Chicago classification for alchasia

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Chicago classification v4.0 achalasia subtypes types I II III manometry

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Here is a complete, well-sourced overview of the Chicago Classification for achalasia:

Chicago Classification of Achalasia (HRM-Based)

High-resolution manometry (HRM) is the gold standard for diagnosing achalasia. The Chicago Classification (CC) - first introduced by Pandolfino et al. in 2008 and currently at version 4.0 - uses two core parameters to classify achalasia:
  • IRP (Integrated Relaxation Pressure): measures EGJ relaxation during swallowing. Abnormal = >15 mmHg (upper limit of normal).
  • Peristalsis pattern: how the esophageal body responds to wet swallows.
A diagnosis of achalasia requires BOTH:
  1. Elevated median IRP (impaired EGJ relaxation)
  2. 100% absent/failed peristalsis (DCI <100 mmHg·s·cm for all swallows)

The Three Subtypes

HRM achalasia subtypes A=Type I, B=Type II, C=Type III
HRM pressure topography plots of the three achalasia subtypes - A: Type I (classic), B: Type II (with pressurization), C: Type III (spastic). - Clinical Gastrointestinal Endoscopy, Fig. 19.3

Type I - Classic Achalasia

FeatureDetail
IRPElevated (>15 mmHg)
Esophageal body100% failed peristalsis - absent contractility, no pressurization
Pressurization<20% of swallows show pan-esophageal pressurization
AppearanceOften dilated esophagus with negligible pressure in the body
  • The esophageal body is essentially silent - no contractions, no pressurization.
  • Often associated with end-stage, dilated esophagus.
  • Treatment response is moderate; more definitive sphincter disruption is required.

Type II - Achalasia with Panesophageal Pressurization

FeatureDetail
IRPElevated (>15 mmHg)
Esophageal body100% failed peristalsis
Pressurization≥20% of swallows show rapid pan-esophageal pressurization (uniform pressure rise, isobaric)
MechanismTrapped fluid column is compressed as esophagus shortens
  • The most common subtype encountered clinically.
  • Carries the best prognosis - treatment response rates of 95-96% with pneumatic dilation or Heller myotomy.
  • The panesophageal pressurization is a passive, homogeneous pressure rise - not a true contraction.

Type III - Spastic (Vigorous) Achalasia

FeatureDetail
IRPElevated (>15 mmHg)
Esophageal body≥20% of swallows show premature/spastic contractions (Distal Latency <4.5 seconds)
DCINormal or elevated in those spastic contractions
PeristalsisNo normal peristalsis; failed peristalsis ± pressurization may also be present
  • Characterized by rapidly propagated, lumen-obliterating contractions in the distal esophagus.
  • Worst prognosis among the subtypes - treatment response is 29-70%.
  • These patients do not respond well to interventions aimed solely at the LES; POEM (per-oral endoscopic myotomy) with a longer myotomy is often preferred.

Summary Table

SubtypeIRPPeristalsisKey FeatureTreatment Response
Type I (Classic)Elevated100% failed, no pressurizationSilent esophagusModerate
Type II (With pressurization)Elevated100% failedPan-esophageal pressurization ≥20% swallowsBest (95-96%)
Type III (Spastic)Elevated≥20% premature/spastic (DL <4.5s)Rapid distal contractionsWorst (29-70%)

Key Updates in Chicago Classification v4.0

The CCv4.0 (Yadlapati et al., 2021) made several refinements:
  1. Abnormal IRP can now be defined based on either supine OR upright position (not both required), if the protocol includes 10 wet swallows in the upright position.
  2. 100% absent peristalsis is strictly required - defined as all swallows having either failed peristalsis OR premature contraction.
  3. Inconclusive diagnoses are formally recognized - if appreciable peristalsis is seen in a different position, the diagnosis shifts toward inconclusive, requiring supportive testing (barium swallow, EndoFLIP, timed barium esophagram).
  4. EGJ Outflow Obstruction (EGJOO) with spastic features is now distinguished from Type III - if IRP is elevated with spasm but some peristalsis is preserved, it is classified as EGJOO with spastic features (a potential achalasia variant).

Clinical Relevance: Treatment Choice by Subtype

  • All types: Pneumatic dilation, laparoscopic Heller myotomy (LHM), POEM
  • Type II: Best outcomes with any modality
  • Type III: POEM is preferred because its longer myotomy (extending into the esophageal body) addresses the spastic segment; LHM may be less effective
  • Type I: Outcomes are reduced further as the degree of esophageal dilatation increases; esophagectomy may be needed in end-stage megaesophagus
Sources: Clinical Gastrointestinal Endoscopy, 3e, p. 308-309 | Sleisenger & Fordtran's GI and Liver Disease, p. 734-735 | Chicago Classification v4.0, Neurogastroenterol Motil 2021
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