Achalasia is a chronic condition with no cure. Treatment options are palliative in nature and aim to reduce the hypertonicity of the LES. This new ACG guideline addresses the diagnosis, treatment, and overall management of adult patients with achalasia and provides recommendations for tailored treatment.

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Achalasia is an oesophagal motility disorder with a global incidence ranging from 0.03 to 1.63 per 1 lakh persons per year. To date, there is no cure for achalasia and all current treatment options are palliative in nature. The ultimate goals of therapy include reducing symptoms, improving oesophagal emptying, and preventing further dilation of the oesophagus.

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Diagnosis of Achalasia

An incorrect GERD diagnosis can significantly delay the diagnosis.

ACG recommends that patients who are initially suspected of having GERD but do not respond to acid-suppressive therapy should be evaluated for achalasia.

Heartburn is commonly found in patients with achalasia, with about 27 to 42% of patients reporting experiencing heartburn. Due to overlapping symptoms, achalasia is often misdiagnosed as gastroesophageal reflux disease (GERD) and patients are treated with proton pump inhibitors (PPI). An incorrect GERD diagnosis can significantly delay achalasia diagnosis.

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Use of oesophagal pressure topography

Based on the inherent benefit of improved detail in describing oesophagal pressurisation and contractile patterns using oesophagal pressure topography and superior accuracy and reproducibility in diagnosing achalasia in both randomised controlled and blinded comparison studies, we recommend using oesophagal pressure topography over conventional line tracing for the diagnosis of achalasia.

High-resolution manometry (HRM) is the current gold standard test for the confirmation of achalasia. Compared to conventional low-resolution pressure tracing manometry, HRM leverages improved space-time resolution and a more intuitive description of contractile and pressure patterns to refine the classification of motor dysfunction. This classification improves accuracy, reproducibility and the ability to distinguish clinically relevant subtypes.

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Classifying achalasia subtypes

Achalasia is divided into 3 distinct manometric subtypes. All 3 subtypes have impaired esophagogastric junction (EGJ) relaxation, but differing patterns of oesophagal pressurisation and contraction.

Based on these observations, we suggest that classifying achalasia subtypes by the Chicago Classification may help inform both prognosis and treatment choice because type II patients have very good outcomes, regardless of which therapy is selected, and type III patients require a more extensive myotomy.

Prior to HRM and oesophagal pressure topography, patients with achalasia were grouped as a single disease and the treatment decisions were not tailored based on physiology or anatomy. Treatment modalities focused on disrupting the lower oesophagal sphincter (LES) via dilation or myotomy. Although this approach offered very good outcomes over a short duration, treatment failures over the first 1–5 years were found to be as high as 10 to 20%.

The achalasia subtypes in the Chicago Classification were created to subtype vigorous achalasia and variants into a more uniform scheme to determine whether these subtypes had different pathogenic features and response to therapy. Studies support that achalasia subtypes defined in the Chicago Classification have prognostic value and varying outcomes across therapies.

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Initial treatment options

The current treatment options in achalasia include pharmacologic, endoscopic, and surgical means. Pharmacologic therapy is the least effective treatment option in achalasia. Calcium channel blockers and nitrates are the two most commonly used medications in treating achalasia. Pharmacotherapy should be reserved for patients with achalasia who are not candidates for definitive therapies of pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), or peroral endoscopic myotomy (POEM) and have failed botulinum toxin injection.

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Botulinum toxin injection

The guideline recommends botulinum toxin injection as first-line therapy for patients with achalasia that are unfit for definitive therapies compared with other less-effective pharmacological therapies.

Botulinum toxin is a useful treatment in achalasia. Most treatment effects are associated with an approximate 50% reduction in the basal LES pressure. This reduction may be sufficient to allow oesophagal emptying when oesophagal pressure rises to a level where it can overwhelm the partially paralysed LES.

Botulinum toxin is an effective treatment option, but its benefits are short-lived, and the medication should not be offered as first-line treatment to patients who are fit for myotomy.

The panel recommends that treatment with botulinum toxin injection does not significantly affect the performance and outcomes of myotomy.

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Pneumatic dilation

Based on no evidence to support routine esophagram and the current shift in practice patterns to perform endoscopy after dilation to rule out and potentially treat perforation endoscopically, we do not suggest obtaining routine gastrograffin esophagram after dilation.

Pneumatic dilation is associated with good to excellent relief of symptoms in 50 to 93% of patients with achalasia. Serial pneumatic dilation is an effective treatment option for short- and long-term symptom and physiologic benefit.

Oesophagal perforation is the most serious complication associated with pneumatic dilation. Early recognition and management of perforation can improve patient outcomes. Antibiotic, parenteral nutrition, and stent placement may be effective in small perforation, but in large and extensive mediastinal contamination, surgical repair through thoracotomy should be preferred.

There is no role for routine esophagram to rule out perforation in post-dilation recovery. This test should be reserved for patients with a clinical suspicion for perforation after dilation.

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Surgical myotomy

Surgical myotomy is one of the 3 definitive therapies for achalasia. Laparoscopic myotomy is the preferred method because of decreased morbidity and faster recovery.

Fundoplication postmyotomy

Based on available data, the panel recommends that myotomy with fundoplication is superior to myotomy without fundoplication in controlling distal oesophagal acid exposure.

The development of GERD after myotomy is a frequent problem, and whether an antireflux procedure should be performed to prevent reflux has been the subject of extensive debate, especially given concerns for increased postoperative dysphagia after a fundoplication.

The addition of fundoplication after myotomy is associated with decreased risk of GERD for thoracotomy, laparotomy, and laparoscopy. The addition of fundoplication to myotomy reduces the incidence of distal oesophagal acid exposure. The benefits of fundoplication are sustained long term.

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Dor and Toupet antireflux procedure after myotomy

Based on current data, the guideline suggests either Dor or Toupet fundoplication to control oesophagal acid exposure in patients with achalasia undergoing surgical myotomy.

Adding a fundoplication is beneficial for reducing the rate of GERD after myotomy, however, there is less certainty on the best approach (anterior Dor or posterior Toupet).

Studies show Toupet fundoplication to be superior to Dor by reducing the length of hospital stay and improving patient’s quality of life.

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Peroral endoscopic myotomy (POEM)

Based on current data, tailored POEM or LHM for type III achalasia is recommended as a more efficacious disruptive therapy of the LES compared with PD.

POEM is a hybrid technique developed to incorporate an endoscopic approach with principles of natural orifice transluminal endoscopic surgery to perform a myotomy. POEM is commonly used in type III achalasia.

With POEM, the length of the myotomy can be tailored and the entire length of the smooth muscle of the oesophagus can be included if necessary. This length can be tailored to findings of the length of the spastic segment noted on high-resolution oesophagal manometry, length of oesophagal wall thickening noted on endoscopic ultrasound, or functional lumen imaging probe (FLIP).

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GERD post-POEM

The panel supports the evidence that in patients with achalasia, POEM compared with LHM with fundoplication or PD is associated with a higher incidence of GERD.

As GERD is a common issue post-POEM, it is suggested to screen patients who undergo POEM for erosive esophagitis or Barrett's oesophagus. Patients who are considering POEM should be advised that lifelong acid suppression with PPIs may potentially be needed.

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Esophagectomy

Based on these limited data, esophagectomy in surgically-fit patients with megaesophagus who have failed other interventions is recommended.

Patients with “end-stage” achalasia and those with untreated achalasia are at risk of aspiration, aspiration pneumonia, and malnutrition. In these set of patients, PD, surgical myotomy, or POEM may be less effective. Those with compromised nutrition may require enteral feeding.

Esophagectomy is associated with a high incidence of postoperative respiratory complications including pneumonia, but the intervention shows reasonably low mortality in carefully selected individuals treated at highly specialized surgical centres. Gastric interposition is the first choice of therapy in most patients undergoing esophagectomy.