Inferior constrictor myotomy in oculopharyngeal muscular dystrophy: clinical and manometric evaluation

Abstract

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant transmitted condition seen mainly in the French Canadian population. Twenty-one patients with an established diagnosis of this rare disease underwent inferior constrictor myotomy. All patients were evaluated clinically and 12 of them underwent pharyngoesophageal manometric evaluation both preoperatively and postoperatively. All patients presented with severe oropharyngeal dysphagia and frequent aspiration together with pharyngooral and pharyngonasal regurgitation. Our results showed that all symptoms were significantly improved after inferior constrictor myotomy. Manometric assessment showed significant differences in pressure, duration and frequency of pharyngeal contraction when compared with a control group. After surgery pharyngeal contraction frequency was significantly lowered toward the normal level but other parameters of pharyngeal function remained unchanged. At the level of the upper esophageal sphincter, resting and closing pressure, relaxation time, relaxation and coordination were studied. There was no significant difference between control subjects and OPMD patients for these variables. Surgery significantly lowered the resting and closing pressure of the sphincter but did not modify significantly the other parameters.