What are the stages of achalasia progression and its management?

Stages of Achalasia

Achalasia is classified into three distinct subtypes (Types I, II, and III) based on the Chicago Classification using high-resolution manometry, not traditional "stages" of disease progression, though end-stage disease represents a separate clinical entity characterized by severe esophageal dilation and tortuosity. 1

Chicago Classification Subtypes

The modern classification system defines achalasia by manometric patterns rather than temporal progression:

Type I Achalasia (Classic)

• Absent contractility throughout the esophageal body 2
• Represents the most common presenting subtype 2
• Often misdiagnosed before high-resolution manometry became available due to esophageal shortening and pseudorelaxation 2

Type II Achalasia (Compression)

• Panesophageal pressurization with at least 20% of swallows 2
• Most common presenting achalasia subtype 2
• Best treatment outcomes among all subtypes, with superior response to pneumatic dilation and laparoscopic Heller myotomy 2

Type III Achalasia (Spastic)

• Premature (spastic) contractions in the distal esophagus 2
• Often mistaken for esophageal spasm before high-resolution manometry 2
• Obstructive physiology includes the distal esophagus 2
• Worst treatment outcomes with standard lower esophageal sphincter-directed therapies 2

End-Stage Achalasia

Beyond the Chicago Classification subtypes, there exists a clinically distinct entity:

• Characterized by progressive tortuosity and dilation of the esophagus with sigmoid deformation 3
• Develops in up to 20% of patients with achalasia over time 3
• Represents failure of primary peristalsis with advanced structural changes 3
• Most published treatment trials have excluded end-stage cases, limiting evidence-based guidance 2
• Can develop 10.1% of patients over 40 years of follow-up, regardless of initial treatment modality 4

Clinical Progression Considerations

While not formal "stages," certain patterns suggest disease evolution:

Early or Incomplete Achalasia

• 12-40% of cases may represent early or incomplete achalasia 2
• Can resolve spontaneously in some instances 2
• May be evolving toward Type III achalasia 2

Disease Trajectory

• 60.7% of patients experience symptom recurrence requiring repeat intervention over long-term follow-up (mean 17.5 years) 4
• Patients may require multiple procedures over decades to maintain symptom control 4
• At 10+ years follow-up, 92% of patients have absent or mild dysphagia with appropriate repeated interventions 4

Diagnostic Algorithm

All patients suspected of achalasia must undergo:

1. High-resolution manometry - gold standard for diagnosis and subtype classification 1
2. Upper endoscopy - mandatory to exclude pseudoachalasia from occult malignancy through careful retroflexed examination of the gastroesophageal junction 1
3. Timed barium esophagram - confirms outflow obstruction and demonstrates structural changes including sigmoid deformation 1
4. Clinical history with medication review - to identify opioid-induced achalasia or other secondary causes 1

Treatment Implications by Subtype

Type I and II Achalasia

• Pneumatic dilation and laparoscopic Heller myotomy are both highly efficacious with similar long-term outcomes 2
• Pneumatic dilation has less morbidity and cost but requires anticipation of repeat dilations over years 2
• POEM is comparable in efficacy but with insufficient data for advanced esophageal dilation, sigmoidization, epiphrenic diverticulum, and hiatal hernia 2

Type III Achalasia

• POEM should be considered the preferred treatment as it allows calibrated myotomy length to the spastic segment 1, 2
• Myotomy length averages 17.2 cm for Type III, with 92% weighted pooled response rate 2
• Standard lower esophageal sphincter-directed therapies have less robust outcomes 2

End-Stage Disease

• Laparoscopic or endoscopic myotomy recommended as initial approach in most patients per international guidelines 3
• Esophagectomy may have benefit as primary treatment in sigmoid esophagus, but should be considered after failed myotomy 3

Critical Pitfalls

• Do not rely on clinical presentation alone - high-resolution manometry is essential for accurate subtype classification which directly impacts treatment selection 1
• Type III achalasia requires longer myotomy - standard lower esophageal sphincter-directed therapy will fail in many cases 2
• Most patients will require repeat interventions over their lifetime - counsel patients that initial treatment is rarely definitive 4
• Post-POEM patients are high risk for reflux esophagitis and should be counseled about potential indefinite proton pump inhibitor therapy 2, 5