Abstract
Achalasia is a primary esophageal motility disorder that involves a failure of the lower esophageal sphincter to relax in response to swallowing. Specifically, the lower esophageal sphincter becomes hypertensive, and there is an absence of peristalsis in the esophagus. The pathophysiology is thought to be due to a loss of inhibitory nerve function from an autoimmune attack that targets the esophageal myenteric nerves. As a result, these abnormalities lead to a functional obstruction at the gastroesophageal junction. In severe cases, achalasia may present as a "sigmoid esophagus," a term used to describe the dilation and distortion of the cervical esophagus. In this case report, we discuss a patient with a known history of achalasia who presented with extra-esophageal symptoms including respiratory distress and tracheal compression from an esophagus dilated with a food impaction. She was found to have a sigmoid esophagus and required direct endoscopy and removal of the food bolus. We will review the pathogenesis of achalasia as well as medical and surgical approaches to treating severe achalasia as presented through other case reports.