Achalasia

Practice Essentials
Achalasia is a primary esophageal motility disorder characterized by the
absence of esophageal peristalsis and impaired relaxation of the lower
esophageal sphincter (LES) in response to swallowing. The LES is
hypertensive in about 50% of patients. These abnormalities cause a
functional obstruction at the gastroesophageal junction (GEJ).
Signs and symptoms
Symptoms of achalasia include the following:
 Dysphagia (most common)
 Regurgitation
 Chest pain
 Heartburn
 Weight loss
Physical examination is noncontributory.
See Presentation for more detail.
Diagnosis
Laboratory studies are noncontributory. Studies that may be helpful include
the following:
 Barium swallow: Bird’s beak appearance, esophageal dilatation (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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 Esophageal manometry (the criterion standard): Incomplete LES
relaxation in response to swallowing, high resting LES pressure, absent
esophageal peristalsis
 Prolonged esophageal pH monitoring to rule out gastroesophageal
reflux disease and determine if abnormal reflux is being caused by
treatment
 Esophagogastroduodenoscopy to rule out cancer of the GEJ or fundus
 Concomitant endoscopic ultrasonography if a tumor is suspected
See Workup for more detail.
Management
The goal of therapy for achalasia is to relieve symptoms by eliminating the
outflow resistance caused by the hypertensive and nonrelaxing LES.
Pharmacologic and other nonsurgical treatments include the following:
 Administration of calcium channel blockers and nitrates decrease LES
pressure (primarily in elderly patients who cannot undergo pneumatic
dilatation or surgery)
 Endoscopic intrasphincteric injection of botulinum toxin to block
acetylcholine release at the level of the LES (mainly in elderly patients
who are poor candidates for dilatation or surgery)
Surgical treatment includes the following:
 Laparoscopic Heller myotomy, preferably with anterior (Dor; more
common) or posterior (Toupet) partial fundoplication
 Peroral endoscopic myotomy (POEM)
Patients in whom surgery fails may be treated with an endoscopic dilatation
first. If this fails, a second operation can be attempted once the cause of
failure has been identified with imaging studies. Esophagectomy is the last
resort.

Source : https://reference.medscape.com/article/169974-overview