What are the guideline sources, guidelines and references for Achalasia ?
Guideline sources The following summarized guidelines for the evaluation and management of achalasia are prepared by our editorial team based on guidelines from the European Society of Gastrointestinal Endoscopy (ESGE 2020), the American Society for Gastrointestinal Endoscopy (ASGE 2020; 2014), the International Society for Diseases of the Esophagus (ISDE 2018), and the American College of Gastroenterology (ACG 2013). 1 2 3 4 5 6 7 8 9 Definition Achalasia is an esophageal motility disorder defined by loss of esophageal peristalsis and incomplete relaxation of lower esophageal sphincter. 6 Epidemiology Proposed causes of achalasia include GEJ obstruction, neuronal degeneration, viral infection, genetic inheritance, and autoimmune disease. 7 Disease course https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 1/8 6/23/23, 1:27 AM Achalasia Pathway Disease course Inflammatory neurodegenerative insult with possible viral involvement within the esophagus results in achalasia, which causes the clinical manifestation of progressive dysphagia, regurgitation, chest pain, heartburn, nocturnal cough, aspiration, and weight loss. The progression of the disease may result in aspiration-pneumonia, Barrett's esophagus, and esophageal cancer. 8 Prognosis and risk of recurrence Achalasia is not associated with an increase in mortality. 9 Calculator Calculator Pathway Diagnostic criteria for achalasia Eckardt clinical staging of achal Achalasia Investigatio Guidelines 1. Screening and diagnosis Diagnosis: suspect achalasia in patients with dysphagia to solids and liquids, and in patients with regurgitation unresponsive to an adequate trial of PPI therapy. B 2. Classification and risk stratification Disease classification: Use the Chicago classification of esophageal motility disorders to assign patients with achalasia to one of three disease subclasses: Situation Guidance Elevated median integrated relaxation pressure (> 15 mmHg) Type I (classic) 100% failed peristalsis Distal contractile integral < 100 mmHg Premature contractions with distal contractile integral < 450 mmHg cm/sec satisfy criteria for failed peristalsis Elevated median integrated relaxation pressure (> 15 mmHg) Type II (with esophageal compression) 100% failed peristalsis Panesophageal pressurization with 20% of swallows https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 2/8 6/23/23, 1:27 AM Achalasia Pathway Contractions may be masked by esophageal pressurization and distal contractile integral should not be calculated Elevated median integrated relaxation pressure (> 15 mmHg) Type III (spastic) No normal peristalsis Premature (spastic) contractions with distal contractile integral > 450 mmHg cm/sec with 20% of swallows May be mixed with panesophageal pressurization. E Severity grading: use the Eckardt score to assess the severity of achalasia symptoms as part of the initial evaluation and follow-up assessment. E 3. Diagnostic investigations Esophageal manometry: As per ISDE 2018 guidelines, consider obtaining high-resolution esophageal manometry as first- line investigation for the diagnosis of achalasia. C As per ACG 2013 guidelines, obtain esophageal manometry in all patients with suspected achalasia without evidence of mechanical obstruction on endoscopy or esophagram. B Barium esophagram: As per ISDE 2018 guidelines: Consider obtaining a timed barium esophagram as part of the diagnostic evaluation and follow-up assessment of patients with achalasia. C Obtain a timed barium esophagram in patients with suspected end-stage achalasia. E As per ACG 2013 guidelines: Obtain a timed barium esophagram to assess esophageal emptying and esophagogastric junction morphology in patients with equivocal results on esophageal manometry. B Look for esophagram findings supporting the diagnosis of achalasia, such as dilation of the esophagus, a narrow esophagogastric junction with "bird-beak" appearance, aperistalsis, and poor emptying of barium. B Upper gastrointestinal endoscopy: As per ISDE 2018 guidelines, perform upper gastrointestinal endoscopy in adult patients with suspected achalasia to exclude neoplastic pseudoachalasia. E As per ACG 2013 guidelines, obtain endoscopic assessment of the GEJ and gastric cardia to rule out pseudoachalasia. B https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 3/8 6/23/23, 1:27 AM Achalasia Pathway 4. Medical management Pharmacologic therapy: As per ISDE 2018 guidelines, avoid using nitrates, calcium blockers or phosphodiesterase inhibitors for symptomatic relief in patients with achalasia. D As per ACG 2013 guidelines, initiate pharmacologic therapy (CCBs or long-acting nitrates) in patients with achalasia unwilling or unable to undergo definitive treatment with either pneumatic dilatation or surgical myotomy, and failed botulinum toxin therapy. B 5. Therapeutic procedures Choice of initial therapy: As per ASGE 2020 guidelines, offer laparoscopic Heller myotomy, pneumatic dilation, and POEM as effective therapeutic modalities in patients with achalasia. Decide between these treatment options based on achalasia type, local expertise, and patient preference. A Show 3 more As per ASGE 2014 guidelines, discuss both endoscopic and surgical treatment options in patients with achalasia. A As per ACG 2013 guidelines: Individualize the choice of initial therapy according to the patient's age, gender, personal preferences, and local institutional expertise. B Perform pneumatic dilation and surgical myotomy at high-volume centers of excellence. B Pneumatic dilatation: As per ESGE 2020 guidelines, use a graded pneumatic dilation protocol in achalasia, starting with a 30-mm dilation and followed by a 35-mm dilation at a planned interval of 2 - 4 weeks, with a subsequent 40-mm dilation when there is insufficient relief, over both a single balloon dilation procedure or the use of a larger balloon from the outset. A As per ISDE 2018 guidelines, perform graded pneumatic dilatations as an effective treatment in patients with esophageal achalasia. B Show 3 more As per ASGE 2014 guidelines, perform pneumatic dilatation with large-caliber balloon dilators in patients being good candidates and desiring endoscopic management. A As per ACG 2013 guidelines, perform either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication as initial therapy for the surgical treatment of patients with achalasia fitting and willing to undergo surgery. B Peroral endoscopic myotomy: As per ASGE 2020 guidelines, consider performing POEM as the preferred treatment in patients with type III achalasia. C Show 2 more https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 4/8 6/23/23, 1:27 AM Achalasia Pathway As per ESGE 2020 guidelines, perform POEM using low-flow CO insufflation. B Show 3 more As per ISDE 2018 guidelines, consider performing POEM as an effective treatment option in patients with achalasia, with results comparable to Heller myotomy for symptom improvement C and to pneumatic dilation for symptom control. C Show 4 more Updated evidence: POEM for achalasia In patients with symptomatic achalasia, POEM was noninferior to laparoscopic Heller's myotomy with respect to clinical success at 2 years. Werner YB et al. N Engl J Med. 2019 Dec 5. Botulinum toxin injection: As per ASGE 2020 guidelines, do not perform botulinum toxin injection as definitive therapy in patients with achalasia. Reserve botulinum toxin injection for patients unfit for other definitive therapies. D As per ESGE 2020 guidelines: Administer botulinum toxin injection (100 units of the toxin diluted in preservative-free saline injected in aliquots of 0.5 -1 mL) using an injection needle in forward view just above the squamocolumnar junction in at least 4 quadrants. A Avoid administering routine injections of botulinum toxin in the esophageal body in addition to injection in the lower esophageal sphincter in patients with type III achalasia. D As per ISDE 2018 guidelines, do not perform botulinum toxin injection in patients < 50 years old. D Show 4 more As per ASGE 2014 guidelines, perform endoscopic botulinum toxin injection for the treatment of patients with achalasia being poor candidates for surgery or pneumatic dilatation. A As per ACG 2013 guidelines, perform endoscopic botulinum toxin injection for the treatment of patients with achalasia being poor candidates for surgery or pneumatic dilatation. B Other endoscopic therapies: Do not perform implantation of retrievable or absorbable temporary stents for the treatment of patients with achalasia. D Do not perform intrasphincteric injection of ethanolamine oleate or polidocanol for the treatment of patients with achalasia. D 6. Perioperative care https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 5/8 6/23/23, 1:27 AM Achalasia Pathway Perioperative antibiotic prophylaxis: administer perioperative antibiotic prophylaxis for POEM. Adapt the choice and duration of antibiotics according to national or local protocols. B 7. Surgical interventions Surgical myotomy: As per ISDE 2018 guidelines, perform laparoscopic Heller myotomy for symptom control in patients with Chicago type I and type II achalasia. B Show 3 more As per ACG 2013 guidelines, perform either laparoscopic surgical myotomy or graded pneumatic dilatation as initial therapy for the treatment of patients with achalasia fitting and willing to undergo surgery. B Esophagectomy: consider performing esophagectomy in patients with end-stage achalasia failed other interventions. C 8. Specific circumstances Pediatric patients: obtain the same work-up in pediatric patients with a provisional diagnosis of achalasia as in adult patients. E Show 3 more Patients with Chagas disease esophagopathy: consider obtaining the same work-up and offering the same treatments for symptom relief in patients with Chagas disease esophagopathy as in patients with idiopathic achalasia, due to the similarities in manometric and clinical features. C Patients with other spastic motility disorders: Be cautious when treating spastic motility disorders other than achalasia with POEM. B Do not use botulinum toxin injections routinely for the treatment of patients with non-achalasia hypercontractile esophageal motility disorders (Jackhammer esophagus, distal esophageal spasm). Administer injections into 4 quadrants of the lower esophageal sphincter and in the lower 1/3 of the esophagus in individual patients, if endoscopic injection of botulinum toxin is chosen. D 9. Patient education General counseling: inform male patients with achalasia about the moderately increased risk of esophageal cancer after 10 years from the initial treatment. E 10. Follow-up and surveillance Post-treatment follow-up: https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 6/8 6/23/23, 1:27 AM Achalasia Pathway As per ISDE 2018 guidelines: Assess patients for symptomatic improvement after treatment for achalasia. E Consider obtaining a timed barium esophagram to assess for recurrence of achalasia. C As per ACG 2013 guidelines, assess patients for symptomatic improvement after treatment for achalasia, and consider obtaining a timed barium esophagram to assess esophageal emptying. B Management of disease recurrence: As per ASGE 2020 guidelines, consider performing pneumatic dilation or redo myotomy using either the same or an alternative myotomy technique (POEM or laparoscopic Heller myotomy) in patients failed initial myotomy. C As per ISDE 2018 guidelines, obtain objective testing in patients with recurrent symptoms after treatment of achalasia, including upper gastrointestinal endoscopy, barium swallow, manometry, and 24-hour pH monitoring. E Show 3 more Surveillance endoscopy: do not perform routine surveillance endoscopy to screen for esophageal cancer. D References 1. Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013 Aug;108 8 1238 49. Open 2. Zaninotto G, Bennett C, Boeckxstaens G et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. 2018 Sep 1;31 9 . Open 3. Bas L A M Weusten, Maximilien Barret, Albert J Bredenoord et al. Endoscopic management of gastrointestinal motility disorders - part 1 European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2020 Jun;52 6 498 515. Open 4. Mouen A Khashab, Marcelo F Vela, Nirav Thosani et al. ASGE guideline on the management of achalasia. Gastrointest Endosc. 2020 Feb;91 2 213 227.e6. Open 5. Pasha SF, Acosta RD, Chandrasekhara V et al. The role of endoscopy in the evaluation and management of dysphagia. Gastrointest Endosc. 2014 Feb;79 2 191 201. Open 6. Dhyanesh A Patel, Hannah P Kim, Jerry S Zifodya et al. Idiopathic (primary) achalasia: a review. 2015 Jul 22;10 89.2015 Jul 22;10 89. Open 7. Woosuk Park, Michael F Vaezi. Etiology and pathogenesis of achalasia: the current understanding. 2005 Jun;100 6 1404 14.2005 Jun;100 6 1404 14. Open 8. Orla M O'Neill, Brian T Johnston, Helen G Coleman. Achalasia: a review of clinical diagnosis, epidemiology, treatment and outcomes. 2013 Sep 21;19 35 5806 12.2013 Sep 21;19 35 5806 12. Open 9. Volker F Eckardt, Tom Hoischen, Gudrun Bernhard. Life expectancy, complications, and causes of death in patients with achalasia: results of a 33-year follow-up investigation. 2008 Oct;20 10 956 60.2008 https://web.pathway.md/diseases/rec1RhD8XTnEbi1MA 7/8 6/23/23, 1:27 AM Achalasia Pathway Oct;20 10 956 60. Open 10. Werner YB, Hakanson B, Martinek J et al. Endoscopic or Surgical Myotomy in Patients with Idiopathic Achalasia. N Engl J Med. 2019 Dec 5;381 23 2219 2229. Open 11. Ines Gockel, Michaela M ller, Johannes Schumacher. Achalasia a Disease of Unknown Cause That Is Often Diagnosed Too Late. Dtsch Arztebl Int. 2012 Mar; 109 12 209 214. Open 12. Zubin Arora, Prashanthi N Thota, Madhusudhan R Sanaka. Achalasia: current therapeutic options. Ther Adv Chronic Dis. 2017 Jun;8 6 7 101 108. Open 13. Dhyanesh A Patel, Brian M Lappas, Michael F Vaezi. An Overview of Achalasia and Its Subtypes. Gastroenterol Hepatol N Y . 2017 Jul;13 7 411 421. Open 14. Fehmi Ates, Michael F Vaezi. The Pathogenesis and Management of Achalasia: Current Status and Future Directions. Gut Liver. 2015 Jul;9 4 449 63. Open 15. Orla M O Neill, Brian T Johnston, and Helen G Coleman. Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomes. World J Gastroenterol. 2013 Sep 21; 19 35 5806 5812. Open 16. H D Allescher, M Storr, M Seige et al. Treatment of achalasia: botulinum toxin injection vs. pneumatic balloon dilation. A prospective study with long-term follow-Up. Endoscopy. 2001 Dec;33 12 1007 17. Open 17. Maura Torres-Aguilera and Jos Mar a Remes Troche. Achalasia and esophageal cancer: risks and links. Clin Exp Gastroenterol. 2018; 11 309 316. Open 18. Alexander J Eckardt, Volker F Eckardt. Treatment and surveillance strategies in achalasia: an update. Nat Rev Gastroenterol Hepatol. 2011 Jun;8 6 311 9. 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