motility disorder of oesophagus
TRANSCRIPT
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Motility disorder of
esophagus
• Dr Kapileshwer Vijay
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ANATOMY & PHYSIOLOGY
Three physiologically distinct neuromuscular units
• Upper Esophageal Sphincter
• Esophageal Body
• Lower Esophageal Sphincter
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Motility Disorders
• Upper esophageal
– UES disorders
– neuromuscular disorders
• Esophageal body
–
achalasia – diffuse esophageal spasm
– nutcracker esophagus
– nonspecific esophagealdysmotility
• LES
– achalasia
– hypertensive LES
• Primary disorders
– achalasia
– diffuse esophageal spasm
– nutcracker esophagus
– nonspecific esophagealdysmotility
• Secondary disorders
– severe esophagitis
– scleroderma
– diabetes
– Parkinson’s
– stroke
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UES Disorders
• Cricopharyngeal hypertension
– elevated UES resting tone
– poorly understood (reflex due
to acid reflux or distension)
• Cricopharyngeal achalasia – incomplete UES relaxation
during swallow
– may be related to Zenker’s
diverticula in some patients
• Clinical manifestations
– localizes as upper (cervical)
dysphagia
– within seconds of swallowing
– coughing, choking, immediateregurgitation, or
nasal regurgitation
• Diagnosis: swallow evaluation &
modified barium swallow
• Treatment - Myotomy
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ACHALASIA CARDIA
HISTRORICAL ASPECTS
• Greek word- failure to relax
• First described in 17 th century by Thomas
Willis : used whale bone for dilation.
• 1880 – Von Miculicz : cardiospasm as the
mechanism.
• 1957 – Code : manometric abnormalities in
Achalasia.
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ACHALASIA CARDIA - PATHOGENESIS
• ETIOLOGY – unknown
• FAMILIAL
• AUTO-IMMUNITY: – Inflammatory infiltrate in myenteric
plexus , predominantly of T-Lymphocyte.
–High prevalence of class II HLAAntigens – DQ –W1, DQ-A1*0101
– Antibodies to myenteric plexus
neurons.
• Infective – Trypanosoma cruzi
–After an attack of Varicella-Zoster
– Association with Guiilain-Barresyndrome
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Pathophysiology
• T‐lymphocyte, eosinophil, and mast cellinfiltration in the myenteric (Auerbach) plexus
Myenteric neural fibrosis• Hypertrophy of the two muscle layers and nerve
fibers
• Degeneration of NO and producing inhibitoryneurons
• Affects relaxation of LES Basal LES pressure rises
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OVERVIEW OF ETIOPATHOGENESIS
Initial Insult
??viral
2° degeneration of vagalefferent neurones and their
cellbodies in DMN
Inflammatory Plexopathy primarilyinvolving NO Nerves
Vigorous AchalasiaDestruction of NO
Neurones
Classic Achalasia2° mucosal and submucosal
inflammation andesophageal dilatation
2° smooth musle
hypertrophy and
degeneration
?DES
?Nutcracker
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- EPIDEMIOLOGY
• Incidence – 6:100 000
• Sex Ratio – Equal
• Age group – 5th to 6th decade
•Hereditary – AR
• Associated disease
– Chagas` DiseaseCardiomyopathy
– Cerebellar ataxia with MEN II
– TRIPLE “A” : Alacrimia,
Achalasia, Adrenal failure.
VENTRAPPEN - CLINICALCLASSIFICATION
1. None
2. Short-lasting episodes of
dysphagia and retrosternalpain < once a week
3. Dysphagia > once a week,
but no regurgitation or
weightloss
4. Frequent dysphagia
accompanied by weightloss
or regurgitation
ACHALASIA CARDIA
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Typical
• Dyspahgia – To both solids andliquids, may be intermittent
- very slowworsening,indolentcourse
• Regurgitation – of bland,undigested food, hours aftermeal
Atypical
• Chest Pain(50%)
• Heartburn – Production of
lactic acid• Weight loss – may be
profound
• Recurrent aspirationpneumonia
ACHALASIA CARDIA – CLINICAL FEATURES
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• PRIMARY EVALUATION
– Barium swallow withfluroscopy
– Esophageal Manometry
– Upper GI Endoscopy
Further evaluation forsecondary achalasia
–
Abdominal and thoracicCT
– Endoscopic Ultrasound
– Endoscopic brushings of GE Junction for cytology
ACHALASIA CARDIA - DIAGNOSIS
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ACHALASIA CARDIA - BARIUM
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ACHALASIA CARDIA
• FLUOROSCOPIC ABNORMALITIES
– Earliest : Breakdown of normal peristalsis into simultaneouscontractions
– Failure of primary peristaltic wave to clear barium
• VIDEOESOPHAGOGRAPHY
– Absence of normal peristalsis in esophageal body
– Incomplete or absent opening of LES / dilatation of esophagus
–100% correlation with manometry.
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Manometery
• Gold standard
• Characteristic featuresregardless of the stage
– Aperistalsis is the
prerequisite.
– Sphincter pressure can benormal in up to 20-30%.
– Sphincter Relaxation maybe complete but of shortduration (< 6 sec) andfunctionally inadequate.
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• To exclude other entities.• Normal mucosa, mild resistance
to passage of scope.
• Failure of relaxation of LES
with air insufflation
• Residual food / fluid
• Dilatation & tortuosity
• Careful retroflexed view TO EXCLUDE CARDIA GROWTH (2-4%)
– Marked resistance to passage of scope.(better appreciated with bigger scope)
ACHALASIA CARDIA - ENDOSCOPY
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• EUS : High frequency 20 Hz
– Thickness of folds
– Submucosal tumor infiltration
– Regional lymphadenopathy
• CECT : - Asymmetric wall thickening
- Extrinsic mass / lymphadenopathy
ACHALASIA CARDIA – OTHER TESTS
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Secondary / Pseudo-achalasia
• Benign : Amyloidosis
Peptic strictures
Post vagotomy effect
• Malignancy :
• Carcinoma stomach, esophagus, pancreas, HCC,Lung, Kidney, breast, prostate.
• Lymphoma
• Peritoneal Mesothelioma
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Achalasia - Treatment
• AIM : Reducing the pressure gradient – relievingsymptoms and preventing further dilatation
• Only palliative
– Pharmacological
– Botulinum Toxin
– Pneumatic Balloon dilatation
– Myotomy
• Open
• Laparoscopic• Thorocoscopic
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Pharmacological therapy• Nitrates : Isosorbide dinitrate
– c GMP
–
5-10mg S/L before each meal. – Onset – 15``, effect lasts for 90 ``.
– Relief in 75-80% But side effects in 30%
• Calcium Channel Blockers : Nifedipine, Diltiazem
– Reduce pressure by 40%, lasts > I hr.
– Effective in 70%
– RCT fail to show any benefit.
• COMPARATIVE TRIAL :
– Iso-sorbide > Nifedipine > Other CCB
85% 50%
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Botulinum Toxin
• Inhibits the Ca++ dep. release of Ach. from nerve terminals.
• Effective in 85% but relapse in 50% in 6 months, due to regeneration of
nerve endings.
• Each vial 100 units
• 80-100 units, in a dilution of 20 units/mL (Lethal dose – 2000u)
• 1 cm above the ‘Z’ line in four quadrants.
• Candidates – older (>50 yrs)
- Vigorous acalasia
- High surgical risk
• Remarkable safety : mild chest discomfort
Reflux < 5%
• Gender/ age/ previous dilations DO NOT predict response rate
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Botulinum Toxin First 6 months
First injection
Immediate Failure 10% Response 90%
30%Early Relapse
Further injections
10%
FAILED 30% IMPROVED 70%
Mean Remission : 1.4 yr(5 month. to 2.5 yrs)
20%
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Pneumatic dilation
• The most effective non-surgical treatment
• Balloon across GE Jn. to tear the circular muscle fibres.• Rigiflex Balloon dilator(Microvasive)
– Double lumen catheter with low compliance polyetylenecylindrical balloon (through the scope)
–
3.0, 3.5, 4.0 cms• Witzel Dilator
– Polyurethane balloon mounted on a forward – viewendoscope. (Balloon inflation under direct vision withoutfluoroscopy)
– 3 psi 2-3 min.
• Efficacy 50-93 %
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COMPLICATIONS OF PNEUMATIC DILATION
– Perforation - 3.3%
– Aspiration - 0.8% – Death - 0.2%
– Haemorrhage - < 0.1%
–
Reflux - 2% (0-9)• Requirement for each subsequent dilation decreases
effectiveness by 50%
• Surgery after 3 unsuccessful dilations
• Initial good response predicts response to subsequent
dilations
• Peristalsis may return in 20%
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MYOTOMY
• Anterior myotomy down to the mucosa till the proximalextent of the LES and 1-2 cm on to the stomach.( 5mm)
• Combination with an anti-reflux procedure is debatable -
loose Nissen fundoplication
- Incomplete Toupet
- Dor Fundoplication
• 80-100% effective, Maintained at 10 years – 65%
•
14% eventually require esophagectomy.• Uncontrolled GERD in 10%(Higher with pH studies)
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Surgery Or Balloon ?
• Efficacy studies
– RCT 100 pts 15 yr follow up
• Favours surgery ( Scendes, Surgery, 1998)
• Cost effectiveness analysis :
– 45 pts follow-up 7 years
• Favours dilation cumulative cost 2.5 times less.
( Parkman, Dig Dis Sci 1993)
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ALGORITHM
PT. With Achalasia
Botulinum toxin 80-100units
pneumatic dilatation
Lap. Myotomy
High Surgical Risk Low surgical risk
FailureFailure Failure Success
Success
SuccessSuccess
Repeat Botulinum toxin
FailureFailure
Graded pneumatic dilatation
Nifedipine / NitratesEsophagectomy
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Addition of Fundoplication
• Richards et al: Randomizedtrial
• Heller myotomy w/ Dor vsw/o Dor ( n = 43)
• Post‐op GERD (by 24‐hr pH
monitoring)• 47.5% in pts w/ Heller
myotomy alone
• 9.1% in pts w/ added Dorfundoplication
•
No difference in LES pressureor dysphagia scores
• Rice et al: Retrospective study
• Heller with and w/o Dor (n =149)
• Decreased incidence of GERD(by 24‐hr pH monitoring)
following fundoplication• Fundoplication did not
decrease esophageal emptyingtime
(assessed by barium
esophagography).
1. Richards WO, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective
randomized double-bline clinical trial. Ann Surg 2004; 240(3):405-415.
2. Rice TW, et al. A physiologic clinical study of achalasia: Should Dor fundoplication be added to Heller myotomy?. J
Thorac Cardiovasc Surg 2005; 130(6):1593-1600
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DIFFUSE ESOPHAGEAL SPASM
• Osgood 1889 – severe chest pain and dysphagia.• Fleshler 1967- DES syndrome
• Familial Clusters
• ETIOPATHOGENESIS :
1. Defect in neural inhibition2. Decreased availability of nitric oxide.
3. Hyper sensiticity to cholinergic agents and pentagastrin stimulation
precipitating chest pain and dysphagia
4. Gastroesophageal reflux (20-50%)
5.Stressful life events.
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CLINICAL FEATURES :
• Recurrent chest pain : - Variable in intensity, frequency and
location – Indistinguishable from cardiac chest pain.
– Most reliable
» Radiation through back
» Variability in amount of exercise precipitatingthe pain
» Associated dysphagia
• Dysphagia : intermittent, non – progressive
–To both liquids and solids, never severe to cause weight loss
– Ppt. By stress, rapid eating, liquids of extreme tempetature.
• Associated fetures of IBS
DIFFUSE ESOPHAGEAL SPASM
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BARIUM STUDY
• Most commonly, Normal
• Disruption of peristalsis
• Tertiaty activity producing segmentation.• Results vary from day to day
• Spastic activity does not correlate with the symptoms.
DIFFUSE ESOPHAGEAL SPASM
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Diffuse Esophageal Spasm
• Numerous nonpropulsive
contractions
• “corkscrew/ rosary bead”
esophagus
• DES requires normal peristalsisinterspersed with 30% +
periods of nonpropulsive
motor activity
• Segmental spasm
• Pseudodiverticulosis
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DIFFUSE ESOPHAGEAL SPASM
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MANOMETRY
• Simultaneous contractions > 20% of wet swallows.
• Pressure exceeding 30 mm Hg.
• Long duration contractions
• Intermittent Normal peristalsis
• Repetitive waves (> 2 peaks)
• Spontaneous contractions
• High amplitude
•Ambulatory manometery
DIFFUSE ESOPHAGEAL SPASM
DIFFUSE ESOPHAGEAL SPASM
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TREATMENT:
• Reassurance
• Treat underlying GERD
– Smooth muscle relaxants
– Nitroglycerin 0.4 mg Sub ling.
– Isosorbide dinitrate10-30 mg four times daily
– Dicycloverine 10-20 mg four times daily
• Calcium Channel Blockers : can decrease high
amplitude contractions but inconsistent – Nifedipine 10-30 mg four times daily
– Diltiazem 60 mg four times daily
DIFFUSE ESOPHAGEAL SPASM
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TREATMENT:Psychotropic Drugs : Reduce the discomfort without any effect
on the motility
– Trazodone 100-150 mg once daily
– Imipramine 50 mg once daily
• Botulinum Toxin patients with
• Pneumatic dilation abn. LES relaxation
•Oesophageal myotomy. Delayed emptying.
DIFFUSE ESOPHAGEAL SPASM
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Nut cracker esophagus
• Dirst described -1979
• Dominant complaint – chestpain
•Most common motilitydisorder in pt. with noncardiac chest pain.(27-48%)
• Dysphagia relativelyuncommon.
• Association with GERD andstress.
• Radiology- Normal
•
Endoscopy- – Low threshfold for pain
– Reproduced by ballondistention
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MANOMETRIC CRITERIA
• NUTCRACKER ESOPHAGUS
– Normal peristalsis
– Increased amplitude of
contractions > 180 mmHg
–Increased duration of contraction > 6 sec.
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treatment
• Medical
– CCB
– Phosphodiesterase
inhibitor
• Surgical
– Long myotomy
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Hypertensive LES
• First described by Code 1960
• Sec to Achlasia
• Clinical
– Dysphagia
– Chest pain
– GERD
– Somatization, nervous
• Radiology
– Ba
• Narrowing of LES
•
Manometery – Normal peristalsis
– LES Pressure > 45 mmHg
– Normal relaxation
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• Treatment
– Medical
• CCB/Nitrate/Botox
•
Surgical – LES Myotomy with
partial fundoplication
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• Contraction wave of < 30 mm Hg in amplitude,
• Do not effectively transport and clear the bolus
• Non Specific Esophageal Motility Disorders.
–INEFFECTIVE ESOPHAGEAL MOTILITY
– HYPOTENSIVE LES
• Associated GERD and ENT complaints
• Could be secondary to chronic acid damage to distalesophagus.
HYPO-CONTRACTING ESOPHAGUS
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HYPOCONTRACTING ESOPHAGUS
Clinical feature
• Mild dysphagia
• Heartburn and acid
regurgitation dominant
symptoms.
• Severe dysphagia – peptic
stricture / esophagitis.
TREATMENT
• Proton Pump Inhibitors.
• Prokinetics.
• Results unreliable
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Thanks