms in 1987. The same clinical assessment was performed in 1995 by an independent evaluator for a total of 37 patients including 12 patients from the first group. As a whole, after a mean follow-up of 6.2 years, surgery succeeded in 18 patients (49%), gave a partial improvement in 12 (32%) and failed in seven (19%). The 12 patients evaluated twice (in 1987 and 1995) have had very good early results, 8-69 months after UES myotomy: dysphagia was totally relieved in eight patients, occurred rarely in three and was moderate in one. Nevertheless, the very long-term follow-up (8 years later) has shown a recurrence of the swallowing and tracheobronchial symptoms in many cases.