Question

Difficulty: MediumGastroenterology and Hepatology

Match each esophageal motility disorder with its most characteristic finding on high-resolution esophageal manometry.

  • AchalasiaIncomplete lower esophageal sphincter (LES) relaxation and absent peristalsis in the smooth muscle esophagus
  • Diffuse esophageal spasmSimultaneous, uncoordinated, non-peristaltic contractions with normal LES relaxation
  • Systemic sclerosis (scleroderma) esophageal involvementAbsent peristalsis in the lower two-thirds of the esophagus with a hypotensive (low resting pressure) LES
  • Jackhammer esophagus (hypercontractile esophagus)Extremely high-amplitude, prolonged, but coordinated peristaltic contractions with normal LES relaxation

Answer

Achalasia matches with incomplete LES relaxation and absent peristalsis. Diffuse esophageal spasm matches with simultaneous contractions and normal LES relaxation. Systemic sclerosis matches with absent peristalsis and a hypotensive LES. Jackhammer esophagus matches with extremely high-amplitude coordinated contractions.
Each disorder has a unique manometric signature: Achalasia features failed LES relaxation and aperistalsis; diffuse esophageal spasm features simultaneous contractions with normal LES relaxation; systemic sclerosis features aperistalsis with a hypotensive LES; and Jackhammer esophagus features normally coordinated but extremely high-amplitude contractions.

Step-by-Step Solution

1
Identify the core physiological defect in achalasia.
The primary issue is the loss of inhibitory neurons, which prevents the LES from relaxing upon swallowing and abolishes distal esophageal peristalsis.
This directly matches achalasia to 'incomplete LES relaxation and absent peristalsis'.
2
Differentiate diffuse esophageal spasm from other motility disorders.
It presents with uncoordinated, simultaneous contractions while normal LES function is preserved.
This matches diffuse esophageal spasm to 'simultaneous, uncoordinated, non-peristaltic contractions with normal LES relaxation'.
3
Determine the effect of systemic sclerosis on the esophagus.
Smooth muscle fibrosis affects the lower two-thirds of the esophagus, weakening the LES and abolishing peristalsis, causing severe acid reflux.
This matches systemic sclerosis to 'absent peristalsis in the lower two-thirds of the esophagus with a hypotensive LES'.
4
Identify the hallmark of Jackhammer esophagus.
It is a hypercontractile disorder characterized by intense, high-amplitude contractions that are normally coordinated.
This matches Jackhammer esophagus to 'extremely high-amplitude, prolonged, but coordinated peristaltic contractions'.

Key Concept

Diagnosis of esophageal motility disorders relies on high-resolution manometry to evaluate lower esophageal sphincter (LES) relaxation and the pattern of esophageal body peristalsis.
Estimated Time:1m 30s
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