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Gastroenterology > Esophagus
Achalasia
Article Last Updated: Jun 30, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Piero Marco Fisichella, MD, Assistant Professor of Surgery, Stritch School of Medicine, Loyola University; Director, Esophageal Motility Center, Loyola University Medical Center
Piero Marco Fisichella is a member of the following medical societies: American College of Surgeons, American Medical Association, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, and Society of American Gastrointestinal and Endoscopic Surgeons
Coauthor(s):
Marco G Patti, MD, Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine
Editors: David Eric Bernstein, MD, Chief, Section of Hepatology, North Shore University Hospital, Director, Associate Professor, Department of Internal Medicine, Division of Hepatology, New York University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Author and Editor Disclosure
Synonyms and related keywords:
hypertensive nonrelaxed esophageal sphincter, primary esophageal motility disorder, lower esophageal sphincter, LES, esophageal peristalsis, dysphagia, regurgitation, chest pain, heartburn, weight loss
Background
Sir Thomas Willis described achalasia in 1672. In 1881, von Mikulicz described the disease as a cardiospasm to indicate that the symptoms were due to a functional problem rather than a mechanical one. In 1929, Hurt and Rake realized that the disease was caused by a failure of the lower esophageal sphincter (LES) to relax. They coined the term achalasia, meaning failure to relax. Achalasia is a primary esophageal motility disorder characterized by failure of a hypertensive LES to relax and the absence of esophageal peristalsis. These abnormalities cause a functional obstruction at the gastroesophageal junction.
Pathophysiology
LES pressure and relaxation are regulated by excitatory (eg, acetylcholine, substance P) and inhibitory (eg, nitric oxide, vasoactive intestinal peptide) neurotransmitters. Persons with achalasia lack nonadrenergic, noncholinergic, inhibitory ganglion cells, causing an imbalance in excitatory and inhibitory neurotransmission. The result is a hypertensive nonrelaxed esophageal sphincter.
Frequency
United States
The incidence of achalasia is approximately 1 per 100,000 people per year.
International
Chagas disease may cause a similar disorder.
Sex
The male-to-female ratio of achalasia is 1:1.
Age
Achalasia typically occurs in adults aged 25-60 years. Fewer than 5% of cases occur in children.
History
Achalasia is characterized by the following symptoms and signs:
- Dysphagia (most common)
- Regurgitation
- Chest pain
- Heartburn
- Weight loss
Physical
Physical examination is noncontributory.
Causes
The cause of achalasia is unknown.
Other Problems to be Considered
Stomach cancer or cancer of the gastroesophageal junction: In the latter, the invasion of the esophageal neural plexus by the tumor can cause nonrelaxation of the LES, thus mimicking achalasia. This condition is known as malignant pseudoachalasia. Since contrast radiography and endoscopy frequently fail to differentiate these 2 entities, patients with a presumed diagnosis of achalasia but who have a shorter duration of symptoms, greater weight loss, and a more advanced age and who are referred for minimally invasive surgery should undergo additional imaging studies to rule out an occult malignancy, because this condition cannot be reliably detected during surgery.
Lab Studies
- Laboratory studies are noncontributory.
Imaging Studies
- Barium swallow
- The esophagus appears dilated, and contrast material passes slowly into the stomach as the LES opens intermittently. The distal esophagus is narrowed and has been described as resembling a bird's beak (see Media file 1).
- The test may show esophageal dilatation (see Media file 2).
Other Tests
- Esophageal manometry is the criterion standard in helping to diagnose the classic findings of achalasia. These findings include the following:
- Incomplete relaxation of the LES in response to swallowing
- High resting LES pressure
- Absent esophageal peristalsis
- Prolonged esophageal pH monitoring is important for the following reasons:
Procedures
- Perform an esophagogastroduodenoscopy (EGD) to rule out cancer of the gastroesophageal junction or fundus. If a tumor is suspected, perform an endoscopic ultrasound at the same time.
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 Vanderbilt University 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
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.
Drug Category: 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.
| Drug Name | Nifedipine (Adalat) |
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| Description | Inhibits transmembrane influx of calcium ions into smooth muscle, which, in turn, inhibits contraction of the muscle fibers. |
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| Adult Dose | 10-30 mg SL 30 min ac; hs prn if nocturnal regurgitation and cough are prominent |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; increased angina; acute myocardial infarction; congestive heart failure |
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| Interactions | Cimetidine can increase blood levels; may decrease blood levels of quinidine; may increase beta-blocker withdrawal symptoms |
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| 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
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| Precautions | May cause lower extremity edema; allergic hepatitis has occurred but is rare |
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Drug Category: Nitrates
These agents relax vascular smooth muscle.
| Drug Name | Isosorbide dinitrate (Isordil) |
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| Description | Has a relaxant effect on smooth muscle fibers of LES. Relaxes vascular smooth muscle by stimulating intracellular cyclic GMP. |
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| Adult Dose | 5 mg SL or 10 mg PO 10-15 min ac |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; anuria; severe dehydration; frank or impending acute pulmonary edema; severe cardiac decompensation |
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| Interactions | 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 |
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| 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
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| 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 |
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Prognosis
- Pneumatic dilatation and laparoscopic myotomy are effective.
- If adequate expertise is available, surgery is preferred.
- Do not use botulinum toxin and medications if performing a pneumatic dilatation or laparoscopic Heller myotomy.
Special Concerns
- Esophageal perforation
- Pneumatic dilatation for achalasia carries a significant and recognized risk of esophageal perforation. Therefore, an informed consent emphasizing this risk of perforation must be obtained from patients prior to the dilatation.
- After the dilatation, administer a small amount of water-soluble contrast material to evaluate for perforation. This should be performed in all patients undergoing the procedure. If no perforation is noted, the patient's diet can be advanced slowly after a period of observation.
- Patients with a small perforation without any evidence of infection or communication with the pleural or peritoneal cavities may receive conservative therapy with broad-spectrum antibiotics and close observation in the hospital.
- A surgical consultation must be obtained as soon as a perforation is identified. Any clinical deterioration or communication with the mediastinum or pleural or peritoneal cavities necessitates surgical intervention.
| Media file 1:
Achalasia. Manometric evaluation of the esophagus in a patient with achalasia. Pertinent findings include absence of propulsive peristalsis in the body of the esophagus (note simultaneous contractions), elevated resting lower esophageal sphincter (LES) pressure, and the absence of LES relaxation. |
 |  View Full Size Image | |
Media type: Graph
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| Media file 2:
Achalasia. Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus. |
 | View Full Size Image | |
Media type: X-RAY
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| Media file 3:
Achalasia. Heller myotomy extending 1.5 cm onto the gastric wall. |
 | View Full Size Image | |
Media type: Image
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| Media file 4:
Achalasia. Dor fundoplication, left row of sutures (after division of short gastric vessels). |
 | View Full Size Image | |
Media type: Image
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Achalasia excerpt Article Last Updated: Jun 30, 2008
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