Snapshot A 45-year-old man presents to his primary care physician complaining of difficulty swallowing solids and liquids. He also reports unintentional weight loss. Introduction Motor disorder of the distal esophagus caused by degeneration of Aurbach's plexus the most common motility disorder Epidemiology more common in people under 50 years of age Pathophysiology autoimmune process causes loss of NO-producing neurons which normally relax the sphincter muscles association with HLA-DQw1 leads to failure of the LES to relax during swallowing results in loss of peristalsis Associated with Chagas' disease amastigotes destroy ganglion cells scleroderma presents in 70% of these patients Presentation Symptoms dysphagia for solids and liquids usually worse for liquids weight loss Evaluation Barium swallow may show narrowing of the distal esophagus loss of peristalsis in the distal two thirds dilated proximal esophagus classic "bird's beak" tapering at the esophageal sphincter Manometry most accurate test that may show increased LES pressure inability of LES to relax decreased peristalsis in the esophageal body diffuse esophageal spasm Upper endoscopy useful in excluding secondary causes of achalasia (i.e. malignancy) use to rule out malignancy shows normal mucosa Differential Diffuse esophageal spasm, GERD, dysphagia, odynophagia, esophageal cancer Treatment Medical management medications to reduce LES tone nifedipine nitrates CCBs botulinum toxin injections wears off in approximately 3-6 months requires reinjection Surgical intervention endoscopic balloon dilation of LES cures 80% leads to perforation in < 3% of patients myotomy with fundoplication more effective and dangerous than pneumatic dilation Prognosis, Prevention, and Complications Prognosis medical and surgical outcomes are similar often require multiple treatments Prevention no preventive measures are available at this time Complications esophageal malignancy secondary to Barrett's esophagus secondary to chronic GERD