Long-term results of cricopharyngeal myotomy in oculopharyngeal muscular dystrophy

Abstract

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. EBM rating: C-4