Motility disorder of esophagus
Dr Kapileshwer Vijay
ANATOMY & PHYSIOLOGY
Three physiologically distinct neuromuscular units
Upper Esophageal Sphincter Esophageal Body Lower Esophageal Sphincter
Motility Disorders
Upper esophageal UES disorders neuromuscular disorders Esophageal body achalasia diffuse esophageal spasm nutcracker esophagus nonspecific esophageal dysmotility LES achalasia hypertensive LES
Primary disorders achalasia diffuse esophageal spasm nutcracker esophagus nonspecific esophageal dysmotility Secondary disorders severe esophagitis scleroderma diabetes Parkinsons stroke
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 Zenkers diverticula in some patients Clinical manifestations localizes as upper (cervical) dysphagia within seconds of swallowing coughing, choking, immediate regurgitation, or nasal regurgitation Diagnosis: swallow evaluation & modified barium swallow Treatment - Myotomy
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.
ACHALASIA CARDIA - PATHOGENESIS
ETIOLOGY unknown FAMILIAL AUTO-IMMUNITY:
Inflammatory infiltrate in myenteric plexus , predominantly of TLymphocyte. High prevalence of class II HLA Antigens DQ W1, DQ-A1*0101 Antibodies to myenteric plexus neurons.
Infective
Trypanosoma cruzi After an attack of VaricellaZoster Association with Guiilain-Barre syndrome
Pathophysiology
Tlymphocyte, eosinophil, and mast cell infiltration in the myenteric (Auerbach) plexus Myenteric neural fibrosis Hypertrophy of the two muscle layers and nerve fibers Degeneration of NO and producing inhibitory neurons Affects relaxation of LES Basal LES pressure rises
OVERVIEW OF ETIOPATHOGENESIS
2 degeneration of vagal efferent neurones and their cellbodies in DMN
Initial Insult ??viral Inflammatory Plexopathy primarily involving NO Nerves
?DES ?Nutcracker
Vigorous Achalasia
Destruction of NO Neurones
2 mucosal and submucosal 2 smooth musle inflammation and hypertrophy and esophageal dilatation degeneration
Classic Achalasia
ACHALASIA CARDIA
- EPIDEMIOLOGY
Incidence 6:100 000 Sex Ratio Equal Age group 5th to 6th decade Hereditary AR Associated disease Chagas` Disease Cardiomyopathy Cerebellar ataxia with MEN II TRIPLE A : Alacrimia, Achalasia, Adrenal failure.
VENTRAPPEN - CLINICAL CLASSIFICATION 1. 2. None Short-lasting episodes of dysphagia and retrosternal pain < once a week Dysphagia > once a week, but no regurgitation or weightloss Frequent dysphagia accompanied by weightloss or regurgitation
3.
4.
ACHALASIA CARDIA CLINICAL FEATURES
Typical
Dyspahgia To both solids and liquids, may be intermittent
- very slow worsening, indolent course
Regurgitation of bland, undigested food, hours after meal
Atypical Chest Pain(50%) Heartburn Production of lactic acid Weight loss may be profound Recurrent aspiration pneumonia
ACHALASIA CARDIA - DIAGNOSIS
PRIMARY EVALUATION
Barium swallow with fluroscopy Esophageal Manometry Upper GI Endoscopy
Further evaluation for secondary achalasia
Abdominal and thoracic CT Endoscopic Ultrasound Endoscopic brushings of GE Junction for cytology
ACHALASIA CARDIA - BARIUM
ACHALASIA CARDIA
FLUOROSCOPIC ABNORMALITIES
Earliest : Breakdown of normal peristalsis into simultaneous contractions 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.
Manometery
Gold standard Characteristic features regardless of the stage
Aperistalsis is the prerequisite. Sphincter pressure can be normal in up to 20-30%. Sphincter Relaxation may be complete but of short duration (< 6 sec) and functionally inadequate.
ACHALASIA CARDIA - ENDOSCOPY
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 (24%)
Marked resistance to passage of scope. (better appreciated with bigger scope)
ACHALASIA CARDIA OTHER TESTS
EUS : High frequency 20 Hz
Thickness of folds Submucosal tumor infiltration Regional lymphadenopathy
CECT : - Asymmetric wall thickening
- Extrinsic mass / lymphadenopathy
Secondary / Pseudo-achalasia
Benign : Amyloidosis
Peptic strictures Post vagotomy effect
Malignancy :
Carcinoma stomach, esophagus, pancreas, HCC, Lung, Kidney, breast, prostate. Lymphoma Peritoneal Mesothelioma
Achalasia - Treatment
AIM : Reducing the pressure gradient relieving symptoms and preventing further dilatation Only palliative
Pharmacological
Botulinum Toxin Pneumatic Balloon dilatation Myotomy
Open Laparoscopic Thorocoscopic
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%
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
Botulinum Toxin First 6 months
First injection
Immediate Failure 10%
Response 90%
30%
Early Relapse
20%
Further injections
10% FAILED 30% IMPROVED 70% Mean Remission : 1.4 yr(5 month. to 2.5 yrs)
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 polyetylene cylindrical balloon (through the scope) 3.0, 3.5, 4.0 cms
Witzel Dilator
Polyurethane balloon mounted on a forward view endoscope. (Balloon inflation under direct vision without fluoroscopy)
3 psi 2-3 min.
Efficacy 50-93 %
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%
MYOTOMY
Anterior myotomy down to the mucosa till the proximal extent 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)
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)
ALGORITHM
PT. With Achalasia
Low surgical risk
Lap. Myotomy
High Surgical Risk
Botulinum toxin 80-100units Graded pneumatic dilatation Success Failure Success
Failure Success Failure pneumatic dilatation Failure Esophagectomy
Repeat Botulinum toxin Failure Success
Nifedipine / Nitrates
Addition of Fundoplication
Richards et al: Randomized trial Heller myotomy w/ Dor vs w/o Dor ( n = 43) Postop GERD (by 24hr pH monitoring) 47.5% in pts w/ Heller myotomy alone 9.1% in pts w/ added Dor fundoplication No difference in LES pressure or dysphagia scores Rice et al: Retrospective study Heller with and w/o Dor (n = 149) Decreased incidence of GERD (by 24hr pH monitoring) following fundoplication Fundoplication did not decrease esophageal emptying time (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
DIFFUSE ESOPHAGEAL SPASM
Osgood 1889 severe chest pain and dysphagia. Fleshler 1967- DES syndrome Familial Clusters ETIOPATHOGENESIS : 1. Defect in neural inhibition 2. 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.
DIFFUSE ESOPHAGEAL SPASM
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 precipitating the 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
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
Numerous nonpropulsive contractions corkscrew/ rosary bead esophagus DES requires normal peristalsis interspersed with 30% + periods of nonpropulsive motor activity
Segmental spasm Pseudodiverticulosis
DIFFUSE ESOPHAGEAL SPASM
DIFFUSE ESOPHAGEAL SPASM
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
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
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 Pneumatic dilation Oesophageal myotomy.
patients with abn. LES relaxation Delayed emptying.
Nut cracker esophagus
Dirst described -1979 Dominant complaint chest pain Most common motility disorder in pt. with non cardiac chest pain.(27-48%) Dysphagia relatively uncommon. Association with GERD and stress.
Radiology- Normal Endoscopy Low threshfold for pain Reproduced by ballon distention
MANOMETRIC CRITERIA
NUTCRACKER ESOPHAGUS
Normal peristalsis Increased amplitude of contractions > 180 mmHg Increased duration of contraction > 6 sec.
treatment
Medical
CCB Phosphodiesterase inhibitor
Surgical
Long myotomy
Hypertensive LES
First described by Code 1960 Sec to Achlasia Clinical
Dysphagia
Radiology
Ba
Narrowing of LES
Manometery
Normal peristalsis LES Pressure > 45 mmHg Normal relaxation
Chest pain GERD Somatization, nervous
Treatment
Medical
CCB/Nitrate/Botox
Surgical
LES Myotomy with partial fundoplication
HYPO-CONTRACTING ESOPHAGUS
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 distal esophagus.
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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