Bird Beak Appearance:
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This barium esophagogram demonstrates achalasia, a motility disorder of the esophagus, characterized by the classic ‘bird beak’ appearance at the gastroesophageal junction. This image highlights the narrowed distal esophagus and proximal dilation.
What Is Achalasia?
Achalasia (also called achalasia cardia or primary achalasia) is a rare disorder where the esophagus loses its ability to push food into the stomach. This happens because:
- The lower esophageal sphincter (LES) fails to relax.
- The esophagus loses normal peristalsis (muscle contractions that move food).
As a result, food gets stuck, leading to esophageal dilation and complications like aspiration pneumonia or even esophageal cancer in severe cases.
Secondary Achalasia (Pseudoachalasia)
Sometimes, similar symptoms occur due to esophageal cancer or other structural blockages—this is called secondary achalasia or pseudoachalasia.
Who Gets Achalasia? (Epidemiology)
- Most common in adults aged 30–70.
- Affects both men and women equally.
- Most cases are idiopathic (no known cause).
- Chagas disease (a parasitic infection) can mimic achalasia.
- Rarely linked to Allgrove syndrome (a genetic disorder).
Symptoms of Achalasia
Patients usually experience:
- Dysphagia (trouble swallowing both solids and liquids—unlike cancer, which mostly affects solids).
- Chest pain or discomfort.
- Regurgitation of undigested food.
- Weight loss (due to eating difficulties).
Complications of Long-Term Achalasia
If untreated, achalasia can lead to:
- Esophageal cancer (~5% of patients).
- Aspiration pneumonia (from food/liquid entering lungs).
- Candida esophagitis (fungal infection).
- Acute airway obstruction (rare but life-threatening).
How Is Achalasia Diagnosed?
According to the Chicago Classification v4.0, achalasia is confirmed via:
1. Esophageal Manometry (Key Test)
- Shows 100% failed peristalsis (no muscle contractions).
- Measures LES pressure (often abnormally high).
2. Barium Swallow (Imaging Test) Bird Beak Appearance
- Reveals “bird beak” or “rat tail” narrowing at the LES.
- Shows esophageal dilation and food stasis.
3. Endoscopy
- Rules out cancer or strictures.
- Checks for mucosal damage.
Types of Achalasia (Chicago Classification of Achalasia)
| Type | Manometric Findings | Prognosis |
|---|---|---|
| Type I (Classic) | No contractions in esophagus | Moderate (81% success with treatment) |
| Type II | Pan-esophageal pressurization | Best (96% success) |
| Type III (Spastic) | Premature, spastic contractions | Worst (66% success) |
Treatment Options for Achalasia
1. Lifestyle Changes
- Eat slowly, drink more water with meals.
- Avoid lying down after eating.
2. Medications (Short-Term Relief)
- Calcium channel blockers (help relax LES temporarily).
3. Pneumatic Dilation (Non-Surgical)
- 90% effective, but carries a 3-5% risk of perforation.
4. Botox Injections
- Relaxes the LES, but effects last only ~12 months.
5. Surgery (Most Effective Long-Term)
- Heller myotomy (cuts LES muscle) + fundoplication (prevents reflux).
- POEM procedure (minimally invasive alternative).
Differential Diagnosis (Conditions That Mimic Achalasia)
- Esophageal cancer (pseudoachalasia)
- Chagas disease
- Scleroderma
- Diffuse esophageal spasm
Prognosis and Long-Term Outlook
- Type II achalasia has the best response to treatment (96%).
- Type III (spastic) is hardest to treat (66%).
- Regular follow-ups are needed to monitor for cancer risk.
Key Takeaways
- Achalasia is a motility disorder causing swallowing difficulties.
- Manometry and barium swallow are key diagnostic tools.
- Surgery (Heller myotomy or POEM) offers the best long-term relief.
- Untreated achalasia increases esophageal cancer risk.
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