Achalasia

Achalasia (primary achalasia) refers to a failure of organised oesophageal peristalsis with an impaired relaxation of the lower oesophageal sphincter (LOS), resulting in often marked dilatation of the oesophagus and food stasis. Obstruction of the distal oesophagus (often due to tumour) has been termed "secondary achalasia" or "pseudoachalasia".

Epidemiology

Primary achalasia is most frequently seen in middle and late adulthood (30-70 years of age) with no gender predilection 6. In most cases achalasia is idiopathic; however, an identical appearance is seen in patients with Chagas disease. Authors differ as to whether they reserve the term achalasia for idiopathic cases, or whether they include Chagas disease.

Clinical presentation

Typically patients present with dysphagia (which is for both solids and liquids, in comparison to dysphagia for solids only in cases of esophageal carcinoma 7), chest pain/discomfort and eventual regurgitation. Initially, symptoms are intermittent. Patients may also present with a complication of long-standing achalasia:

  • oesophageal carcinoma: is the most dreaded complication and is seen in approximately 5%, and most often in the mid oesophagus. It is thought to relate to the chronic irritation of the mucosa by stasis of food and secretions.
  • aspiration pneumonia
  • candida oesophagitis
  • acute airway obstruction: this is a rare complication requiring immediate oesophageal decompression with a nasogastric tube

Pathology

Peristalsis in the distal smooth muscle segment of the oesophagus may be lost due to an abnormality of Auerbach plexus (responsible for smooth muscle relaxation), resulting in weak contractions that are uncoordinated and therefore non-propulsive. The abnormality may also occur in the vagus nerve or the dorsal motor nucleus of the vagus nerve. 

The LOS eventually fails to relax, either partially or completely, with elevated pressures demonstrated manometrically 4. Early in the course of achalasia, this LOS tone may be normal or changes may be subtle.

Radiographic features

Achalasia characteristically involves a short segment (less than 3.5 cm) of the distal oesophagus.

Plain radiograph

Chest x-ray findings include:

  • right convex opacity behind right cardiac border; occasionally left convex opacity if thoracic aorta tortuous
  • air-fluid level due to stasis in thoracic oesophagus filled with retained secretions and food
  • small/absent gastric air bubble
  • anterior displacement and bowing of trachea on the lateral view

The chronic presence of fluid debris in the oesophagus makes patients very prone to aspiration and thus patchy bilateral alveolar opacities representing acute or chronic aspiration pneumonia may be seen.

Fluoroscopy: barium swallow

A barium swallow is able to not only confirm that the oesophagus is dilated but is also able to assess for mucosal abnormalities. Findings include:

  • bird beak sign
  • oesophageal dilatation
  • incomplete LOS relaxation that is not coordinated with oesophageal contraction
  • pooling or stasis of barium in the oesophagus when the oesophagus has become atonic or non-contractile ( a late feature in the disease)
  • uncoordinated, non-propulsive, tertiary contractions (see case 1)
  • failure of normal peristalsis to clear the oesophagus of barium when the patient is in the recumbent position, with no primary waves identified
  • when barium column is high enough (patient standing) the hydrostatic pressure can overcome the LOS pressure allowing passage of oesophageal content
  • A hot or carbonated drink during the exam may help visualize sphincter relaxation and barium emptying.

CT

Patients with uncomplicated achalasia demonstrate a dilated thin walled oesophagus filled with fluid/food debris. 

Overall, CT has little role in directly assessing patients with achalasia, but is useful in assessing common complications. Careful assessment of the wall of the oesophagus should be undertaken to identify any focal regions of thickening which may indicate malignancy. The lungs should be inspected for evidence of aspiration.

Treatment and prognosis

Treatment is aimed at allowing adequate drainage of the oesophagus into the stomach. Options include 4-5:

  • calcium channel blockers
    • ineffective in the long term
    • may be used while preparing for definitive treatment
  • pneumatic dilatation
    • effective in 85% of patients
    • 3-5% risk of bleeding/perforation
  • botulinum toxin injection
    • lasts only ~12 months per treatment
    • may scar the submucosa leading to increased risk of perforation during subsequent myotomy
  • surgical myotomy (e.g. Heller myotomy)
    • 10-30% of patients develop gastro-oesophageal reflux, and thus it is often combined with a fundoplications (e.g. Dor, Toupet, Nissen)
    • the use of per-oral oesophageal myotomy (POEM procedure) is increasing in select patients.

History and etymology

The word achalasia stems from the Greek term "does not relax".

Differential diagnosis

A number of entities may mimic achalasia, forming the so called 'achalasia pattern'. 

Other oesophageal disorders should also be considered:


Oesophageal pathology
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rID: 835
Section: Pathology
Synonyms or Alternate Spellings:
  • Primary achalasia
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