Achalasia Summary



Epidemiology: 10 cases per 100,000 individuals



Defined by inadequate relaxation of the LES and aperistalsis

Primary or idiopathic achalasia cause is unknown.

Secondary achalasia is due to diseases that cause esophageal motor abnormalities.

Achalasia has an insidious onset, and disease progression is gradual.



Causes:

• Idiopathic

• Viral

• Autoimmune

• Neurodegenerative disorders

• Infection (Chagas disease)



Clinical Presentation:

• Dysphagia of both solids and liquids

• Regurgitation of undigested food or saliva

• Chest pain

• Hiccups

• Globus sensation



Achalasia: Testing

The diagnosis of achalasia is established by the presence of aperistalsis in the distal two-thirds of the esophagus and incomplete lower esophageal sphincter (LES) relaxation on manometry.



High-resolution esophageal manometry:

• Absence of peristalsis

• Failure of lower esophageal sphincter to relax



EGD:

• Exclude gastroesophageal junction adenocarcinoma



Barium swallow:

• “Bird’s beak” narrowing of the gastroesophageal junction

• Useful if manometry unclear



DDX:

1. GERD

2. Pseudoachalasia

3. Other esophageal motility disorders:

    • Distal esophageal spasm

    • Jackhammer esophagus



Pseudoachalasia:

Malignant tumor infiltration or other secondary causes leading to myenteric plexus damage and can present similarly to achalasia. Pseudoachalasia has been associated with sudden weight loss later in life.

Three clinical features suggest cancer as a cause of pseudoachalasia:

• Short duration of dysphagia (<1 year)

• Weight loss (>6.8 kg)

• Age older than 55 years



Suspected pseudoachalasia should be evaluated with CT, endoscopy, or endoscopic ultrasonography.



Treatment:

Laparoscopic myotomy of the LES is the first-line therapy for achalasia.



Treatment options include:

• Mechanical disruption of the muscle fibers of the LES (eg, pneumatic dilation, laparoscopic Heller myotomy, or peroral endoscopic myotomy [POEM])

• Or Biochemical reduction in LES pressure (eg, injection of botulinum toxin, use of oral nitrates)



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