Oesophagus achalasia

Question 1

How does food normally get through the lower oesophageal sphincter?

Answer 1

Decrease in the smooth muscle tone

Question 2

How is the lower oesophageal sphincter allowed to relax to allow the passage of food?

Answer 2

Relase of NO, vasoactive intestinal polypeptide from inhibitory neurons

Also interruption of normal cholinergic signalling

Question 3

What are the three things that characterise oesophageal achalasia?

Answer 3

Triad of incomplete lower oesophageal sphincter relaxation

Increased lower oesophageal sphincter tone

Aperistalsis of the oesophagus

Question 4

Describe the cause of primary oesophageal achalasia

Answer 4

Caused by failure of distal oesophageal inhibitor neurons, can be due to degeneration either within the oesophagus or extrinsically in the vagus n

Idiopathic by definition

Question 5

What is an infective cause of secondary oesophageal achalasia?

Answer 5

Trypanosoma cruzi infection, aka Chagas disease

Question 6

How can Chagas disease cause secondary oesophageal achalasia?

Answer 6

Causes destruction of the myenteric plexus, resulting in aperistalsis

Question 7

What are some conditions that can cause an achalasia-like disease?

Answer 7

Diabetic autonomic neuropathy

Infiltrative diseases inc malignancy, amyloidosis, sarcoidosis

Lesions of DRG particularly polio or surgical ablation

Question 8

What are treatment options for achalasia?

Answer 8

Laparoscopic myotomy or botox, to lower the tone in the lower oesophageal sphincter

Question 9

What is the epidemiology of oesophageal achalasia?

Answer 9

No gender preference

Not much age preference (20-60), but unusual before adolescence

Question 10

What symptoms should make you start to think about oesophageal achalasia?

Answer 10

Dysphagia for both liquids and solids

Regurg of undigested food or saliva

Also, heartburn, difficulty belching, chest pain

Question 11

What are the diagnostic steps for oesophageal achalasia?

Answer 11

Manometry is gold standard where the pressures in the lower oesophageal sphincter are measured by a catheter while the pt swallows

Barium swallow specific in only 66%

Question 12

Why should you perform endoscopy in patients with ?oesophageal achalasia?

Answer 12

Because there is a chance that it is pseudo-achalasia secondary to malignancy

Question 13

What are the typical results of a barium oesophagram in oesophageal achalasia?

Answer 13

Dilated oesophagus

Beak like tapering of the oesophagus into the stomach

Aperistalsis and poor emptying of barium into stomach

Question 14

Where in the world is Chagas disease most common?

Answer 14

Central and south america

Question 15

Differentiation between pseudo-achalasia secondary to infiltrative malignancy and primary achalasia can be difficult – what are some features from the history that can help you think of malignancy?

Answer 15

Duration of symptoms

Question 16

Manometry is the gold standard for oesophageal achalasia diagnosis – what is the cut off for lower oesophageal sphincter pressures for a +ve diagnosis?

Answer 16

Nadir must be >8mmHg above gastric pressures after swallowing (can be as high as 45mmHg during rest due to loss of inhibition)

Normally sphincter relaxes to

Question 17

What is the prognosis for people with oesophageal achalasia?

Answer 17

Can progress to oesophageal angulation, tortuosity, severe dilation or megaoesophagus (>6cm)

Some require oesophagectomy

Question 18

Does oesophageal achalasia affect someone’s lifelong chance of developing cancer in the oesophagus?

Answer 18

Yes

Somewhere between 16 and 25 fold increase in the risk of squamous cell carcinoma

But the overall chances are still low