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Achalasia and Gastroesophageal Reflux Disease

1. ACHALASIA

  • MC in 25-60yo. Equal M:F
  • 1o oesophageal motility disorder – characterised by failure of a hypertonic lower oesophageal sphincter (LOS) to relax and the absence of oesophageal peristalsis
  • No food passage from oesophagus to stomach – causes regurgitation. Progressive disorder causes dysphagia of liquids.

Pathology

  • LOS pressure and relaxation are regulated by excitatory (Ach, substance P) and inhibitory (NO, VIP) neurotransmitters – in oesophagus there is imbalance of these neurotransmitters.
  • Neurodegeneration – degeneration of Auerbach’s myenteric plexus along entire length of oesophagus and more so in LOS
    • Leads to hypertension (HTN) of LOS > failure of sphincter to relax on swallowing > elevation of intraluminal oesophageal pressure > oesophageal dilation > loss of peristalsis in body of oesophagus
  • Functional obstruction of gastro-oesophageal junction.
  • Myenteric inflam, depletion of ganglion cells, neural fibrosis, reduced NO and VIP (mediators of LOS relaxation)

Clinical features

  • Dysphagia – only for solids. Liquids pass through normally (may progress to liquids if severe)
    • Nocturnal pulmonary aspiration develops as dysphagia worsens
  • Regurgitation
  • Chest pain – due to oesophageal spasm (AKA cardiospasm)
  • Weight loss

Investigations

  • Barium study – narrowing of oesophagus (dilation in later stages)
  • Biopsy – if squamous cell carcinoma risk
  • XR – bird beak seen
  • Manometry – best
    • Shows failure of LOS to relax during swallowing and absence of peristalsis
    • LOS P >35mmHg
  • Oesophagogastroduodenoscopy

Treatment

  • Initially – pneumatic dilation
  • Botox injection – decreases release of Ach
  • CCB and nitrates to decrease LOS pressure
  • Heller’s myotomy – cut muscles of LOS and cardia
    • Leave mucosa intact

Secondary causes of Achalasia

  • Neurological – stroke, bulbar palsy, MND, MS, poliomyelitis
  • Muscular – Eaton-Lambert myasthenia gravis, MD
  • Autoimmune – systemic sclerosis, polymyositis, SLE, CREST syndrome , scleroderma
  • Endocrine and metastatic disease
  • Chagas disease – Trypanosoma cruzi

DDx

Oesophageal cancer, oesophageal stricture, GORD,  Plummer Vinson Syndrome –

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