title: Current clinical approach to achalasia.
abstract: Idiopathic achalasia is a rare primary motility disorder of the esophagus. The classical features are incomplete relaxation of a frequently hypertensive lower esophageal sphincter (LES) and a lack of peristalsis in the tubular esophagus. These motor abnormalities lead to dysphagia, stasis, regurgitation, weight loss, or secondary respiratory complications. Although major strides have been made in understanding the pathogenesis of this rare disorder, including a probable autoimmune mediated destruction of inhibitory neurons in response to an unknown insult in genetically susceptible individuals, a definite trigger has not been identified. The diagnosis of achalasia is suggested by clinical features and confirmed by further diagnostic tests, such as esophagogastroduodenoscopy (EGD), manometry or barium swallow. These studies are not only used to exclude pseudoachalasia, but also might help to categorize the disease by severity or clinical subtype. Recent advances in diagnostic methods, including high resolution manometry (HRM), might allow prediction of treatment responses. The primary treatments for achieving long-term symptom relief are surgery and endoscopic methods. Although limited high-quality data exist, it appears that laparoscopic Heller myotomy with partial fundoplication is superior to endoscopic methods in achieving long-term relief of symptoms in the majority of patients. However, the current clinical approach to achalasia will depend not only on patients' characteristics and clinical subtypes of the disease, but also on local expertise and patient preferences.
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Eckardt
authors.forename: Volker F
authors.initials: VF
authors.lastname: Eckardt
mesh_terms: D000293:Adolescent; D000328:Adult; D001905:Botulinum Toxins / Q000627:therapeutic use; D003680:Deglutition Disorders / Q000628:therapy; D004724:Endoscopy / Q000379:methods; D004931:Esophageal Achalasia / Q000628:therapy*; D004938:Esophageal Neoplasms / Q000175:diagnosis; D004947:Esophagus / Q000473:pathology; D005762:Gastroenterology / Q000379:methods* / Q000706:statistics & numerical data; D006801:Humans; D008365:Manometry / Q000379:methods; D011379:Prognosis
chemical_list: D001905:Botulinum Toxins
references.citation: Gastrointest Endosc. 2009 Apr;69(4):980
references.pmid: 19327492
references.citation: Gastroenterology. 1992 Nov;103(5):1574-9
references.pmid: 1426877
references.citation: Z Gastroenterol. 2001 Oct;39(10):831-6
references.pmid: 11605151
references.citation: J Gastrointest Surg. 2007 Sep;11(9):1210-2
references.pmid: 18062073
references.citation: J Clin Gastroenterol. 2001 Nov-Dec;33(5):375-7
references.pmid: 11606852
references.citation: Ann Thorac Surg. 1989 Mar;47(3):340-5
references.pmid: 2649031
references.citation: Gastrointest Endosc. 1996 Oct;44(4):398-403
references.pmid: 8905357
references.citation: Dig Dis Sci. 1997 Mar;42(3):580-5
references.pmid: 9073142
references.citation: Ann Surg. 2006 Feb;243(2):196-203
references.pmid: 16432352
references.citation: Gastrointest Endosc Clin N Am. 2001 Apr;11(2):425-34, ix
references.pmid: 11319071
references.citation: N Engl J Med. 1995 Mar 23;332(12):774-8
references.pmid: 7862180
references.citation: Gastroenterology. 2000 Feb;118(2):253-7
references.pmid: 10648452
references.citation: Surg Today. 2006;36(3):235-40
references.pmid: 16493532
references.citation: Gut. 2004 May;53(5):629-33
references.pmid: 15082578
references.citation: Gastroenterology. 2001 Mar;120(4):789-98
references.pmid: 11231931
references.citation: Dig Dis. 2004;22(4):396-401
references.pmid: 15812166
references.citation: World J Surg. 2008 Sep;32(9):1974-9
references.pmid: 18575930
references.citation: J Thorac Cardiovasc Surg. 2004 Sep;128(3):402-7
references.pmid: 15354099
references.citation: Scand J Gastroenterol. 2004 Jul;39(7):634-7
references.pmid: 15370683
references.citation: Ann Surg. 2004 Mar;239(3):371-7
references.pmid: 15075654
references.citation: J Gastrointestin Liver Dis. 2007 Sep;16(3):297-303
references.pmid: 17925926
references.citation: Gut. 2000 May;46(5):597-600
references.pmid: 10764700
references.citation: Gut. 1995 Sep;37(3):305-8
references.pmid: 7590421
references.citation: Endoscopy. 2004 Aug;36(8):690-4
references.pmid: 15280973
references.citation: Endoscopy. 2002 Jul;34(7):519-23
references.pmid: 12170400
references.citation: Dig Dis Sci. 2009 Nov;54(11):2303-11
references.pmid: 19107596
references.citation: Gastrointest Endosc Clin N Am. 2001 Apr;11(2):281-92, vi
references.pmid: 11319062
references.citation: JAMA. 2006 Nov 8;296(18):2227-33
references.pmid: 17090769
references.citation: World J Gastroenterol. 2008 Oct 14;14(38):5900-6
references.pmid: 18855991
references.citation: Gastroenterology. 1996 May;110(5):1410-5
references.pmid: 8613045
references.citation: Scand J Gastroenterol. 2005 Apr;40(4):378-85
references.pmid: 16028431
references.citation: Gut. 2002 Jun;50(6):765-70
references.pmid: 12010876
references.citation: BMC Gastroenterol. 2006 Apr 01;6:12
references.pmid: 16579859
references.citation: Aliment Pharmacol Ther. 1999 Oct;13(10):1347-50
references.pmid: 10540051
references.citation: JAMA. 1995 Nov 1;274(17):1359-62
references.pmid: 7563560
references.citation: Gastrointest Endosc Clin N Am. 2001 Apr;11(2):311-24, vii
references.pmid: 11319064
references.citation: Surg Endosc. 2007 Sep;21(9):1498-502
references.pmid: 17623235
references.citation: Gastroenterology. 1999 Jun;116(6):1300-4
references.pmid: 10348812
references.citation: Clin Gastroenterol Hepatol. 2004 May;2(5):389-94
references.pmid: 15118976
references.citation: World J Surg. 2007 Mar;31(3):470-8
references.pmid: 17308851
references.citation: Gastrointest Endosc. 2006 Apr;63(4):570-80
references.pmid: 16564854
references.citation: Neurogastroenterol Motil. 2009 Jun;21(6):603-8, e20
references.pmid: 19222759
references.citation: Dig Dis Sci. 1991 Aug;36(8):1116-20
references.pmid: 1864206
references.citation: Surg Endosc. 2005 Sep;19(9):1188-92
references.pmid: 16132324
references.citation: Endoscopy. 2001 Dec;33(12):1027-30
references.pmid: 11740645
references.citation: Am J Gastroenterol. 2005 Feb;100(2):270-4
references.pmid: 15667481
references.citation: Am J Surg Pathol. 2002 Jun;26(6):784-8
references.pmid: 12023584
references.citation: Arch Surg. 2005 Sep;140(9):827-33; discussion 833-4
references.pmid: 16172290
references.citation: Am J Gastroenterol. 2004 Dec;99(12):2304-10
references.pmid: 15571574
references.citation: Endoscopy. 2001 Dec;33(12):1007-17
references.pmid: 11740642
references.citation: World J Gastroenterol. 2006 Sep 28;12(36):5763-6
references.pmid: 17007039
references.citation: Am J Gastroenterol. 2002 Jun;97(6):1346-51
references.pmid: 12094848
references.citation: Ann Thorac Surg. 2008 May;85(5):1729-34
references.pmid: 18442574
references.citation: Gastroenterology. 1992 Dec;103(6):1732-8
references.pmid: 1451966
references.citation: Best Pract Res Clin Gastroenterol. 2007;21(4):595-608
references.pmid: 17643903
references.citation: Dig Dis Sci. 2009 Jun;54(6):1237-42
references.pmid: 18975085
references.citation: Ann Surg. 2006 May;243(5):587-91; discussion 591-3
references.pmid: 16632992
references.citation: Ann Surg. 2008 Dec;248(6):986-93
references.pmid: 19092343
references.citation: Gastroenterology. 1991 Sep;101(3):743-8
references.pmid: 1860637
references.citation: Gut. 1987 Jul;28(7):883-7
references.pmid: 3653757
references.citation: Am J Gastroenterol. 2011 Jan;106(1):57-61
references.pmid: 21212754
references.citation: Eur Radiol. 2009 Aug;19(8):1973-80
references.pmid: 19296113
references.citation: Am J Gastroenterol. 1999 May;94(5):1179-85
references.pmid: 10235189
references.citation: Scand J Gastroenterol. 1991 Jul;26(7):798-800
references.pmid: 1896822
references.citation: Ann Surg. 2009 Jan;249(1):45-57
references.pmid: 19106675
references.citation: Am J Gastroenterol. 1999 Dec;94(12):3434-9
references.pmid: 10606299
references.citation: Gut. 1992 Feb;33(2):150-4
references.pmid: 1541407
references.citation: Am J Gastroenterol. 1993 Jan;88(1):34-8
references.pmid: 8420271
references.citation: Gut. 1989 Mar;30(3):299-304
references.pmid: 2651226
references.citation: Gut. 1992 Aug;33(8):1011-5
references.pmid: 1398223
references.citation: Gastroenterology. 2004 Jun;126(7):1872-83
references.pmid: 15188182
references.citation: Am J Gastroenterol. 2006 Apr;101(4):692-7
references.pmid: 16635216
references.citation: Ann Surg. 2004 Sep;240(3):405-12; discussion 412-5
references.pmid: 15319712
references.citation: Eur J Gastroenterol Hepatol. 2008 Oct;20(10):956-60
references.pmid: 18787460
references.citation: Ann Surg. 2008 Dec;248(6):1023-30
references.pmid: 19092347
references.citation: World J Surg. 2001 Jun;25(6):745-9
references.pmid: 11376410
references.citation: Ann Intern Med. 1991 Nov 1;115(9):711-2
references.pmid: 1929039
references.citation: Gastrointest Endosc Clin N Am. 2001 Apr;11(2):325-46, vii
references.pmid: 11319065
references.citation: Dig Dis Sci. 1989 May;34(5):665-71
references.pmid: 2714141
references.citation: Gastroenterology. 2008 Nov;135(5):1526-33
references.pmid: 18722376
references.citation: J Gastrointest Surg. 2008 Jan;12(1):159-65
references.pmid: 17710504
references.citation: Cochrane Database Syst Rev. 2006 Oct 18;(4):CD005046
references.pmid: 17054234