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The management of esophageal achalasia: from diagnosis to surgical treatment

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Updates in Surgery Aims and scope Submit manuscript

Abstract

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.

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References

  1. Willis T (1674) Pharmaceutice rationalis sive diatribe de medicamentorum operationibus in humano corpore. Hagae-Comitis, London

    Google Scholar 

  2. Hurst AF, Rowlands RP (1924) Case of achalasia of the cardia relieved by operation. Proc R Soc Med 17(Clin Sect):45–46

    Google Scholar 

  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):3595–3599

    Article  CAS  PubMed  Google Scholar 

  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):724–728

    Article  CAS  PubMed  Google Scholar 

  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–G406

    CAS  PubMed  Google Scholar 

  6. Yamato S, Spechler SJ, Goyal RK (1992) Role of nitric oxide in esophageal peristalsis in the opossum. Gastroenterology 103(1):197–204

    CAS  PubMed  Google Scholar 

  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):2357–2362

    Article  PubMed  Google Scholar 

  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):667–672

    Article  PubMed  Google Scholar 

  9. Goyal RK, Rattan S, Said SI (1980) VIP as a possible neurotransmitter of non-cholinergic non-adrenergic inhibitory neurones. Nature 288(5789):378–380

    Article  CAS  PubMed  Google Scholar 

  10. Rattan S, Grady M, Goyal RK (1982) Vasoactive intestinal peptide causes peristaltic contractions in the esophageal body. Life Sci 30(18):1557–1563

    Article  CAS  PubMed  Google Scholar 

  11. Ghoshal UC, Daschakraborty SB, Singh R (2012) Pathogenesis of achalasia cardia. World J Gastroenterol 18(24):3050–3057

    Article  CAS  PubMed  Google Scholar 

  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):1119–1124

    PubMed  Google Scholar 

  13. Goldblum JR, Rice TW, Richter JE (1996) Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology 111(3):648–654

    Article  CAS  PubMed  Google Scholar 

  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):445–453

    Article  CAS  PubMed  Google Scholar 

  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):1033–1038

    CAS  Google Scholar 

  16. Verne GN, Sallustio JE, Eaker EY (1997) Anti-myenteric neuronal antibodies in patients with achalasia. A prospective study. Dig Dis Sci 42(2):307–313

    Article  CAS  PubMed  Google Scholar 

  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):15–19

    Article  PubMed  Google Scholar 

  18. Wong RK, Maydonovitch CL, Metz SJ, Baker JR Jr (1989) Significant DQw1 association in achalasia. Dig Dis Sci 34(3):349–352

    Article  CAS  PubMed  Google Scholar 

  19. Latiano A, De Giorgio R, Volta U et al (2006) HLA and enteric antineuronal antibodies in patients with achalasia. Neurogastroenterol Motil 18(7):520–525

    Article  CAS  PubMed  Google Scholar 

  20. Mayberry JF, Atkinson M (1985) A study of swallowing difficulties in first degree relatives of patients with achalasia. Thorax 40(5):391–393

    Article  CAS  PubMed  Google Scholar 

  21. Tullio-Pelet A, Salomon R, Hadj-Rabia S et al (2000) Mutant WD-repeat protein in triple-A syndrome. Nat Genet 26(3):332–335

    Article  CAS  PubMed  Google Scholar 

  22. Mayberry JF, Atkinson M (1985) Studies of incidence and prevalence of achalasia in the Nottingham area. Q J Med 56(220):451–456

    CAS  PubMed  Google Scholar 

  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):791–795

    Article  CAS  PubMed  Google Scholar 

  24. Sadowski DC, Ackah F, Jiang B, Svenson LW (2010) Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil 22(9):e256–e261

    Article  CAS  PubMed  Google Scholar 

  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):1974–1979

    Article  PubMed  Google Scholar 

  26. Crookes PF, Corkill S, DeMeester TR (1997) Gastroesophageal reflux in achalasia. When is reflux really reflux? Dig Dis Sci 42(7):1354–1361

    Article  CAS  PubMed  Google Scholar 

  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):505–510

    Article  CAS  PubMed  Google Scholar 

  28. Spechler SJ, Souza RF, Rosenberg SJ, Ruben RA, Goyal RK (1995) Heartburn in patients with achalasia. Gut 37(3):305–308

    Article  CAS  PubMed  Google Scholar 

  29. Spiess AE, Kahrilas PJ (1998) Treating achalasia: from whalebone to laparoscope. JAMA 280(7):638–642

    Article  CAS  PubMed  Google Scholar 

  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):2144–2149

    Article  CAS  PubMed  Google Scholar 

  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):1359–1362

    Article  CAS  PubMed  Google Scholar 

  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):1413–1418

    Article  CAS  PubMed  Google Scholar 

  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):1488–1494

    Article  CAS  PubMed  Google Scholar 

  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 33:155–158

    Article  CAS  PubMed  Google Scholar 

  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):196–203

    Article  PubMed  Google Scholar 

  36. Patti MG, Diener U, Molena D (2001) Esophageal achalasia: preoperative assessment and postoperative follow-up. J Gastrointest Surg 5(1):11–12

    Article  CAS  PubMed  Google Scholar 

  37. Moonka R, Patti MG, Feo CV et al (1999) Clinical presentation and evaluation of malignant pseudoachalasia. J Gastrointest Surg 3(5):456–461

    Article  CAS  PubMed  Google Scholar 

  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):1011–1015

    Article  CAS  PubMed  Google Scholar 

  39. Patti MG, Herbella FA (2011) Achalasia and other esophageal motility disorders. J Gastrointest Surg 15(5):703–707

    Article  PubMed  Google Scholar 

  40. Woltman TA, Pellegrini CA, Oelschlager BK (2005) Achalasia. Surg Clin North Am 85(3):483–493

    Article  PubMed  Google Scholar 

  41. Eckardt VF, Stauf B, Bernhard G (1999) Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology 116(6):1300–1304

    Article  CAS  PubMed  Google Scholar 

  42. Sanderson DR, Ellis FH Jr, Schlegel JF, Olsen AM (1967) Syndrome of vigorous achalasia: clinical and physiologic observations. Dis Chest 52(4):508–517

    Article  CAS  PubMed  Google Scholar 

  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):27–37

    Article  PubMed  Google Scholar 

  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):1526–1533

    Article  PubMed Central  PubMed  Google Scholar 

  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):1635–1645

    Article  PubMed  Google Scholar 

  46. Vaezi MF, Richter JE (1998) Current therapies for achalasia: comparison and efficacy. J Clin Gastroenterol 27(1):21–35

    Article  CAS  PubMed  Google Scholar 

  47. Gelfond M, Rozen P, Gilat T (1982) Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology 83(5):963–969

    CAS  PubMed  Google Scholar 

  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):312–318

    Article  CAS  PubMed  Google Scholar 

  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):17–22

    Article  CAS  PubMed  Google Scholar 

  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):1259–1262

    CAS  PubMed  Google Scholar 

  51. Bassotti G, Annese V (1999) Review article: pharmacological options in achalasia. Aliment Pharmacol Ther 13(11):1391–1396

    Article  CAS  PubMed  Google Scholar 

  52. Pamphlett R (1989) Early terminal and nodal sprouting of motor axons after botulinum toxin. J Neurol Sci 92(2–3):181–192

    Article  CAS  PubMed  Google Scholar 

  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):159–165

    Article  PubMed  Google Scholar 

  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):1615–1619

    Article  CAS  PubMed  Google Scholar 

  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):411–417

    Article  PubMed  Google Scholar 

  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):832–839

    Article  CAS  PubMed  Google Scholar 

  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):289–296

    Article  PubMed  Google Scholar 

  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):74–79

    Article  CAS  PubMed  Google Scholar 

  59. Inoue H, Minami H, Kobayashi Y et al (2010) Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 42(4):265–271

    Article  CAS  PubMed  Google Scholar 

  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):411–417

    Article  Google Scholar 

  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):3267–3272

    Article  PubMed  Google Scholar 

  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):659–667

    Article  PubMed  Google Scholar 

  63. Heller E (1913) Kardiaplastik beim chronischen kardiospasmus mit dilatation des oesophagus. Mitt Grenzgeb Med Chir 27:141–149

    Google Scholar 

  64. Shimi S, Nathanson LK, Cuschieri A (1991) Laparoscopic cardiomyotomy for achalasia. J R Coll Surg Edinb 36(3):152–154

    CAS  PubMed  Google Scholar 

  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. 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. 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):9–14

    Article  CAS  PubMed  Google Scholar 

  68. Kjellin AP, Granqvist S, Ramel S, Thor KB (1999) Laparoscopic myotomy without fundoplication in patients with achalasia. Eur J Surg 165(12):1162–1166

    Article  CAS  PubMed  Google Scholar 

  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. Liebermann-Meffert D, Allgöwer M, Schmid P, Blum AL (1979) Muscular equivalent of the lower esophageal sphincter. Gastroenterology 76:31–38

    CAS  PubMed  Google Scholar 

  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 218:635–639

    Article  CAS  PubMed  Google Scholar 

  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):1807–1816

    Article  CAS  PubMed  Google Scholar 

  73. Patti MG, Pellegrini CA (2012) Esophageal achalasia 2011: pneumatic dilatation or laparoscopic myotomy? J Gastrointest Surg 16(4):870–873

    Article  PubMed  Google Scholar 

  74. Chen Z, Bessell JR, Chew A, Watson DI (2010) Laparoscopic cardiomyotomy for achalasia: clinical outcomes beyond 5 years. J Gastrointest Surg 14(4):594–600

    Article  PubMed  Google Scholar 

  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. 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):2044–2049

    Article  PubMed  Google Scholar 

  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):580–587

    Article  PubMed  Google Scholar 

  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):870–877

    Article  CAS  PubMed  Google Scholar 

  79. Molena D, Yang SC (2012) Surgical management of end-stage achalasia. Semin Thorac Cardiovasc Surg 24(1):19–26

    Article  PubMed  Google Scholar 

  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):843–847

    Article  CAS  PubMed  Google Scholar 

  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):570–580

    Article  PubMed  Google Scholar 

  82. Lopes AB, Fagundes RB (2012) Esophageal squamous cell carcinoma—precursor lesions and early diagnosis. World J Gastrointest Endosc 4(1):9–16

    Article  PubMed Central  PubMed  Google Scholar 

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  1. Swallowing Center, Department of Surgery, Loyola University Chicago, Health Sciences Campus, 2160 South First Avenue-Room 3226, Maywood, IL, 60153, USA

    Adrian Dobrowolsky & P. Marco Fisichella

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  1. Adrian Dobrowolsky
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  2. P. Marco Fisichella
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Correspondence to P. Marco Fisichella.

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Dobrowolsky, A., Fisichella, P.M. The management of esophageal achalasia: from diagnosis to surgical treatment. Updates Surg 66, 23–29 (2014). https://doi.org/10.1007/s13304-013-0224-1

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