Achalasia

• • Dysphagia

  • Retention of ingested food in the esophagus

  • Radiologic evidence of absent primary peristalsis, dilated body of the esophagus, and a conically narrowed cardioesophageal junction

  • Absent primary peristalsis by manometry and cineradiography

Epidemiology

• • Achalasia is a neuromuscular disorder; esophageal dilation and hypertrophy occur without organic stenosis

  • Primary peristalsis is absent and the cardioesophageal sphincter fails to relax in response to swallowing; the circular muscle layer hypertrophies

  • There is absence, atrophy, or disintegration of the ganglion cells of Auerbach myenteric plexuses and a reduction in nerve fibers within the wall of the esophagus

  • The cause is unknown, but 2 theories exist:

  • 1. A degenerative disease of the neurons

    2. Infection of neurons by a virus (eg, herpes zoster) or other infectious pathogen

  • Achalasia affects males more often than females and may develop at any age; peak incidence ranges from 30 to 60 years

Symptoms and Signs

• • Dysphagia is dominant symptom

  • Weight loss is not usually marked despite the functional obstruction

  • Pain is infrequent

  • Regurgitation of retained esophageal contents is common, especially during the night while the patient sleeps in a recumbent position

  • A variant called vigorous achalasia is characterized by chest pain and esophageal spasms that generate nonpropulsive high-pressure waves in the body of the esophagus

Imaging Findings

• • Contrast radiography and endoscopy

  • -Narrowing at the cardia

    -The dilated body of the esophagus blends into a smooth cone-shaped area of narrowing 3-6 cm long

    -As the disease progresses, the esophagus dilates further and becomes tortuous

  • Manometry

  • -The body of the esophagus is devoid of primary peristaltic waves, but simultaneous disorganized muscular activity may be present

    -Pressure in the gastroesophageal sphincter is increased; relaxation after swallowing is incomplete or absent

• • Symptoms should prompt contrast radiographic or endoscopic studies

  • Endoscopy is essential for establishing the diagnosis and excluding other causes of symptoms

  • Manometry is useful for confirming diagnosis

Rule Out

• • Benign strictures of the lower esophagus

  • Carcinoma at or near the cardioesophageal junction

  • Diffuse esophageal spasm

• • Gastroesophageal contrast radiography

  • Upper GI endoscopy

  • Esophageal manometry

  • pH study, particularly if symptoms of reflux are present or fundoplication is planned

• • Goal is to relieve the functional obstruction either by pneumatic dilation or longitudinal division of all the esophageal muscular layers (Heller myotomy).

Surgery

Indications

• • Advanced disease

  • Failed dilation

  • If no reflux symptoms exist, the need to add an antireflux procedure has not been established

Treatment Monitoring

• • Measure rate of esophageal passage of a technetium Tc 99m-labeled solid meal

  • Periodic esophagoscopy; treatment of achalasia does not lessen the increased risk ...