Achalasia Flashcards
What does Achalasia mean in Greek? What is the definition?
An oesophageal motility disorder of unknown aetiology with loss of oesophageal peristalsis and insufficient LOS relaxation when swallowing
“does not relax” in Greek
How common is achalasia?
- M=F
- Mean age at diagnosis 53yrs
- Incidence 2 in 100,000
Descirbe the presentation of achalasia.
- Dysphagia + posturing to aid swallowing (e.g. arching neck and shoulders or raising the arms or sitting up/standing)
- Regurgitation tastes bland/undigested
- Retrosternal cramps - precipitated by drinking fluids but releived by continued drinking
- Weight loss - gradual and mild
How does regurgitation in achalasia differ from regurgitation in GORD?
In achalasia bland, undigested food or saliva retained in the oesophagus regurgitates when patient is in the recumbent position.
This differs from regurgitation from gastro-oesophageal reflux, where the gastric regurgitate tastes sour.
What are the risk factors for achalasia?
- Allgrove syndrome - achalasia is a characteristic feature, along with alacrima and adrenal insufficiency.
- Herpes and measles viruses
- Autoimmune disease
- HLA II antigens
- Consanguineous parents
What is the aetiology of achalasia?
Unknown cause → inflammatory destruction of inhibitory nitrinergic neurons in the oesophageal myenteric (Auerbach) plexus → loss of peristalsis +incomplete LOS relaxation.
Causes may include infection (Chagas), autoimmunity, genetics (Allgrove syndrome).
What is the myenteric plexus of the oesophagus called?
Which types of nerones are lost in achalasia?
Auerbach plexus
There is selective loss of post-ganglionic INHIBITORY neurones containing NO and vasoactive intestinal peptide. STIMULATORY cholinergic neurones remain intact resulting in sphincter pressure and insufficient relaxation.
NO is the mediator causing relaxation
What are the clinical features of achalasia?
- Dysphagia to solids and liquids*
- Regurgitation
- Retrosternal pain
- Slowly progressive over months or years
Other:
- Posturing to aid swallowing e.g. arching neck and shoulders or sitting up
- Regurgitation
- Gradual weight loss
- Recurrent chest infections
- Globus sensation
- Hiccups
*NB: dysphagia to liquids at presentation suggests oesophageal motility disorder because obstructive disease would have to be very severe for this to occur.
What investigations would you do for achalasia?
Upper GI endoscopy - exclude malignancy
Barium swallow done with fluoroscopy - assesses rate of oesophageal emptying
Oesophageal manometry - shows aperistalsis of the oesophagus and failure of relaxation of the lower oesophageal sphincter
Other:
Chest X-ray - not diagnostic, looks for absence of gastric gas bubble
CT chest - exclude malignancy, looks for asymmetrical thickening
What do these investigations show in achalasia?
- endoscopy
- barium swallow
- oesophageal manometry
Upper GI endoscopy - excludes malignancy; may show mucosa obscured by retained saliva with frothy appearance. In advanced cases oesophagus may be dilated and tortuous and contain food debris (sigmoid oesophagus)
Barium swallow - loss of peristalsis and delayed emptying; dilated oesophagus that tapers smoothly to beak-like narrowing at the gastro-oesophageal junction
Oesophageal manometry - incomplete relaxation of lower oes sphincter with wet swallows and oesophageal aperistalsis; typically high resting lower oes pressure; swallows followed by simultaneous contraction waves, typically low but can be high-amplitude (vigorous achalasia)
You might also do videofluoroscopy.
What type of thickening of the oesophagus can occur in achalasia? What does asymmetrical thickening suggest?
Concentric thickening of oesophageal wall and dilation of oesophagus.
Asymmetric thickening suggests pseudoachalasia (achalasia due to causes other than primary denervation)
What must you exclude in achalasia-like symptoms?
Oesophageal cancer - new-onset dysphagia in patients over 55 is carcinoma until proven otherwise
What is the difference between oesophageal cancer and achalasia?
How do you manage achalasia?
- CCB (i.e. nifedipine, verapamil) or nitrates - lower resting LOS pressure; taken prior to meals
- Pneumatic dilation - balloons used to stretch oesophagus to tear its muscle fibres but high remission (85%). Must be good surgical candidates in case perforation occurs.
- Laparoscopic cardiomyotomy +/- anti-reflux fundoplication - may be most effective; involves surgical division of the LOS.
- New: Peroral endoscopic myotomy (POEM) - shown below
NB: slight increase in the incidence of SCC of the oesophagus in both treated and untreated cases.
Which of these are treatment options for achalasia?
- Conservative only – no immediate treatment necessary
- Oesophageal stent insertion
- Balloon dilatation
- Surgical cardiomyotomy
- Oesophagectomy
- Radiotherapy to the distal oesophagus
- Anti-reflux medication
This patient is symptomatic and needs treatment once diagnosis of achalasia has been confirmed. Oesophageal stents are reserved for inoperable malignant lesions. Oesophagectomy and radiotherapy to the distal oesophagus are used in the treatment of malignancy.
- Balloon dilatation
- Surgical cardiomyotomy
- Anti-reflux medication
- The oesophagus appears normal
- The oesophagus is dilated and contains food contents
- There is an irregular stricture of the distal oesophagus and malignancy is likely
- There is a tight, smooth narrowing of the distal oesophagus in keeping with achalasia
- Multiple filling defects are seen within the oesophagus suggestive of varices
- Gastro-oesophageal reflux is demonstrated and probable benign, peptic stricture is present in the distal oesophagus
- The oesophagus is dilated and contains food contents
- There is a tight, smooth narrowing of the distal oesophagus in keeping with achalasia
This examination is typical of achalasia with tight, smooth narrowing noted of the lower oesophagus, which is dilated proximally and full of food residue.
Typical history:
A 63-year-old female presents to surgical outpatients with a 6-month history of painless, slowly progressive dysphagia. She has experienced difficulties swallowing both solids and liquids. She has lost 3kg in weight and has experienced several chest infections over this period, treated by her GP with antibiotics. She is otherwise well and clinical examination is unremarkable.
- Oesophageal candidiasis
- Gastro-oesophageal reflux
- Achalasia of the oesophagus
- Carcinoma of the oesophagus
- Bulbar palsy
- Extrinsic oesophageal compression from mediastinal malignancy
Achalasia - also associated with cramping discomfort swallowing and some weight
Oesophageal or mediastinal malignancy less likely - due to length of history and occur on bg of reflux disease or start with solids then progress to liquids
Oesophageal candidiasis - in those who are frail and immunosuppressed, esp if on steroids. ahve odynophagia, dysphagia and oral plaques
Bulbar pasly - associated with aspiration and difficulties initiating swallowing. Neuro exam would be abnormal.
Is endoscopy indicated in achalasia?
Yes but only after barium swallow as an oesophagus full of food can make endoscospy difficult
- No significant abnormality
- Pneumomediastinum present
- Dilatation of oesophagus present, seen as gas-filled viscus in superior mediastinum
- Extensive mediastinal adenopathy noted
- Superior mediastinal mass noted – heart size normal
Dilatation of oesophagus present, seen as gas-filled viscus in superior mediastinum
Which tropical disease can produce similar symptoms to achalasia?
The tropical disease trypanosomiasis (Chagas’ disease) may cause similar clinical and imaging appearances.
Achalasia is associated with oesophageal malignancy and endoscopy is needed to exclude this. The cause of achalasia is unknown although it may be neurogenic.
What are the complications of achalasia?
- aspiration pneumonia
- GORD
- oesophageal carcinoma
What is the prognosis with achalasia?
No known cure
Treatment purely symptomatic
Pneumatic dilatation has remission rates of 85.7%
POEM has best outcome in early trials
