Achalasia

(Am J Gastroenterol. 1999;94:3406-12. Gastroenterology. 1999;117:229-32 & 233-54)

DEFINITION:

Achalasia = “lack of relaxation”; The hallmark is the failure of the lower esophageal sphincter (LES) to relax
- Secondary features include aperistalsis of the body of the esophagus
Vigorous Achalasia = prominent contractions can be noticed in body of esophagus (radiographic/manometry)
- These contractions are simultaneous and therefore fulfill manometric definition of aperistalsis
Secondary Achalasia = associated with various diseases: cancer, Chagas’ disease, amyloid, mixed connective tissue disease
Pseudoachalasia = achalasia-like symptoms produced by infiltrating cancer at the GE junction; Consider with ↓ symptom duration & wt loss
Diffuse esophageal spasm vs. Achalasia: at least some normal peristalsis in DES and LES dysfunction to a lesser degree

EPIDEMIOLOGY:

Relatively uncommon: Prevalence 10/10,000; Incidence 0.5 cases/year/100,000 population
Thought to be acquired disorder affecting any age group, however uncommon before the age of 25 (most common 30-60 years old)
♂ = ♀; All races equally affected

ETIOLOGIES:

Remains a mystery; Thought to be associated with a viral infection, particularly Herpes, but no concrete evidence
- Other theories include the possibility of autoimmune disorders
Chagas’ disease has a similarly pathological condition which is due to an infection by Trypanosoma cruzi
- This parasitic antigen has a protein similar to that of myenteric neurons and produces an immunologic attack against the plexus
Pseudoachalasia DDX: cancer of GE junction (most common), amyloidosis, sarcoidosis, postvagotomy, chronic intestinal pseudoobstruction