eMedicine Specialties > Gastroenterology > Esophagus
Achalasia: Treatment & Medication
Updated: Jun 30, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
Medical Care
The goal of therapy for achalasia is to relieve symptoms by eliminating the outflow resistance caused by the hypertensive and nonrelaxing LES. Once the obstruction is relieved, the food bolus can travel through the aperistaltic body of the esophagus by gravity.
- Calcium channel blockers and nitrates are used to decrease LES pressure.
- Approximately 10% of patients benefit from this treatment.
- This treatment is used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery.
- Endoscopic treatment includes an intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES, thereby restoring the balance between excitatory and inhibitory neurotransmitters.
- This treatment has limited value. Only 30% of patients treated endoscopically still have relief of dysphagia 1 year after treatment.
- This treatment can cause an inflammatory reaction at the level of the gastroesophageal junction, making a subsequent myotomy very difficult.
- Use this treatment in elderly patients who are poor candidates for dilatation or surgery.
- Pneumatic dilatation performed by a qualified gastroenterologist is the recommended treatment in those sporadic cases in which surgery is not appropriate.
- A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle fibers while leaving the mucosa intact.
- The success rate is 70-80%, and the perforation rate is approximately 5%.
- If a perforation occurs, emergency surgery is needed to close the perforation and to perform a myotomy.
- As many as 50% of patients may require more than 1 dilatation.
- The incidence of abnormal gastroesophageal reflux after the procedure is approximately 25%.
- A laparoscopic Heller myotomy is considered by many to be the appropriate primary treatment of patients with achalasia (see Surgical Care). A Heller myotomy and a partial fundoplication performed from the chest (thoracoscopic) have a high incidence of gastroesophageal reflux.
Surgical Care
Because of excellent results, a short hospital stay, and a fast recovery time, the primary treatment is considered by many to be a laparoscopic Heller myotomy and partial fundoplication. In the author's experience and in the experience of many authors, this treatment provides a fine balance in relieving symptoms of dysphagia by performing the myotomy and in preventing gastroesophageal reflux by adding a partial wrap. A prospective randomized study from VanderbiltUniversity has shown that a Heller myotomy plus a partial fundoplication was superior to a Heller myotomy alone in regard to the incidence of postoperative reflux. The same authors of this study have also shown that, in patients with achalasia, adding a partial fundoplication not only is more effective in preventing postoperative reflux but also is more cost-effective at a time horizon of 10 years.
However, the use of preoperative endoscopic therapy remains common but has resulted in intraoperative complications (eg, esophageal perforation) and postoperative complications and in a high failure rate.
- Minimally invasive surgery for achalasia is carried out under general anesthesia with the use of 5 trocars. A controlled division of the muscle fibers (myotomy) of the lower esophagus (5 cm) and the proximal stomach (1.5 cm) is carried out (see Media file 3), followed by a partial fundoplication to prevent reflux (see Media files 4-5).
- Patients remain hospitalized for 24-48 hours and return to regular activities in about 2 weeks.
- The operation relieves symptoms in 85-95% of patients, and the incidence of postoperative reflux is 10-15%.
- For patients in whom surgery fails, they may be treated with an endoscopic dilatation first. If this fails, a second operation (extending the previous myotomy onto the anterior gastric wall) can be attempted once the cause of failure has been identified with imaging studies. The last resort is to surgically remove the esophagus (ie, esophagectomy).
In the past, a Heller myotomy was considered to be ineffective in patients with achalasia and a markedly dilated or sigmoid-shaped esophagus. Esophagectomy was the standard treatment. However, Sweet and colleagues have recently shown that (1) a laparoscopic Heller myotomy relieved dysphagia in most patients with achalasia, even when the esophagus was dilated; (2) about 20% of patients required additional treatment; and (3) in the end, swallowing was good in 90% of patients.1 None required an esophagectomy to maintain clinically adequate swallowing.1
Medication
Calcium channel blockers and nitrates both decrease LES pressure but do not improve LES relaxation. Approximately 10% of patients benefit from medical treatment, which should be used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery or as a temporary measure while other treatments are considered.
Calcium channel blockers
These agents interfere with calcium uptake by smooth muscle cells that are dependent on intracellular calcium for contraction. They have a relaxant effect on the LES muscle.
Nifedipine (Adalat)
Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers.
Adult
10-30 mg SL 30 min ac; hs prn if nocturnal regurgitation and cough are prominent
Pediatric
Not established
Cimetidine can increase blood levels; may decrease blood levels of quinidine; may increase beta-blocker withdrawal symptoms
Documented hypersensitivity; increased angina; acute myocardial infarction; congestive heart failure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause lower extremity edema; allergic hepatitis has occurred but is rare
Nitrates
These agents relax vascular smooth muscle.
Isosorbide dinitrate (Isordil)
Has a relaxant effect on smooth muscle fibers of LES. Relaxes vascular smooth muscle by stimulating intracellular cyclic GMP.
Adult
5 mg SL or 10 mg PO 10-15 min ac
Pediatric
Not established
Coadministration with alcohol may cause severe hypotension and cardiovascular collapse; aspirin may increase serum concentrations and effects; coadministration with calcium channel blockers may increase symptomatic orthostatic hypotension (adjust dose of either agent); may decrease effects of heparin
Documented hypersensitivity; anuria; severe dehydration; frank or impending acute pulmonary edema; severe cardiac decompensation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Tolerance to vascular and antianginal effects of nitrates may develop; minimize tolerance by using smallest effective dose, pulsing therapy (intermittent dosing), or alternating with other coronary vasodilators (take last daily dose of short-acting agent no later than 7 pm); caution when administering to patients with glaucoma
More on Achalasia |
| Overview: Achalasia |
| Differential Diagnoses & Workup: Achalasia |
 Treatment & Medication: Achalasia |
| Follow-up: Achalasia |
| Multimedia: Achalasia |
| References |
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References
Sweet MP, Nipomnick I, Gasper WJ, Bagatelos K, Ostroff JW, Fisichella PM, et al. The outcome of laparoscopic Heller myotomy for achalasia is not influenced by the degree of esophageal dilatation. J Gastrointest Surg. Jan 2008;12(1):159-65. [Medline].
Abid S, Champion G, Richter JE, McElvein R, Slaughter RL, Koehler RE. Treatment of achalasia: the best of both worlds. Am J Gastroenterol. Jul 1994;89(7):979-85. [Medline].
Benini L, Sembenini C, Castellani G, Bardelli E, Brentegani MT, Giorgetti P, et al. Pathological esophageal acidification and pneumatic dilitation in achalasic patients. Too much or not enough?. Dig Dis Sci. Feb 1996;41(2):365-71. [Medline].
Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology. Nov 1982;83(5):963-9. [Medline].
Hunter JG, Trus TL, Branum GD, Waring JP. Laparoscopic Heller myotomy and fundoplication for achalasia. Ann Surg. Jun 1997;225(6):655-64; discussion 664-5. [Medline].
Katz PO, Gilbert J, Castell DO. Pneumatic dilatation is effective long-term treatment for achalasia. Dig Dis Sci. Sep 1998;43(9):1973-7. [Medline].
Moonka R, Patti MG, Feo CV, Arcerito M, De Pinto M, Horgan S, et al. Clinical presentation and evaluation of malignant pseudoachalasia. J Gastrointest Surg. Sep-Oct 1999;3(5):456-61. [Medline].
Patti MG, Arcerito M, Tong J, De Pinto M, de Bellis M, Wang A, et al. Importance of preoperative and postoperative pH monitoring in patients with esophageal achalasia. J Gastrointest Surg. Nov-Dec 1997;1(6):505-10. [Medline].
Patti MG, Feo CV, Arcerito M, De Pinto M, Tamburini A, Diener U, et al. Effects of previous treatment on results of laparoscopic Heller myotomy for achalasia. Dig Dis Sci. Nov 1999;44(11):2270-6. [Medline].
Patti MG, Fisichella PM, Perretta S, Galvani C, Gorodner MV, Robinson T, et al. Impact of minimally invasive surgery on the treatment of esophageal achalasia: a decade of change. J Am Coll Surg. May 2003;196(5):698-703; discussion 703-5. [Medline].
Patti MG, Pellegrini CA, Arcerito M, Tong J, Mulvihill SJ, Way LW. Comparison of medical and minimally invasive surgical therapy for primary esophageal motility disorders. Arch Surg. Jun 1995;130(6):609-15; discussion 615-6. [Medline].
Richards WO, Torquati A, Holzman MD, Khaitan L, Byrne D, Lutfi R, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg. Sep 2004;240(3):405-12; discussion 412-5. [Medline].
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. May 2006;243(5):579-84; discussion 584-6. [Medline].
Spiess AE, Kahrilas PJ. Treating achalasia: from whalebone to laparoscope. JAMA. Aug 19 1998;280(7):638-42. [Medline].
Stewart KC, Finley RJ, Clifton JC, Graham AJ, Storseth C, Inculet R. Thoracoscopic versus laparoscopic modified Heller Myotomy for achalasia: efficacy and safety in 87 patients. J Am Coll Surg. Aug 1999;189(2):164-9; discussion 169-70. [Medline].
Torquati A, Lutfi R, Khaitan L, Sharp KW, Richards WO. Heller myotomy vs Heller myotomy plus Dor fundoplication: cost-utility analysis of a randomized trial. Surg Endosc. Mar 2006;20(3):389-93. [Medline].
Vaezi MF, Richter JE, Wilcox CM, Schroeder PL, Birgisson S, Slaughter RL, et al. Botulinum toxin versus pneumatic dilatation in the treatment of achalasia: a randomised trial. Gut. Feb 1999;44(2):231-9. [Medline].
Woltman TA, Oelschlager BK, Pellegrini CA. Surgical management of esophageal motility disorders. J Surg Res. Mar 2004;117(1):34-43. [Medline].
Zaninotto G, Annese V, Costantini M, Del Genio A, Costantino M, Epifani M, et al. Randomized controlled trial of botulinum toxin versus laparoscopic heller myotomy for esophageal achalasia. Ann Surg. Mar 2004;239(3):364-70. [Medline].
Further Reading
Keywords
hypertensive nonrelaxed esophageal sphincter, primary esophageal motility disorder, lower esophageal sphincter, LES, esophageal peristalsis, dysphagia, regurgitation, chest pain, heartburn, weight loss
