Motility disorder of the oesophagus caused by degenerative changes in the inhibitory neurons around the lower oesophagus

Aetiology

• Currently unknown, may be autoimmune

Pathophysiology

• Oesophageal sphincter will not relax properly - food cannot pass into the stomach in the normal manner
• Peristaltic contractions of the oesophagus do not propagate properly -oesophagus becomes increasingly dilated

Clinical features

• Usually presents in the young
• Progressive dysphagia

Investigations

• Barium swallow: for screening -‘bird’s beak’

• Manometry: to confirm, measures pressure within the oesophagus

Management

• Usually involves stretching/slicing of LOS
• Motility disorder of the oesophagus caused by degenerative changes in the inhibitory neurons around the lower oesophagus