Primary Cricopharyngeal Achalasia: A Case Report

Abstract

A case of primary cricopharyngeal achalasia is reported. It is a disease of unknown etiology characterized by a failure of relaxation of the cricopharyngeal muscle which acts as the upper esophageal sphincter.
A 70-year-old man was admitted to the hospital because of severe dysphagia. He had had progressive difficulty in swallowing for 9 years, having been treated by relaxing agents and esophageal dilatation. At the time of admission, he was unable to swallow even his saliva, being dehydrated and had rales over the chest bilaterally. No neuropathy or myopathy was found on examination. However, a barium swallow examination showed arrest of bolus at the cricopharyngeal level and esophagoscopy revealed a tightly contracted cricopharyngeal muscle. After the diagnosis of achalasia was made, the patient underwent extramucosal cricopharyngeal myotomy unilaterally under general anesthesia. The muscle was neither hypertrophic nor atrophic, but contractive. The postoperative course was uneventful and the patient could take normal diet on the 14th postoperative day. A half year postoperatively he has no complaints in swallowing.
Primary cricopharyngeal achalasia is generally seen in elder patients, causing unremitting, progressive and disabling dysphagia, and is hardly curable by conservative treatment. Further, aspiration pneumonitis which endangers the patient's life may result from regurgitation and weight loss may be a predominant symptom. Therefore, an early surgical intervention by cricopharyngeal myotomy is recommended when the diagnosis is established. The operation is safe and simple, and unilateral myotomy would suffice.