This document discusses the classification of esophageal motility disorders based on manometric features. It describes the normal esophageal motility test and features. It then covers various motility disorders including achalasia, atypical LES relaxation disorders, diffuse esophageal spasm, hypercontraction disorders like nutcracker esophagus and isolated hypertensive LES, and ineffective esophageal motility. For each disorder, it provides the manometric criteria for diagnosis and differentiates them based on features of LES relaxation, wave progression, distal wave amplitude. It concludes by discussing the therapeutic implications of this classification system.

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.

5. Normal esophageal motility test

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

22. Achalasia

23. Achalasia

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

32. Diffuse esophageal spasm

33. Spontaneous repetitive contractions

34. Triple-peaked peristaltic contraction
“Abnormal “
Usually indicate DES
Each peak should be at least:
10 % of overall wave amplitude
1 sec in duration

35. Double-peacked contraction
A variant of normal

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

45. Low amplitude (ineffective) contractions

46. Non-transmitted contraction

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

51. Thank You