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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
UGI findings of DES
* Vary in appearance: * normal * Altered progression of bolus due to uncoordinated, simultaneous contractions * "**_corkscrew appearance_**"
26
Associated esophageal condition that may occur with DES
Esophageal pulsion diverticulum
27
Manometric findings for DES
* More than 20% simultanous contractions (\> 30 mmHg) in response to wet swallow * Nl LES function
28
Non-surgical treatment options for DES
* Medical: * Improve dysphagia: * Ca channel blockers * Nitrates * PDE inhibitors * Improve chest pain: * low-dose TCA * Interventional: * Botulinum toxin injection * Balloon dilation
29
Surgical treatment for DES
Long myotomy of esophagus + partial fundoplication (GERD reduction)
30
Diagnosis
Diffuse Esophageal Spasm * Simultaneous contractions of esophageal body * Nl LES tone and relxation
31
Monometric characteristics of nutcracker esophagus occur in \_\_% of pateint with non-cardiac chest pain
27-48% (i.e. 25-50%)
32
Patient population most commonly affected by Nutcracker Esophagus
Females Psychiatric disorders
33
Proposed pathophysiology of Nutcracker Esophagus
* Imbalance between excitatory and inhibitory innervation * Increased esophageal muscle thickness * Increased muscle thickness may be responsible for high-amplitude peristalsis
34
MC presenting symptoms of Nutcracker Esophagus
* Chest pain (MC) * Dysphagia * Depression * Anxiety
35
Manometric findings of Nutcracker Esophagus
* 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
Diagnosis
Normal Esophageal Manometry
37
Diagnosis:
Nutcracker Esophagus * High-amplitude (\>180 mmHg) esophageal contraction * Propogated * Prolonged (\> 6 sec) * Repetative (\>2 peaks) * Increased LES tone (\>40 mmHg)
38
Treatment options for Nutrcacker Esophagus
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
Definition of Hypertensive LES
Resting LES pressure \> 45 mmHg (or \> 95th percentile in nl subjects)
40
Other manometric findings associated with Hypertensive LES
* 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
MC symptoms of nutcracker esophagus
Dysphagia Chest pain Indigestion
42
Other esophageal anatomic disorder commonly associated with hypertensive LES
Large paraesophageal hernia with abnormal esophageal exposure to gastric acid * 24-hr pH monitoring test used to identify these patinets
43
Treatment options for hypertensive LES
Medial managment Distal esophageal myotomy with partial fundoplication (Heller)
44
Results in smooth muscle atrophy and subsequent replacement with scar
Scleroderma
45
Manometric findings of scleroderma
* Peristaltic dysfunction (normal peristalsis of upper 1/3 esophagus d/t striated muscle) * Weakening of LES
46
Barium esophagram findings of scleroderma
* Slightly dilated esophagus * Weak or absent peristalsis * Free reflux often is demonstrated
47
Diagnosis
Achalasia Bird's beak (dilated esophagus)
48
Diagnosis
Nutcracker Esophagus Corkscrew
49
Diagnosis
Normal esophagram
50
Integrated Relaxation Pressure
**_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
How is incomplete relaxation of the LES identified on HRM
Integrated Relaxation pressure of 15mmHg or greater
52
Distal Latency
High resolution manometry meaurement ## Footnote Meaure of peristaltic timing (wave from initiation of swallow to the distal)
53
54
what is eosinophilic esophagitis ?
Eosinophilic esophagitis chonric inflammatory disorder Esophageal dysfunction Intraepithelial infiltration of Eosinophils Thought to be an allergic reaction
55
Eosinophilic esophagitis - what are the classic presenting symptoms ?
1. Dysphagia 2. Food impaction 3. Heart burn un responsive to PPI
56
What does endoscopy of eosinophilic Esophagitis reveal?
1. "Trachealization of the the esophagus" 2. "Feline esophagus" 3. Friable mucosae with edema 4. Single or multiple concentric rings. 5. White papules
57
Biopsy results on Eosinophilic esophagitis ?
Threshold 15-20 Eos / HPF Average is 40; although, may be more
58
Treatment of Eosinophilic Esophagitis?
1. Elimination diet 2. Parenternal or systemic corticosteroids
59
Treatment of Candidial Esophagitis
Fluconazole 100-200 mg daily Superior to keotonazole in patients with HIV
60
Scleroderma - what is it ?
Connective tissue disorder characterized by fibrosis of the skin, vasculature, and internal orrgans.
61
Frequency of GI manifestations with scleroderma ?
80% have some GI involvement 10% present with GI sx. Severe involvement affects 8%. Only 15% of these patients survive 9 years ?
62
Integrated relaxation pressure
A measure of GEJ relaxation while swallowing Averages of 4 non-continues seconds durring the lowest pressure. Incomplete relaxation - IRP \> 15mmHg
63
How to calculate incomplete relaxation off of HRM?
Integrated relaxation pressure. \> 15mmHg
64
HRM diatal latency
measurement of perstaltic timing Normal is defined as \> 4.5 from the inidiation of swallow LESS THAN 4.5 is a spastic swallow
65
Type 1 Achalasia structure characteristics
Type 1: **Late** Achalasia Significant loss of _dilation_ *Loss* of _Elasticity_
66
Type 1 Achalasia Functional Characteristics
Type 1 Achalasia: Late Achalasia 1. Aperistalsis 2. Elevated IRP 3. Incomplete LES relaxation with elevated pressure
67
Characteristics of Type II achlasia
Panesophageal pressurization \> 30mmHg in at least 20% of swallows
68
Type III Achalsia General description ? Quantitative criteria?
Type III Achalasia: "Vigorous Achalsia" Non-peristaltic At least two spastic high-amplitude waves Elevated IRP
69
Treatments most effeective for type III achalasia ?
Botox POEM
70
With what type of achalsia is BoTox most effective
Type III
71
Optimal treatment for Type II achalsia
Heller or pneumatic dilation
72
Optimal treatment for Type I achalsia
Myotomy as initial treatement compared to dilation of Botox

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