New treatment for dysphagia in oculopharyngeal muscular dystrophy. A pilot study onupper oesophageal sphincter dilatation for treatment of dysphagia in patients with oculopharyngeal musculardystrophy


Upper esophageal sphincter (UES) dilatation was done for the treatment of moderate to severe dysphagia with a Maloney bougie in 14 patients with oculopharyngeal muscular dystrophy (OPMD) or with an achalasia dilator in three patients. The severity of dysphagia prior to UES dilatation was evaluated by a 15-point dysphagia score, a pharyngeal and esophageal manometry and a radionuclide pharyngoesophageal transit study. Using actuarial life table, the improvement rate after dilatation with Maloney bougie was 64.3% (95% CI 39.2-89.4) at 3- and 6-month follow-ups, and was 55.7% (95% CI 28.9-82.5) at 12- and 18-month follow-ups. At 3-month post-dilatation, we observed a significant reduction of the mean dysphagia score from 9.6 to 7.2 (P = 0.05). No significant manometric or radionuclide factors were found to predict effective dilatation. The results of this pilot study showed that UES dilatation with Maloney bougie or achalasia dilator may be an effective treatment of moderate dysphagia in patients with OPMD. However, further studies with larger sample sizes are needed to corroborate these results and to assess long-term outcome.

Pour consulter l’article: 10.1016/s0960-8966(97)00092-8


Jean Mathieu, Gilles Lapointe, Anne Brassard, Carmen Tremblay, Bernard Brais, Guy A. Rouleau, Jean-Pierre Bouchard


octobre 1997


Neuromuscular Disorders