Surgery in oculopharyngeal muscular dystrophy.

Oculpharyngeal muscular dystrophy is an autosomal dominant inherited condition seen mainly in patients of French Canadian origin. Fifteen patients with an established diagnosis of the disease were evaluated clinically, manometrically and radiologically before and after cricopharyngeal myotomy. All patients presented with oropharyngeal dysphagia to solids and liquids, pharyngooral and pharyngonasal regurgitation, frequent aspiration at mealtime and chronic aspiration of saliva during the night. When compared with a control group, significant differences were observed in the pressure, duration and frequency of pharyngeal contraction. Cricopharyngeal myotomy did not alter pharyngeal function. At the level of the upper esophageal sphincter, resting and contraction pressure relaxation, relaxation time and coordination were examined. Relaxation time was the only significant abnormality observed between patients with oculopharyngeal muscular dystrophy and control subjects. Surgery significantly lowered the resting and contracting pressures of the sphincter. Relaxation time was brought toward normal. All patients' symptoms were significantly improved by cricopharyngeal myotomy.