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 – pharmacologic therapy

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INITIAL
|
patients awaiting definitive treatment
1st line – 

pharmacologic 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 esophageal emptying.

Although nitrates are probably more effective, they are less well tolerated than nifedipine.[69]

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

Effective treatment option for patients with type 1 and type 2 achalasia.[43]​ Pneumatic dilatation is usually performed on an outpatient basis. Air-inflated balloons are used to apply mechanical stretch to the lower esophageal sphincter to tear its muscle fibers. 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 esophageal sphincter.[44] The balloon is inserted endoscopically or by a combined endoscopic-radiologic 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).[50]

All patients considered for pneumatic dilatation should be suitable surgical candidates, so that perforation can be surgically repaired if required. In one retrospective study, perforation was found to be more common with the "over-the-scope balloon.[46]​ However, another study found an overall perforation rate for pneumatic dilatation of 2%, regardless of balloon type.[47]​ Patients in whom the esophagus is particularly dilated or tortuous, and those with esophageal diverticula or previous surgery, may be at particular risk of perforation.

Pneumatic dilatation is also a second-line option if cardiomyotomy (also known as Heller myotomy) is unsuccessful. Pneumatic dilatation has an initial lower success rate after the first dilatation compared with laparoscopic cardiomyotomy; however, one European achalasia trial found that graded dilatation improves the efficacy, with both techniques having a comparable success rate after 5 years.[52][53]​​

1st line – laparoscopic cardiomyotomy

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ACUTE
|
good surgical candidate
1st line – 

laparoscopic cardiomyotomy

Effective treatment option for patients with type 1 and type 2 achalasia.[43]​ The advent of minimally invasive laparoscopic cardiomyotomy (also known as laparoscopic Heller myotomy), 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.[55][56][57]

Patients with type 1 or type 2 achalasia may be more likely to benefit from laparoscopic cardiomyotomy than those with type 3 achalasia, in whom peroral endoscopic myotomy is the treatment of choice.[28]​​[36]​​​[43]

Mean 5- and 10-year remission rates in excess of 75% have been reported following laparoscopic cardiomyotomy.[55]

Surgical failure often relates to postoperative gastroesophageal reflux.[59] Antireflux 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 postoperative dysphagia was observed with Nissen fundoplication, compared with Dor fundoplication. However, the evidence was of low certainty.[60]

Other postoperative complications reported include mucosal tear, perforation, or postoperative leakage, occurring in <10% of cases.[61]​ Previous nonsurgical interventions do not seem to affect the outcome of surgery, but the procedure may be more technically challenging.[62][63]

1st line – peroral endoscopic myotomy (POEM)

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ACUTE
|
good surgical candidate
1st line – 

peroral endoscopic myotomy (POEM)

POEM is the treatment of choice for patients with type 3 achalasia.[43]​ Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) guidelines suggest using POEM over pneumatic dilatation in patients with achalasia, unless the patient is particularly concerned about the need for continued proton pump inhibitor (PPI) use postoperatively, in which case both POEM or pneumatic dilation may be considered based on shared decision-making.[36]​ POEM may be superior to pneumatic dilation for patients with failed initial POEM or laparoscopic cardiomyotomy (also known as laparoscopic Heller myotomy).[43]​​

POEM is usually performed under general anesthesia. An incision is made in the mid-esophageal mucosa, and the endoscope is tunneled through the submucosa to the gastric cardia. Circular myotomy of the lower esophageal 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 typically observed as inpatients postprocedure to monitor for esophageal perforation and leaks, mediastinitis, bleeding, and cardiopulmonary compromise.[64] 

The precision of POEM and the ability to extend the myotomy proximal to the cardia is particularly advantageous for type 3 achalasia.[43][65]​​​ One randomized trial demonstrated a 2-year success rate of 92% with POEM, compared with 54% with pneumatic dilatation, in treatment-naive patients with achalasia.[54]

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 gastroesophageal reflux in the POEM group.[66]​ One systematic review and meta-analysis found that the efficacy of POEM was similar to that of cardiomyotomy and greater than that of pneumatic dilatation, and the safety outcomes of all three procedures were comparable.[67]

Many patients require long-term PPI therapy for symptomatic reflux or erosive esophagitis.[36][43]​​​ 

Compared with laparoscopic cardiomyotomy, there is less postprocedural pain and faster recovery after POEM.

Given the complexity of this procedure, POEM should be performed by experienced physicians in high-volume centers.[36][65]​​

poor surgical candidate

1st line – onabotulinumtoxinA or abobotulinumtoxinA

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ACUTE
|
poor surgical candidate
1st line – 

onabotulinumtoxinA or abobotulinumtoxinA

The American College of Gastroenterology (ACG) clinical guidelines recommend botulinum toxin injection as the first-line therapy for patients with achalasia who are poor surgical candidates.[42]​​

Injection of onabotulinumtoxinA or abobotulinumtoxinA, both formerly known as botulinum toxin type A, into the lower esophageal sphincter improves dysphagia in about 85% of patients.[71][77][78]

Older patients and those with vigorous achalasia are more likely to respond; however, dysphagia invariably recurs.[71] Although repeat injections can be given, efficacy wanes over time due to development of antibodies against onabotulinumtoxinA and abobotulinumtoxinA.

OnabotulinumtoxinA and abobotulinumtoxinA injections cause severe inflammation and scarring of the gastroesophageal junction, which is said to increase the technical difficulties and risks of cardiomyotomy.[42][63]​ By contrast, prior onabotulinumtoxinA or abobotulinumtoxinA injection does not increase the complication rate of subsequent pneumatic dilatation.[62][79]

OnabotulinumtoxinA or abobotulinumtoxinA injection may also be used as a diagnostic tool when the diagnosis of achalasia is not secure.

Primary options

onabotulinumtoxinA: consult specialist for guidance on dose

OR

abobotulinumtoxinA: consult specialist for guidance on dose

2nd line – pharmacologic therapy

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ACUTE
|
poor surgical candidate
2nd line – 

pharmacologic therapy

Patients who are not suitable candidates or are unwilling to undergo surgery can be maintained on pharmacologic 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 esophageal emptying. However, although nitrates are probably more effective, they are less well tolerated than nifedipine.[69]

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 onabotulinumtoxinA or abobotulinumtoxinA and pharmacologic agents has failed or if severe esophageal 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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