Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomes

World Journal of Gastroenterology

Volumn 19, Issue 35, 2013, Pages 5806-5812

  O'Neill, Orla M ^a   Johnston, Brian T ^b   Coleman, Helen G ^a  

^a QUEEN'S UNIVERSITY BELFAST   (United Kingdom)

^b ROYAL GROUP OF HOSPITALS TRUST   (United Kingdom)

Author keywords

Achalasia; Epidemiology; Incidence; Oesophageal cancer risk; Treatment

Indexed keywords

BOTULINUM TOXIN; CALCIUM CHANNEL BLOCKING AGENT; NITRATE;

ASPIRATION PNEUMONIA; AUTOIMMUNITY; BALLOON DILATATION; CARDIOESOPHAGOMYOTOMY; DISEASE CLASSIFICATION; DYSPHAGIA; ENDOSCOPY; ENVIRONMENTAL EXPOSURE; ESOPHAGEAL ADENOCARCINOMA; ESOPHAGEAL SQUAMOUS CELL CARCINOMA; ESOPHAGOGRAPHY; ESOPHAGUS ACHALASIA; ESOPHAGUS MANOMETRY; ESOPHAGUS MOTILITY; HERPES SIMPLEX VIRUS 1; HUMAN; INCIDENCE; INFLAMMATION; LOWER ESOPHAGUS SPHINCTER; MEGAESOPHAGUS; METAL STENT; MYENTERIC PLEXUS; OUTCOME ASSESSMENT; PREVALENCE; PROGNOSIS; REVIEW; RISK; SURVIVAL; TOPOGRAPHY; TREATMENT RESPONSE;

ACHALASIA; EPIDEMIOLOGY; INCIDENCE; OESOPHAGEAL CANCER RISK; TREATMENT;

ESOPHAGEAL ACHALASIA; HUMANS; PREDICTIVE VALUE OF TESTS; RISK FACTORS; TREATMENT OUTCOME;

EID: 84885575984     PISSN: 10079327     EISSN: 22192840     Source Type: Journal    
DOI: 10.3748/wjg.v19.i35.5806     Document Type: Review

References (60)

1. Cotran RS, Kumar V, Collins T. Robbins Pathologic basis of disease. 6th ed. Philadelphia: WB Saunders, 1999: 778-779
2. Kumar P, Clark M. Clinical Medicine. 4th ed. Edinburgh: WB Saunders, 1998: 229-231
3. Pohl D, Tutuian R. Achalasia: an overview of diagnosis and treatment. J Gastrointestin Liver Dis 2007; 16: 297-303 [PMID: 17925926]
4. Bredenoord AJ, Fox M, Kahrilas PJ, Pandolfino JE, Schwizer W, Smout AJ. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. Neurogastroenterol Motil 2012; 24 Suppl 1: 57-65 [PMID: 22248109 DOI: 10.1111/ j.1365-2982.2011.01834.x]
5. Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W, Post J, Kahrilas PJ. Achalasia: a new clinically relevant classification by high-resolution manometry. Gastroenterology 2008; 135: 1526-1533 [PMID: 18722376 DOI: 10.1053/ j.gastro.2008.07.022]
6. 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: 328-335 [PMID: 22562023 DOI: 10.1053/ j.gastro.2012.04.048]
7. Pandolfino JE, de Ruigh A, Nicodème F, Xiao Y, Boris L, Kahrilas PJ. Distensibility of the esophagogastric junction assessed with the functional lumen imaging probe (FLIP™) in achalasia patients. Neurogastroenterol Motil 2013; 25: 496-501 [PMID: 23413801 DOI: 10.1111/nmo.12097]
8. Boeckxstaens GE. Achalasia: virus-induced euthanasia of neurons? Am J Gastroenterol 2008; 103: 1610-1612 [PMID: 18557706 DOI: 10.1111/j.1572-0241.2008.01967.x]
9. Gockel I, Müller M, Schumacher J. Achalasia--a disease of unknown cause that is often diagnosed too late. Dtsch Arztebl Int 2012; 109: 209-214 [PMID: 22532812 DOI: 10.3238/arztebl. 2012.0209]
10. Park W, Vaezi MF. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol 2005; 100: 1404-1414 [PMID: 15929777 DOI: 10.1111/ j.1572-0241.2005.41775.x]
11. Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology 2010; 139: 369-374 [PMID: 20600038 DOI: 10.1053/j.gastro.2010.06.024]
12. Howard PJ, Maher L, Pryde A, Cameron EW, Heading RC. Five year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh. Gut 1992; 33: 1011-1015 [PMID: 1398223]
13. Birgisson S, Richter JE. Achalasia in Iceland, 1952-2002: an epidemiologic study. Dig Dis Sci 2007; 52: 1855-1860 [PMID: 17420933 DOI: 10.1007/s10620-006-9286-y]
14. Sadowski DC, Ackah F, Jiang B, Svenson LW. Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil 2010; 22: e256-e261 [PMID: 20465592 DOI: 10.1111/j.1365-2982.2010.01511.x]
15. Mayberry JF, Atkinson M. Variations in the prevalence of achalasia in Great Britain and Ireland: an epidemiological study based on hospital admissions. Q J Med 1987; 62: 67-74 [PMID: 3423207]
16. Mayberry JF, Rhodes J. Achalasia in the city of Cardiff from 1926 to 1977. Digestion 1980; 20: 248-252 [PMID: 6967027]
17. Mayberry JF, Atkinson M. Studies of incidence and prevalence of achalasia in the Nottingham area. Q J Med 1985; 56: 451-456 [PMID: 4048387]
18. Arber N, Grossman A, Lurie B, Hoffman M, Rubinstein A, Lilos P, Rozen P, Gilat T. Epidemiology of achalasia in central Israel. Rarity of esophageal cancer. Dig Dis Sci 1993; 38: 1920-1925 [PMID: 8404415]
19. Earlam RJ, Ellis FH, Nobrega FT. Achalasia of the esophagus in a small urban community. Mayo Clin Proc 1969; 44: 478-483 [PMID: 5788257]
20. Galen EA, Switz DM, Zfass AM. Achalasia: incidence and treatment in Virginia. Va Med 1982; 109: 183-186 [PMID: 7080659]
21. Mayberry JF, Atkinson M. Incidence of achalasia in New Zealand, 1980-1984. An epidemiological study based on hospital discharges. J Gastroenterol Hepatol 1988; 3: 247-257
22. Stein CM, Gelfand M, Taylor HG. Achalasia in Zimbabwean blacks. S Afr Med J 1985; 67: 261-262 [PMID: 3983775]
23. Farrukh A, DeCaestecker J, Mayberry JF. An epidemiological study of achalasia among the South Asian population of Leicester, 1986-2005. Dysphagia 2008; 23: 161-164 [PMID: 18027026 DOI: 10.1007/s00455-007-9116-1]
24. 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; 14: 791-795 [PMID: 10482430]
25. Gennaro N, Portale G, Gallo C, Rocchietto S, Caruso V, Costantini M, Salvador R, Ruol A, Zaninotto G. Esophageal achalasia in the Veneto region: epidemiology and treatment. Epidemiology and treatment of achalasia. J Gastrointest Surg 2011; 15: 423-428 [PMID: 21116729 DOI: 10.1007/ s11605-010-1392-7]
26. Podas T, Eaden J, Mayberry M, Mayberry J. Achalasia: a critical review of epidemiological studies. Am J Gastroenterol 1998; 93: 2345-2347 [PMID: 9860390 DOI: 10.1111/ j.1572-0241.1998.00686.x]
27. Zendehdel K, Nyrén O, Edberg A, Ye W. Risk of esophageal adenocarcinoma in achalasia patients, a retrospective cohort study in Sweden. Am J Gastroenterol 2011; 106: 57-61 [PMID: 21212754 DOI: 10.1038/ajg.2010.449]
28. Ng KY, Li KF, Lok KH, Lai L, Ng CH, Li KK, Szeto ML. Tenyear review of epidemiology, clinical features, and treatment outcome of achalasia in a regional hospital in Hong Kong. Hong Kong Med J 2010; 16: 362-366 [PMID: 20890000]
29. Eckardt VF, Hoischen T, Bernhard G. Life expectancy, complications, and causes of death in patients with achalasia: results of a 33-year follow-up investigation. Eur J Gastroenterol Hepatol 2008; 20: 956-960 [PMID: 18787460 DOI: 10.1097/ MEG.0b013e3282fbf5e5]
30. Booy JD, Takata J, Tomlinson G, Urbach DR. The prevalence of autoimmune disease in patients with esophageal achalasia. Dis Esophagus 2012; 25: 209-213 [PMID: 21899655 DOI: 10.1111/j.1442-2050.2011.01249.x]
31. Kraichely RE, Farrugia G, Pittock SJ, Castell DO, Lennon VA. Neural autoantibody profile of primary achalasia. Dig Dis Sci 2010; 55: 307-311 [PMID: 19499338 DOI: 10.1007/ s10620-009-0838-9]
32. Ghoshal UC, Daschakraborty SB, Singh R. Pathogenesis of achalasia cardia. World J Gastroenterol 2012; 18: 3050-3057 [PMID: 22791940 DOI: 10.3748/wjg.v18.i24.3050]
33. Aetiology of achalasia. Accessed 21 July 2012. Available from: URL: http://bestpractice.bmj.com/best-practice/ monograph/872/basics/aetiology.html
34. Lau KW, McCaughey C, Coyle PV, Murray LJ, Johnston BT. Enhanced reactivity of peripheral blood immune cells to HSV-1 in primary achalasia. Scand J Gastroenterol 2010; 45: 806-813 [PMID: 20438398 DOI: 10.3109/00365521003587804]
35. Castagliuolo I, Brun P, Costantini M, Rizzetto C, Palù G, Costantino M, Baldan N, Zaninotto G. Esophageal achalasia: is the herpes simplex virus really innocent? J Gastrointest Surg 2004; 8: 24-30; discussion 30 [PMID: 14746832]
36. Birgisson S, Galinski MS, Goldblum JR, Rice TW, Richter JE. Achalasia is not associated with measles or known herpes and human papilloma viruses. Dig Dis Sci 1997; 42: 300-306 [PMID: 9052510]
37. Kaar TK, Waldron R, Ashraf MS, Watson JB, O'Neill M, Kirwan WO. Familial infantile oesophageal achalasia. Arch Dis Child 1991; 66: 1353-1354 [PMID: 1755653]
38. Mayberry JF, Atkinson M. A study of swallowing difficulties in first degree relatives of patients with achalasia. Thorax 1985; 40: 391-393 [PMID: 4023994]
39. Chuah SK, Hsu PI, Wu KL, Wu DC, Tai WC, Changchien CS. 2011 update on esophageal achalasia. World J Gastroenterol 2012; 18: 1573-1578 [PMID: 22529685 DOI: 10.3748/wjg. v18.i14.1573]
40. Lake JM, Wong RK. Review article: the management of achalasia-a comparison of different treatment modalities. Aliment Pharmacol Ther 2006; 24: 909-918 [PMID: 16948803 DOI: 10.1111/j.1365-2036.2006.03079.x]
41. Kurian AA, Bhayani N, Sharata A, Reavis K, Dunst CM, Swanström LL. Partial anterior vs partial posterior fundoplication following transabdominal esophagocardiomyotomy for achalasia of the esophagus: meta-regression of objective postoperative gastroesophageal reflux and dysphagia. JAMA Surg 2013; 148: 85-90 [PMID: 23324843 DOI: 10.1001/ jamasurgery.2013.409]
42. Boeckxstaens GE, Annese V, des Varannes SB, Chaussade S, Costantini M, Cuttitta A, Elizalde JI, Fumagalli U, Gaudric M, Rohof WO, Smout AJ, Tack J, Zwinderman AH, Zaninotto G, Busch OR. Pneumatic dilation versus laparoscopic Heller' s myotomy for idiopathic achalasia. N Engl J Med 2011; 364: 1807-1816 [PMID: 21561346 DOI: 10.1056/NEJMoa1010502]
43. Spechler SJ. Pneumatic dilation and laparoscopic Heller' s myotomy equally effective for achalasia. N Engl J Med 2011; 364: 1868-1870 [PMID: 21561354 DOI: 10.1056/ NEJMe1100693]
44. Hoogerwerf WA, Pasricha PJ. Pharmacologic therapy in treating achalasia. Gastrointest Endosc Clin N Am 2001; 11: 311-324, vii [PMID: 11319064]
45. Allescher HD, Storr M, Seige M, Gonzales-Donoso R, Ott R, Born P, Frimberger E, Weigert N, Stier A, Kurjak M, Rösch T, Classen M. Treatment of achalasia: botulinum toxin injection vs. pneumatic balloon dilation. A prospective study with long-term follow-Up. Endoscopy 2001; 33: 1007-1017 [PMID: 11740642]
46. Cai XB, Dai YM, Wan XJ, Zeng Y, Liu F, Wang D, Zhou H. Comparison between botulinum injection and removable covered self-expanding metal stents for the treatment of achalasia. Dig Dis Sci 2013; 58: 1960-1966 [PMID: 23397470]
47. Akritidis N, Gousis C, Dimos G, Paparounas K. Fever, cough, and bilateral lung infiltrates. Achalasia associated with aspiration pneumonia. Chest 2003; 123: 608-612 [PMID: 12576387]
48. Orringer MB, Stirling MC. Esophageal resection for achalasia: indications and results. Ann Thorac Surg 1989; 47: 340-345 [PMID: 2649031]
49. Eckardt AJ, Eckardt VF. Current clinical approach to achalasia. World J Gastroenterol 2009; 15: 3969-3975 [PMID: 19705490]
50. Dunaway PM, Wong RK. Risk and surveillance intervals for squamous cell carcinoma in achalasia. Gastrointest Endosc Clin N Am 2001; 11: 425-434, ix [PMID: 11319071]
51. Streitz JM, Ellis FH, Gibb SP, Heatley GM. Achalasia and squamous cell carcinoma of the esophagus: analysis of 241 patients. Ann Thorac Surg 1995; 59: 1604-1609 [PMID: 7771859]
52. Leeuwenburgh I, Scholten P, Alderliesten J, Tilanus HW, Looman CW, Steijerberg EW, Kuipers EJ. Long-term esophageal cancer risk in patients with primary achalasia: a prospective study. Am J Gastroenterol 2010; 105: 2144-2149 [PMID: 20588263 DOI: 10.1038/ajg.2010.263]
53. Sandler RS, Nyrén O, Ekbom A, Eisen GM, Yuen J, Josefsson S. The risk of esophageal cancer in patients with achalasia. A population-based study. JAMA 1995; 274: 1359-1362 [PMID: 7563560]
54. Stein HJ, Siewert JR. Barrett's esophagus: pathogenesis, epidemiology, functional abnormalities, malignant degeneration, and surgical management. Dysphagia 1993; 8: 276-288 [PMID: 8359051]
55. Leeuwenburgh I, Scholten P, Caljé TJ, Vaessen RJ, Tilanus HW, Hansen BE, Kuipers EJ. Barrett's esophagus and esophageal adenocarcinoma are common after treatment for achalasia. Dig Dis Sci 2013; 58: 244-252 [PMID: 23179142 DOI: 10.1007/s10620-012-2157-9]
56. da Rocha JR, Ribeiro U, Sallum RA, Szachnowicz S, Cecconello I. Barrett's esophagus (BE) and carcinoma in the esophageal stump (ES) after esophagectomy with gastric pull-up in achalasia patients: a study based on 10 years follow-up. Ann Surg Oncol 2008; 15: 2903-2909 [PMID: 18618179 DOI: 10.1245/s10434-008-0057-1]
57. Brücher BL, Stein HJ, Bartels H, Feussner H, Siewert JR. Achalasia and esophageal cancer: incidence, prevalence, and prognosis. World J Surg 2001; 25: 745-749 [PMID: 11376410]
58. Coleman HG, Bhat S, Murray LJ, McManus D, Gavin AT, Johnston BT. Increasing incidence of Barrett's oesophagus: a population-based study. Eur J Epidemiol 2011; 26: 739-745 [PMID: 21671079 DOI: 10.1093/jnci/djr203]
59. Zaninotto G, Rizzetto C, Zambon P, Guzzinati S, Finotti E, Costantini M. Long-term outcome and risk of oesophageal cancer after surgery for achalasia. Br J Surg 2008; 95: 1488-1494 [PMID: 18991316 DOI: 10.1002/bjs.6413]
60. Evans JA, Early DS, Fukami N, Ben-Menachem T, Chandrasekhara V, Chathadi KV, Decker GA, Fanelli RD, Fisher DA, Foley KQ, Hwang JH, Jain R, Jue TL, Khan KM, Lightdale J, Malpas PM, Maple JT, Pasha SF, Saltzman JR, Sharaf RN, Shergill A, Dominitz JA, Cash BD. The role of endoscopy in Barrett's esophagus and other premalignant conditions of the esophagus. Gastrointest Endosc 2012; 76: 1087-1094 [PMID: 23164510 DOI: 10.1016/j.gie.2012.08.004]