Approach

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

Treatment includes pharmacological, 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.[39]​​

Surgery can be considered after failed dilatation; pneumatic dilatation can be effective following cardiomyotomy failure. Per-oral endoscopic myotomy (POEM) can be used after treatment with either cardiomyotomy or pneumatic dilatation.[28][40][41]​​

Good surgical candidates: pneumatic dilatation

Many gastroenterologists advocate pneumatic dilatation as the first-line therapy. It is performed on an outpatient basis, under sedation. Air-inflated balloons are used to apply mechanical stretch to the lower oesophageal sphincter to tear its muscle fibres. 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 oesophageal sphincter.[42]

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.[43] Mean clinical remission rates of 81% after 6 months and 77% after 12 months were reported following dilatation of up to 30 mm.[43] 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).[43]

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.[44] However, another study found an overall perforation rate for pneumatic dilatation of 2%, regardless of balloon type.[45] Patients with a dilated or tortuous oesophagus, oesophageal diverticula, or previous surgery at the gastro-oesophageal junction may be at a higher risk of perforation. Most patients with perforation after pneumatic dilatations can be treated conservatively.[46]

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.[47] 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).[48] One retrospective study reported remission rates of 72% at 12 months and 49% at 48 months.[49]

In one randomised clinical trial, reflux oesophagitis was reported in 7% of patients who underwent pneumatic dilatation for newly diagnosed achalasia.[50] Gastro-oesophageal 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 myotomy is as effective, or more effective, than pneumatic dilatation.[51][52][53]

Determination of achalasia subtype based on findings from high-resolution oesophageal manometry may help to predict whether pneumatic dilatation or cardiomyotomy will give a better outcome.[54] Patients with type I or type II achalasia may be more likely to benefit from laparoscopic cardiomyotomy than those with type III achalasia.[28] Pneumatic dilatation may have a lower success rate than laparoscopic cardiomyotomy in patients with type II achalasia.[55]

Remission rates and adverse effects

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] Previous non-surgical interventions do not seem to affect the outcome of surgery, but the procedure may be more technically challenging.[40][59]

Good surgical candidates: peroral endoscopic myotomy (POEM)

POEM is a relatively new technique for the treatment of achalasia. In centres with sufficient expertise, it has emerged as the treatment of choice for 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 gastric cardia is particularly advantageous for type III achalasia.[62]

Success rates and adverse effects

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]

Pending definitive treatment/poor surgical candidate: pharmacological 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.[58] Calcium-channel blockers (e.g., nifedipine or verapamil) or nitrates have been shown to lower the resting or mean oesophageal sphincter pressure.[65][66][67] Variable improvement in dysphagia and chest pain scores are reported.[65]

Sublingual isosorbide dinitrate is more potent and has a faster onset of action compared with nifedipine. Isosorbide dinitrate has been shown to improve oesophageal emptying. Although nitrates are probably more effective, they are less well tolerated and are often replaced with nifedipine.[66] With long-term use, patients may become tolerant to the therapeutic effects of either drug.

Poor surgical candidates: botulinum toxin

Botulinum toxin inhibits the release of acetylcholine from nerve terminals, alleviating the effect of the selective loss of inhibitory neurotransmitters that occurs in achalasia. Endoscopic injection of botulinum toxin into the lower oesophageal sphincter decreases the pressure and improves dysphagia, regurgitation, and chest pain.[68]

The injections are as effective as pneumatic dilatation at relieving symptoms, but the effect tends to be transient.[69][70]​ Older patients respond better than younger people, and botulinum toxin is 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.[69] The effectiveness of repeat injections may be diminished by the formation of antibodies to botulinum toxin.[71]

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]​​

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.[41][72] Oesophagectomy is an option for end-stage disease.[39]​​[73]

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