Abstract
Achalasia and ineffective esophageal motility (IEM) represent the extreme ends of the spectrum of esophageal motility disorders. Achalasia is characterized by aperistalsis in the body of the esophagus and failure of LES relaxation. Patients present with dysphagia for solids and liquids, bland regurgitation, chest pain, and weight loss. The diagnosis is suggested by the barium esophagram and confirmed by manometry. The goal of therapy is to (1) relieve symptoms, (2) improve esophageal emptying by disrupting the poorly relaxing LES, and (3) prevent the development of megaesophagus. Surgical myotomy and pneumatic dilation are the most effective treatments for disrupting the LES gradient. Botulinum toxin and calcium channel blockers may be useful therapies in older patients or those with severe co-morbid illnesses. Ineffective esophageal peristalsis is characterized by the presence of distal esophageal contractions of very low amplitude (<30 mmHg) and/or non-transmitted contractions. It is the most common motility disorder in GERD patients, probably due to impaired cholinergic stimulation along the esophageal body. The diagnosis is best made with esophageal manometry combined with impedance testing to confirm poor bolus transit. Once established, IEM is not improved by acid-suppressive medications, most prokinetic drugs, or anti-reflux surgery.
Keywords
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Willis T. Pharmaceutic rationalis: sive diatriba de medicamentorum; operatimibus in humano corpore. London: Hagae-Comitis; 1674.
Mayberry JF. Epidemiology and demographics of achalasia. Gastrointest Endosc Clin North Am. 2001;11:235–47.
Eckhardt VF, Hoischen T, Bernhard G. Life expectancy, complications and causes of death in patients with achalasia: results of a 33-year follow-up investigation. Eur J Gastroenterol Hepatol. 2008;20:956–60.
Goldblum JR, Whyte RI, Orringer MB, et al. Achalasia: a morphologic study of 42 resected specimens. Am J Surg Pathol. 1994;18:327–37.
Goldblum JR, Rice TW, Richter JE. Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology. 1996;111:648–54.
Holloway RH, Dodds WJ, Helm JF, et al. Integrity of cholinergic innervation to the lower esophageal sphincter in achalasia. Gastroenterology. 1986;90:924–9.
Park W, Vaezi MF. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol. 2005;100:1401–14.
Ruiz-De-Leon A, Mendoza J, De-La-Concha EG, et al. Myenteric antiplexus antibodies and class II HLA in achalasia. Dig Dis Sci. 2002;47:15–9.
Moses PL, Ellis LM, Anees MR, et al. Antineuronal antibodies in idiopathic achalasia and gastroesophageal reflux disease. Gut. 2003;52:629–36.
Niwamoto H, Okamoto E, Fujmoto J, et al. Are human herpes viruses or measles virus associated with esophageal achalasia? Dig Dis Sci. 1995;40:859–64.
Birgissen S, Galinski MS, Goldblum JR, et al. Achalasia is not associated with measles as known herpes or human papilloma viruses. Dig Dis Sci. 1997;42:300–6.
Facco M, Brun P, Zaninotto G, et al. 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.
Eckardt VF, Stauf B, Bernhard G. Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology. 1999;116:1300–4.
DeOliveira JMA, Birgisson S, Doinoff C, et al. Timed-barium swallow: a simple technique for evaluating esophageal emptying in patients with achalasia. Am J Roentgenol. 1997;169:473–9.
Ghosh SK, Pandolfino JE, Rice J, et al. Impaired deglutitive ECJ relaxation in clinical manometry: a quantitative analysis of 400 patients and 75 controls. Am J Physiol Gastrointest Liver Physiol. 2007;293(4):G878–85.
Pandolfino JE, Kwiatek MA, Nealis T, et al. Achalasia: a new clinically relevant classification by high resolution manometry. Gastroenterology. 2008;135:1526–33.
Pandolfino JE, Fox MR, Bredenoord AJ, Kahrilas PJ. High resolution manometry in clinical practice: utilizing pressure topography to classify oeosophageal motility abnormalities. Neurogastroenterol Motil. 2009;21:796–806.
Herbella FAM, Aquino JLB, Stefani-Nokana S, et al. Treatment of achalasia: lessons learned from Chagas’ disease. Dis Esophagus. 2008;21:461–7.
Gockel I, Eckhard VF, Schmitt T, Junginger T. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol. 2005;40:378–85.
Sandler RS, Bozymski EM, Orlando RC. Failure of clinical criteria to distinguish between primary achalasia and achalasia secondary to tumor. Dig Dis Sci. 1982;27:209–13.
Eckhardt VF, Kohne A, Junginger T, et al. Risk factors for diagnostic delay in achalasia. Dig Dis Sci. 1997;42:580–5.
Vela MF, Richter JE, Wachsberger D, et al. Complexities of managing achalasia at a tertiary referral center: use of pneumatic dilation, Heller myotomy and botulinum toxin injection. Am J Gastroenterol. 2004;2:389–94.
Vaezi MF, Baker MF, Achkar E, Richter JE. Timed barium esophagram: better predictor of long-term success after pneumatic dilation than symptoms assessment. Gut. 2002;50:765–70.
Eckardt VF, Aignherr C, Bernhard G. Predictors of outcome in patients with achalasia treated by pneumatic dilation. Gastroenterology. 1992;103:1732–8.
Hulselmans M, Vanuytsel T, Degreef T, et al. Long-term outcome of pneumatic dilation in the treatment of achalasia. Clin Gastroenterol Hepatol. 2010;8:30–5.
Richter JE, Boeckxstaens GE. Management of achalasia: surgery or pneumatic dilation. Gut. 2011;6:869–76.
Kadakia SC, Wong RKH. Graded pneumatic dilation using rigiflex achalasia dilators in patients with primary esophageal achalasia. Am J Gastroenterol. 1993;88:34–8.
Richer JE. Update on the management of achalasia: balloons, surgery and drugs. Exp Rev Gastroenterol Hepatol. 2008;2:435–45.
Zerbid F, Thetiot V, Richy F, et al. Repeated pneumatic dilations as long-term maintenance therapy for esophageal achalasia. Am J Gastroenterol. 2006;101:692–7.
O’Connor JB, Singer ME, Richter JE, et al. The cost-effectiveness of treatment strategies for achalasia. Dig Dis Sci. 2002;47:1516–25.
Karanicolas PJ, Smith SE, Gafni A, et al. The cost of laparoscopic myotomy versus pneumatic dilatation for esophageal achalasia. Surg Endosc. 2007;21:1198–206.
Vantrappen G, Hellemans J, Deloof W, et al. Treatment of achalasia with pneumatic dilation. Gut. 1971;12:268–75.
Eckardt VF, Kanzler G, Westermeier T. Complications and their impact after pneumatic dilation for achalasia: prospective long-term follow-up study. Gastrointest Endosc. 1997;45:349–53.
Ghoshal UC, Kumar S, Saraswat VA, et al. Long-term follow-up after pneumatic dilation for achalasia of the cardia: factors associated with treatment failure and recurrence. Am J Gastroenterol. 2004;99:2304–10.
Vela M, Richter JE, Khandwala E, et al. The long-term efficacy of pneumatic dilation and Heller myotomy for the treatment of achalasia. Clin Gastroenterol Hepatol. 2006;4:580–7.
Kostic S, Kjellin A, Ruth M, et al. Pneumatic dilation or laparoscopic myotomy in the management of newly diagnosed idiopathic achalasia. World J Surg. 2007;31:470–8.
Metman EH, Lagasse JP, Alteroche L, et al. Risk factors for immediate complications after progressive pneumatic dilation for achalasia. Am J Gastroenterol. 1999;94:1179–85.
Guardino J, Vela M, Connor J, Richter JE. Pneumatic dilation for the treatment of achalasia in untreated patients and patients with failed Heller myotomy. J Clin Gastroenterol. 2004;38:855–60.
Heller E. Extramukoese cardinplastik bein chronischen cardiopsasmus mit dilation des oesophagus. Mitt Grenzgeb Med Chir. 1914;27:141–5.
Vaezi MF, Richer JE. Current therapies for achalasia comparison and efficacy. J Clin Gastroenterol. 1998;27:21–35.
Pelligrini C, Wetter LA, Patti M, et al. Thoracoscopic esophagomyotomy. Initial experience with a new approach for achalasia. Ann Surg. 1992;216:291–6.
Oelschlager BK, Chang L, Pellegrini CA. Improved outcomes after extended gastric myotomy for achalasia. Arch Surg. 2003;138:490–5.
Richards W, Torquati A, Holzman M, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg. 2004;240:405–15.
Campos GM, Vittinghoff E, Rabl C, et al. Endoscopic and surgical treatments of achalasia. Ann Surg. 2009;249:45–57.
Constantini MI, Zanninotta G, Guirroli E, et al. The laparoscopic-Dor operation remains effective treatment for achalasia at a minimum of 6 years follow-up. Surg Endosc. 2005;19:345–51.
Portale G, Constantini M, Rizzetto C, et al. Long term outcome of laparoscopic heller-Dor surgery for achalasia: possible detrimental role of previous endoscopic treatment. J Gastrointest Surg. 2005;9:1332–9.
Schuchert MJ, Luketich JD, Landreneau RJ, et al. Minimally-invasive esophagomyotomy in 200 consecutive patients: factors influencing postoperative outcome. Ann Thorac Surg. 2008;85:1729–34.
Snyder CW, Burton RC, Brown LE, et al. Multiple preoperative endoscopic interventions are associated with worse outcome after laparoscopic Heller myotomy for achalasia. J Gastrointest Surg. 2010;14(4):2095–13.
Grockel I, Junginger T, Eckhardt V. Long-term results of conventional myotomy in patients with achalasia: a prospective 20 year analysis. Soc Surg Alimen Tract. 2006;10:1400–8.
Bonatti H, Hinder RA, Klocker J, et al. Long-term results of laparoscopic Heller myotomy with partial fundoplication for the treatment of achalasia. Am J Surg. 2005;190:874–8.
Sharp KW, Khaitan L, Scholz S, et al. 100 Consecutive minimally invasive Heller myotomies: lessons learned. Ann Surg. 2002;235:631–9.
Patti MG, Feo CV, Diener U, et al. Laparoscopic Heller mytomy relieves dysphagia in achalasia when the esophagus is dilated. Surg Endosc. 1999;13:843–6.
Mineo TC, Pompeo E. Long-term outcome in Heller myotomy achalasia sigmoid esophagus. J Thorac Cardiovasc Surg. 2004;128:402–7.
Csendes A, Braghetto I, Burdiles P, Korn O, et al. Very late results of esophagomyotomy for patients with achalasia. Ann Surg. 2006;243(2):196–203.
Lopushinsky SR, Urbach DR. Pneumatic dilation and surgical myotomy for achalasia. J Am Med Assoc. 2006;296:2227–33.
Shimi S, Nathanson LK, Cuschieri A. Laparoscopic cardiomyotomy for achalasia. J R Coll Surg Edinb. 1991;36:152–4.
Boeckxstaens G, Annese V, Chaussade S, et al. Endoscopic pneumodilation versus laparoscopic Heller myotomy with Dor anti-reflux procedure for idiopathic achalasia. N Engl J Med. 2011;364:1807–16.
Hoogerwerf WA, Pasricha PJ. Pharmacologic therapy in treating achalasia. Gastrointest Endosc Clin North Am. 2001;11:311–23.
Bortolotti M, Mari C, Lopilato C, et al. Effects of sildenafil on esophageal motility of patients with idiopathic achalasia. Gastroenterology. 2000;118:253–7.
Annese V, Bassotti G, Coccia G, et al. A multicenter randomized study of intrasphincteric botulinum toxin in patients with oesophageal achalasia. Gut. 2000;46:597–600.
Pasricha PJ, Rai R, Ravich WJ, et al. Botulinum toxin for achalasia: long-term outcome and predictor of response. Gastroenterology. 1996;110:1410–5.
Vaezi MJ, Richter JE, Wilcox CM, et al. Botulinum toxin versus pneumatic dilation in the treatment of achalasia: a randomized trial. Gut. 1999;44:231–9.
Fishman VM, Parkman HP, Schiano TD, et al. Symptomatic improvement in achalasia after botulinum toxin injection of the lower esophageal sphincter. Am J Gastroenterol. 1996;91:1724–30.
Mikaeli J, Fazel A, Montazeri G, et al. Randomized controlled trial comparing botulinum toxin injection to pneumatic dilatation for the treatment of achalasia. Aliment Pharmacol Ther. 2001;15:1389–96.
Panaccione R, Gregor JC, Reynolds RPE, et al. Intrasphincteric botulinum toxin versus pneumatic dilatation for achalasia: a cost minimization analysis. Gastrointest Endosc. 1999;50:492–8.
Ortega JA, Madureri V, Perez I. Endoscopic myotomy in the treatment of achalasia. Gastrointest Endosc. 1980;26:8–10.
Pasricha PJ, Hawari R, Ahmed I, et al. Submuscosal endoscopic esophageal myotomy: a novel experimental approach for the treatment of achalasia. Endoscopy. 2007;39:761–4.
Perretta S, Dallemagne B, Donnatelli G, et al. Transoral endoscopic esophageal myotomy based on esophageal function testing in a survival porcine model. Gastrointest Endosc. 2011;73:111–6.
Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy. 2010;42:265–71.
Micci MA, Learish RD, Li H, et al. Neural stem cell expresses RET, produces nitric oxide and survives transplantation in the GI tract. Gastroenterology. 2001;121:7757–66.
Kahrilas PJ, Dodds WJ, Hogan WJ. Effect of peristaltic dysfunction on esophageal volume clearance. Gastroenterology. 1988;94:73–80.
Kahrilas PJ, Pandolfino JE. Ineffective esophageal peristalsis does not equate to GERD. Am J Gastroenterol. 2003;98:715–7.
Diener U, Patti MG, Molena D, et al. Esophageal dysmotility and gastroesophageal reflux disease. J Gastrointest Surg. 2001;5:260–5.
Tutuian R, Castell DO. Clarification of the esophageal function defect in patients with manometric ineffective esophageal motility studies using combined impedance-manometry. Clin Gastroenterol Hepatol. 2004;2:230–6.
Conchillo M, Nguyen NQ, Sansom M, et al. Multichannel intraluminal impedance monitoring in the evaluation of patients with non-obstructive dysphagia. Am J Gastroenterol. 2005;100:2624–32.
Leite LP, Johnson BT, Barrett J, et al. Ineffective esophageal motility: the primary finding in patients with non-specific esophageal motility disorders. Dig Dis Sci. 1997;42:1859–65.
Fouad YM, Katz PO, Hatlebakk JG, et al. Ineffective esophageal motility: the most common motility abnormality in patients with GERD-associated respiratory symptoms. Am J Gastroenterol. 1999;94:1464–7.
Lund RJ, Wetcher GJ, Raiser F, et al. Laparoscopic toupet for gastroesophageal reflux disease with poor esophageal body motility. J Gastrointest Surg. 1997;1:301–8.
Zhang X, Geboes K, Depoortere I, et al. Effect of repeated cycles of acute esophagitis and healing on esophageal peristalsis; time and length. Am J Physiol. 2005;288:G1339–46.
Singh P, Adamopoulos A, Taylor RH, et al. Oesophageal motor function before and after healing of oesophagitis. Gut. 1992;33:1590–6.
Fibbe C, Layer P, Keller J, et al. Esophageal motility in reflux disease before and after fundoplication: a prospective, randomized, clinical and manometric study. Gastroenterology. 2001;121:5–14.
Cao W, Cheng L, Behar J, et al. Proinflammatory cytokines alter/reduce esophageal circular muscle contraction in experimental cat esophagitis. Am J Physiol Gastrointest Liver Physiol. 2004;287:G1131–9.
Rieder F, Cheng L, Harnett KM, et al. Gastroesophageal reflux disease-associated esophagitis induces endogenous cytokine production leading to otor abnormalities. Gastroenterology. 2007;132:154–65.
Corazziari E, Bontempo I, Anzini F. Effects of cisapride on distal esophageal motility in humans. Dig Dis Sci. 1989;34:1600–5.
Grande L, Lacima G, Ros E, et al. Lack of effect of metoclopramide and domperidone on esophageal peristalsis, and esophageal clearance in reflux esophagitis. A randomized, double-blind study. Dig Dis Sci. 1992;37:583–8.
Agrawal A, Hila A, Tutuian R, et al. Bethanechol improves smooth muscle function in patients with severe ineffective esophageal motility. J Clin Gastroenterol. 2007;41:366–70.
Grossi L, Ciccaglione AF, Marzio L. Effect of the 5-HT1 agonist sumatriptan on oesophageal motor patterns in patients with ineffective oesophageal motility. Neurogastroenterol Motil. 2003;15:9–14.
Montenovo M, Tatum RP, Figueredo, et al. Does combined multichannel intraluminal esophageal impedance and manometry predict post-operative dysphagia after laparoscopic nissen fundoplication? Dis Esophagus. 2009;22:656–63.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2013 Springer Science+Business Media New York
About this chapter
Cite this chapter
Richter, J.E. (2013). Achalasia and Ineffective Esophageal Motility. In: Shaker, R., Belafsky, P., Postma, G., Easterling, C. (eds) Principles of Deglutition. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3794-9_39
Download citation
DOI: https://doi.org/10.1007/978-1-4614-3794-9_39
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-3793-2
Online ISBN: 978-1-4614-3794-9
eBook Packages: MedicineMedicine (R0)