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Classification of esophageal motility
disorders
Samir Haffar M.D.
Indications of esophageal motility study
• Dysphagia Not explained by stenoses or
inflammation of the esophagus
• Chest pain Not explained by heart disease or
other thoracic disorders
Pressure relationship in UES, esophagus,
LES & Stomach
Placement of esophageal motility catheter
within the esophagus
Gastrointest Endoscopy Clin N Am 2005 ; 15 : 243 – 255.
Normal esophageal motility test
Normal esophageal manometric features
• Basal LOS pressure 10 – 45 mm Hg (mid respiratory
pressure measured by station pull
through technique)
• LES relax with swallow Complete (to a level < 8 mm Hg
above gastric pressure)
• Wave progression Peristalsis progressing from UES
through LES at rate of 2 – 8 cm/s
• Distal wave amplitude 30 – 180 mm Hg (average of 10
swallows at 2 recording sites
positioned 3 & 8 cm above LES)
Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
Mid respiratory measurements of LES
Most commonly used
Normal values: 24.4  10.1 mmHg
* Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
End expiratory measurements of LES
Normal values: 15.2  10.7 mmHg
* Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
LES pressure
• The crural diaphragm
• The LES muscle
Reflects pressure generated by
Normal LES Relaxation
Residual Pressure (RP)
Difference between lower pressure achieved & GBP
RP better than percentage of relaxation
Normal RP: 8 mmHg or less
Normal duration of LES relaxation
Little attention has been paid to duration of relaxation
of LES in the literature
Normal values: 11.7 + 0.6 sec (mean + SD)
Hyperclosure LES
LES pressure is often higher for few seconds after
swallow induced relaxation
Velocity of peristaltic wave
How fast contraction moves down
Distance (cm) / time (sec)
Normal value: 2 – 8 cm/sec
This example: 10 / 3 = 3.3 m/sec
Normal esophageal body amplitude
Normal values of DEA*
99 + 44 mmHg
(Mean + 1 SD)
* Distal esophageal amplitude: mean value of amplitude of
10 contractions to wet swallows in 2 most distal transducers
Duration of contraction
Normal duration values
3.9 0.9 sec
Mean + 1 SD
Retrograde contractions
Quite rare
Distal esophagus contracts before proximal esophagus
Raisons for a new classification
• Literature dealing with putative esophageal motility
disorders has evolved over past few decades
• Different groups of investigators have used different
manometric criteria to identify same putative disorder
• Comparison between studies are often difficult
Classification of esophageal motility disorders
• Inadequate LES relaxation
Classic achalasia
Atypical disorders of LES relaxation
• Uncoordinated contraction
Diffuse esophageal spasm
• Hypercontraction
Nutcracker esophagus
Isolated hypertensive LES
• Hypocontraction
Ineffective esophageal motility
Spechler S J & Castell D O. Gut 2001; 49 :145 – 151.
Classic achalasia
• Achalasia is a Greek term that means “does not relax”
• Esophageal disease of unknown cause with degeneration
of neurones in wall of esophagus involving preferentially
NO producing inhibitory neurones
• Of all the proposed esophageal motility disorders,it is
perhaps the best understood & best characterized
Barium of achalasia
Esophagus usually, but not always, dilated
Smooth tapering described as a “ bird-beak ” appearance
Achalasia
Manometric features required for diagnosis
• Incomplete relaxation of LES
Defined as mean swallow induced fall in resting LES
pressure to a nadir value > 8 mm above gastric pressure
• Aperistalsis in the body of esophagus
Simultaneous esophageal contractions < 40 mm Hg
Or no apparent esophageal contractions
Achalasia
Achalasia
Achalasia
Manometric features not required for diagnosis
• LES Elevated resting LES pressure (> 45 mm Hg)
• Esophageal body Resting pressure of esophageal body exceeds
resting pressure in stomach
• UES Elevated UES residual pressure
Decreased duration of UES relaxation
Repetitive UES contractions
Secondary achalasia
• Chagas disease
Protozoan Trypanosoma cruzi
Central & South America
• Malignancies
- Invading esophageal neural plexuses (carcinoma)
- Release of humoral factors (paraneoplastic syndrome)
Primary & secondary achalasia cannot be distinguished
reliably on basis of manometric criteria alone
Clinical suspicion of malignant achalasia
• Old age
• Recent history of dysphagia
• Weight loss
Vigorous achalasia
• Esophageal contractions with amplitudes > 40 mm Hg
• Chest pain may be more prominent or not?
• Injection of botulinum toxin more effective or not?
Atypical disorders of LES relaxation
1 or more manometric features precluding dg of classic
achalasia
• Some preserved peristalsis
• Esophageal contractions with amplitudes > 40 mmHg
• Complete LES relaxation of inadequate duration
Confirmation of dg ultimately requires relief of dysphagia
by treatment decreasing resting LES pressure
Diffuse esophageal spasm (DES)
Condition of unknown etiology characterized by:
Clinically Episodes of dysphagia & chest pain
RadiographicallyTertiary contractions of esophagus
Manometrically Uncoordinated activity in smooth
muscle portion of esophagus
Lack of universally accepted diagnostic criteria for the condition
Segmented or “corkscrew” esophagus
Barium of diffuse esophageal spasm
Manometric features of DES
Required
- Simultaneous contractions in >10% of wet swallows
- Mean simultaneous contraction amplitude >30 mm Hg
Not required
- Spontaneous contractions
- Repetitive contractions
- Multiple peaked contractions
- Intermittent normal peristalsis
If incomplete relaxation of LES is associated
Better classified as atypical disorder of LES relaxation
Diffuse esophageal spasm
Spontaneous repetitive contractions
Triple-peaked peristaltic contraction
“Abnormal “
Usually indicate DES
Each peak should be at least:
10 % of overall wave amplitude
1 sec in duration
Double-peacked contraction
A variant of normal
Hypercontraction
• Nutcracker esophagus
• Isolated hypertensive LES
Disorders of hypercontraction are perhaps the most
controversial of abnormal esophageal motility
patterns because it is not clear that esophageal
hypercontraction has any physiological importance
“Nutcracker oesophagus” is a term coined by
Castell & colleagues for the condition in
which patients with non-cardiac chest pain
&/or dysphagia exhibit peristaltic waves in
the distal oesophagus with mean amplitudes
exceeding normal values by > 2 SD
Richter JE et al. Ann Intern Med 1989 ; 110 : 66 – 78.
Manometric features of nutcracker esophagus
Required
Mean distal esophageal peristaltic wave amplitude >180 mm Hg
(average amplitude of 10 swallows at 2 recording sites positioned
3 & 8 cm above LES)
Not required:
Peristaltic contractions of long duration found commonly (> 6 sec)
Resting pressure in LES is usually normal but may be elevated
In this case: nutcracker esophagus + hypertensive LES
Nutcracker esophagus
• High amplitude peristaltic waves
Nay not interfere with esophageal clearance
May not cause abnormalities on barium contrast
May not correlate with episodes of dysphagia or chest pain
• No relief of pain during treatment with calcium channel
blockers that correct manometric abnormalities
Two types of nutcracker esophagus
• “Statistical nutcracker”
Pressure moderately elevated
More likely stress-related
• “ True nutcrackers”
Very high pressure (up to 500 mmHg)
Frequent prolonged or bizarre-appearing contractions
Some problem with neurologic input to esophagus
Statistical nutcracker esophagus
Amplitude of esophageal contraction: 220 mmHg
True nutcracker esophagus
Amplitude of esophageal contraction: 506.8 mmHg
Manometric features of isolated hypertensive LES
Mean resting LES pressure of > 45 mm Hg
measured in mid respiration using station pull through technique
If also distal peristaltic wave amplitude >180 mm Hg
nutcracker esophagus + hypertensive LES
Ineffective esophageal motility
Manometric features
- Distal esophageal peristaltic wave amplitude <30 mm Hg
- Simultaneous contractions with amplitudes <30 mm Hg
- Failed peristalsis wave: not traverse entire length of distal esoph
- Absent peristalsis
- Patients often have LES hypotension
Hypocontraction in distal esophagus with at least 30% of
wet swallows exhibiting any combination of the followings
Low amplitude (ineffective) contractions
Non-transmitted contraction
“Scleroderma-like” esophageal motility disorders
• Other collagen vascular disorders: MCTD, RA, SLE
• Diabetes mellitus
• Amyloidosis
• Alcoholism
• Myxoedema
• Multiple sclerosis
• Severe GERD
MCTD: Mixed Connective tissue disease
RA: Rhumatoid Arthritis
SLE: Systemic Lupus Erythematous
Use of term “scleroderma esophagus” is discouraged.
If used at all, this term should be restricted only to
patients who have scleroderma.
The term “ineffective esophageal motility” is preferable
to describe patients with constellation of findings typical
of scleroderma
Basal LES LES
relaxation
Wave
progression
Distal wave
amplitude
Achalasia  or nl
Rarely low
Incomplete Simultaneous
No peristaltis
 or nl
Atypical
relaxation of
LES
 or nl or  Incomplete
Short duration
Normal
Simultaneous
 or nl or 
Hypertensive
LES
 Complete Normal Normal
DES  or nl or  Complete Simultaneous
in > 10 %
nl or 
NE  or nl or  Complete Normal 
Ineffective
esophageal
motility
 or normal Complete Normal
Simultaneous
Absent
 > 30 %
Therapeutic implications of this classification
• Inadequate LES relaxation
- Calcium channel blockers
- Pneumatic dilation
- Heller myotomy
- Botulinum toxin injection
• Hypocontraction
- May need teatment for GERD
- May benefit from prokinetic agents
Thank You

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Classification of esophageal motility disorders

  • 1. Classification of esophageal motility disorders Samir Haffar M.D.
  • 2. Indications of esophageal motility study • Dysphagia Not explained by stenoses or inflammation of the esophagus • Chest pain Not explained by heart disease or other thoracic disorders
  • 3. Pressure relationship in UES, esophagus, LES & Stomach
  • 4. Placement of esophageal motility catheter within the esophagus Gastrointest Endoscopy Clin N Am 2005 ; 15 : 243 – 255.
  • 6. Normal esophageal manometric features • Basal LOS pressure 10 – 45 mm Hg (mid respiratory pressure measured by station pull through technique) • LES relax with swallow Complete (to a level < 8 mm Hg above gastric pressure) • Wave progression Peristalsis progressing from UES through LES at rate of 2 – 8 cm/s • Distal wave amplitude 30 – 180 mm Hg (average of 10 swallows at 2 recording sites positioned 3 & 8 cm above LES) Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
  • 7. Mid respiratory measurements of LES Most commonly used Normal values: 24.4  10.1 mmHg * Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
  • 8. End expiratory measurements of LES Normal values: 15.2  10.7 mmHg * Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
  • 9. LES pressure • The crural diaphragm • The LES muscle Reflects pressure generated by
  • 10. Normal LES Relaxation Residual Pressure (RP) Difference between lower pressure achieved & GBP RP better than percentage of relaxation Normal RP: 8 mmHg or less
  • 11. Normal duration of LES relaxation Little attention has been paid to duration of relaxation of LES in the literature Normal values: 11.7 + 0.6 sec (mean + SD)
  • 12. Hyperclosure LES LES pressure is often higher for few seconds after swallow induced relaxation
  • 13. Velocity of peristaltic wave How fast contraction moves down Distance (cm) / time (sec) Normal value: 2 – 8 cm/sec This example: 10 / 3 = 3.3 m/sec
  • 14. Normal esophageal body amplitude Normal values of DEA* 99 + 44 mmHg (Mean + 1 SD) * Distal esophageal amplitude: mean value of amplitude of 10 contractions to wet swallows in 2 most distal transducers
  • 15. Duration of contraction Normal duration values 3.9 0.9 sec Mean + 1 SD
  • 16. Retrograde contractions Quite rare Distal esophagus contracts before proximal esophagus
  • 17. Raisons for a new classification • Literature dealing with putative esophageal motility disorders has evolved over past few decades • Different groups of investigators have used different manometric criteria to identify same putative disorder • Comparison between studies are often difficult
  • 18. Classification of esophageal motility disorders • Inadequate LES relaxation Classic achalasia Atypical disorders of LES relaxation • Uncoordinated contraction Diffuse esophageal spasm • Hypercontraction Nutcracker esophagus Isolated hypertensive LES • Hypocontraction Ineffective esophageal motility Spechler S J & Castell D O. Gut 2001; 49 :145 – 151.
  • 19. Classic achalasia • Achalasia is a Greek term that means “does not relax” • Esophageal disease of unknown cause with degeneration of neurones in wall of esophagus involving preferentially NO producing inhibitory neurones • Of all the proposed esophageal motility disorders,it is perhaps the best understood & best characterized
  • 20. Barium of achalasia Esophagus usually, but not always, dilated Smooth tapering described as a “ bird-beak ” appearance
  • 21. Achalasia Manometric features required for diagnosis • Incomplete relaxation of LES Defined as mean swallow induced fall in resting LES pressure to a nadir value > 8 mm above gastric pressure • Aperistalsis in the body of esophagus Simultaneous esophageal contractions < 40 mm Hg Or no apparent esophageal contractions
  • 24. Achalasia Manometric features not required for diagnosis • LES Elevated resting LES pressure (> 45 mm Hg) • Esophageal body Resting pressure of esophageal body exceeds resting pressure in stomach • UES Elevated UES residual pressure Decreased duration of UES relaxation Repetitive UES contractions
  • 25. Secondary achalasia • Chagas disease Protozoan Trypanosoma cruzi Central & South America • Malignancies - Invading esophageal neural plexuses (carcinoma) - Release of humoral factors (paraneoplastic syndrome) Primary & secondary achalasia cannot be distinguished reliably on basis of manometric criteria alone
  • 26. Clinical suspicion of malignant achalasia • Old age • Recent history of dysphagia • Weight loss
  • 27. Vigorous achalasia • Esophageal contractions with amplitudes > 40 mm Hg • Chest pain may be more prominent or not? • Injection of botulinum toxin more effective or not?
  • 28. Atypical disorders of LES relaxation 1 or more manometric features precluding dg of classic achalasia • Some preserved peristalsis • Esophageal contractions with amplitudes > 40 mmHg • Complete LES relaxation of inadequate duration Confirmation of dg ultimately requires relief of dysphagia by treatment decreasing resting LES pressure
  • 29. Diffuse esophageal spasm (DES) Condition of unknown etiology characterized by: Clinically Episodes of dysphagia & chest pain RadiographicallyTertiary contractions of esophagus Manometrically Uncoordinated activity in smooth muscle portion of esophagus Lack of universally accepted diagnostic criteria for the condition
  • 30. Segmented or “corkscrew” esophagus Barium of diffuse esophageal spasm
  • 31. Manometric features of DES Required - Simultaneous contractions in >10% of wet swallows - Mean simultaneous contraction amplitude >30 mm Hg Not required - Spontaneous contractions - Repetitive contractions - Multiple peaked contractions - Intermittent normal peristalsis If incomplete relaxation of LES is associated Better classified as atypical disorder of LES relaxation
  • 34. Triple-peaked peristaltic contraction “Abnormal “ Usually indicate DES Each peak should be at least: 10 % of overall wave amplitude 1 sec in duration
  • 36. Hypercontraction • Nutcracker esophagus • Isolated hypertensive LES Disorders of hypercontraction are perhaps the most controversial of abnormal esophageal motility patterns because it is not clear that esophageal hypercontraction has any physiological importance
  • 37. “Nutcracker oesophagus” is a term coined by Castell & colleagues for the condition in which patients with non-cardiac chest pain &/or dysphagia exhibit peristaltic waves in the distal oesophagus with mean amplitudes exceeding normal values by > 2 SD Richter JE et al. Ann Intern Med 1989 ; 110 : 66 – 78.
  • 38. Manometric features of nutcracker esophagus Required Mean distal esophageal peristaltic wave amplitude >180 mm Hg (average amplitude of 10 swallows at 2 recording sites positioned 3 & 8 cm above LES) Not required: Peristaltic contractions of long duration found commonly (> 6 sec) Resting pressure in LES is usually normal but may be elevated In this case: nutcracker esophagus + hypertensive LES
  • 39. Nutcracker esophagus • High amplitude peristaltic waves Nay not interfere with esophageal clearance May not cause abnormalities on barium contrast May not correlate with episodes of dysphagia or chest pain • No relief of pain during treatment with calcium channel blockers that correct manometric abnormalities
  • 40. Two types of nutcracker esophagus • “Statistical nutcracker” Pressure moderately elevated More likely stress-related • “ True nutcrackers” Very high pressure (up to 500 mmHg) Frequent prolonged or bizarre-appearing contractions Some problem with neurologic input to esophagus
  • 41. Statistical nutcracker esophagus Amplitude of esophageal contraction: 220 mmHg
  • 42. True nutcracker esophagus Amplitude of esophageal contraction: 506.8 mmHg
  • 43. Manometric features of isolated hypertensive LES Mean resting LES pressure of > 45 mm Hg measured in mid respiration using station pull through technique If also distal peristaltic wave amplitude >180 mm Hg nutcracker esophagus + hypertensive LES
  • 44. Ineffective esophageal motility Manometric features - Distal esophageal peristaltic wave amplitude <30 mm Hg - Simultaneous contractions with amplitudes <30 mm Hg - Failed peristalsis wave: not traverse entire length of distal esoph - Absent peristalsis - Patients often have LES hypotension Hypocontraction in distal esophagus with at least 30% of wet swallows exhibiting any combination of the followings
  • 47. “Scleroderma-like” esophageal motility disorders • Other collagen vascular disorders: MCTD, RA, SLE • Diabetes mellitus • Amyloidosis • Alcoholism • Myxoedema • Multiple sclerosis • Severe GERD MCTD: Mixed Connective tissue disease RA: Rhumatoid Arthritis SLE: Systemic Lupus Erythematous
  • 48. Use of term “scleroderma esophagus” is discouraged. If used at all, this term should be restricted only to patients who have scleroderma. The term “ineffective esophageal motility” is preferable to describe patients with constellation of findings typical of scleroderma
  • 49. Basal LES LES relaxation Wave progression Distal wave amplitude Achalasia  or nl Rarely low Incomplete Simultaneous No peristaltis  or nl Atypical relaxation of LES  or nl or  Incomplete Short duration Normal Simultaneous  or nl or  Hypertensive LES  Complete Normal Normal DES  or nl or  Complete Simultaneous in > 10 % nl or  NE  or nl or  Complete Normal  Ineffective esophageal motility  or normal Complete Normal Simultaneous Absent  > 30 %
  • 50. Therapeutic implications of this classification • Inadequate LES relaxation - Calcium channel blockers - Pneumatic dilation - Heller myotomy - Botulinum toxin injection • Hypocontraction - May need teatment for GERD - May benefit from prokinetic agents