Achalasia: current treatment options

Abstract

Achalasia is a rare esophageal motility disorder, characterized by impaired swallow-induced, lower esophageal sphincter (LES) relaxation and defective esophageal peristalsis. Unfortunately, there are no etiological therapies for achalasia. Patients present with dysphagia, chest pain and regurgitation of undigested food, often leading to weight loss. The currently available treatments have the common aim of relieving symptoms by decreasing the pressure of the LES. This can be achieved with some medications, by inhibiting the cholinergic innervation (botulinum toxin), by stretching (endoscopic dilation) or cutting (surgery) the LES. Recently, other therapeutic options, including per-oral endoscopic myotomy have been developed and are gaining international consensus. The authors report on the benefits and weaknesses of the different therapies and provide an updated approach to the management of achalasia.

Keywords:

• achalasia
• botulinum toxin injection
• dysphagia
• esophageal manometry
• esophageal myotomy
• high-resolution manometry
• POEM esophageal motility disorders
• pneumatic balloon dilation
• surgery

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Key issues

• Esophageal achalasia is due to loss of the ganglion cells in the myenteric plexus of the esophageal body and the lower esophageal sphincter (LES) that leads to progressive dysphagia and reduced esophageal emptying.

• No etiological therapies are available for achalasia. Current treatments aim at reducing the LES pressure and improve esophageal emptying.

• Nitrates and calcium channel blockers, taken 15–30 min before meals, can only partially reduce the LES pressure.

• Single injection of botulinum toxin is relatively effective at short term, but the efficacy decreases with time and patients may require additional treatments.

• Pneumatic dilation causes disruption of the LES fibers by a forceful stretching with an air-filled balloon. The procedure is easy, reproducible, not expensive and generally effective at mid-term follow-up, if performed by using certain standards and in properly selected patients. The ‘graded dilator’ approach is preferred to improve long-term outcomes and minimize complication rate.

• Peroral endoscopic myotomy, the archetype of submucosal endoscopy, theoretically combines long-term benefits of a surgical myotomy with a trans-oral, minimally invasive approach. The procedure still needs long-term evaluation and comparison with other therapeutic options.

• Laparoscopic Heller myotomy is still considered the gold standard treatment for achalasia. Better results are obtained when myotomy is extended for 3 cm on the gastric wall and when an anti-reflux partial fundoplication is added to the myotomy.

• Esophagectomy is used in cases of abnormal esophageal dilation associated with food retention and severe dysphagia or regurgitation, unresponsive to standard surgical or endoscopic treatments.