achalasia

Question 1

define achalasia

Answer 1

an oesophagela motility disorder,

the lower oesophageal sphincter fails to relax (due to degeneration of the myenteric plexus = loss of peristalsis)

= dysphagia for fluids and solids, regurgitation, substernal cramps, reduced weight

longstanding achalasia can = oesophageal cancer

narrowing of the LOS

Question 2

aetiology of achalasia

Answer 2

degeneration of the ganglion cells of the myenteric plexus in the oesophagus wall,

and degeneration of the vagus nerve due to an unknown cause

oesophageal infection with Trypanosoma cruzi seen in Central and South America produces a similar disorder - chagas’ disease

Question 3

epidemiology of achalasia

Answer 3

annual incidence is 1 in 100000

usual presentation age - 25-60yrs

Question 4

sx of achalasia

Answer 4

Insidious onset and gradual progression of:

• intermittent dysphagia involving solids and liquids - sudden onset
• difficulty belching;
• regurgitation (particularly at night);
• heartburn;
• chest pain (atypical/cramping, retrosternal);
• weight loss.
• reflux

Question 5

signs of achalasia

Answer 5

may reveal signs of the complications:

• aspiration pneumonia
• malnutrition and weight loss
• oesophageal malignancy

Question 6

Ix for achalasia

Answer 6

CXR

barium swallow

endoscopy - to exclude malignancy that can mimic achalasia, similar sx and XR - pseudoachalasia

manometry

Serology for antibodies against T. cruzi if Chagas’ disease is suggested by epidemiology and symptoms. Blood film might detect parasites

Question 7

CXR for achalasia

Answer 7

not necessary for dx

fluid level in dilated oesophagus (eg above the heart) - an air-fluid level in upper chest

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

absence of normal gastric air bubble

Question 8

barium swallow for achalasia

Answer 8

dilated oesophagus that tapers down towards the sphincter (beak shaped).

There is absence of peristalsis and sometimes asyncronous, purposeless contractions of the oesophageal body

Question 9

manometry for achalasia

Answer 9

confirms dx

elevated resting LOS pressure (>45mmHg)

incomplete LOS relaxation

absence of peristalsis in the distal (smooth muscle portion) of the oesophagus

Question 10

pharm Mx of achalasia

Answer 10

as bridge while wait definitive rx
* nifedipine or verapamil or isosorbide dinitrate

Question 11

definitive mx of achalasia

Answer 11

surgical:
* pneumatic dilation (balloon used to break down strictures in oesophagus)
* or cardiomyotomy (cut thick outer muscle between oesophagus and stomach)
* or peroral endoscopic myotomy

botilinum for poor surgical candidiates

Question 12

complications of achalasia

Answer 12

aspiration pneumonia
GORD
oesophageal Ca