Achalasia results from progressive degeneration of ganglion cells in the myenteric plexus in the esophageal wall leading to failure of relaxation of the lower esophageal sphincter (LES), often accompanied by a loss of peristalsis in the distal esophagus.
This topic will review the management of achalasia. Our recommendations are largely consistent with a 2013 guideline from the American College of Gastroenterology (ACG) and the American Gastroenterological Association (AGA) guidelines [1,2]. The pathophysiology, etiology, clinical manifestations, and diagnosis of achalasia, as well as a more detailed discussion of pneumatic dilation, botulinum toxin injection, and surgical management of achalasia, are presented separately. (See "Pathophysiology and etiology of achalasia" and "Clinical manifestations and diagnosis of achalasia" and "Pneumatic dilation and botulinum toxin injection for achalasia" and "Operative management of achalasia: Laparoscopic and open Heller myotomy".)
Treatment of achalasia is aimed at decreasing the resting pressure in the lower esophageal sphincter (LES) to a level at which the sphincter no longer impedes the passage of ingested material (image 1) [3,4]. This can be accomplished by mechanical disruption of the muscle fibers of the LES (eg, pneumatic dilation or surgical myotomy) or by biochemical reduction in LES pressure (eg, injection of botulinum toxin, oral nitrates, or calcium channel blockers) (algorithm 1).
Choice of treatment — For patients who are at low surgical risk, either graded pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication should be performed to treat achalasia. Pneumatic dilation and surgical myotomy should be performed in high-volume centers of excellence and the choice of initial therapy should be guided by the patients' age, gender, preference, and local institutional expertise. Patients with the best outcomes after pneumatic dilation are those older than 40 years, women, those with narrow esophageal diameter, and those with a type II pattern by high-resolution manometry [5-10]. (See "Pneumatic dilation and botulinum toxin injection for achalasia", section on 'Predictors of outcome' and "Clinical manifestations and diagnosis of achalasia", section on 'High resolution manometry'.)
For patients who elect to undergo pneumatic dilation, we suggest surgical myotomy if symptoms persist despite three attempts at pneumatic dilation. (See 'Pneumatic dilation' below and "Pneumatic dilation and botulinum toxin injection for achalasia", section on 'Predictors of outcome' and 'Surgical myotomy' below.)