EGJOO

Esophagogastric Junction Outflow Obstruction (EGJOO)

Yaiel G. Rodríguez-Avilés, MD

Table of Contents

Definition
Pathogenesis and Causes
Clinical Presentation
Diagnosis
Treatment

See Also:
*See Esophageal Motility Disorders
*See Achalasia
*See Esophageal Disorders of Peristalsis

Definition

Definition: An Esophageal Motility Disorder Characterized by Impaired Relaxation and/or Increased Resistance to Outflow Across the Esophagogastric Junction (EGJ) but with Preserved Peristalsis

Diagnosis Requires Compatible Symptoms and Supportive Testing ­– A Manometric Diagnosis of EGJOO Alone is Clinically Inconclusive and Does Not, by Itself, Establish Clinically Significant EGJ Obstruction

Pathogenesis and Causes

Pathogenesis

  • Represents a Heterogeneous Disease Subgroup with No Single Pathophysiologic Cause
  • Functional/Neurogenic LES Dysfunction
    • May Represent an Early/Evolving Variant of Achalasia
  • Mechanical/Structural Resistance
    • Extrinsic Factors Raising Outflow Resistance Despite Otherwise Normal LES Smooth Muscle Function
  • Artifactual (False) IRP Elevation
    • Equipment Malfunction or Malposition

Causes

  • Primary/Functional EGJOO
    • Idiopathic
    • Early/Evolving Achalasia
  • Mechanical/Anatomic
    • Hiatal Hernia
    • Esophageal Stricture
    • Prior Fundoplication
    • Prior Bariatric Surgery
    • Esophageal Rings/Webs
    • Eosinophilic Esophagitis
    • Extrinsic Vascular Compression
  • Malignant/Infiltrative
    • Malignancy Involving the EGJ
  • Medication Related
    • Opioid-Induced
  • Artifactual
    • Catheter/Equipment Artifact/Error
    • Central Obesity

Clinical Presentation

Clinically Relevant Symptoms

  • Dysphagia
    • Usually to Solid Foods
  • Non-Cardiac Chest Pain

Additional Symptoms

  • Regurgitation
  • Heartburn (Retrosternal Burning)
  • Cough
  • Aspiration and Recurrent Pneumonia

Per Chicago Classification v4.0: EGJOO is Clinically Relevant Only When the Manometric Pattern is Accompanied by Dysphagia and/or Non-Cardiac Chest Pain and Supported by Objective Evidence of EGJ Obstruction

Diagnosis

Diagnosis of Clinically Relevant EGJOO Requires 3 Things:

  • High-Resolution Manometry Indicating EGJOO
  • Clinically Relevant Symptoms (Dysphagia and/or Non-Cardiac Chest Pain)
  • Supportive Evidence of Obstruction by Timed Barium Esophagram (TBE) and/or Functional Lumen Imaging Probe (FLIP)

Upper Endoscopy/Esophagogastroduodenoscopy (EGD)

  • Should Be Performed in All Patients with Suspected EGJOO
  • Often the First Diagnostic Test Performed
  • Used to Evaluate for Malignancy or Other Mechanical/Anatomic Cause of Obstruction

High-Resolution Manometry (HRM)

  • The Gold Standard Diagnostic Test
  • Findings:
    • Elevated Median Integrated Relaxation Pressure (IRP) in Both Supine and Upright Position
      • Concordant Elevation Required
    • Elevated Intrabolus Pressure in ≥ 20% of Supine Swallows
    • Preserved Peristalsis
    • Does Not Meet Criteria for Achalasia
  • Provocative Maneuvers Increase Diagnostic Confidence
    • Rapid Drink Challenge
    • Solid Swallows
    • Pharmacologic Provocation
  • EGJOO May Coexist with Other Esophageal Motility Disorders:
    • Ineffective Motility
    • Hypercontractile Features
    • Spastic Features

Timed Barium Esophagram (TBE)

  • Used to Demonstrate Objective Impairment of Esophageal Emptying Across the EGJ
  • Findings Supporting Clinically Relevant EGJOO:
    • Delayed Barium Clearance
    • Retained Barium Column
    • Retention of a Barium Tablet at the EGJ
  • Other Findings:
    • Evaluate for Hiatal Hernias
    • Evaluate for Diverticula
    • Identify Other Structural Abnormalities
    • Provides a Quantitative Assessment of Esophageal Emptying

Functional Lumen Imaging Probe (FLIP)

  • Uses Impedance Planimetry by Balloon Catheter to Measure Cross-Sectional Area and Intra-Luminal Pressure
    • The Ratio of the Two Measurements at the Level of the EGJ is Called the Distensibility Index (EGJ-DI)
  • Reduced EGJ-DI Provides Objective Evidence of EGJ Outflow Obstruction in the Appropriate Clinical Context
  • Particularly Useful When:
    • HRM Findings are Inconclusive
    • TBE Findings are Equivocal
    • Additional Objective Evidence of EGJOO is Needed

EGJOO Manometry: Normal Propagation of the Contractile Wavefront with Outflow Obstruction at the EGJ

Treatment

General Approach

  • Primary EGJOO:
    • Observation May Be Appropriate if Symptoms are Mild
    • Definitive LES-Directed Therapy is Generally Reserved for Carefully Selected Patients with Convincing Evidence of Primary Functional EGJOO and Persistent, Clinically Significant Symptoms
      • Pneumatic Dilation (PD) – Less Invasive and Often the Initial Approach
      • Myotomy (Surgical/Heller vs POEM) – More Invasive but Potentially More Durable
      • *Use of PD and Myotomy is Less Well Validated than in Achalasia
    • Botulinum Toxin Injection for Temporary Symptom Relief if Poor Candidate for Definitive LES-Directed Therapy
  • Secondary EGJOO: Treat the Underlying Cause
    • Dilation for Strictures, Rings, and Webs
    • Repair of Hiatal Hernias
    • Oncologic Treatment for Malignancy
    • Discontinuation of Opioids for Medication-Induced EGJOO

Heller Myotomy (HM)

  • Procedure: Complete Division of Both the Circular and Longitudinal Muscle Fibers of the Distal Esophagus and Proximal Stomach to Fully Disrupt the Non-Relaxing Lower Esophageal Sphincter (LES)
    • Preserves the Inner Submucosa and Mucosa
  • Myotomy Extent:
    • Proximal: 4-6 cm onto the Distal Esophagus
    • Distal: 2-3 cm onto the Gastric Cardia
  • Usually Combined with a Partial Fundoplication (Dor or Toupet)
    • Partial Fundoplication Reduces Postoperative GERD by Controlling Distal Esophageal Acid Exposure
    • Complete Nissen Fundoplication is Generally Avoided Because it May Recreate EGJ Obstruction

Peroral Endoscopic Myotomy (POEM)

  • Procedure: Endoscopic Submucosal Tunneling Followed by Division of the LES Muscle
  • Advantages:
    • Can Be Performed After Previous Dilation or Heller Myotomy
    • Avoids Abdominal or Thoracic Surgical Access
  • Disadvantages:
    • No Concomitant Fundoplication
    • Higher Risk of Postoperative GERD and Reflux Esophagitis

Pneumatic Dilation (PD) 

  • Procedure: Endoscopic Dilation of the LES Using a Graded Pneumatic Balloon
  • May Require Repeated/Sequential Graded Dilations: 3.0 cm, 3.5 cm, 4.0 cm
  • Major Complications:
    • Esophageal Perforation
    • GERD

Botulinum Toxin Injection (Botox)

  • Mechanism: Inhibits Acetylcholine Release at the LES
  • Can Provide Short-Term Symptomatic Relief if Poor Candidate for Definitive LES-Directed Therapy
  • Effects are Generally Temporary with High Recurrence and Repeated Injections are Often Required