Abstract

Achalasia is a rare primary esophageal motility disorder characterized by the loss of enteric neurons leading to an absence of peristalsis and impaired relaxation of the lower esophageal sphincter. The subsequent stasis of ingested food not only leads to symptoms of dysphagia, regurgitation, chest pain, and weight loss, but also results in an increased risk of esophageal carcinoma. Structural or functional pulmonary abnormalities occur in more than half of patients and may be due to recurrent aspiration or tracheal compression from a dilated esophagus. Delayed diagnosis or ineffective intervention may lead to progressive dilation of the esophagus and the development of a megaesophagus. Respiratory distress and cardiopulmonary arrest from tracheal or left atrial compression secondary to a megaesophagus are rare complications of achalasia. There is only limited evidence that pneumatic dilation may be used as a first-line therapy for a megaesophagus. The choice of definitive treatment will depend on many factors including achalasia type, patient wishes, performance status, and surgical expertise.

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