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