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 āfailure 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 ā1 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 (Auerbachās) plexus,
cause of which is unknown
ā¢ May be due to neurotropic viruses ( varicella zoster)
ā¢ Different from Hirschsprungās disease of colon as dilated colon
contains normal ganglionic cells
ā¢ Pseudoachalasia produced by adenocarcinoma of cardia
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 āBirdās 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 75ā85% of cases
ā¢ The results are best in patients aged more than 45 years
13. Hellerās 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 (HellerāDorās
operation)
ā¢ It is successful in more than 90% of cases and may be used
after failed dilatation
14. Zenkerās 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 āstreamingā 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
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
400ā500 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
ļØ sensitivity of 90% and a specificity of 100%
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±Davidsonās Principal and Practice of Medicine, 21st edition