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Esophageal Motility Disorders

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Title: Esophageal Motility Disorders


1
Esophageal Motility Disorders
  • Vic V. Vernenkar, D.O.
  • St. Barnabas Hospital
  • Dept. of Surgery

2
Anatomy
  • Active muscular organ with a complex
    neuromuscular structure and integration.
  • The sequential muscular contractions push food
    from above and clear acid and bile reflux from
    below.
  • Specialized sphincter at each end.
  • UES and LES

3
Anatomy
  • UES contracts during inspiration preventing air
    from entering into the GI tract, while the LES
    maintains a steady baseline tone to prevent
    gastric juice from refluxing into the esophagus.
  • LES also contracts during periods of increased
    intraabdominal pressure, preventing reflux due to
    pressure in the abdomen.
  • Inner circular layer, outer longitudinal layer of
    muscle. (ring occlusions and shortens)
    peristalsis.

4
Peristalsis
  • Sequential, coordinated contraction wave that
    travels along the whole length of the esophagus,
    propelling intraluminal contents downstream.
  • Primary wave strips from proximal to distal,
    triggered by swallowing center, 2cm/sec.
  • Secondary wave induced by distension of bolus,
    acts to clear esophagus of retained food.
  • Tertiary contractions are dysfunctional and have
    no role.

5
Motility Disorders
  • Achalasia
  • Primary spastic motility disorders, including
    DES, nutcracker esophagus, hypertensive LES
  • Secondary esophageal motility disorders related
    to DM, scleroderma, alcohol, psychiatric
    disorders, etc.

6
Achalasia
  • Loss of ganglion cells from the wall of the
    esophagus, starting at the LES and going
    proximally.
  • Loss of inhibitory nerves at LES.
  • Circular muscle layer thickened at LES but
    microscopically cells appear normal.

7
Achalasia
8
Achalasia
  • Loss of the inhibitory nerves at the LES causes
    failure of the LES to completely relax, and a
    hypertensive LES pressure over 40mmHg in 60 of
    patients.
  • Loss of nerves along the body of esophagus causes
    aperistalsis, stasis, dilatation.

9
Achalasia
  • Non-peristaltic isolated contractions or
    low-amplitude simultaneous contractions occur.
  • If high-amplitude (60mmHg) simult contractions
    occur it is called Vigorous Achalasia.

10
Achalasia
  • Edrophonium (acet cholesterase inhib) increases
    LES pressure.
  • Atropine reduces the LES pressure in achalasia,
    which is why botulinum toxin can be therapeutic
    (ach release inhibitor).

11
Spastic Motility Disorders
  • Diffuse fragmentation of vagal filaments,
    mitochondrial fragmentation results in functional
    imbalance between excitatory and inhibitory
    pathways.
  • When DES occurs, diffuse muscular hypertrophy as
    much as 2cm has been described in the distal 2/3
    of the esophagus, but wall thickening is also
    found in asymp patients, absent in patients with
    typical sympt and manometric findings too.

12
Diffuse Esophageal Spasm
13
Scleroderma Esophagus
  • Primary defect here is related to smooth muscle
    atrophy and fibrosis.
  • The dysmotility here an absence of peristalsis in
    the esoph body and an atonic LES occur.
  • Motility is preserved at the striated muscle part
    of the esophagus.

14
Frequency
  • Achalasia and DES only a small percentage of
    disorders of motility.
  • Achalasia 1 case per 100,000 per year.
  • Familial clustering occurs but not genetic yet.
  • Nutcracker esoph is most common motility
    disorder, but the most controversial in
    significance.

15
Mortality and Morbidity
  • Achalasia associated with significant progressive
    discomfort, severe dysphagia, malnutrition,
    weight loss, dehydration. Increased incidence of
    SCC with long standing disease.
  • Spastic motility disorders are associated with
    sympt discomfort but not the severity of
    dysphagia as in achalasia.
  • Scleroderma associated with severe acid reflux,
    associated complications, including strictures,
    Barretts, adenocarcinoma.

16
Race, Sex, and Age
  • Racial differences not established.
  • Affects both sexes equally.
  • Achalasia presents in patients 25-60 yrs,
    although it can affect any age group.

17
History
  • Achalasia progressive dysphagia for both solids
    an liquids is a hallmark. Regurge of food in
    dilated esoph common especially at night. Chest
    pain, sensation of heartburn (fermentation).
    Emotional stress or rapid eating makes it worse.
  • Spastic disorders Chest pain hallmark, mimics
    angina, may be related to transient esoph
    ischemia, distension. Dysphagia to solids and
    liquids a common symptom, especially with DES,
    intermittent, non-progressive. Heartburn, regurge.

18
History
  • Scleroderma involves esoph in 75 of patients.
  • Two forms- PSS a progressive form that is more
    fulminant, early involvement with internal
    organs CREST- calcinosis, Raynauds phenomena,
    esoph dysfunction, sclerodactyly, telangiectasia.
  • Severe acid reflux, regurge, dysphagia, erosive
    esophagitis (60), increased incidence of cancer
  • Dysphagia from peptic strictures, poor
    peristalsis.

19
Physical
  • Results of a physical exam are usually
    unrevealing.
  • Pay attention to signs of scleroderma in proper
    clinical setting.
  • A bedside swallowing challenge can be performed
    with a glass of water.
  • Check nutrition and hydration if dysphagia
    reported.

20
Causes
  • Primary disorders are idiopathic in nature.
  • Viral
  • Infectious
  • Environmental
  • Genetic

21
Other Problems
  • Differential diagnosis depends on presenting
    symptoms.
  • CAD, mechanical obstructing lesions, benign or
    malignant should be ruled out.
  • Differential of achalasia includes Chagas disease
    secondary to Trypanosoma cruzi infection and
    pseudoachalasia from GE junction tumors.

22
Chagas Disease
  • Mimics achalasia.
  • Reduviid (kissing) bug bite.
  • Endemic in SA, CA.
  • Septicemia first, then chronic stage ensues.
  • Widespread ganglion destruction throughout the
    body involving heart, gut, GI tract, urinary
    tract, respiratory tract.
  • Symptoms take years to develop.
  • Treatment disrupt LES like in achalasia.

23
Pseudoachalasia
  • Term used to describe clinical picture of GE
    junction obstruction.
  • Present in 5 of patients with clinical and
    manometric diagnosis of achalasia.

24
Pseudoachalasia
  • Clinical presentation is more likely to occur
    with rapidly progressive disease, older age of
    onset, profound weight loss.
  • Workup includes upper endoscopy, biopsies should
    be obtained with any suspicion of malignant
    process. If suspicious lesion found, image with
    CT, MRI, EUS if indicated.
  • In 50 of patients the diagnosis is
    adenocarcinoma of GE junction.

25
Diffuse Esophageal Spasm
  • DES and achalasia can be confused. Manometric
    criteria require that normal esophageal
    peristalsis be present intermittently for DES.
  • LES relaxation, which is incomplete in achalasia,
    should be normal in DES.

26
Workup
  • CXR Dilated esophagus, looks sigmoid like, air
    fliud level, wide mediastinum, absence of gastric
    air bubble.
  • Esophagram In achalasia, dilated, A-F level,
    tapered LES, birds beak appearance. Diverticula
    above LES, hiatal hernia. In DES corkscrew or
    rosary bead esophagus. In scleroderma, slightly
    dilated esophagus, absent peristalsis, free
    reflux.

27
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28
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29
Manometry
  • Achalasia aperistalsis of esophageal body is
    manometric hallmark.
  • DES Normal peristalsis, simultaneous
    contractions in 30 water swallows, incomplete
    LES relaxation, increased LES pressure (40mmHg),
    or repetitive, prolonged(6sec), high-amplitude
    contractions (180mmHg).

30
Manometry
  • Nutcracker Normal patterned peristalsis with
    high amplitude contractions(180mmHg), repetitive
    contractions, increased LES pressure(40mmHg).
  • Hypertensive LES Increased LES pressure
    (40mmHg). Significance of this is questionable.

31
Endoscopy
  • Exclude mechanical and inflammatory lesions that
    are causing dysmotility, structural cause of
    obstruction.
  • Endoscopic US still investigational in managing
    achalasia, used to assist in botulinum toxin
    injection.

32
Pharmacological Treatment
  • Smooth muscle relaxants including Ca channel
    blockers, nitrates. Also used, anticholinergics,
    amyl nitrite, NTG, theophylline,
    ,beta-2-agonists. Experience with these are
    limited in comparison to the first two.

33
Pharmacological Treatment
  • Spastic Disorders antireflux therapy, TCA,
    trazodone.
  • Botulinum toxin injection into the LES used to
    treat pts with achalasia. A potent inhibitor of
    ach release from nerve terminals. May be a good
    alternative for poor surgical candidates,
    disadvantage is high cost and need for repeated
    injections.

34
Endoscopic Therapy
  • EGD with pneumatic dilatation is the standard for
    achalasia. Forceful distension of the LES to 3cm
    with disruption of the circular muscle layer is
    needed for symptomatic relief.
  • Complication is perforation (8)
  • If pressure after is less than 10mmHg, outcome
    excellent.

35
Pneumatic Dilation
36
Pneumatic Dilation
37
Pneumatic Dilation
38
Pneumatic Dilation
39
Surgical Care
  • Surgical treatments target the LES to relieve the
    high pressure.
  • Heller myotomy is procedure of choice for
    achalasia. It decreases the LES pressure across
    GE junction and eliminates dysphagia.
  • Myotomy may lead to GE reflux, so a
    fundoplication may be necessary with the myotomy.

40
Heller Myotomy
41
Fundoplication After Myotomy
42
Surgical Care
  • Heller myotomy is performed thru either
    transthoracic or transabdominal approaches.
  • Efficacy is 60-100 in different series.

43
Surgical Care
  • Esophagectomy with gastric pull up or intestinal
    interposition for patients with advanced disease
    or refractory cases, unresolved symptoms,
    carcinoma, perforation during dilation.
  • Extended Heller Myotomy last resort for DES when
    pain or dysphagia is severe.
  • Operation is a myotomy that starts at the LES and
    goes to thoracic inlet.
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