Oropharyngeal dysphagia in patients with oculopharyngeal muscular dystrophy

Sixteen patients with an established diagnosis of oculopharyngeal muscular dystrophy underwent clinical, radiologic and manometric assessment. Secondary pharyngo-oral and pharyngonasal regurgitations are usually associated with this condition and chronic aspiration with consequent bronchorrhea is common. Such patients may present with marked oropharyngeal dysphagia. Cineradiologic findings correlated well with the mamometric results. The pharynx showed very weak contractions of longer duration than those observed in normal subjects. The proximal esophageal sphincter had a normal resting and closing pressure; however, relaxation and coordination of the sphincter were substantially different from those in a control group. Eleven patients underwent cricopharyngeal myotomy. All had notable improvement of their symptoms. Surgery on the sphincter results in a substantial decrease in its resting pressure; pharyngeal contraction remains unaltered.