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Esophageal Achalasia
Aka: Esophageal Achalasia, Achalasia, Cardiospasm
- See Also
- Esophageal Dysmotility
- Epidemiology
- Rare condition with Incidence <5% of cases presenting to specialty centers
- More common in older adults than younger patients
- Pathophysiology
- Chronic progressive degenerative disorder often with delayed diagnosis
- Delayed diagnosis results in esophageal dilitation, resulting in cases refractory to surgical management
- Chronic esophageal Smooth Muscle denervation
- Deficient distal esophagus Cholinergic innervation
- Decreased tissue nitric oxide
- Inhibitory Neuron loss
- Results
- Loss of normal peristalsis
- Lower Esophageal Sphincter (LES) dysfunction
- LES Incoordinate contraction
- LES tonic contractions
- Constriction of LES in response to Swallowing
- Types
- Achalasia Type 1 (Classic Achalasia)
- No contractility or peristalsis
- Lower esophageal sphincter fails to relax (all Achalasia types)
- Responds to Laparoscopic Heller Myotomy
- Achalasia Type 2 (with esophageal compression)
- No normal peristalsis (but some pressurizations)
- Lower esophageal sphincter fails to relax (all Achalasia types)
- Responds to all treatment options
- Achalasia Type 3 (Spastic Achalasia)
- No normal peristalsis
- Spastic contractions in distal esophagus (>20% of swallows)
- Lower esophageal sphincter fails to relax (all Achalasia types)
- Responds poorly to treatment
- Symptoms
- Dysphagia
- Chest Pain
- Regurgitation of food, Saliva, esophageal secretions
- Weight loss
- Diagnostics
- Upper Endoscopy Achalasia findings
- Food retained in esophagus
- Increased resistance across esophagogastric junction
- High-Resolution Esophageal Manometry
- Required for Achalasia diagnosis
- Performed with Nasogastric Tube with closely positioned pressure sensors measure intraluminal pressure
- Esophageal pressures are measured as the patient swallows various foods and liquids
- Lower esophageal sphincter pressure is also measured before Swallowing and during relaxation
- Upper GI (Barium Esophagram)
- Esophageal dilatation
- Beadlike narrowing of distal segment
- Spasm of lower esophageal sphincter
- Differential Diagnosis
- See Esophageal Dysphagia
- Management
- See Esophageal Dysmotility for general measures
- Myotomy (definitive therapy)
- Laparoscopic Heller Myotomy
- Incises Muscles of the distal esophagus, lower esophageal sphincter and gastric cardia
- Peroral Endoscopic Myotomy
- Newer, more technically challenging, but less invasive procedure than the laparoscopic Heller myotomy
- Incises the same Muscles as the Heller procedure
- Pneumatic dilation (by endoscopy)
- Disrupts lower esophageal sphincter
- Not as effective or longlasting as myotomy (dilation may need to be repeated)
- Onabotulinumtoxin A
- Endoscopic injection into lower esophageal sphincter
- Unknown efficacy, but may be used in patients at too high risk for surgery
- References
- Wilkinson (2020) Am Fam Physician 102(5):291-6 [PubMed]