• Achalasia is a primary motility disorder in which insufficient relaxation of the lower oesophageal sphincter (LOS) and absent peristalsis result in stasis of ingested foods, subsequently leading to oesophageal symptoms of dysphagia, regurgitation, chest pain or weight loss. (UEG 1) The annual incidence is estimated between 1.07 and 2.2 cases per 100,000 individuals, with prevalence rates estimated between 10 and 15.7 per 100,000 individuals. (UEG 2-4)
• This protocol is intended for clinicians involved in the management of adult achalasia patients, including gastroenterologists, endoscopists, radiologists, gastrointestinal (GI) surgeons and primary-care practitioners.

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Abbreviations

Patients who are initially suspected of having GORD but do not respond to acid-suppressive therapy should be evaluated for achalasia. (ACG) - Recommendation Strong, GRADE Very Low

1. Endoscopy should be performed in all patients with symptoms suggestive of achalasia to exclude other diseases. (UEG, ISDE) - Recommendation Expert opinion

2. High-resolution manometry (HRM) is the gold standard for the diagnosis of achalasia in adult patients. (UEG, ACG, ISDE) - Recommendation Strong, GRADE Moderate

3. Timed barium oesophagram is used to diagnose achalasia if manometry not tolerated. (UEG) - Recommendation Conditional, GRADE Moderate

4. Eckardt score should be used as part of the initial and follow-up assessment in patients with achalasia. (ISDE) - Recommendation Expert opinion

Malignant Pseudo-Achalasia

Malignant Pseudo-achalasia must be ruled out in patients with the following recognised risk factors before embarking on treatment:

Age > 55y

Duration of symptoms < 12 months

Weight loss > 10 kg

Severe difficulty passing the LOS with a scope

Patients with the above risk factors must have the following investigations:

1. Urgent Endoscopy and biopsies

2. CT or endoscopic ultrasound (EUS). (UEG) - Recommendation Conditional, GRADE Low

In the treatment of achalasia, symptom relief should be regarded as the primary aim. (UEG, ISDE) - Recommendation Expert opinion

The improvement of objectively measured oesophageal emptying on timed barium oesophagram should be regarded as an important additional treatment aim. (UEG) - Recommendation Expert opinion

General fitness and manometric subtype should be considered when selecting a therapeutic strategy. (UEG, ISDE) - Recommendation Conditional, GRADE Moderate

PD, LHM, and POEM are comparable effective therapies for type I or type II achalasia. (ACG) - Recommendation Conditional, GRADE Low

Oral Pharmacological Therapy

1. The use of calcium blockers, phosphodiesterase inhibitors or nitrates for the treatment of achalasia is not recommended. (UEG, ISDE) - Recommendation Expert opinion. GRADE Low

Endoscopic Pharmacological Therapy

1. Botox should be reserved for patients who are unfit for more invasive treatments, or in whom a more definite treatment needs to be deferred. (UEG, ACG, ISDE) - Recommendation Strong, GRADE Moderate

2. Recommend against Botox in the oesophageal body, even in the presence of type III achalasia. (ISDE) - Recommendation conditional, GRADE Very low

Pneumatic dilation (PD) (type I and II)

1. Graded PD is an effective and relatively safe treatment for oesophageal achalasia. (UEG) - Recommendation Strong, GRADE High

2. Perforation rates are higher than LHM.

Laparoscopic Heller’s Myotomy (LHM) (type I and II)

1. LHM combined with an anti-reflux procedure is an effective and relatively safe therapy for achalasia. (UEG) - Recommendation Strong, GRADE High

2. Myotomy with fundoplication (Dor or Toupet) is superior to myotomy without fundoplication in controlling distal oesophageal acid exposure. (ACG) - Recommendation Strong, GRADE Moderate

Peroral endoscopic myotomy (POEM) (all types)

1. POEM would be a better treatment option in those with type III achalasia. (ACG) - Recommendation Conditional, GRADE Low

2. Reflux is more common after POEM compared to LHM.

Oesophagectomy

1. Patients considered for this treatment must be discussed at a benign UGI MDT and referred to a tertiary unit.

Self-expanding stents

1. Recommend against stent placement for management of long-term dysphagia in patients with achalasia. (ACG, UEG) - Recommendation Strong, GRADE Low

Persistent or recurrent symptoms

Investigations

1. Timed barium oesophagram (TBO) is the first-line test in evaluating continued or recurrent symptoms after definitive therapy. For type III achalasia, repeat HRM to exclude or confirm persistent spastic contractions is recommended. (ACG, UEG, ISDE) - Recommendation Conditional, GRADE Low

2. Repeat endoscopy should be considered in patients with recurrent dysphagia. (UEG) - Recommendation Expert opinion

3. Eckardt score or HRM alone are not to be used to define treatment failure in evaluating continued or recurrent symptoms after definitive therapy for achalasia. (ACG) - Recommendation Strong, GRADE Very low

4. If there is no evidence of impaired oesophageal emptying, empirical treatment with PPI, endoscopy and/or 24-hour pH-(impedance)metry can be considered. (UEG) - Recommendation Expert opinion

Re-Treatment

Treatment for proven recurrent impaired oesophageal emptying should be discussed within the setting of a complex benign UGI MDT once above investigations are completed, and will depend on the initial treatment modality.

1. LHM should be considered before oesophagectomy in patients who have failed PD and POEM if the anatomy is conducive, and there is evidence of incomplete myotomy. (ACG) - Recommendation Strong, GRADE Very low

2. PD is an appropriate and safe treatment option for patients with achalasia post-initial LHM or POEM in need of retreatment. (ACG) - Recommendation Strong, GRADE Moderate

3. POEM is a safe option in patients with achalasia who have previously undergone PD or LHM. (ACG) - Recommendation Strong, GRADE Low

4. Recurrent or persistent dysphagia after POEM should be treated with either re-POEM, LHM or PD. (UEG) - Recommendation Conditional, GRADE Very low

Reflux post achalasia treatment

PPIs are the first-line treatment of GORD after achalasia treatment. Lifelong PPI therapy in patients with oesophagitis > grade A (LA classification) is recommended. (UEG) - Recommendation Expert opinion

Screening for oesophageal cancer

Performing systematic screening for dysplasia and carcinoma is not recommended. However, the threshold of upper GI endoscopy should be low in patients with recurrent symptoms and long-standing achalasia. (ACG, UEG) - Recommendation Conditional, GRADE Low

Megaoesophagus

Oesophagectomy is recommended in surgically fit patients with megaesophagus who have failed other interventions. (ACG) - Recommendation Strong, GRADE Low. Patient considered for oesophagectomy must be discussed within a Complex Benign UGI MDT and referred to a tertiary centre.

1. Zaninotto, G., et al. "The 2018 ISDE achalasia guidelines." Diseases of the Esophagus 31.9 (2018): doy071. Available from: https://academic.oup.com/dote/article/31/9/doy071/5087687?login=true

2. Oude Nijhuis, R. A. B., et al. "European guidelines on achalasia: United European Gastroenterology and European Society of Neurogastroenterology and Motility recommendations." United European gastroenterology journal 8.1 (2020): 13-33. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7005998/

3. Vaezi, Michael F., et al. "ACG Clinical Guidelines: diagnosis and management of achalasia." The American Journal of Gastroenterology115.9 (2020): 1393-1411. Available from: https://journals.lww.com/ajg/fulltext/2020/09000/acg_clinical_guidelines__diagnosis_and_management.18.aspx

This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date. This guideline is for use in Frimley Health Trust hospitals only. Any use outside this location will not be supported by the Trust and will be at the risk of the individual using it.

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