Achalasia: Year of the Zebra

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Achalasia is an esophageal disorder that makes it difficult for food to pass from the esophagus into the stomach, causing symptoms such as difficulty swallowing, regurgitation of undigested food, and chest pain.With achalasia, esophageal peristalsis is impaired due to progressive degeneration of myenteric neurons, which are neurons of the gastrointestinal tract.
Impairment of these neurons prevents food from being effectively moved through the esophagus. It also prevents the lower esophageal sphincter from completely relaxing during swallowing, which stops food from passing, and leads to dilation of the esophagus as food builds up.
Most cases of achalasia occur due to an unknown cause, though there may be secondary causes, including damage to myenteric neurons by Trypanosoma cruzi, the organisms responsible for Chagas disease; or extraesophageal malignancies.Now, the most common symptom of achalasia is difficulty swallowing, also known as dysphagia.
With achalasia, dysphagia appears slowly and gradually, so some people may compensate by eating more slowly or by lifting the neck or throwing the shoulders back when swallowing to improve emptying of the esophagus.
Other symptoms may include regurgitation of undigested food, weight loss, chest pain and heartburn.Diagnosis of achalasia is based on the individual’s symptoms, but tests are needed to confirm the diagnosis.
The barium swallow test is a common screening test for achalasia. It involves swallowing a chalky, thick mixture of barium while x-rays are taken.
The barium swallow test shows dilation of the esophagus and a narrow region at the end classically described as a “bird’s beak” appearance.
However, the gold standard for diagnosis is esophageal manometry, which measures the strength and coordination of the esophageal contractions when an individual swallows.
Manometry typically reveals an absence of peristaltic contractions in the lower esophagus, high pressure in the lower esophageal sphincter at rest, and failure of the lower esophageal sphincter to relax during swallowing.
Although an upper endoscopy is not useful to diagnose achalasia, it may be done to rule out esophageal cancer or secondary causes of achalasia.Treatment for achalasia focuses on relieving the symptoms and reducing the pressure of the lower esophageal sphincter so that it no longer poses a barrier to the passage of food.
The lower esophageal sphincter can be weakened mechanically by procedures that tear or cut the lower esophageal sphincter muscle or weakened by medications that decrease pressure in the lower esophageal sphincter.
The most effective and long-lasting treatment is a Heller laparoscopic myotomy, a surgical intervention where a small cut is made in the lower sphincter to relieve that pressure.
Other options involve pneumatic dilation of the lower esophageal sphincter and botulinum toxin injections. Medical therapy with calcium-channel blockers or sublingual nitrates is a generally ineffective treatment for achalasia, but it can be used in cases where surgery cannot be done and botulinum toxin injections are not effective.
Alright, as a quick recap, achalasia is an esophageal disorder characterized by impaired esophageal motility and incomplete lower esophageal sphincter relaxation that causes progressive dysphagia, regurgitation of food, and chest pain.
The gold standard for diagnosis is esophageal manometry which shows high pressure in the lower esophageal sphincter, incomplete lower sphincter relaxation, and decreased esophageal peristalsis.
Treatment focuses on reducing the pressure of the lower esophageal sphincter, which can be accomplished by procedures that tear or cut the sphincter muscle; or by medications.