Achalasia

What is Achalasia?

Achalasia is a rare disorder of the smooth muscle layer of the esophagus in which muscular ability to move food down the esophagus (peristalsis) is impaired, and the entry to the stomach or the lower esophageal sphincter (LES) fails to relax properly in response to swallowing. Onset of symptoms is usually gradual, with patients complaining of difficulty swallowing both solids and liquid, often regurgitating undigested food, and occasionally having cough or other respiratory complications. Because symptoms are progressive, achalasia should be treated, once the diagnosis is confirmed

Achalasia: Podcast by Lyall A. Gorenstein, MD »  

Causes of Achalasia

Achalasia occurs as a result of loss of innervation of the esophageal muscle, the cause of which is unknown.

Signs & Symptoms of Achalasia

Symptoms of achalasia include difficulty swallowing (dysphagia), regurgitation of undigested food, chest pain that may be perceived as heartburn, and occasionally respiratory complications such as hoarseness or pneumonia from aspirating retained food from the esophagus.

Diagnosis of Achalasia

Diagnosis of achalasia is based on a history of dysphagia (difficulty swallowing) and findings on barium swallow testing and manometry.

An endoscopy of the esophagus, stomach and duodenum (esophagogastroduodenoscopy or EGD) should be performed in order to rule out the possibility of cancer, stricture, or other disorders. Barium swallow or UGI, will demonstrate a smooth tapering of the esophagus just above the stomach. Manometry is critical to confirm the diagnosis. A small solid-state catheter placed into the esophagus, measures esophageal contractions and the function of the lower esophageal sphincter. The characteristic findings are absence of esophageal contractions in the body of the esophagus, and failure of the lower esophageal sphincter to completely relax with swallowing.

Treatments for Achalasia

There are several treatment options for patients with achalasia. Surgical correction provides the most definitive results, and has the best long term outcome, however occasionally in patients with serious medical conditions, non surgical therapy may be preferable. Occasionally patients may get mild temporary relief from medications that relax smooth muscle such as nitrates or calcium channel blockers, however that relief is often minimal and short lived.

Botox

Botulinum toxin (Botox) injected into the lower esophageal sphincter (LES) under endoscopic guidance can be used to temporarily relax the muscle and improve swallowing. Symptom relief after botox therapy usually last 4 to 6 months.

Pneumatic Dilation

Using a specially designed balloon placed into the esophagus, the lower sphincter can be forcefully dilated. This technique splits some of the muscle fibers in the lower sphincter. Approximately 60% of patients experience some improvement in their swallowing after dilation. The procedure is best performed by a gastroenterologist who specializes in achalasia and who has performed many esophageal dilations, because there is a risk of perforating the esophagus, which has to be repaired with surgery.

Because the results of pneumatic dilatation are unpredictable, laparoscopic myotomy is a far better treatment option. Laparoscopic myotomy is safe and results are more predictable.

Laparoscopic Heller Myotomy and Fundoplication

The most effective treatment for achalasia is Heller myotomy (esophagomyotomy), a procedure in which the muscle fibers of the lower esophageal sphincter (LES) are divided. After completing the myotomy, a partial fundoplication or "wrap" at the area of the LES is added in order to prevent acid reflux, which may cause esophagitis and lead to serious damage to the esophagus over time.

Although Heller myotomy can done through the chest, today it is always performed laparoscopically through small incisions in the abdomen, which affords a faster recovery and return to normal activities. Long term studies have shown over 90% of patients still have improved swallowing, eating, and quality of life, ten years after a laparoscopic Heller myotomy.

Per Oral Endoscopic Myotomy (POEM)

Per Oral Endoscopic Myotomy (POEM) is a new and emerging endoscopic technique to treat achalasia. During POEM, the muscle is cut from inside the esophagus without surgery. Data so far indicate that over 90% of patients experience short-term relief of symptoms after the procedure. POEM may prove to combine the benefits of minimally invasive endoscopy with the long-term benefits of surgical myotomy; the technique is currently under careful study at this time.

Lifestyle Changes after Treatment

Patients with achalasia may need to eat slowly, chew food thoroughly, drink plenty of water with meals, and avoid eating near bedtime. This is the case not only before treatment, but frequently, after as well.

By raising the head of the bed or using a wedge-shaped pillow, the patient can encourage emptying of the esophagus into the stomach. After treatment, reflux damage can be inhibited by medications that inhibiting gastric acid secretion. Foods that aggravate reflux should be avoided.

Successful Heller myotomy usually achieves permanent results, but in a small number of patients, swallowing can deteriorate over time. Even after successful treatment of achalasia, the esophagus should be checked every year or two. Some patients may require repeat treatments such as pneumatic dilatations or myotomy. pH testing and endoscopy are sometimes used to check for reflux damage that can lead to a pre-malignant condition known as Barrett's esophagus if untreated.