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Achalasia is an incurable disease that affects the lower esophageal sphincter. The normal lower esophageal sphincter functions to relax by opening to accommodate a food bolus when humans swallow. The nerves are activated during a swallow to signal the lower esophageal sphincter to relax and open as food, liquids, and/or air can pass. With achalasia, the nerves don't work properly and the sphincter cannot relax and open. This causes the inability to get food from the esophagus into the stomach to continue with digestion. Patients usually experience regurgitation of food and liquids and experience chest pain. Weight loss is a common symptom experienced by patients with achalasia.

Causes

There is no known cause, but there are some hypothesized theories that haven't been proven.

Diagnosis

Achalasia is often misdiagnosed as gastroesophageal reflux disease (GERD). Patients will proceed with the standard treatments for GERD without relief. This will typically prompt further investigation revealing a diagnosis of achalasia.

The definitive diagnosis is made with a test called an esophageal manometry. This test is done with a skinny probe with multiple sensors down its entire length that is passed through the patient's nose and into the esophagus. The patient is then asked to swallow small amounts of water, usually 10 swallows are performed, and the probe will be able to sense the function of the esophagus' ability to push the water down into the esophagus and measure the pressure of the lower esophageal sphincter at rest and whether it relaxes. This is the gold standard to make a definitive diagnosis.


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Catheter probe in position with the tracings it provides to show esophageal peristalsis

Sometimes, findings on an upper GI swallow study with barium can help allude to the diagnosis based on the dilated appearance of the esophagus and the abrupt narrowing at the lower esophageal sphincter. During this workup, the patient should have an upper endoscopy to help rule out some other cause for the appearance of the narrowing such as a tumor or scar tissue.


Barium esophagram showing the narrowing of the bottom of the esophagus as it passes into the stomach
 

Treatment

There are several treatments available for achalasia. Achalasia does not go away on its own, and there is no cure. The treatments available only make it possible for esophageal contents to pass through the lower esophageal sphincter and into the stomach.

Pneumatic dilation and botox injections have been used by gastroenterologists for decades that have varied success. Both modalities require serial treatments and have a significant overall failure rate associated with them.

Esophagomyotomy, or commonly called Heller myotomy, is a procedure that has been performed for nearly a century. This procedure is most commonly done through the abdomen, but can also be done through the chest. The procedure is performed to cut the lower esophageal sphincter relieving the obstruction. Relief of symptoms is very high and recurrence rates are low. This has been regarded at the standard of care for patients with achalasia.

Per Oral Endoscopic Myotomy or POEM Procedure

Recently, surgical endoscopists and some gastroenterologists have been able to perform an esophagomyotomy with a purely endoscopic approach. This is called the POEM procedure. The POEM procedure does not require any incisions, and there is very little to no pain. The POEM procedure is done in the operating room under general anesthesia. Post-operative care involves an average of one day in the hospital and patients are back to work in an average of 4 days. Resolutions of symptoms are equivalent to the Heller myotomy. The POEM procedure is available for any patient that can tolerate general anesthesia and have a diagnosis of achalasia.

ANIMATION OF POEM PROCEDURE

The POEM procedure has been used to treat some other esophageal motility disorders, but those are still under investigation.