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Oesophageal achalasia and Cardiospasmus

  • Motor disorder of the oesophagus, due to damaged/degenerated cells in the myenteric plexus
  • Results in complete loss of oesophageal peristalsis and failure of lower oesophageal sphincter (LOS) relaxation
  • Leads to obstruction at the gastro-oesophageal junction (GOJ) and loss of effective propulsion

Etiology

  • Decrease/absence of ganglions in myenteric plexus
  • Most commonly it is idiopathic degeneration of Auerbach’s myenteric plexus
  • Other causes
    • Stress, vitamin B1 def
    • Chagas disease – Trypanosoma cruzi (sleeping sickness)
  • It is a precancerous condition – x7 chance of getting small cell cancer
  • Mean age of onset is between 30-60 years

Pathology

  • Thickening of circular muscle fibres in the distal oesophagus
  • Myenteric inflammation, depletion of ganglion cells and neural fibrosis
  • Levels of NO and VIP are reduced (mediators of LOS relaxation)
  • There is absence of peristalsis, raised LOS pressure and failure of relaxation with functional obstruction of GOJ
  • There is narrowing of the cardia with enormous dilation of the proximal oesophagus
    • Contains foul-smelling fluid – patients are more prone to aspiration pneumonia

Clinical features

  • Triad – dysphagia, regurgitation, weight loss
  • Initially presents as solid food dysphagia – as food is unable to pass into the stomach and accumulates above LOS
  • Eventually there is dysphagia to solids and liquids
  • Chest pain occurs in early stage
  • Regurgitation and recurrent pneumonia
  • Heartburn
  • Malnutrition and general ill health
  • Lung abscess formation

Investigations

  • Barium swallow XR
    • Bird beak sign – narrowing of the lower oesophagus
    • Dilation of proximal oesophagus
  • Oesophageal manometry – gold standard
    • Demonstrates unrelaxed LOS with high resting pressure
    • Shows failure of LOS to relax during swallowing and absence of peristalsis
  • Oesophagoscopy – confirms diagnosis and rules out carcinoma of the oesophagus

Treatment

  • Drugs – botulinum toxin, nitroglycerine, nifedipine
  • Modified Heller’s op – oesophagoardiomyotomy
    • Thickened muscle fibres cut longitudinally
  • Forcible dilation – stretches spasmodic segment
    • Gradual repeat dilations
      • Plummer’s pneumatic dilation – use balloons of 30-40mmHg
      • Negus hydrostatic dilation – dilates GOJ (high risk of perforation)
  • Laparoscopic/thoracoscopic cardiomyotomy
  • Resection – done if there is failure of myotomy or when mega-oesophagus/metaplasia present
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