achalasia

Question 1

definition

Answer 1

Achalasia, from the Greek for ‘does not relax’, is an oesophageal motor disorder of unknown aetiology, characterised by oesophageal aperistalsis and insufficient lower oesophageal sphincter (LOS) relaxation in response to swallowing. This results from loss of inhibitory nitrinergic neurons in the oesophageal myenteric plexus.

Question 2

symptoms

Answer 2

insidious onset.
gradual progression of:
- intermittent dysphagia involving solids and liquids 
- difficulty belching 
- regurgitation (particularly at night)
- heartburn
- chest pain 
- weight loss

• retrosternal pressure/pain (sometimes the feeling is alleviated by cold water)

Question 3

investigations

Answer 3

1st investigations:

• upper GI endoscopy – (this may not be fully sensitive to early achalasia, but is important to exclude malignancy!!)
• barium swallow (+ fluoroscopy)
• oesophageal manometry

others:
CXR
radionucleotide oesophageal emptying study -- used to monitor progress of treatment
timed barium oesophagogram
CT chest

Question 4

risk factors

Answer 4

• allgrove syndrome (triple A disorder: achalasia, alacrima and adrenal insufficiency. caused by ACTH insensitivity)