Wednesday, June 12, 2024

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 appeara

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.

See Treatment and Medication for more detail.

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