Achalasia

Good fifteen minutes left of this morning 🙂

Top end now- achalasia


Achalasia

Definition: non-progressive oesophageal motility disorder where the lower oesophageal sphincter (LOS) fails to relax, and there is loss of peristalsis.

Aetiology/ risk factors:

-due to degeneration of the myenteric plexus in the oesophagus due to unknown cause

Epidemiology: annual incidence is about 1 in 100,000

Usual presentation age = 25-60 years

Symptoms: insidious onset and gradual progression

-intermittent dysphagia (difficulty swallowing)

-difficulty swallowing solids and liquids from the start (does not progress from solids to liquids as a stricture would)

-regurgitation  (particularly at night)

-substernal cramps (retrosternal chest pain)

-heartburn

-difficulty belching

-weight loss

Signs:

-may have signs of complications e.g. aspiration pneumonia or malnutrition

Investigations:

CXR- air-fluid level in dilated oesophagus (above heart)

-may show widened mediastinum and double right heart border (dilated oesophagus)

-absence of normal gastric bubble

Image result for chest x ray achalasia

Achalasia- arrows pointing to massively dilated oesophagus

 

Image result for chest x ray achalasia air-fluid level

Achalasia- see the air-fluid level above the heart?

 

Barium swallow- dilated, tapering oesophagus (beak-shaped)

Image result for barium swallow achalasia

Endoscopy +/- biopsy– to exclude malignancy, which can mimic achalasia

Oesophageal manometry– if barium swallow normal, measures pressures in the oesophagus

-elevated resting LOS pressure (> 45mmHg)

-incomplete LOS relaxation

-absence of peristalsis in the distal, smooth muscle portion, of the oesophagus

Video fluoroscopy– to identify dysmotility

Management:

Endoscopic balloon dilatation or Heller’s cardiotomy/myotomy (muscle fibres of lower oesophageal sphincter at the cardia, where the oesophagus joins the stomach, are cut)

-followed by proton pump inhibitors e.g. omeprazole (to prevent too much reflux now that that the oesophagus is wide open at the bottom)

Non-invasive option– repeated botulinum toxin injections into the LOS

calcium channel blockers (e.g. nifedipine) and nitrates, also relax the sphincter

Complications:

-aspiration pneumonia

-malnutrition, weight loss

-long-standing achalasia may cause oesophageal cancer (15x increased risk)

-surgery for achalasia = gastro-oesophageal reflux disease (GORD)

Prognosis: good prognosis if treated. If untreated, oesophageal dilation may worse, causing pressure on mediastinal structures.

References: Rapid Medicine, Cheese & Onion
Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s