Introduction

Failure of oesophageal peristalsis and of relaxation of lower oesophageal sphincter (LOS) due to degenerative loss of ganglia from Auerbach's plexus i.e. LOS contracted, oesophagus above dilated. Achalasia typically presents in middle-age and is equally common in men and women.

Epidemiology

  • Incidence: 2.00 cases per 100,000 person-years
  • Peak incidence: 50-60 years
  • Sex ratio: 1:1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Clinical features

Clinical features
  • dysphagia of BOTH liquids and solids
  • typically variation in severity of symptoms
  • heartburn
  • regurgitation of food - may lead to cough, aspiration pneumonia etc
  • malignant change in small number of patients

Investigations

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

Treatment
  • 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
  • surgical intervention with a Heller cardiomyotomy should be considered if recurrent or persistent symptoms
  • intra-sphincteric injection of botulinum toxin is sometimes used in patients who are a high surgical risk
  • drug therapy (e.g. nitrates, calcium channel blockers) has a role but is limited by side-effects