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

Thanks