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. Author manuscript; available in PMC: 2024 May 1.
Published in final edited form as: Clin Gastroenterol Hepatol. 2022 Sep 6;21(5):1178–1187.e1. doi: 10.1016/j.cgh.2022.08.030

Table 3.

Prior Epidemiologic Studies of Achalasia, Sorted by Terminal Study Year

Study Location Years Studied Study Design Case Definition Criteria* Cases Reviewed or Claims Codes Validated Number Achalasia Patients Reported Incidence rates (per 100,000/year) Prevalence rate (per 100,000)
Mayberry et al Cardiff, Wales 1926–1977 Retrospective (1) EGD and BE or (2) Manometry Yes 48 0.4 Not Reported
Mayberry et al Nottingham, England 1966–1983 Retrospective (1) EGD and BE or (2) Manometry Yes 53 0.51 8
Arber et al Israel 1973–1983 Prospective 2 of 3 studies: EGD, BE, Manometry Yes 162 0.8–1.1 7.9–12.6
Howard et al Edinburgh, Scotland 1986–1991 Prospective Manometry Yes 36 0.81 Not Reported
Ho et al Singapore 1989–1996 Prospective Manometry Yes 49 0.3 1.8
Birgisson et al Iceland 1952–2002 Retrospective (1) Symptoms of achalasia and (2) exclusion of other esophageal disorders and either (3) BE or (4) Manometry Yes 62 0.55 8.7
Farrukh et al Leicester, England 1986–2005 Retrospective (1) EGD and BE or (2) Manometry Yes 14 0.89 Not Reported
Gennaro et al Italy 2001–2005 Retrospective Claims based – single ICD code for achalasia No 565 1.59 Not Reported
Sadowski et al Alberta, Canada 1995–2008 Retrospective Claims based – single ICD code for achalasia + specialist note + CCP treatment code for balloon dilation or Heller myotomy Yes (no specific criteria) 463 1.63 10.82
Enestvedt et al United States 2000–2008 Retrospective Claims based – EGD performed with ICD code for achalasia associated No 896 Not Reported Not Reported
Kim et al Korea 2007–2011 Retrospective Claims based – single ICD code for achalasia No 3,105 0.39 6.29
Duffield et al Australia 2004–2013 Retrospective Manometry and EGD Yes (gold standard – manometry) 350 2.3–2.8 Not Reported
Samo et al Chicago, United States 2004–2014 Retrospective Claims based – single ICD code for achalasia and free text search for EGJOO Yes (gold standard – manometry) 379 0.77–1.35 4.68–14.42
Harvey et al England 2006–2016 Retrospective Claims based – single ICD code for achalasia Hospital Episode Statistics (HES) – Yes; The Health Improvement Network (THIN) - No HES – 10,509; THIN - 711 HES – 1.99; THIN – 1.53 THIN – 27.1
Sato et al Japan 2005–2017 Retrospective Claims based – single ICD code for achalasia No 385 0.81–1.37 7
Gaber et al United States MarketScan 2001–2018, Medicare 2007–2015 Retrospective Claims based – single inpatient or outpatient ICD code for achalasia No MarketScan – 2900
Medicare – 4907
MarketScan – 10.5
Medicare – 26.0
MarketScan – 18
Medicare – 162.1

EGD = esophagogastroduodenoscopy; BE = barium esophagram; ICD = International Classification of Diseases; EGJOO = esophagogastric junction outflow obstruction

*

Diagnostic studies or claims-based code criteria used in the study to identify achalasia cases