Abstract
Achalasia is a chronic, progressive disease characterized by a manometric pattern showing loss of peristalsis of the distal esophagus, failure of the lower esophageal sphincter (LES) to completely relax with deglutition, and elevated baseline intraluminal esophageal pressure. Even though achalasia is a relatively rare disease, it represents the most common primary esophageal motility disorder and the second functional esophageal disorder most likely to necessitate surgical intervention after gastroesophageal reflux disease (GERD). Esophageal achalasia is an idiopathic disease resulting in damage of the Auerbach myenteric plexus. Over the last decade, the use of high resolution manometry, has allowed for a deeper understanding of this disease.
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Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet. 2014;383:83–93. Comprehensive detailed and updated review on achalasia from renowned experts in the field.
Carlson DA, Pandolfino JE. High-resolution manometry and esophageal pressure topography: filling the gaps of convention manometry. Gastroenterol Clin North Am. 2013;42:1–15. A comprehensive work on features and innovation in HRM from renowned experts.
Ellis FG. The natural history of achalasia of the cardia. Proc R Soc Med. 1960;53:663–6. An historical perspective on the disease and classification based on natural history.
Facco M, Brun P, Baesso I, Costantini M, Rizzetto C, Berto A, Baldan N, Palù G, Semenzato G, Castagliuolo I, Zaninotto G. T cells in the myenteric plexus of achalasia patients show a skewed TCR repertoire and react to HSV-1 antigens. Am J Gastroenterol. 2008;103:1598–609. This study showed an aberrant response to a viral infection or to self-antigens triggered by the infection leading to an immune response against esophageal enteric neurons..
Fisichella PM, Raz D, Palazzo F, Niponmick I, Patti MG. Clinical, radiological, and manometric profile in 145 patients with untreated achalasia. World J Surg. 2008;32:1974–9. Detailing of features of untreated disease with commonly used diagnostic tests.
Goldblum JR, Rice TW, Richter JE. Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology. 1996;111:648–54. Analysis of histopathologic features of achalasia, important to yield better understanding of pathophysiology of the disease.
Kahrilas PJ, Ghosh SK, Pandolfino JE. Esophageal motility disorders in terms of pressure topography: the Chicago Classification. J Clin Gastroenterol. 2008;42:627–35. New classification for esophageal motility disorders using HRM and pressure topography plots..
Pandolfino JE, de Ruigh A, Nicodeme 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. Description of a novel tool with interesting potential in the study of achalasia patients.
Pandolfino JE, Kahrilas PJ. Presentation, diagnosis, and management of achalasia. Clin Gastroenterol Hepatol. 2013;11:887–97. Comprehensive detailed and updated review on achalasia from renowned experts in the field.
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. Identification of 3 subtypes of achalasia with clinical and prognostic relevance.
Park W, Vaezi MF. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol. 2005;100:1404–14. An interesting insight in hypothesis on pathophysiology and etiogenesis of achalasia.
Sadowski DC, Ackah F, Jiang B, Svenson LW. Achalasia: incidence, prevalence and survival. A population-based study. Neurogastroenterol Motil. 2010;22:256–61. The first North American population-based study of achalasia epidemiology using a governmental administrative database.
Sandler RS, Nyren O, Ekbom A, Eisen GM, Yuen J, Josefsson S. The risk of esophageal cancer in patients with achalasia. A population-based study. JAMA. 1995;274:1359–62. Large population study on risk of cancer in achalasia patients compared to general population.
Triadafilopoulos G, Boeckxstaens GE, Gullo R, Patti MG, Pandolfino JE, Kahrilas PJ, et al. The Kagoshima consensus on esophageal achalasia. Dis Esophagus. 2012;25:337–48. A summary of the state of the art of knowledge on achalasia from international experts in this field.
Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013;108:1238–49. Detailed and updated review of key steps in diagnosis and treatment of achalasia.
Verne GN, Hahn AB, Pineau BC, Hoffman BJ, Wojciechowski BW, Wu WC. Association of HLA-DR and -DQ alleles with idiopathic achalasia. Gastroenterology. 1999;117:26–31. Experimental study showing an association between idiopathic achalasia and HLA alleles in a race-specific manner.
Verne GN, Sallustio JE, Eaker EY. Anti-myenteric neuronal antibodies in patients with achalasia. A prospective study. Dig Dis Sci. 1997;42:307–13. Study showing the presence of circulating neural antibodies in patients with achalasia..
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Mungo, B., Molena, D. (2014). Achalasia: Pathophysiology and Diagnostic Evaluation. In: Fisichella, P., Allaix, M., Morino, M., Patti, M. (eds) Esophageal Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-04337-1_10
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DOI: https://doi.org/10.1007/978-3-319-04337-1_10
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