Oesophageal motility disorders

Section: UGI Sub-section: Oesophagus Curriculum: Curriculum, page 95

Overview

Divide based on the Chicago Classification

• Disorders of oesophagogastric junction Outflow
  • Achalasia
  • Oesophagogastric junction outflow obstruction
• Disorders of Peristalsis
  • Distal oesophageal spasm
  • Nutcracker oesophagus
  • Ineffective oesophageal motility
  • Absent contractility

Alternative causes

• Due to progressive damage induced by an underlying collagen vascular or neuromuscular disorders
  • Scleroderma
  • Dermatomyositis
  • Polymyositis
  • Lupus erythematosus
  • Chagas disease
  • Myasthenia gravis

General Points

• Relatively rare
• Present with variety of symptoms
  • Dysphagia, chest pain, heart burn, regurgitation, weight loss
  • Usually vague and non-specific
  • Severity of symptoms do not always correlate well to manometry
• Need to exclude other organ systems
  • Cardiac, respiratory, peptic ulcers, pancreatic/biliary
  • Need to ask about symptoms specific to connective tissue diseases
• Workup
  • Manometry
  • Barium Swallow
  • OGD to visualise mucosa, strictures, hernia, diverticulum, oesophagitis, masses etc
  • CT if considering extrinsic cause
  • pH testing if reflux thought cause of disease

Chicago Classification

• Version 4 published 2021 Esophageal motility disorders on high-resolution manometry- Chicago classification version 4.0©, page 1
• Categorizes esophageal motility disorders via an algorithmic scheme using metrics from esophageal High Resolution Manometry
• Endoscopy prior to classification
  • Rule out obstruction
  • Rule out Hiatus hernia

Divide based on the Chicago Classification

• Disorders of oesophagogastric junction Outflow

  • Achalasia
  • Oesophagogastric junction outflow obstruction

• Disorders of Peristalsis

  • Distal oesophageal spasm
  • Nutcracker oesophagus
  • Ineffective oesophageal motility
  • Absent contractility

Classification	Disorder	Definition
Disorders of EGJ Outflow	Type I Achalasia	Abnormal median IRP & 100% failed peristalsis
	Type II Achalasia	Abnormal median IRP, 100% failed peristalsis, & ≥20% swallows with panesophageal pressurization
	Type III Achalasia∞	Abnormal median IRP & ≥20% swallows with premature/spastic contraction and no evidence of peristalsis
	EGJ Outflow Obstruction*†	Abnormal median IRP (supine andupright), ≥20% elevated intrabolus pressure (supine), and not meeting criteria for achalasia
Disorders of Peristalsis	Absent Contractility	Normal median IRP (supine and upright) & 100% failed peristalsis
	Distal Esophageal Spasm*	Normal median IRP & ≥20% swallows with premature/spastic contraction
	Hypercontractile Esophagus*	Normal median IRP & ≥20% hypercontractile swallows
	Ineffective Esophageal Motility	Normal median IRP, with >70% ineffective swallows or ≥50% failed peristalsis
∞CCv4.0 recognizes that the distinction between type III achalasia and conclusive EGJOO can be difficult and was vague in CCv3.0. In CCv4.0 achalasia is defined by 100% absent peristalsis which is inclusive of swallows that are either failed or premature and Type III achalasia should not have evidence of normal peristalsis (normal or ineffective swallows)
†Patients with EGJ obstruction and evidence of peristalsis would fulfill strict criteria for EGJOO and may have features suggestive of achalasia or other patterns of peristalsis defined by criteria used for disorders of peristalsis: EGJOO with spastic features (presence of ≥ 20% premature swallows), EGJOO with hypercontractile features, EGJOO with ineffective motility, or EGJOO with no evidence of disordered peristalsis.
*Denote manometric patterns of unclear clinical relevance. A clinically relevant conclusive diagnosis requires additional information which may include clinically relevant symptoms and/or supportive testing (as detailed in the document).
Distal Contractile Integral (DCI); integrated relaxation pressure (IRP); esophagogastric junction (EGJ)

Classifies motility disorders based on manometry findings

• Uses diagnostic algorithm to progressively identify physiologic dysfunction Achalasia

1. GOJ outflow obstruction
2. Major disorders of peristalsis
3. Minor disorders of peristalsis

Step 1 – Assess GOJ

• OGD - exclude hiatus hernia and obstructing lesion
• Assessment of GOJ relaxation
  • Integrated relaxation pressure (Achalasia or GOJ outflow obstruction)
• This divides into Disorders of EGJ Outflow vs Disorders of Peristalsis Step 2 – Characterise oesophageal contractions
• Peristaltic vigour and pattern
• Premature – never seen in normal (? T3 Achalasia or DES) Step 3 – Characterise pressure patterns, if present