Definition:
Achalasia is a disorder of the esophagus.

Cause:
Achalasia is caused by the muscle at the end of the esophagus failing to open as much as it needs to for the food to enter the stomach. Failure of the lower esophageal sphincter (LES) to relax defines the disorder. The LES is a muscular ring at the junction of the esophagus and stomach. It normally relaxes during swallowing.

A constricted LES causes the esophagus to repeatedly contract to try to force the food into the stomach. The wave-like contraction of smooth muscles pushing against a closed LES causes severe chest pain. (These esophageal contractions are called peristalsis.)

Over time, the esophagus may loose the ability to contract completely. The final result is that the esophagus becomes a wide tube with no ability to propel food at all. Achalasia is a rare disorder, may occur at any age, especially in middle-aged or older adults. Children may develop achalasia as well. We have seen children as young as 11 months with documented achalasia requiring surgery.

A small proportion occurs as a secondary result of other conditions, such as Chagas disease (this is only seen in Central America).

Symptoms:

• Difficulty swallowing liquids and solids
• Chest pain which may be worse after eating
• Regurgitation of food
• Unintentional weight loss
• GE Reflux or Heartburn
• Cough

Work-up:
An upper GI x-ray test or barium esophagogram is done as the first test. The patient will swallow a liquid which outlines the appearance of the esophagus on live X-ray. This may show absence of peristalsis, an enlarged esophagus, and typically a narrowing (“bird�s beak”) at the bottom of the esophagus. The definitive test for achalasia, however, is esophageal manometry. During this test, pressure measurements are taken in the esophagus.

Differential Diagnosis:
Due to the similarity of symptoms, achalasia can be misdiagnosed as other disorders, such as gastroesophageal reflux disease (GERD), hiatus hernia, and even psychosomatic disorders.

Treatment options:

• Medication:
  • Drugs that reduce LES pressure may be helpful while waiting for surgical treatment.
    • Calcium channel blockers such as nifedipine nitrates, nitroglycerin or isosorbide dinitrate.
  • Many patients experience side effects such as headache and swollen feet, and the drugs may stop helping after several months.
  • Endoscopic injection of the esophageal muscle with botulinum toxin (called “Botox”), can paralyze the lower esophageal sphincter and prevent spasms. The relief, however, is only temporary, and symptoms return quickly in most patients.
  • Inflammation caused by Botox injections increase the difficulty of later laparosopic Heller myotomy. This therapy is only recommended for patients who are not surgical candidates..
• Balloon (pneumatic) dilation: The muscle fibers are stretched and slightly torn by forceful inflation of a balloon placed inside the lower esophageal sphincter. There is always a small risk of a perforation which would have to be fixed by surgery right away. Gastroesophageal reflux (GERD) occurs after pneumatic dilation in some patients. Pneumatic dilation causes some scarring which may increase the difficulty of Heller myotomy if this surgery is needed later. Pneumatic dilation is most effective on the long term in patients over the age of 40; the benefits tend to be shorter-lived in younger patients. This treatment may need to be repeated with larger balloons for maximum effectiveness.
• Surgery: Laparoscopic Heller myotomy improves 90% of achalasia patients.

The term “myotomy” mean cutting muscle. The Heller esophageal myotomy is a separation of the muscle from the lining of the esophagus, followed by a cutting of the muscle. See photo below. The cut along the esophagus, starts above the LES and must extend down to the stomach.

The myotomy only cuts through the outermost muscle layers which are causing the functional blockage. The inner mucosal layer is left intact. A partial wrap of the stomach called a ‘Dor” fundoplication is added to prevent GE reflux.

After surgery, patients should stay on a soft diet for several weeks to avoid pain with swallowing (called dysphagia) which can follow this surgery.

Prognosis:
Even after a well-performed, initially successful Heller myotomy, swallowing may still deteriorate over time. Patient may still require an occasional pneumatic dilation, a repeat myotomy, or even surgical revision of the esophagus later.