BACKGROUND: Congenital cricopharyngeal achalasia is a rare condition in which a newborn presents with dysphagia, choking, nasal reflux, and salivation. Awareness of this condition is important because simple myotomy of the cricopharyngeus muscle often solves the problem. METHODS: The diagnosis and the surgical management of cricopharyngeal achalasia were reviewed based on 4 cases experienced over the last 13 years. RESULTS: The severity of the symptoms and the age of onset were dependent on the severity of achalasia. The cine-fluoroscopic swallow is an important diagnostic procedure. Surgery should be performed early enough for infants to learn how to swallow properly. The surgical procedure involves complete myotomy of cricopharyngeus, allowing the submucosa of the esophagus to bulge out in the posterior midline region. Postoperatively, nasopharyngeal reflux may resolve immediately or decrease over several months, depending on the timing of surgical intervention and the patient's ability to learn the act of swallowing. CONCLUSIONS: Early surgical intervention for this disease is recommended to achieve early recovery from dysphagia and to establish buccopharyngeal swallowing during the appropriate period of development.
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