Must suspect achalasia if dysphagia to solids and liquids and if regurgitation unresponsive to PPI trial (prevalence = 10 in 100,000).

All patients with suspected achalasia without mechanical obstruction on endoscopy or esophagram should undergo esophageal motility testing to confirm diagnosis.

Achalasia diagnosis is supported by dilated esophagus, narrow esophagogastric junction with “bird beak” appearance, aperistalsis, incomplete lower esophageal sphincter relaxation, poor emptying of barium on esophagram.

If motility testing equivocal, barium esophagram recommended to assess esophageal emptying and esophagogastric junction.

Endoscopy to assess gastroesophageal junction and cardia is recommended in all achalasia patients to rule out pseudoachalasia.

Graded pneumatic dilation (PD) or laparoscopic surgical myotomy with partial fundoplication are recommended as initial achalasia therapy (if fit and willing to have surgery).

Surgical myotomy and PD should be performed at high-volume centers of excellence.

Initial therapy should be guided by patient age, gender, preference and local institutional experience.

In poor surgical candidates, botulinum toxin injection is recommended.

In patients unwilling or unfit for PD or surgery who fail botulinum toxin, pharmacologic therapy recommended (e.g. calcium channel blockers, long-acting nitrates).

Post-therapy follow-up may include esophagram to assess symptom relief and esophageal emptying.

Surveillance endoscopy for esophageal cancer not recommended (400 endoscopies to detect 1 cancer).