Esophageal Motility Disorders

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Esophageal Motility Disorders

• Vic V. Vernenkar, D.O.
• St. Barnabas Hospital
• Dept. of Surgery

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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

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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.

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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.

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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.

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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.

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Achalasia
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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.

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Achalasia

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

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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).

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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.

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Diffuse Esophageal Spasm
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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.

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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.

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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.

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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.

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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.

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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.

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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.

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Causes

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

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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.

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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.

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Pseudoachalasia

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

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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.

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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.

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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.

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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).

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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.

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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.

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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.

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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.

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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.

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Pneumatic Dilation
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Pneumatic Dilation
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Pneumatic Dilation
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Pneumatic Dilation
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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.

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Heller Myotomy
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Fundoplication After Myotomy
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Surgical Care

• Heller myotomy is performed thru either
  transthoracic or transabdominal approaches.
• Efficacy is 60-100 in different series.

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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.