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Epidemiology, Evaluation, and Classification of Achalasia and Other Esophageal Motility Disorders

  • Wayne S. Lee
  • Ali Rezaie
  • Miguel A. BurchEmail author
Chapter

Abstract

Dysphagia is an increasingly encountered symptom in an enlarging elderly segment of the population. Achalasia is an incurable disorder characterized by lack of deglutitive relaxation of the esophagogastric junction and esophageal aperistalsis. The pathophysiology is likely multifactorial, with the end result of inhibitory neural degeneration in Auerbach’s plexus, collagen deposition, and muscular hypertrophy. Evaluation for esophageal dysmotility includes meticulous focused history. Clinical symptoms for achalasia may be quantified with the Eckardt scoring system. Diagnostic studies include barium swallow, upper endoscopy, pH study, computed tomography, and esophageal manometry. The Chicago classification should be used to sequentially stratify patients with esophageal dysmotility based on manometric findings.

Keywords

Dysphagia Esophageal dysmotility Achalasia Diffuse esophageal spasm Nutcracker Jackhammer esophagus Pseudoachalasia Esophageal high-resolution manometry Chicago classification 

References

  1. 1.
    Madhavan A, LaGorio LA, Crary MA, Dahl WJ, Carnaby GD. Prevalence of and risk factors for dysphagia in the community dwelling elderly: a systematic review. J Nutr Health Aging. 2016;20(8):806–15.CrossRefGoogle Scholar
  2. 2.
    Mayberry JF, Atkinson M. Studies of incidence and prevalence of achalasia in the Nottingham area. Q J Med. 1985;56(220):451–6.PubMedGoogle Scholar
  3. 3.
    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(237):67–74.PubMedGoogle Scholar
  4. 4.
    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(8):1011–5.CrossRefGoogle Scholar
  5. 5.
    Sadowski DC, Ackah F, Jiang B, Svenson LW. Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil. 2010;22(9):e256–61.CrossRefGoogle Scholar
  6. 6.
    Mayberry JF. Epidemiology and demographics of achalasia. Gastrointest Endosc Clin N Am. 2001;11(2):235–48. vCrossRefGoogle Scholar
  7. 7.
    Samo S, Carlson DA, Gregory DL, Gawel SH, Pandolfino JE, Kahrilas PJ. Incidence and prevalence of achalasia in Central Chicago, 2004–2014, since the widespread use of high-resolution manometry. Clin Gastroenterol Hepatol. 2016;15(3):366–73.CrossRefGoogle Scholar
  8. 8.
    Sonnenberg A. Time trends of US hospitalization for esophageal disease. J Clin Gastroenterol. 2014;48(8):e71–5.CrossRefGoogle Scholar
  9. 9.
    Sonnenberg A. Hospitalization for achalasia in the United States 1997–2006. Dig Dis Sci. 2009;54(8):1680–5.CrossRefGoogle Scholar
  10. 10.
    Enestvedt BK, Williams JL, Sonnenberg A. Epidemiology and practice patterns of achalasia in a large multi-centre database. Aliment Pharmacol Ther. 2011;33(11):1209–14.CrossRefGoogle Scholar
  11. 11.
    Stuckey BG, Mastaglia FL, Reed WD, Pullan PT. Glucocorticoid insufficiency, achalasia, alacrima with autonomic motor neuropathy. Ann Intern Med. 1987;106(1):61–3.CrossRefGoogle Scholar
  12. 12.
    Evsyutina YV, Trukhmanov AS, Ivashkin VT. Family case of achalasia cardia: case report and review of literature. World J Gastroenterol. 2014;20(4):1114–8.CrossRefGoogle Scholar
  13. 13.
    Gordillo-Gonzalez G, Guatibonza YP, Zarante I, Roa P, Jacome LA, Hani A. Achalasia familiar: report of a family with an autosomal dominant pattern of inherence. Dis Esophagus. 2011;24(1):E1–4.CrossRefGoogle Scholar
  14. 14.
    Johnston BT, Colcher A, Li Q, Gideon RM, Castell JA, Castell DO. Repetitive proximal esophageal contractions: a new manometric finding and a possible further link between Parkinson’s disease and achalasia. Dysphagia. 2001;16(3):186–9.CrossRefGoogle Scholar
  15. 15.
    Moore SW. Down syndrome and the enteric nervous system. Pediatr Surg Int. 2008;24(8):873–83.CrossRefGoogle Scholar
  16. 16.
    Zarate N, Mearin F, Hidalgo A, Malagelada JR. Prospective evaluation of esophageal motor dysfunction in down’s syndrome. Am J Gastroenterol. 2001;96(6):1718–24.CrossRefGoogle Scholar
  17. 17.
    Qualman SJ, Haupt HM, Yang P, Hamilton SR. Esophageal Lewy bodies associated with ganglion cell loss in achalasia. Similarity to Parkinson’s disease. Gastroenterology. 1984;87(4):848–56.PubMedGoogle Scholar
  18. 18.
    Moran T, Lawlor P, Brennan M, Ravi N, Reynolds JV. Oesophageal manometry: 10-year audit from a specialist centre, and early experience with high-resolution manometry. Ir J Med Sci. 2015;184(2):379–87.CrossRefGoogle Scholar
  19. 19.
    Kahrilas PJ, Pandolfino JE. Ineffective esophageal motility does not equate to GERD. Am J Gastroenterol. 2003;98(4):715–7.CrossRefGoogle Scholar
  20. 20.
    Conchillo JM, Nguyen NQ, Samsom M, Holloway RH, Smout AJ. Multichannel intraluminal impedance monitoring in the evaluation of patients with non-obstructive dysphagia. Am J Gastroenterol. 2005;100(12):2624–32.CrossRefGoogle Scholar
  21. 21.
    Goldblum JR, Whyte RI, Orringer MB, Appelman HD. Achalasia. A morphologic study of 42 resected specimens. Am J Surg Pathol. 1994;18(4):327–37.CrossRefGoogle Scholar
  22. 22.
    Gockel I, Bohl JR, Doostkam S, Eckardt VF, Junginger T. Spectrum of histopathologic findings in patients with achalasia reflects different etiologies. J Gastroenterol Hepatol. 2006;21(4):727–33.CrossRefGoogle Scholar
  23. 23.
    Ellis FG. The natural history of achalasia of the cardia. Proc R Soc Med. 1960;53:663–6.PubMedPubMedCentralGoogle Scholar
  24. 24.
    Robertson CS, Martin BA, Atkinson M. Varicella-zoster virus DNA in the oesophageal myenteric plexus in achalasia. Gut. 1993;34(3):299–302.CrossRefGoogle Scholar
  25. 25.
    Becker J, Niebisch S, Ricchiuto A, Schaich EJ, Lehmann G, Waltgenbach T, et al. Comprehensive epidemiological and genotype-phenotype analyses in a large European sample with idiopathic achalasia. Eur J Gastroenterol Hepatol. 2016;28(6):689–95.CrossRefGoogle Scholar
  26. 26.
    de Oliveira RB, Rezende Filho J, Dantas RO, Iazigi N. The spectrum of esophageal motor disorders in Chagas’ disease. Am J Gastroenterol. 1995;90(7):1119–24.PubMedGoogle Scholar
  27. 27.
    Mearin F, Garcia-Gonzalez MA, Strunk M, Zarate N, Malagelada JR, Lanas A. Association between achalasia and nitric oxide synthase gene polymorphisms. Am J Gastroenterol. 2006;101(9):1979–84.CrossRefGoogle Scholar
  28. 28.
    Paladini F, Cocco E, Cascino I, Belfiore F, Badiali D, Piretta L, et al. Age-dependent association of idiopathic achalasia with vasoactive intestinal peptide receptor 1 gene. Neurogastroenterol Motil. 2009;21(6):597–602.CrossRefGoogle Scholar
  29. 29.
    Grubel C, Borovicka J, Schwizer W, Fox M, Hebbard G. Diffuse esophageal spasm. Am J Gastroenterol. 2008;103(2):450–7.CrossRefGoogle Scholar
  30. 30.
    Mittal RK, Kassab G, Puckett JL, Liu J. Hypertrophy of the muscularis propria of the lower esophageal sphincter and the body of the esophagus in patients with primary motility disorders of the esophagus. Am J Gastroenterol. 2003;98(8):1705–12.CrossRefGoogle Scholar
  31. 31.
    Champion JK, Delise N, Hunt T. Myenteric plexus in spastic motility disorders. J Gastrointest Surg. 2001;5(5):514–6.CrossRefGoogle Scholar
  32. 32.
    Konturek JW, Gillessen A, Domschke W. Diffuse esophageal spasm: a malfunction that involves nitric oxide? Scand J Gastroenterol. 1995;30(11):1041–5.CrossRefGoogle Scholar
  33. 33.
    Lee JI, Park H, Kim JH, Lee SI, Conklin JL. The effect of sildenafil on oesophageal motor function in healthy subjects and patients with nutcracker oesophagus. Neurogastroenterol Motil. 2003;15(6):617–23.CrossRefGoogle Scholar
  34. 34.
    Huang L, Pimentel M, Rezaie A. Do Jackhammer contractions lead to achalasia? A longitudinal study. Neurogastroenterol Motil. 2016;29(3).  https://doi.org/10.1111/nmo.12953
  35. 35.
    Pandolfino JE, Gawron AJ. Achalasia: a systematic review. JAMA. 2015;313(18):1841–52.CrossRefGoogle Scholar
  36. 36.
    Eckardt VF, Stauf B, Bernhard G. Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology. 1999;116(6):1300–4.CrossRefGoogle Scholar
  37. 37.
    Peghini PL, Johnston BT, Leite LP, Castell DO. Mucosal acid exposure sensitizes a subset of normal subjects to intra-oesophageal balloon distension. Eur J Gastroenterol Hepatol. 1996;8(10):979–83.CrossRefGoogle Scholar
  38. 38.
    Sengupta JN. An overview of esophageal sensory receptors. AM J Med. 2000;108(Suppl 4a):87s–9s.CrossRefGoogle Scholar
  39. 39.
    Sinan H, Tatum RP, Soares RV, Martin AV, Pellegrini CA, Oelschlager BK. Prevalence of respiratory symptoms in patients with achalasia. Dis Esophagus. 2011;24(4):224–8.CrossRefGoogle Scholar
  40. 40.
    Gockel I, Eckardt VF, Schmitt T, Junginger T. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol. 2005;40(4):378–85.CrossRefGoogle Scholar
  41. 41.
    Eckardt VF, Aignherr C, Bernhard G. Predictors of outcome in patients with achalasia treated by pneumatic dilation. Gastroenterology. 1992;103(6):1732–8.CrossRefGoogle Scholar
  42. 42.
    Gockel I, Junginger T. The value of scoring achalasia: a comparison of current systems and the impact on treatment–the surgeon’s viewpoint. Am Surg. 2007;73(4):327–31.PubMedGoogle Scholar
  43. 43.
    Ren Y, Tang X, Chen Y, Chen F, Zou Y, Deng Z, et al. Pre-treatment Eckardt score is a simple factor for predicting one-year peroral endoscopic myotomy failure in patients with achalasia. Surg Endosc. 2017;31:3234–41.CrossRefGoogle Scholar
  44. 44.
    Duranceau A, Liberman M, Martin J, Ferraro P. End-stage achalasia. Dis Esophagus. 2012;25(4):319–30.CrossRefGoogle Scholar
  45. 45.
    Roman S, Huot L, Zerbib F, Bruley d, Varannes S, Gourcerol G, Coffin B, et al. High-resolution manometry improves the diagnosis of esophageal motility disorders in patients with dysphagia: a randomized multicenter study. Am J Gastroenterol. 2016;111(3):372–80.CrossRefGoogle Scholar
  46. 46.
    Kahrilas PJ, Bredenoord AJ, Fox M, Gyawali CP, Roman S, Smout AJ, et al. The Chicago classification of esophageal motility disorders, v3.0. Neurogastroenterol Motil. 2015;27(2):160–74.CrossRefGoogle Scholar
  47. 47.
    Ghosh SK, Pandolfino JE, Rice J, Clarke JO, Kwiatek M, Kahrilas PJ. Impaired deglutitive EGJ relaxation in clinical esophageal manometry: a quantitative analysis of 400 patients and 75 controls. Am J Physiol Gastrointest Liver Physiol. 2007;293(4):G878–85.CrossRefGoogle Scholar
  48. 48.
    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(5):1526–33.CrossRefGoogle Scholar
  49. 49.
    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.CrossRefGoogle Scholar
  50. 50.
    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.CrossRefGoogle Scholar

Copyright information

© SAGES 2019

Authors and Affiliations

  1. 1.Department of Minimally Invasive SurgeryCedars-Sinai Medical CenterLos AngelesUSA
  2. 2.Department of Medicine, GI Motility ProgramCedars-Sinai Medical CenterLos AngelesUSA

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