[7] Oesophageal Motility Disorders

Question 1

What are oesophageal motility disorders?

Answer 1

A group of conditions characterised by abnormalities in oesophageal peristalsis

Question 2

How does the prevalence of oesophageal motility disorders compare to other oesophageal disorders?

Answer 2

They are less common than mechanical and inflammatory diseases of the oesophagus

Question 3

How do oesophageal motility disorders typically manifest?

Answer 3

With difficulty swallowing solids and liquids

Question 4

What are the major causes of oesophageal dysmotility?

Answer 4

Achalasia

Diffuse oesophageal spasm

Question 5

How long is the oesophagus?

Answer 5

25cm

Question 6

What is the upper third of the oesophagus composed of?

Answer 6

Skeletal muscle

Question 7

What is the middle third of the oesophagus composed of?

Answer 7

It is a transition zone comprised of both skeletal and smooth muscle

Question 8

What is the lower third of the oesophagus composed of?

Answer 8

Smooth muscle

Question 9

What is the upper oesophageal sphincter comprised of?

Answer 9

Skeletal muscle

Question 10

What is the purpose of the upper oesophageal sphincter?

Answer 10

It prevents air from entering the GI tract

Question 11

What is the lower oesophageal sphincter composed of?

Answer 11

Smooth muscle

Question 12

What is the purpose of the lower oesophageal sphincter?

Answer 12

It prevents reflux from the stomach

Question 13

What propels ingested food down the oesophagus?

Answer 13

Peristaltic waves

Question 14

What controls the peristaltic waves in the oesophagus?

Answer 14

Oesophageal myenteric neurones

Question 15

What is the primary peristaltic wave of the oesophagus under the control of?

Answer 15

Swallowing centre

Question 16

What is the secondary peristaltic wave of the oesophagus controlled by?

Answer 16

It is activated in response to distention

Question 17

What happens as food descends the oesophagus?

Answer 17

The lower oesophageal sphincter relaxes, and remains so until food has passed

Question 18

What is achalasia?

Answer 18

A primary motility disorder of the oesophagus, characterised by failure of smooth muscle relaxation

Question 19

How common is achalasia?

Answer 19

Relatively rare (1 per 100,000)

Question 20

What is the mean age of diagnosis of achalasia?

Answer 20

About 50 years

Question 21

What is the pathophysiology of achlasia?

Answer 21

Unknown

Question 22

What is a common histological feature of achlasia?

Answer 22

Progressive destruction of the ganglion cells in the myenteric plexus.

Question 23

What does the failure of smooth muscle to relax in achalasia cause?

Answer 23

An inability of the oesophagus to relax, and a high resting tone and failure of relaxation of the lower oesophageal sphincter

Question 24

What is the result of failure of the oesophagus to relax in achalasia?

Answer 24

Causes difficulty in passing food boluses down the oesophagus

Question 25

What is the result of the high resting tone and failure of relaxation of the lower oesophageal sphincter in achalasia?

Answer 25

Means that the food bolus may get stuck, and fail to pass into the stomach

Question 26

What is the result of the failure of food to pass into the stomach in achalasia?

Answer 26

Produces the symptoms of vomiting, discomfort, and developing poor nutritional status

Question 27

Why is achlasia a progressive disease?

Answer 27

The current theory in its pathophysiology suggests that as the ganglionitis progresses, there is destruction of more and more neurones and subsequent worsening of severity of the condition

Question 28

How will achalasia classically present?

Answer 28

``` With progressive dysphagia when ingesting solids and liquids Regurgitation of food Coughing Chest pain Weight loss ```

Question 29

How can achalasia cause coughing?

Answer 29

Due to overspill and aspiration, especially at night

Question 30

What may be found on examination with achalasia?

Answer 30

There are rarely any obvious signs of note, except for visible weight loss in longstanding or severe cases

Question 31

What are the main differential diagnoses for achalasia?

Answer 31

Other oesophageal motility disorders GORD Oesophageal malignancy Angina

Question 32

What needs to be excluded in any patient presenting with dysphagia?

Answer 32

Oesophageal cancer

Question 33

What is the result of the need to exclude oesophageal cancer in patients with dysphagia?

Answer 33

Nearly all patients will require urgent endoscopy

Question 34

What may be found on endoscopy in achalasia?

Answer 34

May be normal | Rarely, there is a tight lower oesophageal sphincter (which may suddenly give way)

Question 35

What is the gold standard for diagnosis of achalasia?

Answer 35

Oesophageal manometry

Question 36

What happens in oesophageal manometry?

Answer 36

A pressure sensitive probe is inserted into the oesophagus (tip placed 5cm above lower oesophageal sphincter). Then, the pressure of the sphincter and the surrounding muscle is measured

Question 37

What are the key features of oesophageal manometry in achalasia?

Answer 37

Absence of oesophageal peristalsis Failure of relaxation of the lower oesophageal sphincter High resting lower oesophageal sphincter tone

Question 38

What may barium swallows show in achalasia?

Answer 38

May show proximal dilation of the oesophagus, with a characteristic 'birds beak' appearance distally

Question 39

What is involved in the conservative management of achalasia?

Answer 39

Sleep with many pillows Eating slowly and chewing foods thoroughly Taking plenty of fluids with meals

Question 40

What medications can be used in the conservative management of achalasia?

Answer 40

Calcium channel blockers or nitrates | Botox injections

Question 41

How useful are calcium channel blockers/nitrates in achalasia?

Answer 41

They can be partly effective for temporary relief, but their action is typically short live

Question 42

How are botox injections given in achalasia?

Answer 42

They are injected into the lower oesophageal sphincter by endoscopy

Question 43

How long are botox injections effective for in achalasia?

Answer 43

A few months at most

Question 44

What are the surgical techniques that can be used in achalasia?

Answer 44

Endoscopic balloon dilation | Laparoscopic Heller myotomy

Question 45

What happens in endoscopic balloon dilation for achalasia?

Answer 45

A balloon is inserted into the lower oesophageal sphincter, which is dilated to stretch the muscle fibres

Question 46

What % of patients have a good response to endoscopic balloon dilation?

Answer 46

75%

Question 47

What are the risks with endoscopic balloon dilation?

Answer 47

Perforation | Need for further intervention

Question 48

What is the risk of perforation with endoscopic balloon dilation?

Answer 48

5%

Question 49

What happens in a laparoscopic Heller myotomy?

Answer 49

There is division of the specific fibres of the lower oesophageal sphincter which fail to relax

Question 50

What % of patients with achalasia have a long-term improvement in swallowing with a laparoscopic Heller myotomy?

Answer 50

85%

Question 51

What is the advantage of a laparoscopic Heller myotomy over endoscopic balloon dilation?

Answer 51

It has a lower side effect profile

Question 52

By how much does having long-standing achalasia increase the risk of oesophageal cancer?

Answer 52

8-16x, although the absolute risk remains small

Question 53

What is diffuse oesophageal spasm (DOS)?

Answer 53

A disease characterised by multi-focal, high amplitude contractions of the oesophagus

Question 54

What is diffuse oesophageal spasm thought to be caused by?

Answer 54

Dysfunction of the oesophageal inhibitory nerves

Question 55

What can DOS progress too in some patients?

Answer 55

Achalasia

Question 56

How will patients with DOS typically present?

Answer 56

Severe dysphagia to both solids and liquids | Central chest pain, usually exacerbated by food

Question 57

What may the pain from DOS respond well to?

Answer 57

Nitrates

Question 58

What is the problem with the pain from DOS responding well to nitrates?

Answer 58

it can make it difficult to distinguish from angina

Question 59

What feature might differentiate NOS pain from angina?

Answer 59

NOS pain is rarely exertional

Question 60

What is found on examination in NOS?

Answer 60

Examination is usually normal

Question 61

How is DOS investigated?

Answer 61

In the same manner as other motility disorders, with the definitive diagnosis being made via manometry

Question 62

What is found on endoscopy in DOS?

Answer 62

Usually normal

Question 63

What does manometry characteristically show in DOS?

Answer 63

A pattern of repetitive, simultaneous, and ineffective contractions of the oesophagus May also be dysfunction of the lower oesophageal sphincter

Question 64

What may a barium swallow show in DOS?

Answer 64

'Corkscrew' appearance

Question 65

What is the initial management of DOS?

Answer 65

Agents that act to relax the oesophageal smooth muscle, typically nitrates or calcium channel blockers as first line

Question 66

How effective are nitrates or calcium channel blockers at managing DOS?

Answer 66

They limit the strongest contractions, so provide symptomatic improvement, although their long-term efficacy is unceratin

Question 67

Which patients with DOS might benefit from pneumatic dilation?

Answer 67

Patients with DOS and documented hypertension of the lower oesophageal sphincter

Question 68

When is myotomy used in DOS?

Answer 68

Reserved for the most severe cases

Question 69

Why must myotomy be used with caution in DOS?

Answer 69

Due to its invasive nature

Question 70

Describe the excision used in myotomy for DOS?

Answer 70

It is extensive, involving the entire spasmic segment and the lower oesophageal sphincter

Question 71

What other conditions are associated with oesophageal dysmotility?

Answer 71

A number of autoimmune and connective tissue disorders

Question 72

How is oesophageal dysmotility managed when it is secondary to another condition?

Answer 72

Treatment is directed at the underlying cause, with nutritional modification and PPIs as required