Overview
This lecture covers esophageal motility disorders, their causes, mechanisms, complications, diagnostic approach, and treatment options.
Pathophysiology & Types
- Esophageal motility disorders result from dysfunction of esophageal nerves or muscles, impairing peristalsis.
- The primary symptom is dysphagia (difficulty swallowing) for both solids and liquids.
- Complications can include aspiration and increased risk of esophageal cancer (especially in achalasia and scleroderma).
Major Esophageal Motility Disorders
- Achalasia: Loss of myenteric plexus function decreases nitric oxide and VIP, leading to high lower esophageal sphincter (LES) tone and poor mid/distal esophageal motility.
- Often idiopathic, but Trypanosoma cruzi infection is a secondary cause; classic triad: megaesophagus, megacolon, dilated cardiomyopathy.
- Diffuse Esophageal Spasm: Normal LES tone, but high, uncoordinated contractions in mid/distal esophagus causing dysphagia and chest pain.
- Esophageal Scleroderma: Atrophy and fibrosis of esophagus lowers both LES tone and mid/distal motility; associated with CREST syndrome.
Diagnosis
- Barium Swallow:
- Achalasia: Bird’s beak appearance (dilated esophagus with narrowing at LES).
- Diffuse Esophageal Spasm: Corkscrew appearance (uncoordinated contractions).
- Scleroderma: Often normal findings.
- EGD with Biopsy: Mainly used to rule out malignancy.
- Manometry (Gold Standard):
- Achalasia: Low mid/distal contractility, high LES tone.
- Diffuse Spasm: High-amplitude, uncoordinated mid/distal contractions, normal LES tone.
- Scleroderma: Low contractility and LES tone.
Treatment
- Achalasia:
- Medical: Calcium channel blockers (e.g., amlodipine), long-acting nitrates, or botulinum toxin for patients not suitable for surgery.
- Severe/refractory: Pneumatic dilation (preferred in older patients), Heller myotomy (preferred in younger/good surgical candidates).
- Diffuse Esophageal Spasm:
- Medical therapy preferred: Calcium channel blockers and nitrates to reduce smooth muscle contractions; rarely pneumatic dilation or botulinum toxin.
- Esophageal Scleroderma:
- No specific motility treatment; use PPIs to prevent esophagitis.
Key Terms & Definitions
- Dysphagia — difficulty swallowing.
- Myenteric (Auerbach) Plexus — nerve network controlling esophageal muscle contraction.
- Lower Esophageal Sphincter (LES) — muscular ring controlling passage from esophagus to stomach.
- CREST Syndrome — Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia.
Action Items / Next Steps
- Review pathophysiology and distinguishing features of each disorder.
- Memorize classic diagnostic imaging findings.
- Study drug mechanisms and indications for each treatment.
- Prepare for exam questions on CREST syndrome and Trypanosoma cruzi triad.