Advances in the Treatment of Achalasia

Esophagus (E Dellon, Section Editor)

Opinion statement

Achalasia, although rare, remains one of the most commonly diagnosed disorders of esophageal motility. It results from an idiopathic loss of ganglion cells responsible for esophageal motility and relaxation of the lower esophageal sphincter (LES). As a result, patients present with worsening dysphagia to both liquids and solids and often suffer from significant regurgitation of retained food in the esophagus. When the diagnosis of achalasia is suspected, patients should undergo evaluation with esophageal motility testing, endoscopic examination, and contrast esophagram. Once the diagnosis of achalasia has been established, options for treatment rely on controlling patient symptoms. Medical options are available, but their effectiveness is inconsistent. Endoscopic options include injection of botulinum toxin, which can achieve good short-term results, and pneumatic balloon dilation (PBD), considered the most effective non-surgical option. Surgical options, including laparoscopic, open, or endoscopic myotomy, and provide long-lasting results. This chapter will review achalasia and the treatment options available.


Achalasia Pneumatic dilation Myotomy POEM Peroral endoscopic myotomy Manometry FLIP 

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology. 2010;139(2):369–74.PubMedCrossRefGoogle Scholar
  2. 2.
    Fisichella PM, Raz D, Palazzo F, Niponmick I, Patti MG. Clinical, radiological, and manometric profile in 145 patients with untreated achalasia. World J Surg. 2008;32(9):1974–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Eckardt VF, Köhne U, Junginger T, Westermeier T. Risk factors for diagnostic delay in achalasia. Dig Dis Sci. 1997;42(3):580–5.PubMedCrossRefGoogle Scholar
  4. 4.
    Goldblum JR, Rice TW, Richter JE. Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology. 1996;111(3):648–54.PubMedCrossRefGoogle Scholar
  5. 5.
    Pandolfino JE, Kahrilas PJ. AGA technical review on the clinical use of esophageal manometry. Gastroenterology. 2005;128(1):209–24.PubMedCrossRefGoogle Scholar
  6. 6.
    Spechler SJ, Castell DO. Classification of oesophageal motility abnormalities. Gut. 2001;49(1):145–51.PubMedCrossRefGoogle Scholar
  7. 7.
    Clouse RE, Staiano A. Topography of normal and high-amplitude esophageal peristalsis. Am J Physiol. 1993;265(6 Pt 1):G1098–107.PubMedGoogle Scholar
  8. 8.
    Pratap N, Reddy DN. Can achalasia subtyping by high-resolution manometry predict the therapeutic outcome of pneumatic balloon dilatation?: author’s reply. J Neurogastroenterol Motil. 2011;17(2):205.PubMedCentralPubMedCrossRefGoogle Scholar
  9. 9.
    Salvador R, Costantini M, Zaninotto G, Morbin T, Rizzetto C, Zanatta L, et al. The preoperative manometric pattern predicts the outcome of surgical treatment for esophageal achalasia. J Gastrointest Surg. 2010;14(11):1635–45.PubMedCrossRefGoogle Scholar
  10. 10.
    Andersson M, Lundell L, Kostic S, Ruth M, Lönroth H, Kjellin A, et al. Evaluation of the response to treatment in patients with idiopathic achalasia by the timed barium esophagogram: results from a randomized clinical trial. Dis Esophagus. 2009;22(3):264–73.PubMedCrossRefGoogle Scholar
  11. 11.
    Kahrilas PJ, Kishk SM, Helm JF, Dodds WJ, Harig JM, Hogan WJ. Comparison of pseudoachalasia and achalasia. Am J Med. 1987;82(3):439–46.PubMedCrossRefGoogle Scholar
  12. 12.
    Sandler RS, Bozymski EM, Orlando RC. Failure of clinical criteria to distinguish between primary achalasia and achalasia secondary to tumor. Dig Dis Sci. 1982;27(3):209–13.PubMedCrossRefGoogle Scholar
  13. 13.•
    Rohof WO, Hirsch DP, Kessing BF, Boeckxstaens GE. Efficacy of treatment for patients with achalasia depends on the distensibility of the esophagogastric junction. Gastroenterology. 2012;143(2):328–35. This article describes the use of a functional luminal imaging probe to evaluate the distensibility of the esophagogastric junction which has shown to be promising in evaluating patients response to therapy.PubMedCrossRefGoogle Scholar
  14. 14.
    Wallin L, Boesby S. Prospective clinical and manometric study comparing pneumatic dilatation and sublingual nifedipine in the treatment of oesophageal achalasia. Gut. 1992;33(2):288.PubMedCrossRefGoogle Scholar
  15. 15.
    Bortolotti M, Coccia G, Brunelli F, Sarti P, Mazza M, Bagnato F, et al. Isosorbide dinitrate or nifedipine: which is preferable in the medical therapy of achalasia? Ital J Gastroenterol. 1994;26(8):379–82.PubMedGoogle Scholar
  16. 16.
    Annese V, Bassotti G, Coccia G, Dinelli M, D’Onofrio V, Gatto G, et al. A multicentre randomised study of intrasphincteric botulinum toxin in patients with oesophageal achalasia. GISMAD Achalasia Study Group. Gut. 2000;46(5):597–600.PubMedCrossRefGoogle Scholar
  17. 17.
    Zaninotto G, Annese V, Costantini M, Del Genio A, Costantino M, Epifani M, et al. Randomized controlled trial of botulinum toxin versus laparoscopic heller myotomy for esophageal achalasia. Ann Surg. 2004;239(3):364–70.PubMedCrossRefGoogle Scholar
  18. 18.
    Smith CD, Stival A, Howell DL, Swafford V. Endoscopic therapy for achalasia before Heller myotomy results in worse outcomes than heller myotomy alone. Ann Surg. 2006;243(5):579–84. discussion 584–6.PubMedCrossRefGoogle Scholar
  19. 19.
    Vela MF, Richter JE, Khandwala F, Blackstone EH, Wachsberger D, Baker ME, et al. The long-term efficacy of pneumatic dilatation and Heller myotomy for the treatment of achalasia. Clin Gastroenterol Hepatol. 2006;4(5):580–7.PubMedCrossRefGoogle Scholar
  20. 20.
    Campos GM, Vittinghoff E, Rabl C, Takata M, Gadenstätter M, Lin F, et al. Endoscopic and surgical treatments for achalasia: a systematic review and meta-analysis. Ann Surg. 2009;249(1):45–57.PubMedCrossRefGoogle Scholar
  21. 21.
    Zerbib F, Thétiot V, Richy F, Benajah D-A, Message L, Lamouliatte H. Repeated pneumatic dilations as long-term maintenance therapy for esophageal achalasia. Am J Gastroenterol. 2006;101(4):692–7.PubMedCrossRefGoogle Scholar
  22. 22.
    Ott DJ, Richter JE, Wu WC, Chen YM, Castell DO, Gelfand DW. Radiographic evaluation of esophagus immediately after pneumatic dilatation for achalasia. Dig Dis Sci. 1987;32(9):962–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Guardino JM, Vela MF, Connor JT, Richter JE. Pneumatic dilation for the treatment of achalasia in untreated patients and patients with failed Heller myotomy. J Clin Gastroenterol. 2004;38(10):855–60.PubMedCrossRefGoogle Scholar
  24. 24.
    Lynch KL, Pandolfino JE, Howden CW, Kahrilas PJ. Major complications of pneumatic dilation and Heller myotomy for achalasia: single-center experience and systematic review of the literature. Am J Gastroenterol. 2012;107(12):1817–25.PubMedCrossRefGoogle Scholar
  25. 25.
    Morino M, Rebecchi F, Festa V, Garrone C. Preoperative pneumatic dilatation represents a risk factor for laparoscopic Heller myotomy. Surg Endosc. 1997;11(4):359–61.PubMedCrossRefGoogle Scholar
  26. 26.
    Ali A, Pellegrini CA. Laparoscopic myotomy: technique and efficacy in treating achalasia. Gastrointest Endosc Clin N Am. 2001;11(2):347–58. vii.PubMedGoogle Scholar
  27. 27.
    Richards WO, Torquati A, Holzman MD, Khaitan L, Byrne D, Lutfi R, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg. 2004;240(3):405–12. discussion 412–5.PubMedCrossRefGoogle Scholar
  28. 28.
    Lopushinsky SR, Urbach DR. Pneumatic dilatation and surgical myotomy for achalasia. JAMA. 2006;296(18):2227–33.PubMedCrossRefGoogle Scholar
  29. 29.••
    Boeckxstaens GE, Annese V, des Varannes SB, Chaussade S, Costantini M, Cuttitta A, et al. Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. N Engl J Med. 2011;364(19):1807–16. Randomized, controlled trial comparing pneumatic dilation versus laparoscopic Heller's myotomy that demonstrated similar short and long term results.PubMedCrossRefGoogle Scholar
  30. 30.
    Kostic S, Kjellin A, Ruth M, Lönroth H, Johnsson E, Andersson M, et al. Pneumatic dilatation or laparoscopic cardiomyotomy in the management of newly diagnosed idiopathic achalasia. Results of a randomized controlled trial. World J Surg. 2007;31(3):470–8.PubMedCrossRefGoogle Scholar
  31. 31.
    Kostic S, Johnsson E, Kjellin A, Ruth M, Lönroth H, Andersson M, et al. Health economic evaluation of therapeutic strategies in patients with idiopathic achalasia: results of a randomized trial comparing pneumatic dilatation with laparoscopic cardiomyotomy. Surg Endosc. 2007;21(7):1184–9.PubMedCrossRefGoogle Scholar
  32. 32.
    Imperiale TF, O’Connor JB, Vaezi MF, Richter JE. A cost-minimization analysis of alternative treatment strategies for achalasia. Am J Gastroenterol. 2000;95(10):2737–45.PubMedCrossRefGoogle Scholar
  33. 33.
    O’Connor JB, Singer ME, Imperiale TF, Vaezi MF, Richter JE. The cost-effectiveness of treatment strategies for achalasia. Dig Dis Sci. 2002;47(7):1516–25.PubMedCrossRefGoogle Scholar
  34. 34.
    Kadakia SC, Wong RK. Pneumatic balloon dilation for esophageal achalasia. Gastrointest Endosc Clin N Am. 2001;11(2):325–46. vii.PubMedGoogle Scholar
  35. 35.
    Sumiyama K, Gostout CJ, Rajan E, Bakken TA, Knipschield MA. Transesophageal mediastinoscopy by submucosal endoscopy with mucosal flap safety valve technique. Gastrointest Endosc. 2007;65(4):679–83.PubMedCrossRefGoogle Scholar
  36. 36.•
    Inoue H, Minami H, Kobayashi Y, Sato Y, Kaga M, Suzuki M, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy. 2010;42(4):265–71. The initial paper describing the use of peroral endoscopic myotomy for the treatment of achalasia.PubMedCrossRefGoogle Scholar
  37. 37.••
    Bhayani NH, Kurian AA, Dunst CM, Sharata AM, Rieder E, Swanstrom LLA (Oct. 2013) Comparative Study on Comprehensive, Objective Outcomes of Laparoscopic Heller Myotomy With Per-Oral Endoscopic Myotomy (POEM) for Achalasia. Ann Surg. The most thorough study evaluating peroral endoscopic myotomy with laparoscopic Heller's myotomy.Google Scholar
  38. 38.
    Teitelbaum EN, Boris L, Arafat FO, Nicodème F, Lin Z, Kahrilas PJ, et al. Comparison of esophagogastric junction distensibility changes during POEM and Heller myotomy using intraoperative FLIP. Surg Endosc. 2013;27(12):4547–55.PubMedCrossRefGoogle Scholar
  39. 39.
    Li Q-L, Chen W-F, Zhou P-H, Yao L-Q, Xu M-D, Hu J-W, et al. Peroral endoscopic myotomy for the treatment of achalasia: a clinical comparative study of endoscopic full-thickness and circular muscle myotomy. J Am Coll Surg. 2013;217(3):442–51.PubMedCrossRefGoogle Scholar
  40. 40.
    Von Renteln D, Fuchs K–H, Fockens P, Bauerfeind P, Vassiliou MC, Werner YB, Fried G, Breithaupt W, Heinrich H, Bredenoord AJ, Kersten JF, Verlaan T, Trevisonno M, and Rösch T (Aug. 2013) Peroral endoscopic myotomy for the treatment of achalasia: an international prospective multicenter study. Gastroenterology 145(2) pp. 309–11.e1–3Google Scholar
  41. 41.
    Bonatti H, Hinder RA, Klocker J, Neuhauser B, Klaus A, Achem SR, et al. Long-term results of laparoscopic Heller myotomy with partial fundoplication for the treatment of achalasia. Am J Surg. 2005;190(6):874–8.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  1. 1.Department of SurgeryUniversity Hospitals Case Medical CenterClevelandUSA

Personalised recommendations