Overview of the treatment of achalasia
- Stuart J Spechler, MD
Stuart J Spechler, MD
- Berta M and Cecil O Patterson Professor of Medicine
- University of Texas Southwestern Medical Center at Dallas
Achalasia results from progressive degeneration of ganglion cells in the myenteric plexus in the esophageal wall leading to failure of relaxation of the lower esophageal sphincter (LES) accompanied by a loss of peristalsis in the distal esophagus.
This topic will review the management of achalasia. Our recommendations are largely consistent with a 2013 guideline from the American College of Gastroenterology (ACG) and the American Gastroenterological Association (AGA) guidelines [1,2]. The pathophysiology, etiology, clinical manifestations, and diagnosis of achalasia, as well as a more detailed discussion of pneumatic dilation, botulinum toxin injection, and surgical management of achalasia, are presented separately. (See "Pathophysiology and etiology of achalasia" and "Achalasia: Pathogenesis, clinical manifestations, and diagnosis" and "Pneumatic dilation and botulinum toxin injection for achalasia" and "Surgical myotomy for achalasia".)
Treatment of achalasia is aimed at decreasing the resting pressure in the lower esophageal sphincter (LES) to a level at which the sphincter no longer impedes the passage of ingested material (image 1) [3,4]. This can be accomplished by mechanical disruption of the muscle fibers of the LES (eg, pneumatic dilation [PD], surgical myotomy or per-oral endoscopic myotomy) or by pharmacological reduction in LES pressure (eg, injection of botulinum toxin, oral nitrates, or calcium channel blockers) (algorithm 1). For any of the invasive therapies, results are best for patients with type II achalasia. Unfortunately, no treatment can reverse the degeneration of ganglion cells, restore the lost esophageal neurons, and hence, normalize esophageal function. Consequently, available treatments do not normalize swallowing; they merely improve it. Moreover, the efficacy of all these treatments tends to decrease with time. Consequently, patients will require long-term follow-up and will frequently need repeated or alternative treatments .
Choice of treatment — For patients who are at low surgical risk, either graded pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication should be performed to treat achalasia. Per-oral endoscopic myotomy (POEM) is a promising new endoscopic technique for performing myotomy, but its proper role in the treatment of achalasia is not yet clear. Pneumatic dilation and surgical myotomy should be performed in high-volume centers of excellence, and the choice of initial therapy should be guided by the patients' age, gender, preference, and local institutional expertise. Patients with the best outcomes after pneumatic dilation are those older than 40 years, women, those with narrow esophageal diameter, and those with a type II pattern by high-resolution manometry [6-11]. (See "Pneumatic dilation and botulinum toxin injection for achalasia", section on 'Predictors of outcome' and "Achalasia: Pathogenesis, clinical manifestations, and diagnosis", section on 'High-resolution manometry'.)
For patients who elect to undergo pneumatic dilation, we suggest surgical myotomy if symptoms persist, despite three attempts at pneumatic dilation. (See 'Pneumatic dilation' below and "Pneumatic dilation and botulinum toxin injection for achalasia", section on 'Predictors of outcome' and 'Surgical myotomy' below.)
- Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol 2013; 108:1238.
- Spechler SJ. American gastroenterological association medical position statement on treatment of patients with dysphagia caused by benign disorders of the distal esophagus. Gastroenterology 1999; 117:229.
- Spiess AE, Kahrilas PJ. Treating achalasia: from whalebone to laparoscope. JAMA 1998; 280:638.
- Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet 2014; 383:83.
- Kahrilas PJ. Treating achalasia; more than just flipping a coin. Gut 2016; 65:726.
- Boeckxstaens GE, Annese V, des Varannes SB, et al. Pneumatic dilation versus laparoscopic Heller's myotomy for idiopathic achalasia. N Engl J Med 2011; 364:1807.
- Vela MF, Richter JE, Khandwala F, et al. The long-term efficacy of pneumatic dilatation and Heller myotomy for the treatment of achalasia. Clin Gastroenterol Hepatol 2006; 4:580.
- Rohof WO, Salvador R, Annese V, et al. Outcomes of treatment for achalasia depend on manometric subtype. Gastroenterology 2013; 144:718.
- Vantrappen G, Hellemans J, Deloof W, et al. Treatment of achalasia with pneumatic dilatations. Gut 1971; 12:268.
- Vaezi MF, Baker ME, Achkar E, Richter JE. Timed barium oesophagram: better predictor of long term success after pneumatic dilation in achalasia than symptom assessment. Gut 2002; 50:765.
- Pandolfino JE, Kwiatek MA, Nealis T, et al. Achalasia: a new clinically relevant classification by high-resolution manometry. Gastroenterology 2008; 135:1526.
- Kostic S, Johnsson E, Kjellin A, et al. Health economic evaluation of therapeutic strategies in patients with idiopathic achalasia: results of a randomized trial comparing pneumatic dilatation with laparoscopic cardiomyotomy. Surg Endosc 2007; 21:1184.
- 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.
- Zerbib F, Thétiot V, Richy F, et al. Repeated pneumatic dilations as long-term maintenance therapy for esophageal achalasia. Am J Gastroenterol 2006; 101:692.
- Elliott TR, Wu PI, Fuentealba S, et al. Long-term outcome following pneumatic dilatation as initial therapy for idiopathic achalasia: an 18-year single-centre experience. Aliment Pharmacol Ther 2013; 37:1210.
- Campos GM, Vittinghoff E, Rabl C, et al. Endoscopic and surgical treatments for achalasia: a systematic review and meta-analysis. Ann Surg 2009; 249:45.
- West RL, Hirsch DP, Bartelsman JF, et al. Long term results of pneumatic dilation in achalasia followed for more than 5 years. Am J Gastroenterol 2002; 97:1346.
- Morino M, Rebecchi F, Festa V, Garrone C. Preoperative pneumatic dilatation represents a risk factor for laparoscopic Heller myotomy. Surg Endosc 1997; 11:359.
- Vantrappen G, Hellemans J. Treatment of achalasia and related motor disorders. Gastroenterology 1980; 79:144.
- Hunter JG, Trus TL, Branum GD, Waring JP. Laparoscopic Heller myotomy and fundoplication for achalasia. Ann Surg 1997; 225:655.
- Vogt D, Curet M, Pitcher D, et al. Successful treatment of esophageal achalasia with laparoscopic Heller myotomy and Toupet fundoplication. Am J Surg 1997; 174:709.
- Ancona E, Anselmino M, Zaninotto G, et al. Esophageal achalasia: laparoscopic versus conventional open Heller-Dor operation. Am J Surg 1995; 170:265.
- Holzman MD, Sharp KW, Ladipo JK, et al. Laparoscopic surgical treatment of achalasia. Am J Surg 1997; 173:308.
- Arreola-Risa C, Sinanan M, Pellegrini CA. Thoracoscopic Heller's myotomy. Treatment of achalasia by the videoendoscopic approach. Chest Surg Clin N Am 1995; 5:459.
- Luketich JD, Fernando HC, Christie NA, et al. Outcomes after minimally invasive esophagomyotomy. Ann Thorac Surg 2001; 72:1909.
- Lopushinsky SR, Urbach DR. Pneumatic dilatation and surgical myotomy for achalasia. JAMA 2006; 296:2227.
- Lynch KL, Pandolfino JE, Howden CW, Kahrilas PJ. Major complications of pneumatic dilation and Heller myotomy for achalasia: single-center experience and systematic review of the literature. Am J Gastroenterol 2012; 107:1817.
- Gockel I, Timm S, Sgourakis GG, et al. Achalasia--if surgical treatment fails: analysis of remedial surgery. J Gastrointest Surg 2010; 14 Suppl 1:S46.
- Kumbhari V, Behary J, Szczesniak M, et al. Efficacy and safety of pneumatic dilatation for achalasia in the treatment of post-myotomy symptom relapse. Am J Gastroenterol 2013; 108:1076.
- Yaghoobi M, Mayrand S, Martel M, et al. Laparoscopic Heller's myotomy versus pneumatic dilation in the treatment of idiopathic achalasia: a meta-analysis of randomized, controlled trials. Gastrointest Endosc 2013; 78:468.
- Spechler SJ. Pneumatic dilation and laparoscopic Heller's myotomy equally effective for achalasia. N Engl J Med 2011; 364:1868.
- Moonen A, Annese V, Belmans A, et al. Long-term results of the European achalasia trial: a multicentre randomised controlled trial comparing pneumatic dilation versus laparoscopic Heller myotomy. Gut 2016; 65:732.
- Wang L, Li YM, Li L. Meta-analysis of randomized and controlled treatment trials for achalasia. Dig Dis Sci 2009; 54:2303.
- Smith CD, Stival A, Howell DL, Swafford V. Endoscopic therapy for achalasia before Heller myotomy results in worse outcomes than heller myotomy alone. Ann Surg 2006; 243:579.
- Wen ZH, Gardener E, Wang YP. Nitrates for achalasia. Cochrane Database Syst Rev 2004; :CD002299.
- Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology 1982; 83:963.
- Coccia G, Bortolotti M, Michetti P, Dodero M. Prospective clinical and manometric study comparing pneumatic dilatation and sublingual nifedipine in the treatment of oesophageal achalasia. Gut 1991; 32:604.
- Bassotti G, Annese V. Review article: pharmacological options in achalasia. Aliment Pharmacol Ther 1999; 13:1391.
- Pasricha PJ, Hawari R, Ahmed I, et al. Submucosal endoscopic esophageal myotomy: a novel experimental approach for the treatment of achalasia. Endoscopy 2007; 39:761.
- von Renteln D, Inoue H, Minami H, et al. Peroral endoscopic myotomy for the treatment of achalasia: a prospective single center study. Am J Gastroenterol 2012; 107:411.
- Inoue H, Minami H, Kobayashi Y, et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 2010; 42:265.
- Swanstrom LL, Kurian A, Dunst CM, et al. Long-term outcomes of an endoscopic myotomy for achalasia: the POEM procedure. Ann Surg 2012; 256:659.
- Verlaan T, Rohof WO, Bredenoord AJ, et al. Effect of peroral endoscopic myotomy on esophagogastric junction physiology in patients with achalasia. Gastrointest Endosc 2013; 78:39.
- Inoue H, Ikeda H, Onimaru M, et al. Clinical results in 300 cases of POEM for esophageal achalasia: A single institute registered prospective study (abstract). Gastrointest Endosc 2013; 77:AB121.
- Von Renteln D, Fuchs KH, Fockens P, et al. Endoscopic versus surgical myotomy for idiopathic achalasia: Results of a prospective multicenter study and comparison with laparoscopic surgery (abstract). Gastrointest Endosc 2013; 77:AB122.
- Li QL, Chen WF, Zhou PH, et al. Peroral endoscopic myotomy for the treatment of achalasia: a clinical comparative study of endoscopic full-thickness and circular muscle myotomy. J Am Coll Surg 2013; 217:442.
- Inoue H, Sato H, Ikeda H, et al. Per-Oral Endoscopic Myotomy: A Series of 500 Patients. J Am Coll Surg 2015; 221:256.
- Kumbhari V, Tieu AH, Onimaru M, et al. Peroral endoscopic myotomy (POEM) vs laparoscopic Heller myotomy (LHM) for the treatment of Type III achalasia in 75 patients: a multicenter comparative study. Endosc Int Open 2015; 3:E195.
- Orenstein SB, Raigani S, Wu YV, et al. Peroral endoscopic myotomy (POEM) leads to similar results in patients with and without prior endoscopic or surgical therapy. Surg Endosc 2015; 29:1064.
- Hirota WK, Zuckerman MJ, Adler DG, et al. ASGE guideline: the role of endoscopy in the surveillance of premalignant conditions of the upper GI tract. Gastrointest Endosc 2006; 63:570.
- Eckardt AJ, Eckardt VF. Editorial: Cancer surveillance in achalasia: better late than never? Am J Gastroenterol 2010; 105:2150.