The current guidelines on the diagnosis and treatment of achalasia as published in the American Journal of Gastroenterology by the American College of Gastroenterology are summarized below.

Achalasia is an incurable disease with an incidence of 1/100,000 occurring equally among men and women. Achalasia is characterized by insufficient relaxation of the lower esophageal sphincter and loss of esophageal peristalsis, leading to difficulty in swallowing with dysphagia to solids and liquids, regurgitation, and occasional chest pain with or without weight loss. Achalasia is often misdiagnosed as gastroesophageal reflux disease (GERD). Achalasia must be suspected in those with dysphagia to solids and liquids and in those with regurgitation unresponsive to an adequate trial of proton pump inhibitor (PPI) therapy.
Endoscopic finding of retained saliva with a puckered gastroesophageal junction or a barium swallow showing a dilated esophagus with birds beaking in a symptomatic patient should prompt appropriate diagnostic and therapeutic strategies.
Graded pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy for the treatment of achalasia. Surgical myotomy has shown excellent results in most patients and remains the surgery of choice, with more being done laparoscopically. The benefit of adding a fundoplication was demonstrated and a cost–utility analysis found that myotomy plus fundoplication was more cost effective than myotomy alone. Retreatment of patients is likely required within 5 years; retreatment should be individualized based on local expertise.
The guidelines also call for follow-up for symptom relief and esophagus emptying results through use of barium esophagram. Endoscopy surveillance for esophageal cancer is not recommended.

DIAGNOSIS OF ACHALASIA

• All patients with suspected achalasia who do not have evidence of a mechanical obstruction on endoscopy or esophagogram should undergo esophageal motility testing before a diagnosis of achalasia can be confirmed.
• The diagnosis of achalasia is supported by esophagogram findings including dilation of the esophagus, a narrow esophago-gastric junction with “ bird-beak ” appearance, aperistalsis, and poor emptying of barium.
• Barium esophagogram is recommended to assess esophageal emptying and esophago-gastric junction morphology in those with equivocal motility testing.
• Endoscopic assessment of the gastroesophageal junction and gastric cardia is recommended in all patients with achalasia to rule out pseudoachalasia.

TAILORED APPROACH TO TREATING ACHALASIA

• Either graded pneumatic dilation (PD) or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy for the treatment of achalasia in those fit and willing to undergo surgery.
• PD and surgical myotomy should be performed in high-volume centers of excellence.
• The choice of initial therapy should be guided by patients’ age, gender, preference, and local institutional expertise.
• Botulinum toxin therapy is recommended in patients who are not good candidates for more definitive therapy with PD or surgical myotomy.
• Pharmacologic therapy for achalasia is recommended for patients who are unwilling or cannot undergo definitive treatment with either PD or surgical myotomy and have failed botulinum toxin therapy.

PATIENT FOLLOW-UP

• Patient follow-up after therapy may include assessment of both symptom relief and esophageal emptying by barium esophagogram.
• Surveillance endoscopy for esophageal cancer is not recommended.

In summary the new guidelines for diagnosis include:

1. Performing an esophageal motility test on all patients suspected of having achalasia (the manometric findings of irregular muscle contractions and incomplete lower esophageal sphincter relaxation without mechanical obstruction establish the diagnosis of achalasia in the appropriate setting);
2. Using esophagogram findings to support a diagnosis;
3. Using an esophagogram for patients with equivocal motility testing;
4. Using endoscopic assessment of the gastroesophageal junction and gastric cardia to rule out pseudoachalasia.

Treatment recommendations offer a tailored approach:

1. Initial therapy should be either graded pneumatic dilation (PD) or laparoscopic surgical myotomy with a partial fundoplication in patients fit to undergo surgery;
2. Procedures should be performed in high-volume centers of excellence;
3. Initial therapy should be based on patient age, sex, preference, and local institutional expertise;
4. Botulinum toxin therapy is recommended for patients not suited for PD or surgery;
5. Pharmacologic therapy can be used for patients not undergoing PD or myotomy and who have failed botulinum toxin therapy (nitrates and calcium channel blockers are most commonly used).