Updates in Surgery

, Volume 66, Issue 1, pp 23–29 | Cite as

The management of esophageal achalasia: from diagnosis to surgical treatment

Review Article


The goal of this review is to illustrate our approach to patients with achalasia in terms of preoperative evaluation and surgical technique. Indications, patient selection and management are herein discussed. Specifically, we illustrate the pathogenetic theories and diagnostic algorithm with current up-to-date techniques to diagnose achalasia and its manometric variants. Finally, we focus on the therapeutic approaches available today: medical and surgical. A special emphasis is given on the surgical treatment of achalasia and we provide the reader with a detailed description of our pre and postoperative management.


Achalasia Pneumatic dilatation Heller myotomy Esophageal function testing Laparoscopic repair 


Conflict of interest

The authors have no conflicts of interest.


  1. 1.
    Willis T (1674) Pharmaceutice rationalis sive diatribe de medicamentorum operationibus in humano corpore. Hagae-Comitis, LondonGoogle Scholar
  2. 2.
    Hurst AF, Rowlands RP (1924) Case of achalasia of the cardia relieved by operation. Proc R Soc Med 17(Clin Sect):45–46Google Scholar
  3. 3.
    Crist J, Gidda JS, Goyal RK (1984) Intramural mechanism of esophageal peristalsis: roles of cholinergic and noncholinergic nerves. Proc Natl Acad Sci USA 81(11):3595–3599PubMedCrossRefGoogle Scholar
  4. 4.
    Mearin F, Mourelle M, Guarner F et al (1993) Patients with achalasia lack nitric oxide synthase in the gastro-oesophageal junction. Eur J Clin Invest 23(11):724–728PubMedCrossRefGoogle Scholar
  5. 5.
    Murray J, Du C, Ledlow A, Bates JN, Conklin JL (1991) Nitric oxide: mediator of nonadrenergic noncholinergic responses of opossum esophageal muscle. Am J Physiol 261(3 Pt 1):G401–G406PubMedGoogle Scholar
  6. 6.
    Yamato S, Spechler SJ, Goyal RK (1992) Role of nitric oxide in esophageal peristalsis in the opossum. Gastroenterology 103(1):197–204PubMedGoogle Scholar
  7. 7.
    De Giorgio R, Di Simone MP, Stanghellini V et al (1999) Esophageal and gastric nitric oxide synthesizing innervation in primary achalasia. Am J Gastroenterol 94(9):2357–2362PubMedCrossRefGoogle Scholar
  8. 8.
    Khelif K, De Laet MH, Chaouachi B, Segers V, Vanderwinden JM (2003) Achalasia of the cardia in Allgrove’s (triple A) syndrome: histopathologic study of 10 cases. Am J Surg Pathol 27(5):667–672PubMedCrossRefGoogle Scholar
  9. 9.
    Goyal RK, Rattan S, Said SI (1980) VIP as a possible neurotransmitter of non-cholinergic non-adrenergic inhibitory neurones. Nature 288(5789):378–380PubMedCrossRefGoogle Scholar
  10. 10.
    Rattan S, Grady M, Goyal RK (1982) Vasoactive intestinal peptide causes peristaltic contractions in the esophageal body. Life Sci 30(18):1557–1563PubMedCrossRefGoogle Scholar
  11. 11.
    Ghoshal UC, Daschakraborty SB, Singh R (2012) Pathogenesis of achalasia cardia. World J Gastroenterol 18(24):3050–3057PubMedCrossRefGoogle Scholar
  12. 12.
    de Oliveira RB, Rezende Filho J, Dantas RO, Iazigi N (1995) The spectrum of esophageal motor disorders in Chagas’ disease. Am J Gastroenterol 90(7):1119–1124PubMedGoogle Scholar
  13. 13.
    Goldblum JR, Rice TW, Richter JE (1996) Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology 111(3):648–654PubMedCrossRefGoogle Scholar
  14. 14.
    Raymond L, Lach B, Shamji FM (1999) Inflammatory aetiology of primary oesophageal achalasia: an immunohistochemical and ultrastructural study of auerbach’s plexus. Histopathology 35(5):445–453PubMedCrossRefGoogle Scholar
  15. 15.
    Storch WB, Eckardt VF, Wienbeck M et al (1995) Autoantibodies to auerbach’s plexus in achalasia. Cell Mol Biol (Noisy-le-grand) 41(8):1033–1038Google Scholar
  16. 16.
    Verne GN, Sallustio JE, Eaker EY (1997) Anti-myenteric neuronal antibodies in patients with achalasia. A prospective study. Dig Dis Sci 42(2):307–313PubMedCrossRefGoogle Scholar
  17. 17.
    Ruiz-de-Leon A, Mendoza J, Sevilla-Mantilla C et al (2002) Myenteric antiplexus antibodies and class II HLA in achalasia. Dig Dis Sci 47(1):15–19PubMedCrossRefGoogle Scholar
  18. 18.
    Wong RK, Maydonovitch CL, Metz SJ, Baker JR Jr (1989) Significant DQw1 association in achalasia. Dig Dis Sci 34(3):349–352PubMedCrossRefGoogle Scholar
  19. 19.
    Latiano A, De Giorgio R, Volta U et al (2006) HLA and enteric antineuronal antibodies in patients with achalasia. Neurogastroenterol Motil 18(7):520–525PubMedCrossRefGoogle Scholar
  20. 20.
    Mayberry JF, Atkinson M (1985) A study of swallowing difficulties in first degree relatives of patients with achalasia. Thorax 40(5):391–393PubMedCrossRefGoogle Scholar
  21. 21.
    Tullio-Pelet A, Salomon R, Hadj-Rabia S et al (2000) Mutant WD-repeat protein in triple-A syndrome. Nat Genet 26(3):332–335PubMedCrossRefGoogle Scholar
  22. 22.
    Mayberry JF, Atkinson M (1985) Studies of incidence and prevalence of achalasia in the Nottingham area. Q J Med 56(220):451–456PubMedGoogle Scholar
  23. 23.
    Ho KY, Tay HH, Kang JY (1999) A prospective study of the clinical features, manometric findings, incidence and prevalence of achalasia in Singapore. J Gastroenterol Hepatol 14(8):791–795PubMedCrossRefGoogle Scholar
  24. 24.
    Sadowski DC, Ackah F, Jiang B, Svenson LW (2010) Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil 22(9):e256–e261PubMedCrossRefGoogle Scholar
  25. 25.
    Fisichella PM, Raz D, Palazzo F, Niponmick I, Patti MG (2008) Clinical, radiological, and manometric profile in 145 patients with untreated achalasia. World J Surg 32(9):1974–1979PubMedCrossRefGoogle Scholar
  26. 26.
    Crookes PF, Corkill S, DeMeester TR (1997) Gastroesophageal reflux in achalasia. When is reflux really reflux? Dig Dis Sci 42(7):1354–1361PubMedCrossRefGoogle Scholar
  27. 27.
    Patti MG, Arcerito M, Tong J et al (1997) Importance of preoperative and postoperative pH monitoring in patients with esophageal achalasia. J Gastrointest Surg 1(6):505–510PubMedCrossRefGoogle Scholar
  28. 28.
    Spechler SJ, Souza RF, Rosenberg SJ, Ruben RA, Goyal RK (1995) Heartburn in patients with achalasia. Gut 37(3):305–308PubMedCrossRefGoogle Scholar
  29. 29.
    Spiess AE, Kahrilas PJ (1998) Treating achalasia: from whalebone to laparoscope. JAMA 280(7):638–642PubMedCrossRefGoogle Scholar
  30. 30.
    Leeuwenburgh I, Scholten P, Alderliesten J et al (2010) Long-term esophageal cancer risk in patients with primary achalasia: a prospective study. Am J Gastroenterol 105(10):2144–2149PubMedCrossRefGoogle Scholar
  31. 31.
    Sandler RS, Nyren O, Ekbom A, Eisen GM, Yuen J, Josefsson S (1995) The risk of esophageal cancer in patients with achalasia. A population-based study. JAMA 274(17):1359–1362PubMedCrossRefGoogle Scholar
  32. 32.
    Lehman MB, Clark SB, Ormsby AH, Rice TW, Richter JE, Goldblum JR (2001) Squamous mucosal alterations in esophagectomy specimens from patients with end-stage achalasia. Am J Surg Pathol 25(11):1413–1418PubMedCrossRefGoogle Scholar
  33. 33.
    Zaninotto G, Rizzetto C, Zambon P, Guzzinati S, Finotti E, Costantini M (2008) Long-term outcome and risk of oesophageal cancer after surgery for achalasia. Br J Surg 95(12):1488–1494PubMedCrossRefGoogle Scholar
  34. 34.
    Meijssen MA, Tilanus HW, van Blankenstein M, Hop WC, Ong GL (1992) Achalasia complicated by oesophageal squamous cell carcinoma: a prospective study in 195 patients. Gut 33:155–158PubMedCrossRefGoogle Scholar
  35. 35.
    Csendes A, Braghetto I, Burdiles P, Korn O, Csendes P, Henriquez A (2006) 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 243(2):196–203PubMedCrossRefGoogle Scholar
  36. 36.
    Patti MG, Diener U, Molena D (2001) Esophageal achalasia: preoperative assessment and postoperative follow-up. J Gastrointest Surg 5(1):11–12PubMedCrossRefGoogle Scholar
  37. 37.
    Moonka R, Patti MG, Feo CV et al (1999) Clinical presentation and evaluation of malignant pseudoachalasia. J Gastrointest Surg 3(5):456–461PubMedCrossRefGoogle Scholar
  38. 38.
    Howard PJ, Maher L, Pryde A, Cameron EW, Heading RC (1992) Five year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh. Gut 33(8):1011–1015PubMedCrossRefGoogle Scholar
  39. 39.
    Patti MG, Herbella FA (2011) Achalasia and other esophageal motility disorders. J Gastrointest Surg 15(5):703–707PubMedCrossRefGoogle Scholar
  40. 40.
    Woltman TA, Pellegrini CA, Oelschlager BK (2005) Achalasia. Surg Clin North Am 85(3):483–493PubMedCrossRefGoogle Scholar
  41. 41.
    Eckardt VF, Stauf B, Bernhard G (1999) Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology 116(6):1300–1304PubMedCrossRefGoogle Scholar
  42. 42.
    Sanderson DR, Ellis FH Jr, Schlegel JF, Olsen AM (1967) Syndrome of vigorous achalasia: clinical and physiologic observations. Dis Chest 52(4):508–517PubMedCrossRefGoogle Scholar
  43. 43.
    Pandolfino JE, Ghosh SK, Rice J, Clarke JO, Kwiatek MA, Kahrilas PJ (2008) Classifying esophageal motility by pressure topography characteristics: a study of 400 patients and 75 controls. Am J Gastroenterol 103(1):27–37PubMedCrossRefGoogle Scholar
  44. 44.
    Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W, Post J, Kahrilas PJ (2008) Achalasia: a new clinically relevant classification by high-resolution manometry. Gastroenterology 135(5):1526–1533PubMedCentralPubMedCrossRefGoogle Scholar
  45. 45.
    Salvador R, Costantini M, Zaninotto G et al (2010) The preoperative manometric pattern predicts the outcome of surgical treatment for esophageal achalasia. J Gastrointest Surg 14(11):1635–1645PubMedCrossRefGoogle Scholar
  46. 46.
    Vaezi MF, Richter JE (1998) Current therapies for achalasia: comparison and efficacy. J Clin Gastroenterol 27(1):21–35PubMedCrossRefGoogle Scholar
  47. 47.
    Gelfond M, Rozen P, Gilat T (1982) Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology 83(5):963–969PubMedGoogle Scholar
  48. 48.
    Gelfond M, Rozen P, Keren S, Gilat T (1981) Effect of nitrates on LOS pressure in achalasia: a potential therapeutic aid. Gut 22(4):312–318PubMedCrossRefGoogle Scholar
  49. 49.
    Rozen P, Gelfond M, Salzman S, Baron J, Gilat T (1982) Radionuclide confirmation of the therapeutic value of isosorbide dinitrate in relieving the dysphagia in achalasia. J Clin Gastroenterol 4(1):17–22PubMedCrossRefGoogle Scholar
  50. 50.
    Traube M, Dubovik S, Lange RC, McCallum RW (1989) The role of nifedipine therapy in achalasia: results of a randomized, double-blind, placebo-controlled study. Am J Gastroenterol 84(10):1259–1262PubMedGoogle Scholar
  51. 51.
    Bassotti G, Annese V (1999) Review article: pharmacological options in achalasia. Aliment Pharmacol Ther 13(11):1391–1396PubMedCrossRefGoogle Scholar
  52. 52.
    Pamphlett R (1989) Early terminal and nodal sprouting of motor axons after botulinum toxin. J Neurol Sci 92(2–3):181–192PubMedCrossRefGoogle Scholar
  53. 53.
    Sweet MP, Nipomnick I, Gasper WJ et al (2008) The outcome of laparoscopic heller myotomy for achalasia is not influenced by the degree of esophageal dilatation. J Gastrointest Surg 12(1):159–165PubMedCrossRefGoogle Scholar
  54. 54.
    Bassotti G, D’Onofrio V, Battaglia E et al (2006) Treatment with botulinum toxin of octo-nonagerians with oesophageal achalasia: a two-year follow-up study. Aliment Pharmacol Ther 23(11):1615–1619PubMedCrossRefGoogle Scholar
  55. 55.
    Chuah SK, Wu KL, Hu TH, Tai WC, Changchien CS (2010) Endoscope-guided pneumatic dilation for treatment of esophageal achalasia. World J Gastroenterol 16(4):411–417PubMedCrossRefGoogle Scholar
  56. 56.
    Katzka DA, Castell DO (2011) Review article: an analysis of the efficacy, perforation rates and methods used in pneumatic dilation for achalasia. Aliment Pharmacol Ther 34(8):832–839PubMedCrossRefGoogle Scholar
  57. 57.
    Weber CE, Davis CS, Kramer HJ, Gibbs JT, Robles L, Fisichella PM (2012) Medium and long-term outcomes after pneumatic dilation or laparoscopic heller myotomy for achalasia: a meta-analysis. Surg Laparosc Endosc Percutan Tech 22(4):289–296PubMedCrossRefGoogle Scholar
  58. 58.
    Tuset JA, Lujan M, Huguet JM, Canelles P, Medina E (2009) Endoscopic pneumatic balloon dilation in primary achalasia: predictive factors, complications, and long-term follow-up. Dis Esophagus 22(1):74–79PubMedCrossRefGoogle Scholar
  59. 59.
    Inoue H, Minami H, Kobayashi Y et al (2010) Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 42(4):265–271PubMedCrossRefGoogle Scholar
  60. 60.
    von Renteln D, Inoue H, Minami H et al (2012) Peroral endoscopic myotomy for the treatment of achalasia: a prospective single center study. Am J Gastroenterol 107(3):411–417CrossRefGoogle Scholar
  61. 61.
    Ren Z, Zhong Y, Zhou P et al (2012) Perioperative management and treatment for complications during and after peroral endoscopic myotomy (POEM) for esophageal achalasia (EA) (data from 119 cases). Surg Endosc 26(11):3267–3272PubMedCrossRefGoogle Scholar
  62. 62.
    Swanstrom LL, Kurian A, Dunst CM, Sharata A, Bhayani N, Rieder E (2012) Long-term outcomes of an endoscopic myotomy for achalasia: the POEM procedure. Ann Surg 256(4):659–667PubMedCrossRefGoogle Scholar
  63. 63.
    Heller E (1913) Kardiaplastik beim chronischen kardiospasmus mit dilatation des oesophagus. Mitt Grenzgeb Med Chir 27:141–149Google Scholar
  64. 64.
    Shimi S, Nathanson LK, Cuschieri A (1991) Laparoscopic cardiomyotomy for achalasia. J R Coll Surg Edinb 36(3):152–154PubMedGoogle Scholar
  65. 65.
    Patti MG, Pellegrini CA, Horgan S et al (1999) Minimally invasive surgery for achalasia: an 8-year experience with 168 patients. Ann Surg 230(4):587–593 (discussion 593–594)Google Scholar
  66. 66.
    Richards WO, Torquati A, Holzman MD et al (2004) Heller myotomy versus heller myotomy with dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg 240(3):405–412 (discussion 412–415)Google Scholar
  67. 67.
    Burpee SE, Mamazza J, Schlachta CM et al (2005) Objective analysis of gastroesophageal reflux after laparoscopic heller myotomy: an anti-reflux procedure is required. Surg Endosc 19(1):9–14PubMedCrossRefGoogle Scholar
  68. 68.
    Kjellin AP, Granqvist S, Ramel S, Thor KB (1999) Laparoscopic myotomy without fundoplication in patients with achalasia. Eur J Surg 165(12):1162–1166PubMedCrossRefGoogle Scholar
  69. 69.
    Oelschlager BK, Chang L, Pellegrini CA (2003) Improved outcome after extended gastric myotomy for achalasia. Arch Surg 138(5):490–495 (discussion 495–497)Google Scholar
  70. 70.
    Liebermann-Meffert D, Allgöwer M, Schmid P, Blum AL (1979) Muscular equivalent of the lower esophageal sphincter. Gastroenterology 76:31–38PubMedGoogle Scholar
  71. 71.
    Mattioli S, Pilotti V, Felice V, DiSimone M, D’Ovidio F, Gozzetti G (1993) Intraoperative study on the relationship between the lower esophageal sphincter pressure and the muscular components of the gastro-esophageal junction in achalasic patients. Ann Surg 218:635–639PubMedCrossRefGoogle Scholar
  72. 72.
    Boeckxstaens GE, Annese V, des Varannes SB et al (2011) Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. N Engl J Med 364(19):1807–1816PubMedCrossRefGoogle Scholar
  73. 73.
    Patti MG, Pellegrini CA (2012) Esophageal achalasia 2011: pneumatic dilatation or laparoscopic myotomy? J Gastrointest Surg 16(4):870–873PubMedCrossRefGoogle Scholar
  74. 74.
    Chen Z, Bessell JR, Chew A, Watson DI (2010) Laparoscopic cardiomyotomy for achalasia: clinical outcomes beyond 5 years. J Gastrointest Surg 14(4):594–600PubMedCrossRefGoogle Scholar
  75. 75.
    Malthaner RA, Tood TR, Miller L, Pearson FG (1994) Long-term results in surgically managed esophageal achalasia. Ann Thorac Surg 58(5):1343–1346 (discussion 1346–1347)Google Scholar
  76. 76.
    Gaissert HA, Lin N, Wain JC, Fankhauser G, Wright CD, Mathisen DJ (2006) Transthoracic heller myotomy for esophageal achalasia: analysis of long-term results. Ann Thorac Surg 81(6):2044–2049PubMedCrossRefGoogle Scholar
  77. 77.
    Vela MF, Richter JE, Khandwala F et al (2006) The long-term efficacy of pneumatic dilatation and heller myotomy for the treatment of achalasia. Clin Gastroenterol Hepatol 4(5):580–587PubMedCrossRefGoogle Scholar
  78. 78.
    Patti MG, Molena D, Fisichella PM et al (2001) Laparoscopic heller myotomy and dor fundoplication for achalasia: analysis of successes and failures. Arch Surg 136(8):870–877PubMedCrossRefGoogle Scholar
  79. 79.
    Molena D, Yang SC (2012) Surgical management of end-stage achalasia. Semin Thorac Cardiovasc Surg 24(1):19–26PubMedCrossRefGoogle Scholar
  80. 80.
    Patti MG, Feo CV, Diener U et al (1999) Laparoscopic heller myotomy relieves dysphagia in achalasia when the esophagus is dilated. Surg Endosc 13(9):843–847PubMedCrossRefGoogle Scholar
  81. 81.
    Hirota WK, Zuckerman MJ, Adler DG et al (2006) ASGE guideline: the role of endoscopy in the surveillance of premalignant conditions of the upper GI tract. Gastrointest Endosc 63(4):570–580PubMedCrossRefGoogle Scholar
  82. 82.
    Lopes AB, Fagundes RB (2012) Esophageal squamous cell carcinoma—precursor lesions and early diagnosis. World J Gastrointest Endosc 4(1):9–16PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2013

Authors and Affiliations

  1. 1.Swallowing Center, Department of SurgeryLoyola University ChicagoMaywoodUSA

Personalised recommendations