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Motility disorder of
Esophagus
Dr. Jeetendra Bhandari
Introduction
ā€¢ Disruption of coordinated, sequential motility pattern limiting motion of
delivery of food and fluid
ā€¢ Common symptoms dysphagia and chest pain
ā€¢ Not very common
ā€¢ May be part of a more diffuse gastrointestinal motility problem
ā€¢ May be associated with GERD
Achalasia
Introduction
ā€¢ Greek ā€“ ā€˜failure to relaxā€™
ā€¢ Primary esophageal motility disorder characterized by
ā€¢ A hypertonic LES which fails to relax in response to swallowing wave
ā€¢ Failure of propagated esophageal contraction, leading to progressive dilatation of
gullet
ā€¢ Incidence ā€“1 per 100,000 people
ā€¢ Trypanosoma cruzi (Chagas disease) causes similar syndrome
Pathogenesis
ā€¢ It is due to loss of ganglionic cells in Myentric (Auerbachā€™s) plexus,
cause of which is unknown
ā€¢ May be due to neurotropic viruses ( varicella zoster)
ā€¢ Different from Hirschsprungā€™s disease of colon as dilated colon
contains normal ganglionic cells
ā€¢ Pseudoachalasia produced by adenocarcinoma of cardia
Hypertensive non-relaxed esophageal
sphincter
Imbalance in excitatory and inhibitory
neurotransmission
Lack of non-adrenergic, non-cholinergic
inhibitory ganglion cells
Pathophysiology
Clinical features
ā€¢ History
ā€¢ Weight loss (predominant)
ā€¢ Dysphagia (both to solid and liquid foods)
ā€¢ Regurgitation (especially at night)
ā€¢ Chest pain
ā€¢ Heart burn
ā€¢ Predisposes to SCC of esophagus
Investigations
ā€¢ Manometry
ā€¢ Gold standard for diagnosis (raised resting pressure)
ā€¢ Barium Studies
ā€¢ Endoscopy
ā€¢ Rule out other diseases (esophagitis or cancer) and GERD
Manometry
ā€¢ In typical achalasia, the manometry tracings show five classic
findings
ā€¢ Two abnormalities of the LES and three of the esophageal body
1. The LES will be hypertensive with pressures usually above 35 mm Hg,
2. LES will fail to relax with deglutition
3. The body of the esophagus will have a pressure above baseline
4. No evidence of progressive peristalsis
5. Low-amplitude waveforms indicating a lack of muscular tone
Barium Swallow
ā€¢ Esophagus appears dilated and
contrast material passes slowly
into stomach as LES opens
intermittently
ā€¢ Distal esophagus narrowed
resembling ā€œBirdā€™s Beakā€
Management
ā€¢ Respond well to treatment among motility disorders
ā€¢ Medical
ā€¢ To relieve symptoms
ā€¢ Calcium channel blockers and nitrates used to decrease LES pressure
ā€¢ Botulinum toxin
ā€¢ Acts by interfering with cholinergic excitatory neuronal activities at LES
ā€¢ Not permanent and has to be injected every few months endoscopically
ā€¢ For elderly patient if surgery contraindicated
Pneumatic dilatation
ā€¢ Involves stretching the cardia with a balloon to disrupt the muscle
and render it less competent
ā€¢ Perforation is the major complication
ā€¢ The risk of perforation increases with bigger balloons
ā€¢ should be used cautiously for progressive dilatation over a period of weeks
ā€¢ Forceful dilatation is curative in 75ā€“85% of cases
ā€¢ The results are best in patients aged more than 45 years
Hellerā€™s myotomy
ā€¢ This involves cutting the muscle of the lower esophagus and
cardia
ā€¢ The major complication is gastro-esophageal reflux
ā€¢ most surgeons therefore add a partial anterior fundoplication (Hellerā€“Dorā€™s
operation)
ā€¢ It is successful in more than 90% of cases and may be used
after failed dilatation
Zenkerā€™s diverticulum (pharyngeal pouch)
ļ±Posterior protrusion of esophageal wall above cricopharyngeal
sphincter through the natural weak point (the dehiscence of
Killian) between the oblique and horizontal (cricopharyngeus)
fibres of the inferior pharyngeal constrictor
ļ±exact mechanism to its formation is unknown, but it involves loss
of the coordination between pharyngeal contraction and opening
of the upper sphincter
Zenkerā€™s Diverticulum
ā€¢ As the pouch enlarges, it tends to fill with food on eating, and
the fundus descends into the mediastinum
ā€¢ This leads to halitosis and esophageal dysphagia
ā€¢ Coughing out of same food several hours after ingestion
a small pharyngeal pouch with a
prominent cricopharyngeal
impression and ā€˜streamingā€™ of
barium, indicating partial obstruction
a large pouch extending behind the
oesophagus towards the thoracic inlet
Treatment
ā€¢ Endoscopically with a linear cutting stapler to divide septum
between the diverticulum and the upper esophagus, producing a
diverticulo-esophagostomy,
or
ā€¢ Open surgery involving pouch excision, pouch suspension
(diverticulopexy) and/or myotomy of the cricopharyngeus
ā€¢ All techniques have good results
Other diverticula
ā€¢ Mid-esophageal diverticula
ā€¢ Usually small pulsion diverticula of no particular consequence
ā€¢ The underlying motility disorder does not usually require treatment
ā€¢ Some pulsion diverticula may fistulate into the trachea
ā€¢ Epiphrenic diverticula
ā€¢ pulsion diverticula situated in the lower esophagus above the diaphragm
Epiphrenic diverticulum proximal to the gastro-esophageal sphincter.
(a) Small and asymptomatic
(b) large, symptomatic and appearing as a gas-filled bubble on the chest
radiograph
Barium swallow showing mid- and distal esophageal diverticula
Disorders of body of esophagus
Diffuse Esophageal Spasm
ā€¢ Incoordinate contractions of the esophagus, causing dysphagia and/or chest
pain
ā€¢ The condition may be dramatic, with spastic pressures on manometry of
400ā€“500 mmHg
ā€¢ Marked hypertrophy of the circular muscle and a corkscrew esophagus on
barium swallow
ā€¢ These abnormal contractions are more common in the distal two-thirds of
the oesophageal body
Contdā€¦
ā€¢ Making diagnosis when chest pain is the only symptom may be
difficult
ā€¢ Prolonged ambulatory esophageal manometry that correlates
episodes of chest pain with manometric abnormalities may
establish the diagnosis
ļ‚Ø sensitivity of 90% and a specificity of 100%
Corkscrew esophagus in diffuse esophageal spasm
Treatment
ā€¢ No proven pharmacological or endoscopic treatment
ā€¢ Calcium channel antagonists, vasodilators and endoscopic dilatation have
only transient effects
ā€¢ Severity and frequency of symptoms may be tolerated by most patients
Treatment contdā€¦
ā€¢ Combination of chest pain and dysphagia is sufficiently severe sometimes
that malnutrition begins
ā€¢ In these patients, extended esophageal myotomy up to the aortic arch may
be required
ā€¢ Surgical treatment of diffuse spasm is more successful in improving
dysphagia than chest pain
ā€¢ Caution should be exercised in patients in whom chest pain is the only
symptom
Nutcracker Esophagus
ā€¢ Described as an esophagus with hypertensive peristalsis or high-
amplitude peristaltic contractions in which peristaltic pressures of more
than 180 mmHg develop
ā€¢ A condition in which extremely forceful peristaltic activity leads to
episodic chest pain and dysphagia
ā€¢ Most painful of all esophageal motility disorders
References
ļ±Bailey and Love Short Practice of Surgery 26th Edition
ļ±Davidsonā€™s Principal and Practice of Medicine, 21st edition
Mortility disorder of oesophagus

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Mortility disorder of oesophagus

  • 2. Introduction ā€¢ Disruption of coordinated, sequential motility pattern limiting motion of delivery of food and fluid ā€¢ Common symptoms dysphagia and chest pain ā€¢ Not very common ā€¢ May be part of a more diffuse gastrointestinal motility problem ā€¢ May be associated with GERD
  • 4. Introduction ā€¢ Greek ā€“ ā€˜failure to relaxā€™ ā€¢ Primary esophageal motility disorder characterized by ā€¢ A hypertonic LES which fails to relax in response to swallowing wave ā€¢ Failure of propagated esophageal contraction, leading to progressive dilatation of gullet ā€¢ Incidence ā€“1 per 100,000 people ā€¢ Trypanosoma cruzi (Chagas disease) causes similar syndrome
  • 5. Pathogenesis ā€¢ It is due to loss of ganglionic cells in Myentric (Auerbachā€™s) plexus, cause of which is unknown ā€¢ May be due to neurotropic viruses ( varicella zoster) ā€¢ Different from Hirschsprungā€™s disease of colon as dilated colon contains normal ganglionic cells ā€¢ Pseudoachalasia produced by adenocarcinoma of cardia
  • 6. Hypertensive non-relaxed esophageal sphincter Imbalance in excitatory and inhibitory neurotransmission Lack of non-adrenergic, non-cholinergic inhibitory ganglion cells Pathophysiology
  • 7. Clinical features ā€¢ History ā€¢ Weight loss (predominant) ā€¢ Dysphagia (both to solid and liquid foods) ā€¢ Regurgitation (especially at night) ā€¢ Chest pain ā€¢ Heart burn ā€¢ Predisposes to SCC of esophagus
  • 8. Investigations ā€¢ Manometry ā€¢ Gold standard for diagnosis (raised resting pressure) ā€¢ Barium Studies ā€¢ Endoscopy ā€¢ Rule out other diseases (esophagitis or cancer) and GERD
  • 9. Manometry ā€¢ In typical achalasia, the manometry tracings show five classic findings ā€¢ Two abnormalities of the LES and three of the esophageal body 1. The LES will be hypertensive with pressures usually above 35 mm Hg, 2. LES will fail to relax with deglutition 3. The body of the esophagus will have a pressure above baseline 4. No evidence of progressive peristalsis 5. Low-amplitude waveforms indicating a lack of muscular tone
  • 10. Barium Swallow ā€¢ Esophagus appears dilated and contrast material passes slowly into stomach as LES opens intermittently ā€¢ Distal esophagus narrowed resembling ā€œBirdā€™s Beakā€
  • 11. Management ā€¢ Respond well to treatment among motility disorders ā€¢ Medical ā€¢ To relieve symptoms ā€¢ Calcium channel blockers and nitrates used to decrease LES pressure ā€¢ Botulinum toxin ā€¢ Acts by interfering with cholinergic excitatory neuronal activities at LES ā€¢ Not permanent and has to be injected every few months endoscopically ā€¢ For elderly patient if surgery contraindicated
  • 12. Pneumatic dilatation ā€¢ Involves stretching the cardia with a balloon to disrupt the muscle and render it less competent ā€¢ Perforation is the major complication ā€¢ The risk of perforation increases with bigger balloons ā€¢ should be used cautiously for progressive dilatation over a period of weeks ā€¢ Forceful dilatation is curative in 75ā€“85% of cases ā€¢ The results are best in patients aged more than 45 years
  • 13. Hellerā€™s myotomy ā€¢ This involves cutting the muscle of the lower esophagus and cardia ā€¢ The major complication is gastro-esophageal reflux ā€¢ most surgeons therefore add a partial anterior fundoplication (Hellerā€“Dorā€™s operation) ā€¢ It is successful in more than 90% of cases and may be used after failed dilatation
  • 14. Zenkerā€™s diverticulum (pharyngeal pouch) ļ±Posterior protrusion of esophageal wall above cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor ļ±exact mechanism to its formation is unknown, but it involves loss of the coordination between pharyngeal contraction and opening of the upper sphincter
  • 16. ā€¢ As the pouch enlarges, it tends to fill with food on eating, and the fundus descends into the mediastinum ā€¢ This leads to halitosis and esophageal dysphagia ā€¢ Coughing out of same food several hours after ingestion
  • 17. a small pharyngeal pouch with a prominent cricopharyngeal impression and ā€˜streamingā€™ of barium, indicating partial obstruction a large pouch extending behind the oesophagus towards the thoracic inlet
  • 18. Treatment ā€¢ Endoscopically with a linear cutting stapler to divide septum between the diverticulum and the upper esophagus, producing a diverticulo-esophagostomy, or ā€¢ Open surgery involving pouch excision, pouch suspension (diverticulopexy) and/or myotomy of the cricopharyngeus ā€¢ All techniques have good results
  • 19. Other diverticula ā€¢ Mid-esophageal diverticula ā€¢ Usually small pulsion diverticula of no particular consequence ā€¢ The underlying motility disorder does not usually require treatment ā€¢ Some pulsion diverticula may fistulate into the trachea ā€¢ Epiphrenic diverticula ā€¢ pulsion diverticula situated in the lower esophagus above the diaphragm
  • 20. Epiphrenic diverticulum proximal to the gastro-esophageal sphincter. (a) Small and asymptomatic (b) large, symptomatic and appearing as a gas-filled bubble on the chest radiograph
  • 21. Barium swallow showing mid- and distal esophageal diverticula
  • 22. Disorders of body of esophagus
  • 23. Diffuse Esophageal Spasm ā€¢ Incoordinate contractions of the esophagus, causing dysphagia and/or chest pain ā€¢ The condition may be dramatic, with spastic pressures on manometry of 400ā€“500 mmHg ā€¢ Marked hypertrophy of the circular muscle and a corkscrew esophagus on barium swallow ā€¢ These abnormal contractions are more common in the distal two-thirds of the oesophageal body
  • 24. Contdā€¦ ā€¢ Making diagnosis when chest pain is the only symptom may be difficult ā€¢ Prolonged ambulatory esophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis ļ‚Ø sensitivity of 90% and a specificity of 100%
  • 25. Corkscrew esophagus in diffuse esophageal spasm
  • 26. Treatment ā€¢ No proven pharmacological or endoscopic treatment ā€¢ Calcium channel antagonists, vasodilators and endoscopic dilatation have only transient effects ā€¢ Severity and frequency of symptoms may be tolerated by most patients
  • 27. Treatment contdā€¦ ā€¢ Combination of chest pain and dysphagia is sufficiently severe sometimes that malnutrition begins ā€¢ In these patients, extended esophageal myotomy up to the aortic arch may be required ā€¢ Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain ā€¢ Caution should be exercised in patients in whom chest pain is the only symptom
  • 28. Nutcracker Esophagus ā€¢ Described as an esophagus with hypertensive peristalsis or high- amplitude peristaltic contractions in which peristaltic pressures of more than 180 mmHg develop ā€¢ A condition in which extremely forceful peristaltic activity leads to episodic chest pain and dysphagia ā€¢ Most painful of all esophageal motility disorders
  • 29. References ļ±Bailey and Love Short Practice of Surgery 26th Edition ļ±Davidsonā€™s Principal and Practice of Medicine, 21st edition

Editor's Notes

  1. Balloon of 30-35 mm diameter