Classification of esophageal motility disorders
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Transcript of Classification of esophageal motility disorders
Classification of esophageal motilitydisorders
Samir Haffar M.D.
Indications of esophageal motility study
• Dysphagia Not explained by stenoses orinflammation of the esophagus
• Chest pain Not explained by heart disease orother thoracic disorders
Pressure relationship in UES, esophagus,
LES & Stomach
Placement of esophageal motility catheterwithin 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 Hgabove gastric pressure)
• Wave progression Peristalsis progressing from UESthrough LES at rate of 2 – 8 cm/s
• Distal wave amplitude 30 – 180 mm Hg (average of 10swallows at 2 recording sitespositioned 3 & 8 cm above LES)
Richter JE et al. Dig Dis Sci 1984 ; 29 : 134 – 40.
Mid respiratory measurements of LESMost 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 RelaxationResidual 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 contractionsQuite rare
Distal esophagus contracts before proximal esophagus
Raisons for a new classification
• Literature dealing with putative esophageal motilitydisorders has evolved over past few decades
• Different groups of investigators have used differentmanometric criteria to identify same putative disorder
• Comparison between studies are often difficult
Classification of esophageal motility disorders
• Inadequate LES relaxationClassic achalasiaAtypical disorders of LES relaxation
• Uncoordinated contractionDiffuse esophageal spasm
• HypercontractionNutcracker esophagusIsolated hypertensive LES
• HypocontractionIneffective 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 degenerationof neurones in wall of esophagus involving preferentiallyNO 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, dilatedSmooth 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 LESpressure to a nadir value > 8 mm above gastric pressure
• Aperistalsis in the body of esophagusSimultaneous esophageal contractions < 40 mm HgOr 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 pressureDecreased duration of UES relaxationRepetitive UES contractions
Secondary achalasia
• Chagas diseaseProtozoan Trypanosoma cruziCentral & South America
• Malignancies- Invading esophageal neural plexuses (carcinoma)- Release of humoral factors (paraneoplastic syndrome)
Primary & secondary achalasia cannot be distinguishedreliably 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 classicachalasia
• Some preserved peristalsis
• Esophageal contractions with amplitudes > 40 mmHg
• Complete LES relaxation of inadequate duration
Confirmation of dg ultimately requires relief of dysphagiaby treatment decreasing resting LES pressure
Diffuse esophageal spasm (DES)
Condition of unknown etiology characterized by:
Clinically Episodes of dysphagia & chest pain
Radiographically Tertiary contractions of esophagus
Manometrically Uncoordinated activity in smoothmuscle 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 associatedBetter 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 contractionA variant of normal
Hypercontraction
• Nutcracker esophagus
• Isolated hypertensive LES
Disorders of hypercontraction are perhaps the mostcontroversial of abnormal esophageal motilitypatterns 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 elevatedIn this case: nutcracker esophagus + hypertensive LES
Nutcracker esophagus
• High amplitude peristaltic wavesNay not interfere with esophageal clearance May not cause abnormalities on barium contrastMay 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 elevatedMore 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 Hgnutcracker 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% ofwet 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 diseaseRA: 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 preferableto describe patients with constellation of findings typical
of scleroderma
Basal LES LES relaxation
Wave progression
Distal wave amplitude
Achalasia Ý or nl Rarely low
Incomplete SimultaneousNo peristaltis
or nl
Atypical relaxation of LES
or nl or Ý IncompleteShort duration
NormalSimultaneous
or nl or Ý
Hypertensive LES
Ý Complete Normal Normal
DES or nl or Ý Complete Simultaneous in > 10 %
nl or Ý
NE or nl or Ý Complete Normal Ý
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