Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Dysphagia• Retention of ingested food in the esophagus• Radiologic evidence of absent primary peristalsis, dilated body of the esophagus, and a conically narrowed cardioesophageal junction• Absent primary peristalsis by manometry and cineradiography +++ Epidemiology + • Achalasia is a neuromuscular disorder; esophageal dilation and hypertrophy occur without organic stenosis• Primary peristalsis is absent and the cardioesophageal sphincter fails to relax in response to swallowing; the circular muscle layer hypertrophies• There is absence, atrophy, or disintegration of the ganglion cells of Auerbach myenteric plexuses and a reduction in nerve fibers within the wall of the esophagus• The cause is unknown, but 2 theories exist: 1. A degenerative disease of the neurons 2. Infection of neurons by a virus (eg, herpes zoster) or other infectious pathogen• Achalasia affects males more often than females and may develop at any age; peak incidence ranges from 30 to 60 years +++ Symptoms and Signs + • Dysphagia is dominant symptom• Weight loss is not usually marked despite the functional obstruction• Pain is infrequent• Regurgitation of retained esophageal contents is common, especially during the night while the patient sleeps in a recumbent position• A variant called vigorous achalasia is characterized by chest pain and esophageal spasms that generate nonpropulsive high-pressure waves in the body of the esophagus +++ Imaging Findings + • Contrast radiography and endoscopy-Narrowing at the cardia-The dilated body of the esophagus blends into a smooth cone-shaped area of narrowing 3-6 cm long-As the disease progresses, the esophagus dilates further and becomes tortuous• Manometry -The body of the esophagus is devoid of primary peristaltic waves, but simultaneous disorganized muscular activity may be present-Pressure in the gastroesophageal sphincter is increased; relaxation after swallowing is incomplete or absent + • Symptoms should prompt contrast radiographic or endoscopic studies• Endoscopy is essential for establishing the diagnosis and excluding other causes of symptoms• Manometry is useful for confirming diagnosis +++ Rule Out + • Benign strictures of the lower esophagus• Carcinoma at or near the cardioesophageal junction• Diffuse esophageal spasm + • Gastroesophageal contrast radiography• Upper GI endoscopy• Esophageal manometry• pH study, particularly if symptoms of reflux are present or fundoplication is planned + • Goal is to relieve the functional obstruction either by pneumatic dilation or longitudinal division of all the esophageal muscular layers (Heller myotomy). +++ Surgery +++ Indications + • Advanced disease• Failed dilation• If no reflux symptoms exist, the need to add an antireflux procedure has not been established +++ Treatment Monitoring + • Measure rate of esophageal passage of a technetium Tc 99m-labeled solid meal• Periodic esophagoscopy; treatment of achalasia does not lessen the increased risk ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free a profile for additional features.