An oesophegeal motor disorder

Failure of oesophageal peristalsis and of relaxation of the lower oesophageal sphincter (LOS) due to degenerative loss of ganglia from myenteric (Auerbach's) plexus i.e. LOS contracted, oesophagus above dilated.

Aetiology: Unknown aetiology, may be linked to an infection

Epidemiology: Achalasia typically presents in middle-age and is equally common in men and women.

PC

• Key Sx - dysphagia of BOTH liquids and solids
• Retrosternal pressure/pain
• typically variation in severity of symptoms
• regurgitation of food
  • may lead to cough, aspiration pneumonia etc
• heartburn (uncommon)

Investigations

• oesophageal manometry
  • excessive LOS tone which doesn't relax on swallowing
  • considered the most important diagnostic test
• barium swallow
  • shows grossly expanded oesophagus, fluid level
  • 'bird's beak' appearance
• chest x-ray
  • wide mediastinum
  • fluid level

Management

• drug therapy (e.g. nitrates, calcium channel blockers prior to meals) - has a role but is limited by side-effects - Can be used whilst awaiting definitive Tx.
• pneumatic (balloon) dilation is increasingly the preferred first-line option
  • less invasive and quicker recovery time than surgery
  • patients should be a low surgical risk as surgery may be required if complications occur