Approach

There is no known cure for achalasia, and treatment is symptomatic to reduce dysphagia. The aim is to decrease lower esophageal sphincter pressure and improve esophageal emptying. There are no interventions that can restore esophageal peristalsis. Although swallowing usually improves significantly with treatment, it never returns completely to normal and the patient can only swallow while upright.

Treatment includes pharmacologic, endoscopic, and surgical modalities. Each modality has specific advantages and disadvantages, and choice depends on local expertise and patient preference. As the clinical circumstances change, different treatment modalities may become appropriate.

Initial treatment will depend on whether the patient is a surgical candidate. All patients considered for pneumatic dilatation should be fit enough to undergo surgery so complications can be managed surgically if required.[42]​​

Good surgical candidates

For type 1 and type 2 achalasia, pneumatic dilation, laparoscopic cardiomyotomy (also known as laparoscopic Heller myotomy), and peroral endoscopic myotomy (POEM) are all effective treatment options.[43]​ Treatment decisions should be made collaboratively, considering individual patient factors, disease characteristics, patient preferences, and institutional expertise. For type 3 achalasia, POEM is the treatment of choice.[36][43]​​

Treatment approaches may vary geographically. In Europe, serial dilations are often used as first-line therapy, followed by more definitive management. In contrast, large centers in the US typically start with either POEM or laparoscopic cardiomyotomy.

Good surgical candidates: pneumatic dilatation

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. However, 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 most commonly used balloon dilators enable graded dilatation under fluoroscopic guidance or are passed "over-the-scope."

One systematic review and meta-analysis found that a graded approach, starting with 30 mm dilatation and followed by 35 mm and 40 mm dilatation in the event of inadequate symptom relief, was the safest and most efficient method to dilatation in patients with achalasia.[45] Mean clinical remission rates of 81% after 6 months and 77% after 12 months were reported following dilatation of up to 30 mm.[45] Perforation was more common during initial dilatation. The risk of perforation using a 30 mm balloon was low (1%), and subsequent 35 mm dilatation was safer than initial 35 mm dilatation (0.97% vs. 9.3%, respectively).[45]

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 with a dilated or tortuous esophagus, esophageal diverticula, or previous surgery at the gastroesophageal junction may be at a higher risk of perforation. Most patients with perforation after pneumatic dilatations can be treated conservatively.[48]

Remission rates and adverse effects

Prospectively collected data suggest remission rates of 40% at 5 years and 36% after 10-15 years after a single pneumatic dilatation.[49] In one subsequent 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] One retrospective study reported remission rates of 72% at 12 months and 49% at 48 months.[51]​ Pneumatic dilatation has a lower 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]

In one randomized clinical trial, reflux esophagitis was reported in 7% of patients who underwent pneumatic dilatation for newly diagnosed achalasia.[54] Gastroesophageal reflux is usually mild and responds well to acid suppression.

Good surgical candidates: laparoscopic cardiomyotomy

The advent of minimally invasive laparoscopic cardiomyotomy, which has lower morbidity than the open procedure, has made surgery a more attractive option.

Systematic reviews and meta-analyses suggest that laparoscopic cardiomyotomy is as effective, or more effective, than pneumatic dilatation.[55][56][57]

Determination of achalasia subtype based on findings from high-resolution esophageal manometry may help to predict whether pneumatic dilatation or cardiomyotomy will give a better outcome.[58] 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 POEM is the treatment of choice).[28]​​[36]​​[43]​ 

Remission rates and adverse effects

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]

Good surgical candidates: POEM

POEM is the treatment of choice for 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.[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 post-procedure 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 gastric cardia is particularly advantageous for type 3 achalasia.​[43][65]

Success rates and adverse effects

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 post-procedural 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] ​

Pending definitive treatment/poor surgical candidate: pharmacologic therapy

Drug treatment is used as an initial therapy pending definitive treatment or as a first-line treatment for patients who are poor surgical candidates.[61] Calcium-channel blockers (e.g., nifedipine or verapamil) or nitrates have been shown to lower the resting or mean esophageal sphincter pressure.[68][69][70] Variable improvement in dysphagia and chest pain scores are reported.[68]

Sublingual isosorbide dinitrate is more potent and has a faster onset of action compared with nifedipine. Isosorbide dinitrate has been shown to improve esophageal emptying. Although nitrates are probably more effective, they are less well tolerated and are often replaced with nifedipine.[69] With long-term use, patients may become tolerant to the therapeutic effects of either drug.

Poor surgical candidates: onabotulinumtoxinA and abobotulinumtoxinA

OnabotulinumtoxinA and abobotulinumtoxinA, both formerly known as botulinum toxin type A, inhibit the release of acetylcholine from nerve terminals, alleviating the effect of the selective loss of inhibitory neurotransmitters that occurs in achalasia. Endoscopic injection of onabotulinumtoxinA or abobotulinumtoxinA into the lower esophageal sphincter decreases the pressure and improves dysphagia, regurgitation, and chest pain.[71]

The injections are as effective as pneumatic dilatation at relieving symptoms, but the effects tend to be transient.[72][73]​ Older patients respond better than younger people, and onabotulinumtoxinA or abobotulinumtoxinA are useful in patients who are too frail to undergo more invasive procedures. Initial response is in excess of 80%, but this drops to 68% to 75% after 2 years, even with repeat treatment sessions.[72] The effectiveness of repeat injections may be diminished by the formation of antibodies to onabotulinumtoxinA and abobotulinumtoxinA.[74]

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]

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

Progressive disease despite treatment

In frail and older patients, a gastrostomy is a possibility to allow feeding.​[75] Esophagectomy is an option for end-stage disease.[42]​​[76]

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