Digestive Diseases and Sciences

, Volume 38, Issue 2, pp 233–244 | Cite as

Epidemiology of hospitalization for achalasia in the United States

  • Amnon Sonnenberg
  • Benson T. Massey
  • Daniel J. McCarty
  • Steven J. Jacobsen
Original Articles


Achalasia is an uncommon esophageal motility disorder of unknown etiology. To gain insights into possible etiologic risk factors, demographic and comorbidity data were obtained from Medicare hospital discharge data files from 1986–1989 on patients aged 65 and older. Age-adjusted sex- and race-specific occurrence rates were calculated for each US state. The rate of comorbid illness occurrence in achalasia patients was compared to that of the entire hospitalized Medicare population. Records of 15,000 achalasia discharges were available for analysis. Achalasia discharge rates increased linearly from age 65 to 94 years. They were similar in males and females as well as whites and nonwhites. High rates were observed in the South and low rates in most states of the East North Central region around the Great Lakes and in the Pacific region. The same geographic pattern was observed in men and women as well as in the two separate subsets of data representing the periods 1986–1987 and 1988–1989. Achalasia was associated with a significantly increased risk for pulmonary complications, malnutrition, and gastroesophageal cancer. The concordant occurrence of achalasia in patients with Parkinson's disease, depressive disorder, and various other myoneural disorders indicated a possible etiologic relationship. Achalasia appears to represent the clinical end point of several different pathways. Besides aging, different neurologic diseases may contribute to a loss in control of esophageal motility. The geographic pattern could suggest the influence of environmental factors.

Key Words

Medicare esophageal motility complications of achalasia malnutrition esophageal cancer pseudoachalasia aging Parkinson's disease neuronal degeneration 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Reynolds JC, Parkman HP: Achalasia. Gastroenterol Clin North Am 18:223–255, 1989Google Scholar
  2. 2.
    Misiewicz JJ, Waller SL, Anthony PP, Gummer JWP: Achalasia of the cardia: Pharmacology and histopathology of isolated cardiac sphincteric muscle from patients with and without achalasia. Q J Med 149:17–30, 1969Google Scholar
  3. 3.
    Cassella RR, Brown AL Jr, Sayre GP, Ellis FH Jr: Achalasia of the esophagus: Pathologic and etiologic considerations. Ann Surg 160:474–486, 1964Google Scholar
  4. 4.
    Cassella RR, Ellis FH Jr, Brown AL: Fine-structure changes in achalasia of the esophagus. Am J Pathol 46:279–288, 1965Google Scholar
  5. 5.
    Earlam RJ, Ellis FH Jr, Nobrega FT: Achalasia of the esophagus in a small urban community. Proc Mayo Clin 44:478–483, 1969Google Scholar
  6. 6.
    Mayberry JF, Rhodes J: Achalasia in the city of Cardiff from 1926 to 1977. Digestion 20:248–252, 1980Google Scholar
  7. 7.
    Mayberry JF, Atkinson M: Studies of incidence and prevalence of achalasia in the Nottingham area. Q J Med 56:451–456, 1985Google Scholar
  8. 8.
    World Health Organization: The International Classification of Diseases 9th revision. Clinical Modification, 2nd ed. DHHS Pub. No. (PHS) 80-1260. Washington, DC, Public Health Service. US Government Printing Office, 1980Google Scholar
  9. 9.
    Kahn HA, Sempos CT: Statistical Methods in Epidemiology. New York, Oxford University Press, 1989, pp 85–105Google Scholar
  10. 10.
    US Bureau of the Census. US Census of Population: 1980, Vol 1, Chapter B. General Population Characteristics. Washington, DC, US Government Printing Office, 1982Google Scholar
  11. 11.
    Office of Population Censuses and Surveys: Occupational mortality. The Registrar General's decennial supplement for Great Britain, 1979–80, 1982–83. Series DS No. 6. Part I, commentary. London, Her Majesty's Stationery Office, 1986, pp 19–28Google Scholar
  12. 12.
    Kleinbaum DG, Kupper LL, Muller KE: Applied Regression Analysis and Other Multivariable Methods, 2nd ed. Boston, PWS-Kent Publishing Company, 1988, pp 219–220Google Scholar
  13. 13.
    Wychulis AR, Woolam GL, Andersen HA, Ellis FH Jr: Achalasia and carcinoma of the esophagus. JAMA 215:1638–1641, 1971Google Scholar
  14. 14.
    Bailar JC III, Ederer F: Significance factors for the ratio of a Poisson variable to its expectation. Biometrics 20:639–643, 1964Google Scholar
  15. 15.
    Susser M: What is a cause and how do we know one? A grammar for pragmatic epidemiology. Am J Epidemiol 133:635–648, 1991Google Scholar
  16. 16.
    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 62:67–74, 1987Google Scholar
  17. 17.
    Royal College of General Practitioners, Office of Population Censuses and Surveys, Department of Health and Social Security: Morbidity statistics from general practice 1970–71, socio-economic analyses. Studies on medical and population subjects, No 46, London, Her Majesty's Stationery Office, 1982Google Scholar
  18. 18.
    Sonnenberg A, Sonnenberg GS: Occupational mortality from gastric and duodenal ulcer. Br J Ind Med 43:50–55, 1986Google Scholar
  19. 19.
    Johanson JF, Sonnenberg A: The prevalence of hemorrhoids and chronic constipation: An epidemiologic study. Gastroenterology 98:380–386, 1990Google Scholar
  20. 20.
    Sonnenberg A: Disability from inflammatory bowel disease among employees in West Germany. Gut 30:367–370, 1989Google Scholar
  21. 21.
    Rogot E, Sorlie PD, Johnson NJ, Glover CS, Treasure DW: A mortality study of one million persons by demographic, social, and economic factors: 1979–1981 follow-up. US National Longitudinal Mortality Study. US Department of Health, Education, and Welfare. Public Health Service. NIH publication No. 88-2896, Washington, DC, US Government Printing Office, 1988Google Scholar
  22. 22.
    Sagan LA: The Health of Nations. New York, Basic Books, 1987Google Scholar
  23. 23.
    Smith GD, Bartley M, Blane D: The Black report on socioeconomic inequalities in health 10 years on. Br Med J 301:373–377, 1990Google Scholar
  24. 24.
    Morris JN: Inequalities in health: Ten years and little further on. Lancet 336:491–493, 1990Google Scholar
  25. 25.
    West RR: Specificity of association in analysis of mortality and inference on causality. Int J Epidemiol 20:984–988, 1991Google Scholar
  26. 26.
    Chassin MR, Brook RH, Park RE, Keesey J, Fink A, Kosecoff J, Kahn K, Merrick N, Solomon DH: Variations in the use of medical and surgical services by the Medicare population. N Engl J Med 314:285–290, 1986Google Scholar
  27. 27.
    Just-Viera JO, Haight C: Achalasia and carcinoma of the esophagus. Surg Gynecol Obstet 128:1081–1095, 1969Google Scholar
  28. 28.
    Lortat-Jacob JL, Richard CA, Fekete F, Testart J: Cardiospasm and esophageal carcinoma: Report of 24 cases. Surgery 66:969–975, 1969Google Scholar
  29. 29.
    Bolivar JC, Herendeen TL: Carcinoma of the esophagus and achalasia. Ann Thorac Surg 10:81–89, 1970Google Scholar
  30. 30.
    Hankins JR, McLaughlin JS: The association of carcinoma of the esophagus with achalasia. J Thorac Cardiovasc Surg 69:355–360, 1970Google Scholar
  31. 31.
    Carter R, Brewer LA III Achalasia and esophageal carcinoma: studies in early diagnosis for improved surgical management. Am J Surg 130:114–120, 1975Google Scholar
  32. 32.
    Norton GA, Postlethwait RW, Thompson WM: Esophageal carcinoma: A survey of populations at risk. South J Med 73:25–27, 1980Google Scholar
  33. 33.
    Meijssen MAC, Tilanus HW, van Blankenstein M, Hop WCJ, Ong GL: Achalasia complicated by oesophageal squamous cell carcinoma: A prospective study in 195 patients. Gut 33:155–158, 1992Google Scholar
  34. 34.
    Sandler SR, Bozymski EM, Orlando RC: Failure of clinical criteria to distinguish between primary achalasia and achalasia secondary to tumor. Dig Dis Sci 27:209–213, 1982Google Scholar
  35. 35.
    Goldschmiedt M, Peterson WL, Spielberger R, Lee EL, Kurtz SF, Feldman M: Esophageal achalasia secondary to mesothelioma. Dig Dis Sci 34:1285–1298, 1989Google Scholar
  36. 36.
    Fredens K, Tøttrup A, Kristensen IB, Dahl R, Jacobsen NO, Funch-Jensen P, Thommesen P: Severe destruction of esophageal nerves in a patient with achalasia secondary to gastric cancer. A possible role of eosinophil neurotoxic proteins. Dig Dis Sci 34:297–303, 1989Google Scholar
  37. 37.
    Wambach G, Weller P, Zeidler D: Sekundäre Achalasie im Rahmen einer Polyneuropathie bei kleinzelligem Bronchialkarzinom. Internist 28:79–81, 1987Google Scholar
  38. 38.
    Gibberd FB, Gleeson JA, Gossage AAR, Wilson RSE: Oesophageal dilatation in Parkinson's disease. J Neurol Neurosurg Psychiatry 37:938–940, 1974Google Scholar
  39. 39.
    Palmer ED: Dysphagia in Parkinsonism. JAMA 229:1349, 1974Google Scholar
  40. 40.
    Dumas F, Laurent F, Miremont F, Ferrer X, Raymond JM, Baldit C, Couzigou P, Amouretti M: Pharyngoesophageal swallowing disorders in Parkinson's disease. Gastroenterology 98:A347, 1990 (abstract)Google Scholar
  41. 41.
    Ghandour E, Hutton TJ, Long T, Anuras S: Gastrointestinal symptoms in patients with Parkinson's disease. Gastroenterology 98:A353, 1990 (abstract)Google Scholar
  42. 42.
    Wakabayashi K, Takahashi H, Takeda S, Ohama E, Ikuta F: Lewy bodies in the enteric nervous system in Parkinson's disease. Arch Histol Cytol 52(suppl):191–194, 1989Google Scholar
  43. 43.
    Oyanagi K, Wakabayashi K, Ohama E, Takeda S, Horikawa Y, Morita T, Ikuta F: Lewy bodies in the lower sacral parasympathetic neurons of a patient with Parkinson's disease. Acta Neuropathol 80:558–559, 1990Google Scholar
  44. 44.
    Qualman SJ, Haupt HM, Yang P, Hamilton SR: Esophageal Lewy bodies associated with ganglion cell loss in achalasia. Similarity to Parkinson's disease. Gastroenterology 87:848–856, 1984Google Scholar
  45. 45.
    Foster PN, Stewart M, Lowe JS, Atkinson M: Achalasia-like disorder of the oesophagus in von Recklinghausen's neurofibromatosis. Gut 28:1522–1526, 1987Google Scholar
  46. 46.
    Murphy MS, Gardner-Medwin D, Eastham EJ: Achalasia of the cardia associated with hereditary cerebellar ataxia. Am J Gastroenterol 84:1329–1330, 1989Google Scholar
  47. 47.
    Maayan C, Oren A, Goldin E, Dinour D, Goldberg M, Mogle P: Megaesophagus and recurrent apnea in an adult patient with familial dysautonomia. Am J Gastroenterol 85:729–732, 1990Google Scholar
  48. 48.
    Stuckey BG, Mastaglia FL, Reed WD, Pullan PT: Glucocorticoid insufficiency, achalasia, alacrima with autonomic and motor neuropathy. Ann Intern Med 107:62–64, 1987Google Scholar
  49. 49.
    Cardi M, De Marchi C, Felli F, Paoletti PL, Paolini A: Acalasia cardiale e stress emotivo. Considerazioni su 47 casi clinici. Min Med 75:405–408, 1984Google Scholar
  50. 50.
    Berkson J: Limitations of the application of fourfold table analysis to hospital data. Biometrics 2:47–53, 1946Google Scholar
  51. 51.
    Mausner JS, Kramer S: Epidemiology: An Introductory Text. Philadelphia, WB Saunders Company, 1985, pp 180–192Google Scholar
  52. 52.
    Krakauer H, Bailey RC: Epidemiologic oversight of the medical care provided to Medicare beneficiaries. Stat Med 10:521–540, 1991Google Scholar

Copyright information

© Plenum Publishing Corporation 1993

Authors and Affiliations

  • Amnon Sonnenberg
    • 1
    • 2
  • Benson T. Massey
    • 1
    • 2
  • Daniel J. McCarty
    • 1
    • 2
  • Steven J. Jacobsen
    • 1
    • 2
  1. 1.Division of Gastroenterology, Division of Biostatistics and EpidemiologyVA Medical CenterMilwaukee
  2. 2.Medical College of WisconsinMilwaukee

Personalised recommendations