Related Subjects:
| Achalasia
|American Trypanosomiasis (Chagas Disease)
| Oesophageal Carcinoma
| Diffuse Oesophageal spasm
| Diffuse Oesophageal Perforation - Rupture
| Gastro-Oesophageal Reflux
Lost pleasure with nosh
Chest fills, an audible slosh
Foul fermenting food
Swallow study, contrast squeak
Wide tube tapers to bird’s beak Link to author
About
- Achalasia is a rare primary motility disorder of the oesophagus
- The immediate concern in all older patient is to exclude oesophageal cancer.
- Achalasia - from the Greek "fails to relax" and causes dysphagia
Epidemiology
- Usually in younger age groups aged 30-60 but all age groups can be affected
- Incidence is one person in 100,000 per year
Aetiology
- The Lower oesophageal sphincter (LOS) fails to relax with decreased peristalsis
- This causes a functional obstruction to the passage of food
- There is a loss of ganglionic cells in myenteric complex in oesophagus
- Reduction in the synthesis of nitric oxide and vasoactive intestinal polypeptide
- Degeneration of nerves from the vagus
- Similar to Chagas disease seen in South America.
Clinical
- Dysphagia equal to solids and liquids. Inability to belch
- Patient drinks fluids after eating to force food across LOS
- Valsalva like manoeuvres to force through food
- Worse when eating in a hurry or stressed
- May regurgitate undigested food held above LOS in the oesophagus
- Chest pain may be seen and some weight loss
- Hiccoughs and reflux symptoms
Investigations
- Needs barium swallow, an upper endoscopy, and oesophageal manometry to confirm the diagnosis
- CXR - shows a wide and dilated oesophagus with fluid level with absent fundal gas shadow. May show any chest infection due to aspiration.
- Barium meal - dilated oesophagus above LOS with a rat's tail or "beaked" appearance as barium trickles through
- Manometry shows raised pressure at LOS usually > 45mmHg and failure to relax and asynchronous contractions with loss of distal oesophageal peristalsis and insufficient LES relaxation with swallowing.
- Endoscopy shows a dilated oesophagus and excludes a stricture
- Cholecystokinin usually ? LOS tone but in Achalasia tone ?
- Serology for Trypanosoma cruzi if the patient is from an affected area
Differential
- Oesophageal cancer: Dysphagia with achalasia is equally to solids and liquids but with malignancy solids are affected first and only later fluids and saliva
- Oesophageal Stricture - benign or malignant
- Chagas disease: endemic areas. Check serology if likely
Management
- Calcium antagonists (Nifedipine 10 mg s/l capsule) and nitrates e.g. GTN spay [NTG] given before meals may reduce LOS tone but side effects include flushing and headache.
- Botulinum toxin is a potent neurotoxin that inhibits the release of acetylcholine at presynaptic terminals of motor neurons. A single injection of botulinum toxin has been shown to be effective in about 85% of patients with achalasia, but its effect diminishes over time
- Endoscopic pneumatic balloon dilation of the LOS is more commonly used to tear the muscles at the LOS but has a risk of perforation.
- Surgical treatment is Heller's extramucosal cardiomyotomy which may include a fundoplication to reduce subsequent reflux. It can be done via a thoracic or abdominal approach
Complications
- Aspiration and pneumonia especially overnight
- Increased risk of oesophageal squamous carcinoma x 15 population
- Malnutrition and weight loss
References