Esophagus: Motility Disorders

Chicago Classification – Version 3

Integrated Relaxation Pressure (IRP) Elevated

  • Achalasia – Aperistalsis
    • Type I: No Contractile Activity
    • Type II: Pan-Esophageal Pressurization in ≥ 20% of Swallows
    • Type III: Premature/Spastic Contractions in ≥ 20% of Swallows
  • EGJOO – Peristalsis

Integrated Relaxation Pressure (IRP) Normal

  • Major Disorders of Peristalsis
    • DES – Premature Contractions ≥ 20% of Swallows
    • Jackhammer Esophagus – DCI (Distal Contractile Integral) > 8,000 mmHg ≥ 20% of Swallows
    • Absent Contractility – No Scorable Contraction
  • Minor Disorders of Peristalsis
    • Ineffective Esophageal Motility – ≥ 50% of Swallows Ineffective
    • Fragmented Peristalsis – ≥ 50% of Swallows Fragmented

Normal Manometry 1

Achalasia

Basics

  • Definition: Failure/Incomplete Relaxation of the LES with Esophageal Aperistalsis
  • The Most Common Esophageal Motility Disorder
  • Cause: Autoimmune Destruction of Neural Ganglion Cells in the Myenteric Plexus
  • Risk Factors:
    • Autoimmune Disorders
    • Neurodegenerative Disorders
    • Diabetes Type 1
  • Increased Risk of Squamous Cell Carcinoma

Symptoms

  • Classic Triad:
    • Dysphagia to Both Solids & Liquids (Most Common)
    • Regurgitation
    • Weight Loss
  • Heartburn
  • Chest Pain
  • Difficulty with Belching

Diagnosis

  • Manometry
    • Required to Establish Diagnosis
    • Findings: Incomplete LES Relaxation & Aperistalsis
  • Barium Esophagram
    • Preform if Manometric Findings are Equivocal
    • Findings: Dilated Esophagus, Narrowed EG Junction & Delayed Emptying
      • “Bird’s Beak” Appearance
  • Upper Endoscopy
    • Required to Rule Out Cancer Which Can Mimic
    • Bx: T Cell & Eosinophil Infiltration of Myenteric Plexus

Chicago Classification – Version 3

  • Type I (Classic Achalasia):
    • High Integrated Relaxation Pressure (IRP)
    • Aperistalsis
    • No Contractile Activity
      • Distal Contractile Integral (DCI/Strength of Distal Contraction) < 100 mmHg
  • Type II:
    • High Integrated Relaxation Pressure (IRP)
    • Aperistalsis
    • Pan-Esophageal Pressurization in ≥ 20% of Swallows
  • Type III (Spastic Achalasia):
    • High Integrated Relaxation Pressure (IRP)
    • Aperistalsis
    • Premature/Spastic Contractions in ≥ 20% of Swallows
      • Distal Contractile Integral (DCI/Strength of Distal Contraction) < 450 mmHg

Treatment

  • Heller Myotomy
    • Historical Gold Standard (Still the “Safe Answer” on Testing)
    • Dysphagia Relief
      • Type I/II: 90%
      • Type III: 50%
  • Pneumatic/Balloon Dilation
    • Graded Dilations are Superior to Single Dilation
    • Emerging as the First-Line Initial Treatment
    • Must Be Good Surgical Candidates as Perforation May Require Surgical Repair
    • Compared to Heller Myotomy:
      • Single Dilation is Inferior but Graded Dilations are Equivalent
      • More Adverse Events
      • Similar Quality of Life
  • Peroral Endoscopic Myotomy (POEM)
    • Must Be Good Surgical Candidates as Perforation May Require Surgical Repair
    • Compared to Heller Myotomy:
      • Similar Results
      • Lower Complications
      • Shorter Hospital Stay & Faster Recovery
    • Highest Risk of GERD – Ablates LES, No Antireflux Procedure
  • Options if Unable to Tolerate Surgery:
    • Endoscopic Botulinum Toxin (Botox) Injections into LES
    • Pharmacology: Nitrates, CCB or PDE-5 Inhibitors
    • *Less Effective

Similar Findings

  • Chagas Disease
    • Parasite: Trypanosoma cruzi
    • Predominantly in Central/South America
    • Produces Similar Sx
  • Pseudoachalasia
    • Similar Imaging Findings but Due to CA
  • Allgrove Syndrome:
    • Autosomal Recessive Disorder
    • Triad: Achalasia, Adrenal Insufficiency & Alacrima (Reduced Tear Production)

Achalasia Manometry; Type I – No Contractility with Incomplete LES Relaxation; Type II – Penesophageal Pressurization; Type III – Premature Spastic Contractions 1

“Birds Beak” on Esophagram

Esophagogastric Junction Outflow Obstruction (EGJOO)

Basics

  • Obstruction from Intrinsic or Extrinsic Compression
  • May Be a Precursor to Achalasia

Causes

  • Idiopathic
  • Pseudoachalasia from Malignancy
  • Hiatal Hernia
  • Stricture
  • Scarring
  • Vascular Obstruction from Diseased Aortic Arch

Diagnosis

  • Manometry
    • Chicago Classification – Version 3:
      • High Integrated Relaxation Pressure (IRP)
      • Preserved Peristalsis
  • Barium Esophagram
  • Upper Endoscopy
    • Required to Rule Out Cancer or Other Structural Abnormality

Treatment

  • Based on Symptoms
  • Options: PPI, Botox Injection, Pneumatic Dilations, POEM or Myotomy

EGJOO Manometry 1

Diffuse Esophageal Spasm (DES)

Basics

  • High-Amplitude Uncoordinated/Unorganized Contractions with No Peristalsis
  • Premature Rapidly Propagated Contractions from Impaired Inhibitory Innervation
  • Associated with Psychiatric Disorders

Symptoms

  • Dysphagia to Both Solids & Liquids (Most Common)
  • Regurgitation
  • Heartburn
  • Globus Sensation
  • Chest Pain

Diagnosis

  • Manometry
    • Required to Establish Diagnosis
    • Findings: High-Amplitude Uncoordinated/Unorganized Contractions with No Peristalsis
    • Chicago Classification – Version 3:
      • Normal Integrated Relaxation Pressure (IRP)
      • Premature Contractions ≥ 20% of Swallows with Distal Latency < 4.5 Seconds
  • Barium Esophagram
    • Findings: “Corkscrew” Pattern
  • Upper Endoscopy
    • Required to Rule Out Cancer or Other Structural Abnormality

Treatment

  • Primary Tx: Diltiazem (CCB) #1 or Imipenem (TCA)
    • Other Meds: Trazodone, Venlafaxine or PDE-5 Inhibitors
  • Other Options:
    • Peroral Endoscopic Myotomy (POEM)
    • Surgical Myotomy
    • Endoscopic Botulinum Toxin (Botox) Injections

DES Manometry 1

“Corkscrew” on Esophagram 2

Jackhammer (Nutcracker) Esophagus

Basics

  • High-Amplitude Coordinated Contractions with Peristalsis
  • Extreme Asynchronous Contractions from Excessive Cholinergic Drive
  • Can Involve Esophageal Body or Be Limited to the GE Junction

Symptoms

  • Dysphagia
  • Chest Pain (More Prevalent Than in Other Motility Disorders)
  • Regurgitation
  • Heartburn
  • Globus Sensation

Diagnosis

  • Manometry
    • Required to Establish Diagnosis
    • Findings: High-Amplitude Coordinated Contractions with Peristalsis
    • Chicago Classification – Version 3:
      • Normal Integrated Relaxation Pressure (IRP)
      • Distal Contractile Integral (DCI/Strength of Distal Contraction) > 8,000 mmHg
        • ≥ 20% of Swallows
  • Barium Esophagram
    • Findings: Normal
  • Upper Endoscopy
    • Required to Rule Out Cancer or Other Structural Abnormality

Treatment

  • Primary Tx: Diltiazem (CCB) #1 or Imipenem (TCA)
    • Other Meds: Trazodone, Venlafaxine or PDE-5 Inhibitors
  • Other Options:
    • Peroral Endoscopic Myotomy (POEM)
    • Surgical Myotomy
    • Endoscopic Botulinum Toxin (Botox) Injections

Hypercontractile Esophagus Manometry 1

Absent Contractility

Basics

  • Most Often Associated with Connective Tissue Disorders (Scleroderma)
  • Chicago Classification – Version 3
    • Normal Integrated Relaxation Pressure (IRP)
    • No Scorable Contraction

Scleroderma (Systemic Sclerosis)

  • Massive Reflux & Dysphagia (Loss of LES Tone)
  • Most Common Site: Esophagus (Fibrous Replacement of Smooth Muscle)
  • Dx:
    • Manometry: Low LES Pressure & No Peristalsis
  • Tx:
    • Primary Tx: PPI & Reglan
    • Roux-en-Y Gastric Bypass if Severe
      • Avoid Fundoplication Alone (Scleroderma Can Also Affect Gastric Motility)

Absent Contractility Manometry 1

Minor Disorders of Peristalsis

Ineffective Esophageal Motility (IEM)

  • ≥ 50% of Swallows Ineffective
  • Most Often Caused by Distal Damage from Chronic GERD
  • Often Have More Mild Symptoms & Require Less Intervention
  • Manometry
    • Chicago Classification – Version 3:
      • Normal Integrated Relaxation Pressure (IRP)
      • ≥ 50% of Swallows Ineffective
  • Tx: Control of GERD

Fragmented Peristalsis

  • ≥ 50% of Swallows Fragmented
  • Often Have More Mild Symptoms & Require Less Intervention
  • Manometry
    • Chicago Classification – Version 3:
      • Normal Integrated Relaxation Pressure (IRP)
      • ≥ 50% of Swallows Fragmented
        • (DCI > 450 mmHg with > 5 cm Break)
  • Poorly Described in Literature

IEM Manometry 1

Fragmented Peristalsis Manometry 1

References

  1. Herbella FA, Armijo PR, Patti MG. A pictorial presentation of 3.0 Chicago Classification for esophageal motility disorders. Einstein (Sao Paulo). 2016 Jul-Sep;14(3):439-442. (License: CC BY-4.0)
  2. Samo S, Carlson DA, Kahrilas PJ, Pandolfino JE. Ineffective Esophageal Motility Progressing into Distal Esophageal Spasm and Then Type III Achalasia. ACG Case Rep J. 2016 Dec 21;3(4):e183. (License: CC BY-NC-ND-4.0)