Achalasia - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Achalasia is an esophageal motor disorder characterized by a loss of esophageal peristalsis and failure of the lower esophageal sphincter to relax in response to swallowing.
The most common presenting symptoms are dysphagia to solids and liquids, regurgitation, and retrosternal pain. These can be slowly progressive over months or years.
The first investigation for any patient with dysphagia is usually endoscopy to exclude malignancy. Subsequent barium swallow studies and esophageal manometry are often required to establish the diagnosis of achalasia.
Treatment is symptomatic, not curative, and is primarily aimed at relieving dysphagia; options include pharmacologic, endoscopic, and surgical procedures.

Quick Reference

History & Exam
- Key Factors
- Other Factors
More information...
Diagnostics Tests
More information...
Treatment Options
More information...

Definition

Epidemiology

Etiology

Pathophysiology

Images

Classic appearance of dilated esophagus with tapered beak-like narrowing on barium study

Citations

Key Articles

Eckardt VF. Clinical presentations and complications of achalasia. Gastrointest Endosc Clin N Am. 2001 Apr;11(2):281-92.[Abstract]
Zaninotto G, Bennett C, Boeckxstaens G, et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. 2018 Sep 1;31(9).[Abstract][Full Text]
Vaezi MF, Pandolfino JE, Yadlapati RH, et al. ACG clinical guidelines: diagnosis and management of achalasia. Am J Gastroenterol. 2020 Sep;115(9):1393-411.[Abstract][Full Text]

Referenced Articles

1. Sadowski DC, Ackah F, Jiang B, et al. Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil. 2010 Sep;22(9):e256-61.[Abstract]
2. van Hoeij FB, Ponds FA, Smout AJ, et al. Incidence and costs of achalasia in The Netherlands. Neurogastroenterol Motil. 2018 Feb;30(2).[Abstract]
3. Duffield JA, Hamer PW, Heddle R, et al. Incidence of achalasia in South Australia based on esophageal manometry findings. Clin Gastroenterol Hepatol. 2017 Mar;15(3):360-5.[Abstract]
4. Samo S, Carlson DA, Gregory DL, et al. Incidence and prevalence of achalasia in central Chicago, 2004-2014, since the widespread use of high-resolution manometry. Clin Gastroenterol Hepatol. 2017 Mar;15(3):366-73.[Abstract]
5. Kim E, Lee H, Jung HK, et al. Achalasia in Korea: an epidemiologic study using a national healthcare database. J Korean Med Sci. 2014 Apr;29(4):576-80.[Abstract][Full Text]
6. Stein CM, Gelfand M, Taylor HG. Achalasia in Zimbabwean blacks. S Afr Med J. 1985 Feb 16;67(7):261-2.[Abstract]
7. Ho KY, Tay HH, Kang JY. A prospective study of the clinical features, manometric findings, incidence and prevalence of achalasia in Singapore. J Gastroenterol Hepatol. 1999 Aug;14(8):791-5.[Abstract]
8. Birgisson S, Richter JE. Achalasia in Iceland, 1952-2002: an epidemiologic study. Dig Dis Sci. 2007 Aug;52(8):1855-60.[Abstract]
9. Harvey PR, Thomas T, Chandan JS, et al. Incidence, morbidity and mortality of patients with achalasia in England: findings from a study of nationwide hospital and primary care data. Gut. 2019 May;68(5):790-5.[Abstract]
10. Marlais M, Fishman JR, Fell JM, et al. UK incidence of achalasia: an 11-year national epidemiological study. Arch Dis Child. 2011 Feb;96(2):192-4.[Abstract]
11. Haisley KR, Preston JF, Dolan JP, et al. Twenty-year trends in the utilization of Heller myotomy for achalasia in the United States. Am J Surg. 2017 Aug;214(2):299-302.[Abstract]
12. Mayberry JF, Mayell MJ. Epidemiological study of achalasia in children. Gut. 1988 Jan;29(1):90-3.[Abstract][Full Text]
13. Sonnenberg A, Massey BT, McCarty DJ, et al. Epidemiology of hospitalization for achalasia in the United States. Dig Dis Sci. 1993 Feb;38(2):233-44.[Abstract]
14. Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet. 2014 Jan 4;383(9911):83-93.[Abstract][Full Text]
15. Booy JD, Takata J, Tomlinson G, et al. The prevalence of autoimmune disease in patients with esophageal achalasia. Dis Esophagus. 2012 Apr;25(3):209-13.[Abstract]
16. Kaar TK, Waldron R, Ashraf MS, et al. Familial infantile esophageal achalasia. Arch Dis Child. 1991 Nov;66(11):1353-4.[Abstract]
17. Patt H, Koehler K, Lodha S, et al. Phenotype-genotype spectrum of AAA syndrome from Western India and systematic review of literature. Endocr Connect. 2017 Nov;6(8):901-13.[Abstract][Full Text]
18. Roucher-Boulez F, Brac de la Perriere A, Jacquez A, et al. Triple-A syndrome: a wide spectrum of adrenal dysfunction. Eur J Endocrinol. 2018 Mar;178(3):199-207.[Abstract][Full Text]
19. Wouters MM, Lambrechts D, Becker J, et al. Genetic variation in the lymphotoxin-alpha (LTA)/tumour necrosis factor-alpha (TNFalpha) locus as a risk factor for idiopathic achalasia. Gut. 2014 Sep;63(9):1401-9.[Abstract]
20. Ates F, Vaezi MF. The pathogenesis and management of achalasia: current status and future directions. Gut Liver. 2015 Jul;9(4):449-63.[Abstract][Full Text]
21. Park W, Vaezi MF. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol. 2005 Jun;100(6):1404-14.[Abstract]
22. Blam ME, Delfyett W, Levine MS, et al. Achalasia: a disease of varied and subtle symptoms that do not correlate with radiographic findings. Am J Gastroenterol. 2002 Aug;97(8):1916-23.[Abstract]
23. Eckardt VF. Clinical presentations and complications of achalasia. Gastrointest Endosc Clin N Am. 2001 Apr;11(2):281-92.[Abstract]
24. Eckardt VF, Stauf B, Bernhard G. Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology. 1999 Jun;116(6):1300-4.[Abstract]
25. Kopelman Y, Triadafilopoulos G. Endoscopy in the diagnosis and management of motility disorders. Dig Dis Sci. 2011 Mar;56(3):635-54.[Abstract]
26. Expert Panel on Gastrointestinal Imaging; Levy AD, Carucci LR, et al. ACR Appropriateness Criteria®. Dysphagia. J Am Coll Radiol. 2019 May;16(5s):S104-15.[Abstract][Full Text]
27. Spechler SJ, Castell DO. Classification of esophageal motility abnormalities. Gut. 2001 Jul;49(1):145-51.[Abstract]
28. Zaninotto G, Bennett C, Boeckxstaens G, et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. 2018 Sep 1;31(9).[Abstract][Full Text]
29. Fox M. Multiple rapid swallowing in idiopathic achalasia: from conventional to high resolution manometry. Neurogastroenterol Motil. 2007 Sep;19(9):780-1.[Abstract]
30. Kahrilas PJ, Bredenoord AJ, Fox M, et al. Expert consensus document: Advances in the management of oesophageal motility disorders in the era of high-resolution manometry: a focus on achalasia syndromes. Nat Rev Gastroenterol Hepatol. 2017 Nov;14(11):677-88.[Abstract][Full Text]
31. Mariani G, Boni G, Barreca M, et al. Radionuclide gastroesophageal motor studies. J Nucl Med. 2004 Jun;45(6):1004-28.[Abstract]
32. Kostic SV, Rice TW, Baker ME, et al. Timed barium esophagogram: a simple physiologic assessment for achalasia. J Thorac Cardiovasc Surg. 2000 Nov;120(5):935-43.[Abstract][Full Text]
33. Andersson M, Kostic S, Ruth M, et al. Characteristics of timed barium esophagogram in newly diagnosed idiopathic achalasia: clinical and manometric correlates. Acta Radiol. 2007 Feb;48(1):2-9.[Abstract]
34. McMahon BP, Frøkjaer JB, Liao D, et al. A new technique for evaluating sphincter function in visceral organs: application of the functional lumen imaging probe (FLIP) for the evaluation of the oesophago-gastric junction. Physiol Meas. 2005 Oct;26(5):823-36.[Abstract]
35. Carlson DA, Gyawali CP, Kahrilas PJ, et al. Esophageal motility classification can be established at the time of endoscopy: a study evaluating real-time functional luminal imaging probe panometry. Gastrointest Endosc. 2019 Dec;90(6):915-23.e1.[Abstract][Full Text]
36. Pandolfino JE, Kwiatek MA, Nealis T, et al. Achalasia: a new clinically relevant classification by high-resolution manometry. Gastroenterology. 2008 Nov;135(5):1526-33.[Abstract][Full Text]
37. Fox M, Hebbard G, Janiak P, et al. High-resolution manometry predicts the success of oesophageal bolus transport and identifies clinically important abnormalities not detected by conventional manometry. Neurogastroenterol Motil. 2004 Oct;16(5):533-42.[Abstract]
38. Gockel I, Eckardt VF, Schmitt T, et al. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol. 2005 Apr;40(4):378-85.[Abstract]
39. Storm AC, Fishman DS, Buxbaum JL, et al. American Society for Gastrointestinal Endoscopy guideline on informed consent for GI endoscopic procedures. Gastrointest Endosc. 2022 Feb;95(2):207-15.[Abstract][Full Text]
40. ASGE Standards of Practice Committee, Coelho-Prabhu N, Forbes N, et al. Adverse events associated with EGD and EGD-related techniques. Gastrointest Endosc. 2022 Sep;96(3):389-401.e1.[Abstract][Full Text]
41. American College of Radiology. ACR appropriateness criteria: dysphagia. 2018 [internet publication].[Full Text]
42. Vaezi MF, Pandolfino JE, Yadlapati RH, et al. ACG clinical guidelines: diagnosis and management of achalasia. Am J Gastroenterol. 2020 Sep;115(9):1393-411.[Abstract][Full Text]
43. Vela MF, Richter JE, Wachsberger D, et al. Complexities of managing achalasia at a tertiary referral center: use of pneumatic dilatation, Heller myotomy, and botulinum toxin injection. Am J Gastroenterol. 2004 Jun;99(6):1029-36.[Abstract]
44. Spechler SJ. American Gastroenterological Association medical position statement on treatment of patients with dysphagia caused by benign disorders of the distal esophagus. Gastroenterology. 1999 Jul;117(1):229-33.[Abstract][Full Text]
45. Borhan-Manesh F, Kaviani MJ, Taghavi AR. The efficacy of balloon dilation in achalasia is the result of stretching of the lower esophageal sphincter, not muscular disruption. Dis Esophagus. 2016 Apr;29(3):262-6.[Abstract]
46. van Hoeij FB, Prins LI, Smout AJPM, et al. Efficacy and safety of pneumatic dilation in achalasia: A systematic review and meta-analysis. Neurogastroenterol Motil. 2019 Jul;31(7):e13548.[Abstract][Full Text]
47. Borotto E, Gaudric M, Danel B, et al. Risk factors of oesophageal perforation during pneumatic dilatation for achalasia. Gut. 1996 Jul;39(1):9-12.[Abstract][Full Text]
48. Katzka DA, Castell DO. Review article: an analysis of the efficacy, perforation rates and methods used in pneumatic dilation for achalasia. Aliment Pharmacol Ther. 2011 Oct;34(8):832-9.[Abstract]
49. Vanuytsel T, Lerut T, Coosemans W, et al. Conservative management of esophageal perforations during pneumatic dilation for idiopathic esophageal achalasia. Clin Gastroenterol Hepatol. 2012 Feb;10(2):142-9.[Abstract]
50. Eckardt VF, Gockel I, Bernhard G. Pneumatic dilation for achalasia: late results of a prospective follow up investigation. Gut. 2004 May;53(5):629-33.[Abstract][Full Text]
51. Cheng P, Shi H, Zhang Y, et al. Clinical effect of endoscopic pneumatic dilation for achalasia. Medicine (Baltimore). 2015 Jul;94(28):e1193.[Abstract][Full Text]
52. Legros L, Ropert A, Brochard C, et al. Long-term results of pneumatic dilatation for relapsing symptoms of achalasia after Heller myotomy. Neurogastroenterol Motil. 2014 Sep;26(9):1248-55.[Abstract][Full Text]
53. Ponds FA, Fockens P, Lei A, et al. Effect of peroral endoscopic myotomy vs pneumatic dilation on symptom severity and treatment outcomes among treatment-naive patients with achalasia: a randomized clinical trial. JAMA. 2019 Jul 9;322(2):134-44.[Abstract][Full Text]
54. Weber CE, Davis CS, Kramer HJ, et al. Medium and long-term outcomes after pneumatic dilation or laparoscopic Heller myotomy for achalasia: a meta-analysis. Surg Laparosc Endosc Percutan Tech. 2012 Aug;22(4):289-96.[Abstract]
55. Bonifácio P, de Moura DTH, Bernardo WM, et al. Pneumatic dilation versus laparoscopic Heller's myotomy in the treatment of achalasia: systematic review and meta-analysis based on randomized controlled trials. Dis Esophagus. 2019 Feb 1;32(2).[Abstract][Full Text]
56. Schoenberg MB, Marx S, Kersten JF, et al. Laparoscopic Heller myotomy versus endoscopic balloon dilatation for the treatment of achalasia: a network meta-analysis. Ann Surg. 2013 Dec;258(6):943-52.[Abstract]
57. Rohof WO, Salvador R, Annese V, et al. Outcomes of treatment for achalasia depend on manometric subtype. Gastroenterology. 2013 Apr;144(4):718-25.[Abstract][Full Text]
58. Andolfi C, Fisichella PM. Meta-analysis of clinical outcome after treatment for achalasia based on manometric subtypes. Br J Surg. 2019 Mar;106(4):332-41.[Abstract]
59. Csendes A, Braghetto I, Burdiles P, et al. Very late results of esophagomyotomy for patients with achalasia: clinical, endoscopic, histologic, manometric, and acid reflux studies in 67 patients for a mean follow-up of 190 months. Ann Surg. 2006 Feb;243(2):196-203.[Abstract]
60. Midya S, Ghosh D, Mahmalat MW. Fundoplication in laparoscopic Heller's cardiomyotomy for achalasia. Cochrane Database Syst Rev. 2022 Dec 8;(12):CD013386.[Abstract][Full Text]
61. Lake JM, Wong RK. Review article: the management of achalasia - a comparison of different treatment modalities. Aliment Pharmacol Ther. 2006 Sep 15;24(6):909-18.[Abstract]
62. Ali A, Pellegrini CA. Laparoscopic myotomy: technique and efficacy in treating achalasia. Gastrointest Endosc Clin N Am. 2001 Apr;11(2):347-58,[Abstract]
63. Kohn GP, Dirks RC, Ansari MT, et al. SAGES guidelines for the use of peroral endoscopic myotomy (POEM) for the treatment of achalasia. Surg Endosc. 2021 May;35(5):1931-48.[Abstract]
64. NOSCAR POEM White Paper Committee., Stavropoulos SN, Desilets DJ, et al. Per-oral endoscopic myotomy white paper summary. Gastrointest Endosc. 2014 Jul;80(1):1-15.[Abstract][Full Text]
65. Kahrilas PJ, Katzka D, Richter JE. Clinical practice update: the use of per-oral endoscopic myotomy in achalasia: expert review and best practice advice from the AGA Institute. Gastroenterology. 2017 Nov;153(5):1205-11.[Abstract]
66. Schlottmann F, Luckett DJ, Fine J, et al. Laparoscopic heller myotomy versus peroral endoscopic myotomy (POEM) for achalasia: a systematic review and meta-analysis. Ann Surg. 2018 Mar;267(3):451-60.[Abstract]
67. Dirks RC, Kohn GP, Slater B, et al. Is peroral endoscopic myotomy (POEM) more effective than pneumatic dilation and Heller myotomy? A systematic review and meta-analysis. Surg Endosc. 2021 May;35(5):1949-62.[Abstract]
68. Triadafilopoulos G, Aaronson M, Sackel S, et al. Medical treatment of esophageal achalasia: double-blind crossover study with oral nifedipine, verapamil, and placebo. Dig Dis Sci. 1991 Mar;36(3):260-7.[Abstract]
69. Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology. 1982 Nov;83(5):963-9.[Abstract]
70. Rozen P, Gelfond M, Salzman S, et al. Radionuclide confirmation of the therapeutic value of isosorbide dinitrate in relieving the dysphagia in achalasia. Journal Clin Gastroenterol. 1982 Feb;4(1):17-22.[Abstract]
71. Annese V, Bassotti G, Coccia G, et al; GISMAD Achalasia Study Group. A multicentre randomised study of intrasphincteric botulinum toxin in patients with esophageal achalasia. Gut. 2000 May;46(5):597-600.[Abstract][Full Text]
72. Vaezi MF, Richter JE, Wilcox CM, et al. Botulinum toxin versus pneumatic dilatation in the treatment of achalasia: a randomised trial. Gut. 1999 Feb;44(2):231-9.[Abstract][Full Text]
73. Leyden JE, Moss AC, MacMathuna P. Endoscopic pneumatic dilation versus botulinum toxin injection in the management of primary achalasia. Cochrane Database Syst Rev. 2014;(12):CD005046.[Abstract][Full Text]
74. Hoogerwerf WA, Pasricha PJ. Pharmacologic therapy in treating achalasia. Gastrointest Endosc Clin N Am. 2001 Apr;11(2):311-24.[Abstract]
75. Gomes CA Jr, Andriolo RB, Bennett C, et al. Percutaneous endoscopic gastrostomy versus nasogastric tube feeding for adults with swallowing disturbances. Cochrane Database Syst Rev. 2015;(5):CD008096.[Abstract][Full Text]
76. Duranceau A, Liberman M, Martin J, et al. End-stage achalasia. Dis Esophagus. 2012 May;25(4):319-30.[Abstract]
77. Wehrmann T, Kokabpick H, Jacobi V, et al. Long-term results of endoscopic injection of botulinum toxin in elderly achalasic patients with tortuous megaesophagus or epiphrenic diverticulum. Endoscopy. 1999 Jun;31(5):352-8.[Abstract]
78. Annese V, Bassotti G, Coccia G, et al; the GISMAD Achalasia Study Group. Comparison of two different formulations of botulinum toxin A for the treatment of esophageal achalasia. Aliment Pharmacol Ther. 1999 Oct;13(10):1347-50.[Abstract]
79. Mikaeli J, Bishehsari F, Montazeri G, et al. Injection of botulinum toxin before pneumatic dilatation in achalasia treatment: a randomized-controlled trial. Aliment Pharmacol Ther. 2006 Sep 15;24(6):983-9.[Abstract]
80. Stefanidis D, Richardson W, Farrell TM, et al. SAGES guidelines for the surgical treatment of esophageal achalasia. Surg Endosc. 2012 Feb;26(2):296-311.[Abstract]
81. Frankhuisen R, van Herwaarden MA, Heijkoop R, et al. Persisting symptoms and decreased health-related quality-of-life in a cross-sectional study of treated achalasia patients. Aliment Pharmacol Ther. 2007 Sep 15;26(6):899-904.[Abstract]
82. Brucher BL, Stein HJ, Bartels H, et al. Achalasia and esophageal cancer: incidence, prevalence, and prognosis. World J Surg. 2001 Jun;25(6):745-9.[Abstract]
83. Tustumi F, Bernardo WM, da Rocha JRM, et al. Esophageal achalasia: a risk factor for carcinoma. A systematic review and meta-analysis. Dis Esophagus. 2017 Oct 1;30(10):1-8.[Abstract]
84. Dunaway PM, Wong RK. Risk and surveillance intervals for squamous cell carcinoma in achalasia. Gastrointest Endosc Clin N Am. 2001 Apr;11(2):425-34.[Abstract]
85. Zendehdel K, Nyrén O, Edberg A, et al. Risk of esophageal adenocarcinoma in achalasia patients, a retrospective cohort study in Sweden. Am J Gastroenterol. 2011 Jan;106(1):57-61.[Abstract]
86. Ravi K, Geno DM, Katzka DA. Esophageal cancer screening in achalasia: is there a consensus? Dis Esophagus. 2015 Apr;28(3):299-304.[Abstract]