Chapter 13 - Epidemiology, Diagnosis, and Medical Management of Achalasia

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Abstract

Achalasia is a rare esophageal disorder of unknown etiology. Its pathophysiology is well understood, and involves the loss of esophageal myenteric neurons, primarily the inhibitory neurons. Such loss results in absent peristaltic activity and impaired relaxation of the lower esophageal sphincter. Dysphagia for solids and liquids is the most common symptom. Esophageal manometry is the key diagnostic procedure, particularly early in the course of the disease. Recent advances in manometric techniques established subtypes of achalasia that can be used to predict response to medical or surgical therapy. Endoscopy and radiology mostly support the diagnosis and help exclude other lesions. Treatment modalities are aimed at reducing the barrier to flow by disrupting lower esophageal sphincter integrity and function. Pneumatic balloon dilation is the main mode of medical therapy, while pharmacotherapy and botulinum toxin injection are reserved for high-risk patients.

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