title: Distal esophageal spasm: an update.
abstract: Distal esophageal spasm (DES) is an esophageal motility disorder that presents clinically with chest pain and/or dysphagia and is defined manometrically as simultaneous contractions in the distal (smooth muscle) esophagus in ≥20% of wet swallows (and amplitude contraction of ≥30 mmHg) alternating with normal peristalsis. With the introduction of high resolution esophageal pressure topography (EPT) in 2000, the definition of DES was modified. The Chicago classification proposed that the defining criteria for DES using EPT should be the presence of at least two premature contractions (distal latency<4.5 s) in a context of normal EGJ relaxation. The etiology of DES remains insufficiently understood, but evidence links nitric oxide (NO) deficiency as a culprit resulting in a disordered neural inhibition. GERD frequently coexists in DES, and its role in the pathogenesis of symptoms needs further evaluation. There is some evidence from small series that DES can progress to achalasia. Treatment remains challenging due in part to lack of randomized placebo-controlled trials. Current treatment agents include nitrates (both short and long acting), calcium-channel blockers, anticholinergic agents, 5-phosphodiesterase inhibitors, visceral analgesics (tricyclic agents or SSRI), and esophageal dilation. Acid suppression therapy is frequently used, but clinical outcome trials to support this approach are not available. Injection of botulinum toxin in the distal esophagus may be effective, but further data regarding the development of post-injection gastroesophageal reflux need to be assessed. Heller myotomy combined with fundoplication remains an alternative for the rare refractory patient. Preliminary studies suggest that the newly developed endoscopic technique of per oral endoscopic myotomy (POEM) may also be an alternative treatment modality.
authors.affiliation: Divisions of Gastroenterology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Achem.Sami@Mayo.edu
authors.forename: Sami R
authors.initials: SR
authors.lastname: Achem
authors.forename: Lauren B
authors.initials: LB
authors.lastname: Gerson
mesh_terms: D000465:Algorithms; D004106:Dilatation / Q000379:methods; D018450:Disease Progression; D004931:Esophageal Achalasia / Q000601:surgery; D015155:Esophageal Spasm, Diffuse / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D049630:Esophageal Sphincter, Lower / Q000601:surgery; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D008365:Manometry / Q000379:methods
chemical_list: D005765:Gastrointestinal Agents
references.citation: Aliment Pharmacol Ther. 2012 Mar;35(5):493-500
references.pmid: 22239853
references.citation: Dig Dis Sci. 2003 Nov;48(11):2124-31
references.pmid: 14705816
references.citation: Dig Dis Sci. 1997 Oct;42(10):2138-45
references.pmid: 9365149
references.citation: Dis Esophagus. 2011 Jul;24(5):354-9
references.pmid: 21143695
references.citation: Aliment Pharmacol Ther. 1990 Aug;4(4):411-6
references.pmid: 2103759
references.citation: South Med J. 1985 Mar;78(3):312-5
references.pmid: 3975747
references.citation: Dis Mon. 2008 Sep;54(9):642-70
references.pmid: 18725006
references.citation: Med J Aust. 1948 Dec 4;2(23):658-61
references.pmid: 18106499
references.citation: Am J Physiol Gastrointest Liver Physiol. 2011 Dec;301(6):G1093-8
references.pmid: 21903762
references.citation: J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):436-8
references.pmid: 23114472
references.citation: Gastrointest Endosc. 2013 Jan;77(1):149-50
references.pmid: 22482919
references.citation: Aust N Z J Surg. 1982 Apr;52(2):193-7
references.pmid: 6952865
references.citation: J Clin Gastroenterol. 1999 Apr;28(3):228-32
references.pmid: 10192608
references.citation: Am J Med. 1992 May 27;92(5A):98S-105S
references.pmid: 1595773
references.citation: Ann Intern Med. 1987 Apr;106(4):593-7
references.pmid: 3826958
references.citation: N Engl J Med. 1995 Mar 23;332(12):774-8
references.pmid: 7862180
references.citation: Science. 1953 Mar 27;117(3039):334-39
references.pmid: 13048675
references.citation: Aliment Pharmacol Ther. 2012 Jan;35(1):5-14
references.pmid: 22077344
references.citation: South Med J. 1986 Jul;79(7):847-9
references.pmid: 3726585
references.citation: Am J Gastroenterol. 2012 Dec;107(12):1926-7
references.pmid: 23211862
references.citation: J Neurogastroenterol Motil. 2012 Jan;18(1):100-1
references.pmid: 22323994
references.citation: Gastroenterol Clin North Am. 2008 Dec;37(4):859-78, ix
references.pmid: 19028322
references.citation: Dig Liver Dis. 2012 Jul;44(7):569-75
references.pmid: 22475443
references.citation: Dysphagia. 2012 Mar;27(1):115-23
references.pmid: 22215281
references.citation: J Thorac Cardiovasc Surg. 1992 Oct;104(4):859-68; discussion 868-9
references.pmid: 1405682
references.citation: Gastroenterology. 1987 Apr;92(4):1027-36
references.pmid: 3549420
references.citation: J Thorac Cardiovasc Surg. 1977 Nov;74(5):736-43
references.pmid: 916713
references.citation: Neurology. 1987 Apr;37(4):616-23
references.pmid: 3561773
references.citation: Gastroenterology. 1996 May;110(5):1410-5
references.pmid: 8613045
references.citation: Dig Dis Sci. 1983 Jun;28(6):481-5
references.pmid: 6615585
references.citation: Ann Intern Med. 1984 Feb;100(2):242-5
references.pmid: 6691670
references.citation: Dis Esophagus. 2013 Jul;26(5):470-4
references.pmid: 22816880
references.citation: J Thorac Cardiovasc Surg. 1975 Jul;70(1):126-32
references.pmid: 1152494
references.citation: Am J Gastroenterol. 2002 Jul;97(7):1640-6
references.pmid: 12135012
references.citation: Br J Surg. 2007 Sep;94(9):1113-8
references.pmid: 17497756
references.citation: Am J Gastroenterol. 2006 Mar;101(3):464-9
references.pmid: 16542281
references.citation: Gastroenterology. 2011 Aug;141(2):469-75
references.pmid: 21679709
references.citation: N Engl J Med. 1994 May 19;330(20):1411-7
references.pmid: 8159194
references.citation: Endoscopy. 2010 Apr;42(4):265-71
references.pmid: 20354937
references.citation: Gastrointest Endosc. 2001 Dec;54(6):18A
references.pmid: 11762324
references.citation: Ann Thorac Surg. 1982 Sep;34(3):230-6
references.pmid: 7114943
references.citation: Dig Dis Sci. 1996 Oct;41(10):2025-31
references.pmid: 8888717
references.citation: AJR Am J Roentgenol. 2004 Aug;183(2):409-13
references.pmid: 15269034
references.citation: J Neurogastroenterol Motil. 2012 Jan;18(1):6-12
references.pmid: 22323983
references.citation: Dig Dis Sci. 2005 Sep;50(9):1605-10
references.pmid: 16133957
references.citation: Gastroenterology. 1998 Jul;115(1):42-9
references.pmid: 9649457
references.citation: J Gastrointest Surg. 2008 Jun;12(6):1133-45
references.pmid: 18071832
references.citation: Gut. 2001 Jul;49(1):145-51
references.pmid: 11413123
references.citation: Gastrointest Radiol. 1992 Summer;17(3):189-92
references.pmid: 1612298
references.citation: Digestion. 2012;85(3):236-42
references.pmid: 22472689
references.citation: Am J Med. 1992 May 27;92(5A):129S-131S
references.pmid: 1595758
references.citation: Pain Physician. 2012 Mar-Apr;15(2):E131-42
references.pmid: 22430659
references.citation: Digestion. 1987;36(2):81-3
references.pmid: 3569678
references.citation: Arch Otolaryngol Head Neck Surg. 1987 Jun;113(6):603-5
references.pmid: 3566940
references.citation: Dis Esophagus. 2012 Apr;25(3):214-21
references.pmid: 21951821
references.citation: Neurogastroenterol Motil. 2012 Mar;24 Suppl 1:57-65
references.pmid: 22248109
references.citation: Neurogastroenterol Motil. 2010 Oct;22(10):1056-e279
references.pmid: 20565688
references.citation: J Hand Surg Am. 1993 Sep;18(5):883-7
references.pmid: 8228064
references.citation: J Laparoendosc Adv Surg Tech A. 2009 Apr;19(2):195-8
references.pmid: 19260789
references.citation: Gastroenterology. 1995 Oct;109(4):1241-8
references.pmid: 7557091
references.citation: Am J Gastroenterol. 2000 Oct;95(10):2720-30
references.pmid: 11051340
references.citation: Scand J Gastroenterol. 1995 Nov;30(11):1041-5
references.pmid: 8578161
references.citation: Gastroenterology. 1958 May;34(5):782-96
references.pmid: 13538146
references.citation: Ann Thorac Surg. 1987 Jan;43(1):25-31
references.pmid: 3541814
references.citation: Neurology. 1993 Apr;43(4):834-6
references.pmid: 8469349
references.citation: Dig Dis Sci. 1987 Jun;32(6):583-92
references.pmid: 3568945