Achalasia

Question 1

Define Achalasia:

Answer 1

A condition in which the normal muscular activity of the oesophagus is disturbed (absent or uncoordinated) due to:

Failure or Incomplete Relaxation of the Lower oesophageal sphincter

Question 2

What actually is Achalasia?

Answer 2

Basically when the inhibitory neurones of the Myenteric plexus which control the LOS stop producing inhibitory neurotransmitters (e.g. Nitic Oxide)

These normally are released when swallowing to relax the LOS

When they stop being released, there is increased tone in the LOS

Question 3

Describe the Aetiology of both Primary and Secondary Achalasia:

Answer 3

Primary: Idiopathic degeneration of Inhibitory Ganglia of the Myenteric plexus regulating the LOS

Secondary: Oesophageal infection with Trypanosoma cruzi seen in Central/South America produces a similar disorder known as Chagas Disease.

Or could be due to extraoesophageal malignancies

Question 4

Describe the Aetiology of Achalasia:

(Sex, Age, Incidence)

Answer 4

Affects both sexes equally

Can occur at any age (25-60)

Annual incidence: 1/100,000

Question 5

What are the presenting symptoms of Achalasia?

(6)

Answer 5

Insidious onset and gradual progression of:

• Intermittent dysphagia involving solids and liquids
• Difficulty belching
• Regurgitation (particularly at night)
• Heartburn
• Chest pain (atypical/cramping, retrosternal)
• Weight loss (because they are eating less)

Question 6

What type of Oesophageal disorder is Achalasia?

Answer 6

Functional Oesophageal Disorder

(As opposed to inflammatory or mechanical)

Question 7

What physical signs may be present on examination of a patient with Achalasia?

Answer 7

Malnutrition

Weight loss

Aspiration pneumonia (complication)

Question 8

What Investigations are appropriate for Achalasia?

Answer 8

Gold Standard: Manometry

Barium Swallow

CXR

Endoscopy is usually performed to exclude other disease including malignancy which may present similarly

Question 9

What is Manometry and what would it show in Achalasia?

Answer 9

Oesophageal Motility study:

• A measure of pressure in the Oesophagus using a catheter
• Used to assess motility/peristalsis in the UOS, Oesophageal Body and LOS
• Normally shows an increase in pressure progressing down the oesophagus and then a lowering in pressure in the LOS as the bolus is passed into the stomach

In Achalasia:

• Normal in Upper Oesophagus
• Decreased peristalsis in oesophageal body
• Increased pressure in the LOS

Question 10

Achalasia causes an increased risk of what?

Answer 10

Oesophageal Squamous cell carcinoma & Adenocarcinoma

Question 11

What would you expect to see on a barium swallow of a patient with Achalasia?

Answer 11

‘Bird-beak Sign’

• Dilated oesophagus which smoothly tapers down to the sphincter

Question 12

What may a CXR show in Achalasia?

Answer 12

Widened mediastinum

Double right heart border (dilated oesophagus)

Air‐fluid level in the upper chest

Absence of the normal gastric air bubble

Question 13

A patient presents to you and you suspect Achalasia. ODQ he reveals he recently returned from a trip to Central America.

What further investigations in addition to the normal tests for achalasia may be appropriate?

Answer 13

Serology for antibodies against Trypanosoma cruzi

If +ve suspect Chagas Disease

Blood film may also show parasites

Question 14

Suggest two possible treatments for Achalasia:

Answer 14

Balloon dilation of the obstruction at the LOS

Manage the increased tone of the LOS with endoscopic botox injections into the LOS