Achalasia Recommendations

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Authors: Danny French and Anne-Sophie Laliberté

Introduction

Achalasia is a rare primary motility disorder characterized by insufficient relaxation of the lower esophageal sphincter and absent peristalsis. It is more common between 40 and 60 years old and affects both sexes equally. Typical symptoms included dysphagia, regurgitation, weight loss, and atypical chest pain.

Assessment recommendations

High-resolution manometry

–        We strongly recommend high-resolution manometry has the gold standard for achalasia diagnosis and sub-classification.

–        Chicago classification version 3 or 4 should be used.

 

Timed barium swallow

–        We recommend a timed barium swallow as an objective evaluation of the disease and outcomes after myotomy.

–        We recommend timed barium swallow over barium swallow, but barium swallow is a good diagnostic test.

 

Gastroscopy

–        We strongly recommend gastroscopy to rule out pseudoachalasia (ex. Esophageal cancer, peptic stricture, etc.) and assess the esophageal mucosae. But we recommended against gastroscopy alone to make the diagnosis.

 

Eckardt score

–        We recommend the Eckardt score as a simple tool to assess achalasia severity of symptoms.

 

Treatment recommendations

The goal of the treatment is to relieve the symptoms of the patient since there is no curative treatment for achalasia.

Medical treatment with nitrates, calcium channel blockers, or phosphodiesterase inhibitors

–        There is no convincing evidence that nitrates or calcium blockers are effective for symptomatic relief.

–        There is no evidence that phosphodiesterase inhibitors are effective for symptomatic relief.

–        We recommended that medical treatment should not be used as first-line therapy.

 

Botulinum toxin injection

–        We recommend against the injection of Botox as first-line therapy except for patients unfit for surgery

 

Myotomy: Laparoscopic myotomy with or without partial fundoplication, POEM, or pneumatic dilatation

–        The decision between treatment should be based on local expertise, achalasia type, and patient preference.

–        Laparoscopic Heller myotomy, POEM (per oral endoscopic myotomy) and pneumatic dilatation are all effective treatments for achalasia.

–        We recommend POEM for type 3 achalasia if available.

–        Patients that undergo POEM should be counseled regarding the increased risk of postoperative reflux compared to Heller myotomy with partial fundoplication.

–        We recommend adding a partial fundoplication to Heller myotomy to reduce post-operative GERD.

–        Myotomy should be considered as the first-line therapy instead of esophagectomy for the sigmoid esophagus compare to esophagectomy.

Recurrence

–        We recommend routine repeat objective investigations: Timed barium swallow, repeat high-resolution manometry, and gastroscopy.

–        For patients with failed myotomy (Heller or POEM) we recommend redo myotomy or the use of pneumatic dilatation.

–        We recommend esophagectomy in a patient with end-stage achalasia with failed myotomy.

Follow up recommendations

  • We strongly recommend clinical follow-up since achalasia is a chronic disorder with a risk of symptoms recurrence.
  • We recommend treating symptomatic GERD with PPI.
  • We recommend that achalasia patients should be informed of their high risk of aspiration and moderately increased risk of squamous cell carcinoma at 10 or more years from the primary treatment.
  • No association was recommended for routine gastroscopy, but we recommended a low threshold for symptomatic patients

REFERENCES

  1. International high Resolution Manometry Working Group. The Chicago Classification of Esopahgeal Motility Disordes, v3.0. Neurogastroenterol Motil 2015;27(2): 160-174.
  2. Niebich, S. Hadzjusufovic, E, Mehdorn, M. et al. Achalasia- an unnecessary long way to diagnosis. Dis of the esophagus .2017;30:1-6.
  3. Khashab, M.A. Vela, M. F. Thosani, N. et al. ASGE guideline on the management of Achalasia. Gastrointestinal endoscopy. 2020;91(2):213-227.
  4. Nijhuis RAB, Zaninotto G. Roman, S. European Guideline on Achalasia. United European Gastroenterology Journal2020. 8(1): 13-34.
  5. Zaninotto, G. Bennett, Boeckxstaens, G. et al. The 2018 ISDE achalasia guidelines. Disease of the esophagus 2018;31:1-29.
  6. Jung, H-K. Hong, S.J. Lee, O.Y. et al. 2019 Seoul Consensus on Esophageal Achalasia Guidelines. J Neurogastroenterol Motil. 2020;26(2) :2093-0879.
  7. Vaezi, M.F. Pandolfino, J.E. Yadlapati, R.H. et al.ACG Clinical Guidelines : Diagnosis and Management of Achalasia. Am J Gastroenterol 2020;115:1393-1411.