Achalasia - an update

Abstract

Achalasia is an esophageal motility disorder of unknown cause, characterized by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The overall success rate of graded pneumatic dilation is 78%, with women and older patients responding best. Laparoscopic myotomy, usually combined with a partial fundoplication, has an overall success rate of 87%. Young patients, especially men, are the best candidates for surgical myotomy. Botulinum toxin injection into the lower esophageal sphincter and smooth muscle relaxants are usually reserved for older patients or those with co-morbid illness. The prognosis for achalasia patients to return to near normal swallowing is good, but the disease is rarely "cured" with a single procedure and intermittent touch-up procedures may be required.

Keywords: Achalasia; Balloon dilation; Botulinum toxin; Esophageal sphincter lower; Muscle, smooth.

Figure 1

Achalasia subtypes by high resolution manometry. (A) Type I (classic achalasia) - there is no significant pressurization within the esophageal body (all dark blue) and impaired lower esophageal sphincter (LES) relaxation (IRP = 42 mmHg). (B) Type II (achalasia with compression) - water swallows cause rapid pan-esophageal pressurization which may exceed LES pressure, causing the esophagus to empty. (C) Type III (spastic achalasia) - although this is also associated with rapidly propagated pressurization, the pressurization is attributable to an abnormal lumen obliterating contraction. (Modified from: Pandolfino JE, Fox MR, Bredenoord AJ, Kahrilas PJ. High resolution manometry in clinical practice: utilizing pressure topography to classify oeosophageal motility abnormalities. Neurogastroenterol Motil 2009;21:796-806). UES, upper esophageal sphincter; IRP, integrated relaxation pressure; CFV, contractile front velocity.

Figure 2

Suggested algorithm for the treatment of achalasia. Healthy patients with low risk of complications after surgery can be offered potentially definitive therapy with either pneumatic dilation or laparoscopic myotomy. Failures are best referred to Esophageal Centers of Excellence with expertise in pneumatic dilation, repeat myotomy, and esophagectomy. High risk patients, especially the elderly, are best treated with botulinum toxin injections. (Updated from the American College of Gastroenterology Practice Guidelines: Diagnosis and management of achalasia. American College of Gastroenterology Practice Parameter Committee. 1999;94:3406-3412, with permission).