Makindo Medical Notes.com

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
@DrCindyCooper

Achalasia is primarily caused by the degeneration of the nerves in the oesophagus, particularly the ganglion cells in the myenteric plexus.

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

• Bloods: FBC, U&E, LFTs, Glucose
• CXR: shows a wide and dilated oesophagus with fluid level with absent fundal gas shadow. May show any chest infection due to aspiration pneumonia due to regurgitation and aspiration of food into the lungs.
• Barium swallow: dilated oesophagus above LOS and "bird's beak" appearance, where the lower oesophagus narrows sharply due to the tight LOS.
• Manometry : This is the gold standard test for diagnosing achalasia. There is raised pressure at LOS usually > 45 mmHg 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 and tumour
• 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) or 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. A rare but serious complication of treatments like pneumatic dilation.
• 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
• Manage reflux: Eating slowly, chewing food thoroughly, and drinking plenty of water with meals can help manage symptoms. Elevating the Head of the Bed can help reduce nocturnal regurgitation. Avoiding Large Meals Before Bedtime: This can also help reduce the risk of nighttime symptoms.
• Cancer risk: There is also a slightly increased risk of oesophageal cancer in patients with long-standing achalasia, so regular follow-up and monitoring are important.

Complications

• Aspiration and pneumonia especially overnight
• Increased risk of oesophageal squamous carcinoma x 15 population
• Malnutrition and weight loss

References

• Guidelines for the Surgical Treatment of Esophageal Achalasia