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Classification of esophageal motility disorders
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Classification of esophageal motility disorders

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    Classification of esophageal motility disorders Classification of esophageal motility disorders Presentation Transcript

    • 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