Approach Considerations

A diagnosis of achalasia should be considered when patients present with dysphagia, chest pain, and refractory reflux symptoms after an endoscopy does not reveal a mechanical obstruction or an inflammatory cause of esophageal symptoms.^[9]

The American College of Gastroenterology released new guidelines for the diagnosis and management of achalasia in July 2013.^{[10, 11]}Recommendations for the proper diagnosis of the disorder include the following:

Performing an esophageal motility test on all patients suspected of having achalasia
Using esophagram findings to support a diagnosis
Using barium esophagram, as recommended for patients with equivocal motility testing
Endoscopic assessment of the gastroesophageal junction and gastric cardia, as recommended, to rule out pseudoachalasia

Laboratory studies are generally noncontributory.

Esophageal pressure topography (EPT) may be the preferred assessment modality of esophageal motility over conventional line tracings (CLT).^[12] Six attending gastroenterologists and six gastroenterology fellows from 3 academic centers interpreted each of the 40 studies using both EPT and CLT formats: Among all raters, the odds of an incorrect exact esophageal motility diagnosis were 3.3 times higher with CLT than with EPT, and the odds of incorrect identification of a major motility disorder were 3.4 times higher with CLT than with EPT.

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 the image below).

Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.

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Other Tests

Esophageal manometry (see the image below) is the criterion standard in helping to diagnose the classic findings of achalasia.^[13]These findings include the following:

Incomplete relaxation of the LES in response to swallowing
High resting LES pressure
Absent esophageal peristalsis

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.
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Prolonged esophageal pH monitoring is important for the following reasons:

To rule out gastroesophageal reflux disease (GERD)
To determine if abnormal reflux is being caused by treatment.^[14]

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.

Treatment & Management

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Media Gallery

Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.
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.
Heller myotomy extending 1.5 cm onto the gastric wall.
Dor fundoplication, left row of sutures (after division of short gastric vessels).
Completed Dor fundoplication.

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Achalasia Workup

Approach Considerations

Imaging Studies

Barium swallow

Other Tests

Procedures

References

Tables

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