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Esophagus Master > Esophageal Motility Disorders > Flashcards

Esophageal Motility Disorders Flashcards

1
Q

MC Categories of Esophageal Motility Disorders

A
  • Achalasia
  • Diffuse esophageal spasm
  • Nutcracker esophagus
  • Hypertensive LES
  • Scleroderma
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2
Q

A common cause of esophageal dysmotility that must be ruled out before a diagnosis of altered esophageal motor function is made

A

GERD

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3
Q

Other less common diseases that may affect esophageal motility include

A
  • Connective tissue disorders
  • DM
  • Dermatomyositus
  • Amyloidosis
  • Chronic ideopathic intenstial pseudo-obstruction
  • Alcoholism
  • Chaga’s disease
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4
Q

Definition of achalasia

A

Primary esophageal motility disorder charcterized by:

Aperistalsis of distal esophagus

Failure of complete LES relaxation

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5
Q

Early achalasia findings may include:

A

Disordered peristalsis with normal LES function

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6
Q

Pathophysiology of Achalasia

A

Degeneration of ganglion cells in esophageal myoenteric plexus

  • Destruction of inhibitory neurons (contain NO and VIP) due to inflammatory process
    • Initiating event may be viral, and inflammatory process autoimmune in origin
    • Elevated antibody titers to herpes virus
    • Predominant T-lymphocyte inflammatory infiltrates
    • Autoantibodies to myoenteric plexus
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7
Q

Natural hisotry of achalasia

A
  • Loss of esophageal propulsion and lack of LES relaxation
    • Stasis of food bolus until intraesophageal pressure great enough allow transit into stomach
      • Esophageal dilation
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8
Q

MC presentation of achalasia

A

Dysphagia

  • Progressive
  • Liquids and solids
  • Exacerbated by emotional stress and fast eating
  • Regurgitation of undigested food and aspiration: MC in advanced phase of achalsia when esophagus is dilated
  • Chest pain and indigesion common: often misleading to diagnosis of GERD
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9
Q

2 classic barium swallow findings for achalsia

A

Bird’s beak narrowing of GEJ

Air-fluid level in distal esophagus

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10
Q

Role of endoscopy in diagnostic evaluation of suspected achalsia

A

Rule out pseudoachalsia due to esophageal, gastric, or pancreatic cancer involving distal esophagus or esophageal diaphragmatic hernia

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11
Q

Gold standard diagnostic test for achalasia

A

Esophagel mannometry

  • Aperistalsis
  • Failure of LES relaxation
    • LES hypertensive
  • Resting esophageal pressure elevated and non-peristaltic
  • Simultaneous contractions seen in reponse to swallow
    • Esophagus very dilated: low amplitude
    • Vigerous achalsia: high amplitude and duration simultanous contractions (rare)
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12
Q

Medical treatment options and success for achalasia

A
  • Options: reduce LES tone
    • anticholangerics
    • nitrates
    • Ca channel blockers
    • Beta blockers
    • PDE inhibitors
  • Success: low efficacy with high rates of adverse effects
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13
Q

Success of endoscopic balloon diation for achalasia

A

50-90% with multiple sessions in experienced hands

  • Risk of perforation: 2-5% per dilation
  • Less effective in young patients
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14
Q

RCT results (endoscopic dilation vs surgery) for achalsia

A

Near complete relief of symptoms at 5-year follow-up:

  • Endoscopic balloon dilation: 51%
  • Surgery: 95%
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15
Q

Success of intersphincteric botulinum toxin injection for achalasia

A

60-70% (short lived)

  • Requires repeated injections
  • Tx may lead to GERD
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16
Q

Standard of Care treatment for achalasia

A

Heller myotomy with fundoplication

  • Laparoscopic distal esophageal myotomy
    • myotomy 6 cm with extension of at leat 1.5 cm onto stomach
  • Partial fundoplication (Dor or Toupet)
    • RCT demonstrated higher postop GERD without fundoplication
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17
Q

Surgical treatment options for end-stage achalsia or failure of Heller myotomy with fundoplication

A

Esophagectomy with reconstruction (gastric pull-up or colonic interposition)

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18
Q

Most frequent reason for performing esophagectomy in achalasia

A

Iatrogenic perforation

  • Other indication: esophageal cancer or dysplasia
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19
Q

Achalasia a known risk factor for what

A

Esophageal cancer

  • Endoscopic surveillence offered even after surgical intervention and resolution of symptoms
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20
Q

Diagnosis

A

Achalasis

  • Aperistalsis in esophageal body
  • Failure of relaxation of LES
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21
Q

Definition of Diffuse Esophageal Spasm (DES)

A

Rare esophageal motility disorder characterized by:

  • non-peristaltic, simultaneous contractions of distal esophagus
    • occur either spontaneously or in response to swallow
  • intact LES function and relaxation
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22
Q

Pathophysiology of DES

A

Unknown

Hypotheses:

  • dysfunction of inhibitoary nerves (similar to achalasia)
  • early stage of myoenteric plexus denervation disease
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23
Q

% of cases of DES that will progress to achalsia

A

3-5%

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24
Q

MC presentation of DES

A
  • Chest pain
    • rest
    • after swallow
    • associated with hih-amplitude and long-duration esophageal contractions
  • Dysphagia
    • non-progressive (different from achalasia which is progressive)
    • liquids and solids
    • intermittent
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25
Q

UGI findings of DES

A
  • Vary in appearance:
    • normal
    • Altered progression of bolus due to uncoordinated, simultaneous contractions
      • “corkscrew appearance”
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26
Q

Associated esophageal condition that may occur with DES

A

Esophageal pulsion diverticulum

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27
Q

Manometric findings for DES

A
  • More than 20% simultanous contractions (> 30 mmHg) in response to wet swallow
  • Nl LES function
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28
Q

Non-surgical treatment options for DES

A
  • Medical:
    • Improve dysphagia:
      • Ca channel blockers
      • Nitrates
      • PDE inhibitors
    • Improve chest pain:
      • low-dose TCA
  • Interventional:
    • Botulinum toxin injection
    • Balloon dilation
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29
Q

Surgical treatment for DES

A

Long myotomy of esophagus + partial fundoplication (GERD reduction)

30
Q

Diagnosis

A

Diffuse Esophageal Spasm

  • Simultaneous contractions of esophageal body
  • Nl LES tone and relxation
31
Q

Monometric characteristics of nutcracker esophagus occur in __% of pateint with non-cardiac chest pain

A

27-48% (i.e. 25-50%)

32
Q

Patient population most commonly affected by Nutcracker Esophagus

A

Females

Psychiatric disorders

33
Q

Proposed pathophysiology of Nutcracker Esophagus

A
  • Imbalance between excitatory and inhibitory innervation
  • Increased esophageal muscle thickness
    • Increased muscle thickness may be responsible for high-amplitude peristalsis
34
Q

MC presenting symptoms of Nutcracker Esophagus

A
  • Chest pain (MC)
  • Dysphagia
  • Depression
  • Anxiety
35
Q

Manometric findings of Nutcracker Esophagus

A
  • High-amplitude (>180 mmHg) contractions in distal esophagus
  • Prolonged contractons (>6sec) common but not required for diagnosis
  • Peristaltic waves normally propogated, repeative (>2), and persistent
  • May have hypertensive LES (> 40 mmHg)
36
Q

Diagnosis

A

Normal Esophageal Manometry

37
Q

Diagnosis:

A

Nutcracker Esophagus

  • High-amplitude (>180 mmHg) esophageal contraction
  • Propogated
  • Prolonged (> 6 sec)
  • Repetative (>2 peaks)
  • Increased LES tone (>40 mmHg)
38
Q

Treatment options for Nutrcacker Esophagus

A

Similar to DES

  • Anticholinergic and smooth-muscle relaxing agents (Nitrates, Ca channel blockers, PDE inhibitors)
  • TCA (decreases chest pain)
  • Botulinum toxin, esophageal dilation, surgery (long myotomy) in select cases
39
Q

Definition of Hypertensive LES

A

Resting LES pressure > 45 mmHg (or > 95th percentile in nl subjects)

40
Q

Other manometric findings associated with Hypertensive LES

A
  • 50% of patients with peristaltic waves characteristic of nutcracker esophagus
  • Some patients have incompete relaxation of LES and elevated intrabolus pressures suggesting outflow obstruction
41
Q

MC symptoms of nutcracker esophagus

A

Dysphagia

Chest pain

Indigestion

42
Q

Other esophageal anatomic disorder commonly associated with hypertensive LES

A

Large paraesophageal hernia with abnormal esophageal exposure to gastric acid

  • 24-hr pH monitoring test used to identify these patinets
43
Q

Treatment options for hypertensive LES

A

Medial managment

Distal esophageal myotomy with partial fundoplication (Heller)

44
Q

Results in smooth muscle atrophy and subsequent replacement with scar

A

Scleroderma

45
Q

Manometric findings of scleroderma

A
  • Peristaltic dysfunction (normal peristalsis of upper 1/3 esophagus d/t striated muscle)
  • Weakening of LES
46
Q

Barium esophagram findings of scleroderma

A
  • Slightly dilated esophagus
  • Weak or absent peristalsis
  • Free reflux often is demonstrated
47
Q

Diagnosis

A

Achalasia

Bird’s beak (dilated esophagus)

48
Q

Diagnosis

A

Nutcracker Esophagus

Corkscrew

49
Q

Diagnosis

A

Normal esophagram

50
Q

Integrated Relaxation Pressure

A

High Resolution Manometry Measurement

  • A measure of the GEJ relaxation durring swallowing
  • Average of 4 non-continuous seconds of the lowest pressure during the deglutitive window ofthe swallow.
  • Incomplete GEJ Relaxation is identified by an IRP of 15mmHg or higher.
51
Q

How is incomplete relaxation of the LES identified on HRM

A

Integrated Relaxation pressure of

15mmHg or greater

52
Q

Distal Latency

A

High resolution manometry meaurement

Meaure of peristaltic timing

(wave from initiation of swallow to the distal)

54
Q

what is eosinophilic esophagitis ?

A

Eosinophilic esophagitis

chonric inflammatory disorder

Esophageal dysfunction

Intraepithelial infiltration of Eosinophils

Thought to be an allergic reaction

55
Q

Eosinophilic esophagitis - what are the classic presenting symptoms ?

A
  1. Dysphagia
  2. Food impaction
  3. Heart burn un responsive to PPI
56
Q

What does endoscopy of eosinophilic Esophagitis reveal?

A
  1. “Trachealization of the the esophagus”
  2. “Feline esophagus”
  3. Friable mucosae with edema
  4. Single or multiple concentric rings.
  5. White papules
57
Q

Biopsy results on Eosinophilic esophagitis ?

A

Threshold 15-20 Eos / HPF

Average is 40; although, may be more

58
Q

Treatment of Eosinophilic Esophagitis?

A
  1. Elimination diet
  2. Parenternal or systemic corticosteroids
59
Q

Treatment of Candidial Esophagitis

A

Fluconazole 100-200 mg daily

Superior to keotonazole in patients with HIV

60
Q

Scleroderma - what is it ?

A

Connective tissue disorder characterized by fibrosis of the skin, vasculature, and internal orrgans.

61
Q

Frequency of GI manifestations with scleroderma ?

A

80% have some GI involvement

10% present with GI sx.

Severe involvement affects 8%.

Only 15% of these patients survive 9 years ?

62
Q

Integrated relaxation pressure

A

A measure of GEJ relaxation while swallowing

Averages of 4 non-continues seconds durring the lowest pressure.

Incomplete relaxation - IRP > 15mmHg

63
Q

How to calculate incomplete relaxation off of HRM?

A

Integrated relaxation pressure.

> 15mmHg

64
Q

HRM diatal latency

A

measurement of perstaltic timing

Normal is defined as > 4.5 from the inidiation of swallow

LESS THAN 4.5 is a spastic swallow

65
Q

Type 1 Achalasia

structure characteristics

A

Type 1: Late Achalasia

Significant loss of dilation

Loss of Elasticity

66
Q

Type 1 Achalasia

Functional Characteristics

A

Type 1 Achalasia: Late Achalasia

  1. Aperistalsis
  2. Elevated IRP
  3. Incomplete LES relaxation with elevated pressure
67
Q

Characteristics of Type II achlasia

A

Panesophageal pressurization > 30mmHg in at least 20% of swallows

68
Q

Type III Achalsia

General description ?

Quantitative criteria?

A

Type III Achalasia: “Vigorous Achalsia”

Non-peristaltic

At least two spastic high-amplitude waves

Elevated IRP

69
Q

Treatments most effeective for type III achalasia ?

A

Botox

POEM

70
Q

With what type of achalsia is BoTox most effective

71
Q

Optimal treatment for Type II achalsia

A

Heller or pneumatic dilation

72
Q

Optimal treatment for Type I achalsia

A

Myotomy as initial treatement compared to dilation of Botox

Esophagus Master (11 decks)

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