Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

patients awaiting definitive treatment

1st line – pharmacological therapy

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INITIAL
|
patients awaiting definitive treatment
1st line – 

pharmacological therapy

This can be used as a bridge treatment while awaiting definitive intervention. The agents used are either calcium-channel blockers (i.e., nifedipine, verapamil) or nitrates, taken prior to meals.

Patients report a variable improvement in dysphagia and chest pain.

Sublingual isosorbide dinitrate is more potent and has a faster onset of action than nifedipine. It has also been shown to improve oesophageal emptying.

Although nitrates are probably more effective, they are less well tolerated than nifedipine.[66]

Maximum effect occurs in 5-30 minutes with isosorbide dinitrate and 30-120 minutes with calcium-channel blockers.

With long-term use, patients may become tolerant to the effects. Adverse effects of either treatment, such as hypotension and headaches, may limit their use.

Primary options

isosorbide dinitrate: 5-20 mg orally (immediate-release) three times daily; 2.5 to 5 mg sublingually three times daily

OR

nifedipine: 10-30 mg orally (immediate-release) three times daily

OR

verapamil: 80-160 mg orally (immediate-release) three times daily

ACUTE

good surgical candidate

1st line – pneumatic dilatation

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ACUTE
|
good surgical candidate
1st line – 

pneumatic dilatation

Pneumatic dilatation is usually performed under conscious sedation on an outpatient basis. Air-inflated balloons are used to apply mechanical stretch to the lower oesophageal sphincter to tear its muscle fibres. There is some evidence to suggest that the clinical effectiveness of balloon dilatation is not the result of muscular disruption, but of circumferential stretching of the lower oesophageal sphincter.[42] The balloon is inserted endoscopically or by a combined endoscopic-radiological approach.

In one prospective 10-year follow-up study, endoscopic pneumatic dilatation for achalasia was associated with remission rates of 85.7% (6-36 months), 61.9% (37-60 months), and 40% (>60 months).[48]

All patients considered for pneumatic dilatation should be suitable surgical candidates, so that perforation can be surgically repaired if required. There is a perforation rate of up to 5%. Patients in whom the oesophagus is particularly dilated or tortuous, and those with oesophageal diverticula or previous surgery, may be at particular risk of perforation.

Pneumatic dilatation is also a second-line option if cardiomyotomy is unsuccessful. Pneumatic dilatation may have a lower success rate than laparoscopic cardiomyotomy in patients with type II achalasia.[55]

1st line – laparoscopic cardiomyotomy

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ACUTE
|
good surgical candidate
1st line – 

laparoscopic cardiomyotomy

The advent of minimally invasive laparoscopic cardiomyotomy, which has lower morbidity than the open procedure, has made surgery a more attractive option. Cardiomyotomy may be a first-line treatment depending on local expertise, especially in younger patients, or second-line after failed pneumatic dilatation. Systematic reviews and meta-analyses suggest that laparoscopic myotomy is as effective as, or more effective than, pneumatic dilatation.[52][51][53]

Patients with type I or type II achalasia may be more likely to benefit from laparoscopic cardiomyotomy than those with type III achalasia.[28]

Mean 5- and 10-year remission rates in excess of 75% have been reported following laparoscopic cardiomyotomy.[51]

Surgical failure often relates to postoperative gastro-oesophageal reflux.[56] Anti-reflux fundoplication at the time of cardiomyotomy is advocated to address this problem. One Cochrane review compared different types of fundoplications to identify the best suited technique to control acid reflux without worsening dysphagia. No differences between Dor and Toupet fundoplications were observed, and increased post-operative dysphagia was observed with Nissen fundoplication, compared with Dor fundoplication. However, the evidence was of low certainty.[57]​

Other postoperative complications reported include mucosal tear, perforation, or postoperative leakage, occurring in <10% of cases.[58]

1st line – peroral endoscopic myotomy

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ACUTE
|
good surgical candidate
1st line – 

peroral endoscopic myotomy

Peroral endoscopic myotomy (POEM) is a relatively new technique for the treatment of achalasia. In centres with sufficient expertise, it is the treatment of choice for patients with type III achalasia. Society of American Gastrointestinal and Endoscopic Surgeons guidelines recommend using POEM over pneumatic dilatation in patients with achalasia.[60]

POEM is usually performed under general anaesthesia. An incision is made in the mid-oesophageal mucosa, and the endoscope is tunnelled through the submucosa to the gastric cardia. Circular myotomy of the lower oesophageal sphincter and gastric cardia is performed. The length of the myotomy is at least 6 cm. On completion of the dissection the mucosal defect is closed with endoscopic clips. Patients are observed as inpatients post-procedure to monitor for oesophageal perforation and leaks, mediastinitis, bleeding, and cardiopulmonary compromise.[61]

The precision of POEM and the ability to extend the myotomy proximal to the cardia is particularly advantageous for type III achalasia.[62] One randomised trial demonstrated a 2-year success rate of 92% with POEM, compared with 54% with pneumatic dilatation, in treatment-naive patients with achalasia.[50]

One meta-analysis found that POEM was more effective than laparoscopic cardiomyotomy at relieving dysphagia after 2 years follow-up, although there was a much higher incidence of gastro-oesophageal reflux in the POEM group.[63]​ One systematic review and meta-analysis found that the efficacy of POEM was similar to that of Heller myotomy and greater than that of pneumatic dilatation, and the safety outcomes of all three procedures were comparable.[64]

Many patients may require long-term proton pump inhibitor therapy for symptomatic reflux or erosive oesophagitis.

Compared with laparoscopic cardiomyotomy, there is less post-procedural pain and faster recovery after POEM.

Given the complexity of this procedure, POEM should be performed by experienced physicians in high-volume centres.[62]

poor surgical candidate

1st line – botulinum toxin A

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ACUTE
|
poor surgical candidate
1st line – 

botulinum toxin A

The 2020 American College of Gastroenterology clinical guidelines recommend botulinum toxin injection as the first-line therapy for patients with achalasia who are poor surgical candidates.[39]​​

Injection of botulinum toxin into the lower oesophageal sphincter improves dysphagia in about 85% of patients.[68][74][75]

Older patients and those with vigorous achalasia are more likely to respond; however, dysphagia invariably recurs.[68] Although repeat injections can be given, efficacy wanes over time due to development of antibodies against the botulinum toxin.

Botulinum toxin injections cause severe inflammation and scarring of the gastro-oesophageal junction, which is said to increase the technical difficulties and risks of cardiomyotomy.[39][59]​ By contrast, prior botulinum toxin injection does not increase the complication rate of subsequent pneumatic dilatation.[40][76]

Botulinum toxin injection may also be used as a diagnostic tool when the diagnosis of achalasia is not secure.

Primary options

botulinum toxin type A: consult specialist for guidance on dose

2nd line – pharmacological therapy

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ACUTE
|
poor surgical candidate
2nd line – 

pharmacological therapy

Patients who are not suitable candidates or are unwilling to undergo surgery can be maintained on pharmacological therapy, but tolerance can develop with long-term use. Patients report a variable improvement in symptoms of dysphagia and chest pain.

Typically either calcium-channel blockers (i.e., nifedipine, verapamil) or nitrates are used. Sublingual isosorbide dinitrate is more potent and has a faster onset of action than nifedipine. It has been shown to improve oesophageal emptying. However, although nitrates are probably more effective, they are less well tolerated than nifedipine.[66]

Adverse effects of either treatment, such as hypotension and headaches, may limit their use.

Maximum effect occurs in 5 to 30 minutes with isosorbide dinitrate and 30 to 120 minutes with calcium-channel blockers.

Primary options

isosorbide dinitrate: 5-20 mg orally (immediate-release) three times daily; 2.5 to 5 mg sublingually three times daily

OR

nifedipine: 10-30 mg orally (immediate-release) three times daily

OR

verapamil: 80-160 mg orally (immediate-release) three times daily

3rd line – gastrostomy

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ACUTE
|
poor surgical candidate
3rd line – 

gastrostomy

In a frail, older patient who is a poor surgical candidate, a gastrostomy may be considered if previous therapy with botulinum toxin and pharmacological agents has failed or if severe oesophageal dilatation is present.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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