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Esophageal Motility Disorders

Esophageal Motility Disorders

1. Embryology

A. Muscular epithelial-lined tube
B. Derived from primitive foregut
C. Second week of embryologic development
D. Mesoderm forms and separates ectoderm from endoderm--providesmaterial necessary for connective tissue, muscular coats, serous coverings

2. Histology and Final Development

A. Adventitia: outer loose connective tissue containing nerves, lymphatics, blood vessels
B. Muscularis: two layers of muscle--outer longitudinal and an inner circular
C. Submucosa: connects muscularis with the mucosa--strongest layer--elastic tissue; collagenous fibers; network of vessels & nerves
D. Mucosa: squamous, columnar; Z-line

3. Vascularity

A. Arterial: 3 sources
1) Inferior and superior thyroid arteries: cervical esophagus
2) Tracheobronchial, aortic arch and esophageal branches: body of esophagus
3) Left gastric and splenic arteries: GEJ
B. Veins: drainage pattern similar to lymphatics
C. Submucosal plexus--communicating veins--perforating veins that pierce the muscularis
D. Eventually drain into inferior thyroid, azygous, hemiazygous, left gastric, splenic, left gastroepiploic systems

4. Lymphatics:

A. Submucosal lymphatics form long channels that run parallel to esophageal axis
B. May travel long distances before draining into regional nodes

5. Innervation

A. Nerves: extraesophageal
B. Sympathetic: cervical, thoracic chains; celiac plexus and ganglia
C. Parasympathetic: vagus nerve muscular plexus around the circular layer of the muscularis (Auerbach's plexus)
D. Submucosal plexus (Meissner's plexus)
E. Auerbach's and Meissner's plexus are interconnected and are responsible for the fine- control mechanism of esophageal function

6. Nerves: central

A. Nucleus ambiguous
B. Dorsal motor nucleus of Vagus through the myenteric plexus
C. Both work together on the longitudinal and circular muscles to propel a bolus--peristalsis

7. Peristalsis

A. Primary: normal propulsive wave in response to the stimulation of normal voluntary deglutition
B. Secondary: normal wave without voluntary deglutition: best defense
C. Tertiary: abnormal; may occur spontaneously or following deglutition

8. Normal function

A. Upper Esophageal Sphincter (UES)
B. Level of cricoid cartilage C 5-6
C. Composed of cricopharyngeus and inferior pharyngeal constrictors
D. Remains contracted between swallows due to continuous stimulation by IX and XI
E. Resting tone: 45 - 65 mmHg (range 32 -101 mmHg)
F. Swallow: inhibition of all motor nerve stimulation; UES opens; closes; rebound; baseline pressures

9. Esophageal Body

A. Proximal striated muscle: direct innervation to its motor end plate from nucleus ambiguous
B. Smooth muscle: indirect neural input from dorsal motor nucleus (X) via myenteric plexus
C. Innervation: longitudinal muscle shortens; circular muscle contracts; peristalsis
D. Duration and amplitude: weaker in proximal esophagus; stronger, longer in distal esophagus

10. Lower esophageal sphincter (LES)

A. Specialized muscle arrangement 3 - 4 cm above gastroesophageal junction (GEJ)
B. High pressure zone with resting tone 15 - 25 mmHg (24.8 mmHg)
C. Influenced by neural and hormonal factors; drugs
D. Relaxes at time of swallowing; closes with passage of contraction through sphincter

11. Diagnostic Techniques

A. Radiology
B. Endoscopy
1) Reflux Disease
2) Esophageal Malignancies
3) Esophageal Biopsies
C. Motility Studies
D. 24 Hour pH Monitoring
1) Acid Perfusion Tests (Bernstein's Test)
E. Radionuclide Emptying Studies

12. Oropharyngeal Dysphagia

A. Neurologic: central vs peripheral
B. Myogenic
C. Cricopharyngeal Muscle Dysfunction
D. Iatrogenic
E. Lower esophageal disease

13. Idiopathic Motor Disorders

A. Hypomotility Disorders
1) Achalasia
B. Hypermotility Disorders
1) Diffuse Esophageal Spasm
C. Hyperperistalsis (Nutcracker; Supersqueeze)
D. Hypertensive LES
E. Nonspecific Esophageal Motility Disorders

14. Achalasia

A. Young adults -- 1 in 100,000 -- Cause unknown
B. Loss of control at the postganglionic, nonadrenergic and noncholinergic inhibitory nerves -- LES dysfunction and esophageal body changes
C. ? denervation; Chagas' Disease
D. Compounded by physical & psychologic stress
E. LES: normal or increased resting pressure
1) Incomplete or absent relaxation

15. Symptoms: dysphagia; odynophagia; regurgitation; aspiration and its complications; made worse by cold liquids and stressful situations

A. Squamous cell carcinoma 1-10%
B. Peristalsis absent in esophageal body; high resting pressure
C. Contractions weak at all recording levels
D. Esophageal dilatation; megaesophagus
E. Barium swallow: "bird's beak" esophagus

16. CXR: widened mediastinum; air-fluid level in posterior mediastinum; absence of gastric bubble

A. Endoscopy: changes vary with stage
1) Normal
2) Food/fluid retention in fasting state
3) Thickened mucosa with wide folds
4) Hyperemia: stasis esophagitis

17. Treatment

A. Medication: calcium channel blockers--decrease LES resting pressures--improves symptoms, not emptying
1) Early disease with minimal symptoms
B. Dilatation: stretches/ruptures fibers of the LES
1) Lowers LES resting pressure; improves emptying
2) Good to excellent short term results in 65% of patients
3) Esophageal rupture seen in 4%
C. Reflux seen in 7 - 17%

18. Esophageal myotomy: improves obstructive symptoms more effectively than dilatation

A. Can be done via left thoracotomy, laparotomy, or scope
B. 5 - 7 cm myotomy on distal esophagus
C. Extends 1 cm onto gastric wall
D. Mucosa dissected from muscularis
E. 90% relief of dysphagia short and long term
F. ? concomitant antireflux procedure: partial fundoplication

19. Diffuse Esophageal Spasm

A. Rare--cause is unknown; muscle hypertrophy; degenerative changes in Vagus branches
B. Symptoms: odynophagia, dysphagia, unexplained chest pain
1) Anxious individuals
2) Symptoms worsened by stress
3) Must differentiate from CAD

20. Simultaneous segmental contractions on x-ray

A. Corkscrew / rosary bead esophagus
B. Diverticulum: intermittent or epiphrenic
1) Endoscopy: usually normal
2) Peristalsis:>30% repetitive tertiary contractions
3) Duration and amplitude occasionally abnormal
4) LES: occas. hypertensive; occas. incomplete relaxation

21. Treatment

A. Nitroglycerin
B. Calcium channel blockers: decreases amplitude of contraction & reduces LES pressure
C. Control anxiety and precipitating factors
D. Esophageal myotomy: results not as good as in achalasia; good to excellent results in 67 - 70%
E. If LES is transected must do a partial fundoplication
F. Dilatation if hypertensive LES is documented

22. Nutcracker or Supersqueeze Esophagus

A. Normal peristalsis
B. Contraction amplitude is > 2 standard deviations above normal > 180 mmHg in distal esophagus
C. Duration of contractions >6 sec.
D. LES: occas. hypertensive; usually normal
E. Primary symptoms is chest pain
F. Strong emotional influence / hypochondriacal

23. Treatment

A. Psychologic assessment and support
B. Calcium channel blockers
C. Dilatation or myotomy are of little or no benefit
D. Hypertensive LES
E. Resting pressure > 45 mmHg; normal relaxation and peristalsis
F. Conservative medical / psychiatric management

24. Idiopathic Gastroesophageal Reflux

A. Peristalsis and contraction amplitude are normal
B. LES is weak (gradient < 6 mmHg) allowing gastric acid to reflux and bathe the lining of the lower esophagus--responsible for 60 - 70 % of all GERD
C. Sphincter failure
D. Primary muscle dysfunction

25. Increased exposure to acid further weakens the LES leading to further reflux and mucosal damage

A. Esophageal contraction abnormalities with poor amplitude and aperistalsis
B. Correlation between the severity of sphincter hypotension and extent of functional abnormalities in the esophageal body
1) (DeMeester)
2) ? correction cures abnormalities

26. Reflux Disease and Scleroderma

A. Seen in 90% of patients with scleroderma
B. Atrophy of smooth muscle components
C. Fibrous infiltration
D. Incompetence of LES with disappearance of propulsive and emptying mechanisms
E. Antireflux procedures not as successful though some improvement is seen
F. Reflux

27. Idiopathic Gastroesophageal Reflux

A. Frequent association with Type I hiatal hernia
1) Alterations in the anatomy of the hiatus
2) Phrenoesophageal membrane
B. Secondary causes
1) Delay of gastric emptying
2) Pyloric stenosis
3) Gastric mass
4) Poor esophageal wall muscle tone (scleroderma)

28. Reflux: two factors must occur

A. Acid-peptic or pancreaticobiliary secretions must reach the esophagus with increased frequency
B. Esophagus must be unable to clear those refluxed materials back into the stomach
C. Treatment
1) Medical treatment
2) Surgical treatment if medical treatment fails

29. Diagnosis

A. Barium swallow
B. Fiberoptic / rigid endoscopy
C. Multiple biopsies if findings consistent with severe esophagitis, stricture, Barrett's epithelium, ulceration
D. Esophageal function test (manometry, acid reflux, perfusion and clearing tests, 24 hr pH monitoring)

30. Indications for surgery

A. Symptomatic after 3 months of medical therapy
B. Persistent esophagitis, stricture, aspiration, bleeding
C. Positive 24 hr pH study
D. Manometry suggesting dysfunctional LES and adequate esophageal motility

(peak amplitude > 30 mmHg)

E. Barrett's mucosa if biopsies are benign (no CIS)

31. Operation of choice

A. Restoration of the anatomic and physiologic relationships
B. of the LES at the GEJ
C. 360 degree wrap (normal esophageal motility)
D. 270 degree wrap (dysfunctional esophagus)
F. Esophageal resection (rare)
G. Totally unyielding (fibrotic) esophagus
H. Barrett's esophagus with CIS or frank malignancy
I. Gastroplasty: falling out of favor
J. Procedures
K. Nissen Fundoplication
L. Belsey Mark IV
M. Hill Fundoplication
N. Collis Gastroplasty
O. Collis-Belsey Procedure

EXTENDED OUTLINE

natomy and functional evaluation 1. Anatomy

A. Begins (transition from pharynx to esophagus) at lower end of sixth cervical vertebra/cricoid cartilage
B. Ends (transition to stomach) at 11th thoracic vertebra
C. Esophagus is midline, passing to the left in lower neck and upper thorax, then back to midline, then to left again in lower thorax to pass through diaphragmatic hiatus
D. Follows curve of vertebral column except to pass anteriorly to pass through diaphragmatic hiatus
E. Sites of perforation during rigid esophagoscopy:
1) Cricopharyngeus
2) Terminal left anterior deviation
F. Measurements
1) Incisors to cardia = 38-40cm (men), 36-38 (women)
2) Cricopharyngeus to cardia =23-30cm, avg. 25
3) Incisors to cricopharyngeus = 14-15cm
4) Incisors to tracheal bifurcation/indentation of aortic arch = 24-26cm
G. Anatomic relations of esophagus
1) Trachea and cervical spine
2) Recurrent laryngeal nerves - in tracheoesophageal groove - left is closer to esophagus
3) Above tracheal bifurcation, esophagus passes to the right of the aorta
4) From arch down, esophagus lies to the right of the aorta
5) 8th vertebra - left wall of esophagus is covered only by mediastinal pleura - common site of perforation in Boerhaave’s syndrome
6) Passing through diaphragmatic hiatus, phrenoesophageal membrane surrounds
7) 2cm of abdominal esophagus between membrane and cardia - subjected to positive pressure
8) Thoracic duct - through diaphragm behind aorta - in thorax, dorsal to esophagus, from 5th thoracic vertebra up, it passes to left, then departs from esophagus in neck to join L SCV at junction of IJV
H. Musculature of the esophagus
1) Opening is collared by cricopharyngeus muscle
2) Outer longitudinal layer and inner circular layer
3) Circular muscle is elliptical
4) Upper esophagus is only striated muscle
5) At upper/middle 1/3 junction - 50% smooth muscle
I. Arterial supply of the esophagus
1) Cervical = inferior thyroid artery (mainly) + common carotid, SCA
2) Thoracic = bronchial arteries (75% have one R and 1-2 L)
3) Abdominal = branches of left gastric and inferior phrenic arteries
4) After penetrating esophagus, arteries branch in T to form longitudinal anastomoses
5) Esophagus can be mobilized from stomach to aortic arch w/o devascularization
J. Venous drainage
1) Cervical = inferior thyroid
2) Thoracic = bronchial, azygos and hemiazygous veins
3) Abdominal = cardiac vein
K. Innervation
1) Parasympathetic = vagus
2) Cricopharyngeus and cervical esophagus - recurrent laryngeal nerves
3) RLN injury causes vocal cord paralysis and dysfunction of cricopharyngeus and of cervical esophageal motility, predisposing to aspiration
4) Esophageal plexus receives fibers from vagus and from thoracic sympathetic chain
L. Lymphatic drainage
1) Submucosal plexus - lymph flow is longitudinal - extensive submucosal spread (of tumor) can occur

2. Normal structure and function

A. Pharyngeal phase of swallowing
1) Tongue is piston - propels food bolus as soft palate is closed
2) Swallowing is reflex, once initiated
3) Larynx is elevated and epiglottis covers opening of larynx
4) Pharyngeal pressure increases to 45mm Hg
5) Food propelled by pressure gradient into thoracic esophagus
6) Upper, striated portion of esophagus relaxes, then contracts within 0.5 seconds to twice its resting level of 30mm Hg
7) A peristaltic wave of 30mm Hg begins in the esophagus
8) Afferent nerves of pharynx are glossopharyngeal and superior laryngeal branch of vagus
9) Efferent nerves arise from CN V, VII, X, XI, XII and C1-3
10) Motor disorders of pharyngeal swallowing:
a) Incomplete upper sphincter relaxation
b) Loss of skeletal portion of cervical esophagus
B. Esophageal phase of swallowing
1) Pressure gradient of -6mm Hg in thoracic esophagus to +6mm Hg intraabdominal
2) Lower 1/3 of esophagus is most important
a) Peristaltic wave of 30-120 mm Hg
b) Rises to a peak in 1 sec, lasts 0.5 sec, then subsides in 1.5 sec
c) Wave moves down the esophagus at 2-4 cm/sec, reaches distal esophagus 9 sec after swallow starts
d) Vagal modulated wave
e) If vagi are preserved, muscle can be divided and propagate wave
f) Vagal fibers end in myenteric plexus
g) No known sympathetic innervation of the esophagus
C. Pathologic states
1) Diffuse esophageal spasm - simutaneous contraction
2) Achalasia - failure of LES relaxation
3) Scleroderma - loss of contraction of smooth muscle portion of esophagus

3. The antireflux mechanism

A. LES
1) No distinct anatomic sphincter, but muscular architecture of cardia acts like a sphincter
2) Gastric contraction results in increased LES pressure
B. Resting LES pressure
1) Correlates with incidence of GERD
2) Truncal vagotomy has no effect
3) Atropine (and other anti-cholinergics) reduces LES tone but does not cause GER
4) In pharmacologic doses:
a) Secretin, cholecystokinin, glucagon, prostaglandins reduce LES pressure
b) Gastrin, bombesin, motilin augment it
5) Low LES tone in GERD is probably due to abnormal myogenic function
6) Results of antireflux operations are independent of changes in resting LES pressure
7) Myotomy can be performed along the length of the LES without resulting in reflux
C. Phrenoesophageal ligament
D. Intra-abdominal esophagus
1) Laplace’s law - pressure required to distend a soft tube is inversely proportional to its diameter
2) Small-diameter esophagus requires high intragastric pressure to allow reflux
3) LES competence directly proportional to length of intra-abdominal esophagus in cadaver studies by DeMester

4. GERD

A. Results from decrease in LES pressure, shortening of the intra-abdominal esophagus or both
B. Competence of cardia
1) Requires adequate LES pressure + intrabdominal length
2) 80% prob of GERD when LES <5mmHG (independent of length)
3) 80% prob of GERD when length <1cm (independent of pressure)
4) Low incidence when pressure > 20mmHg and >2cm abd length
C. Gastric function
1) Delayed gastric emptying
2) intragastric pressure and distention shorten intraabdominal length
D. Overall LES length is also a factor in GERD
E. Esophageal clearance
1) Gravity, salivation and swallowing
2) Pts w/complications of GERD (Barrett’s and stricture) have higher proportion of weak amplitude and simultaneous contractions
3) frequency of swallows (0.87 2.59/min) during episodes of reflux
4) Any impairment of motility may exposure time
F. LES relaxation-abnormal will increased exposure
G. Hiatal hernia
H. Phrenoesophageal ligament and snug hiatus prevent distention of abdominal esophagus
I. Antirreflux operations restore to normal the failed components of a mechanically defective sphincter

5. Objective assessment of esophagus

A. Esophageal and upper GI barium studies
1) accuracy with video/cine
2) accuracy with solid and liquid boluses
3) Intraluminal abnormalities, landmarks
4) Some motor dysfunction - spastic contractions
5) Mucosal lesions better seen with double contrast
6) GERD- reflux only seen in 40% of those with manometry proven
B. Esophagoscopy
1) Any patient who reports dysphagia
2) Confirm structural abnormalities w/bx
3) Hiatal hernia = a pouch lined with gastric rugal folds lying 2cm above crural indentation (identify w/a sniff)
4) Esophagitis
a) Grade I= reddening w/o ulceration
b) Grade II= erosive and invasive, not circumferential
c) Grade III= confluence of erosions (cobblestone) - no stricturing
d) Grade IV= complications
5) Stricture
a) Multiple biopsies
b) Dilate
6) Barrett’s
a) Difficulty visualizing squamo columnar jxn
b) Mucosa is red, more luxuriant
c) Biopsy proximal to lesion to determine junction w/nl squamous mucosa
d) Surveillance = 4 circumferential biopsies a t 2cm intervals
7) Submucosal lesions - do not biopsy
C. The acid perfusion test
1) 0.1N HCl or H2O infused 15 cm above LES
2) Pt reports symptoms
3) Positive test is pt reporting symptoms w/acid relieved by saline
4) Reduced sensitivity in pts w/stricture or Barrett’s
D. Manometry
1) Indications
a) Motor abnormality of esophagus suspected
b) Dysphagia or odynophagia w/o definite structural abnormality on Ba swallow
c) Confirm dx of achalasia, esophageal spasm, scleroderma
d) GERD - assess esophageal clearance prior to surgery
e) Determine LES pressure, total and intra abdominal length
2) Pressure-measuring catheter is withdrawn rapidly or stepwise across cardia
3) Measurements
a) Relaxation of LES to gastric levels during swallow
b) Respiratory inversion point - reference point for LES
c) Response to 10 pharyngeal swallows-wet swallows are more sensitive
4) Achalasia (Fig 39-21)
a) LES does not fully relax
b) All waves in body are simultaneous
c) No primary peristaltic waves are seen
d) Resting pressure of body is usually elevated
5) Scleroderma (Fig 39-22)
a) All muscular function of distal esophagus is obliterated
b) No high pressure zone
c) No contractions in body (lower 2/3 of esophagus)
6) Simultaneous, repetitive or broad-based powerful contractions
a) Partial obstruction
b) Esophageal spasm
E. 24-hour esophageal motility monitoring
1) Advantages
a) Multiplies amount of data
b) Various physiologic conditions
2) Limitations of stationary monitoring
a) Pt is supine
b) Limited to 10 swallows
3) Technique
a) Drugs are stopped 48h before test
b) 3 transducers - 5, 10, 15 cm above upper border of LES
c) Pt diary of eating, position, sleeping, symptoms
4) Diagnostic criteria (Table 39-2)
5) Little correlation with stationary manometry - especially for normal or nutcracker by ambulatory
6) Primarily useful in pts with noncardiac chest pain
a) Amplitude and duration of contractions associated w/ pain are similar to asymptomatic
b) Frequency of contractions prior to episodes is increased
c) Esophageal claudication
d) Long esophageal myotomy can eliminate ability of esophagus to produce these bursts of abnormal activity
7) Other findings
a) Esophageal contractility deteriorates with mucosal injury
b) Assess esophageal clearance function = peristaltic contractions with amplitude > 30mm Hg
F. 3-D imaging of LES
1) Overall length or intra abdominal length below 5th percentile can nulify normal LES pressure
2) Increases the sensitivity of esophageal manometry in identifying pts who will benefit from “early” antireflux surgery (i.e., before the development of mucosal injury)
G. Esophageal pH tests
1) pH electrode withdrawl test
a) Normal is sharp rise in pH from stomach to 5-7 in esophagus
b) 20% false poitive - abandoned
2) SART
a) pH electrode 5cm above LES - 0.1N Hcl infused into stomach - pt performs maneuvers
b) > 2 drops in pH = abnormal cardia
3) Acid clearance test
a) Performed after SART
b) Acid infused into esophagus
c) Normal=pH > 5 with < 10 swallows
4) 24-hour pH monitoring
a) Most sensitive method for reflux-related problems
b) Indications
(1) GERD symptoms, other tests equivocal
(2) Prior to antireflux operation
(3) Atypical GERD symptoms
(4) Dysphagia and motor disorder (?GERD)
(5) Recurrent symptoms after esophageal or gastric surgery
c) Technique
(1) pH electrode 5cm above LES
(2) Acid reflux = pH <4
(3) Alkaline reflux = pH >7
(4) Restrict intake to food pH 5-6
d) Measure
(1) Cumulative time pH < 4 as percentage of time supine, total, upright
(2) Frequency of episodes of pH<4/24h
(3) Duration of longest episode
(4) Number of episodes > 5 min
H. Radionuclide studies
1) Localization of Barrett’s - not used
2) Dx and quantitation of GERD - not physiologic
3) Measure esophageal transit - ?screening test prior to manometry
4) Measurement of gastric emptying
I. Bilirubin monitoring with fiberoptic probe
1) Complications are related to acid and alkaline reflux
2) 5cm above LES
3) Uses bilirubin as a marker of exposure to duodenal contents