Long-term results of cricopharyngeal myotomy in oculopharyngeal muscular dystrophy

OBJECTIVE: To analyze long-term results of extramucosal cricopharyngeal myotomy in oculopharyngeal muscular dystrophy. STUDY DESIGN: The preoperative and postoperative evaluations including symptoms, type of feeding, weight, and functional examinations were retrospectively evaluated in 39 patients. Results were defined postoperatively as successful, partial, or failed. SETTING: University hospital. RESULTS: In the short term, 25 patients showed a complete remission of symptoms, 10 showed a marked improvement, and 4 exhibited no improvement at all (success rate of 90%). Long-term evaluation during a mean follow-up of 4 years showed that of the 35 improved patients, 12 exhibited a recurrence of dysphagia (mean time of 39 months). In 3 of these 12 patients, a revision transmucosal endoscopic cricopharyngeal myotomy was performed with a successful outcome in 2. Of the 4 patients who initially showed no improvement, 1 was improved by transmucosal myotomy. CONCLUSION: Extramucosal cricopharyngeal myotomy improves dysphagia in oculopharyngeal muscular dystrophy patients during the first few years but one third of the patients exhibited a recurrence of symptoms within 3 years. Revision by transmucosal endoscopic myotomy may be performed.