ACHALASIA

What is achalasia?

 Achalasia is a disorder of the gullet (oesophagus) in which the muscles fail to move food along properly and the valve at the lower end of the oesophagus fails to open, preventing food passing into the stomach normally. It is an uncommon condition that is diagnosed in 1 in 100,000 people annually, affects males and females equally and is commonest between 30 -60 years of age.

What causes achalasia?

Damage to or loss of the nerves in the wall of the gullet (oesophagus). The underlying cause for this remains largely unknown, however there may be some association with certain viral or autoimmune conditions (in which the immune system attacks healthy cells and tissues). In patients with achalasia there is thought be an increased likelihood of developing oesophageal cancer in the future.

What symptoms does it cause?

The commonest symptoms include difficulty swallowing (dysphagia), pain on swallowing and/or regurgitation of undigested food after meals. In some patients this can cause choking, coughing , heartburn or weight loss.

In some patients there may be no symptoms and it is incidentally detected during investigations for other diseases.

How is it diagnosed?

Three investigations are normally required:-

Upper GI endoscopy

A thin telescope is passed via the mouth using local anaesthetic and/or sedation to inspect the gullet (oesophagus), stomach and duodenum.

Barium swallow

A white dye containing barium is swallowed and its movement along the gullet towards the stomach is videoed using x-ray.

Oesophageal high resolution manometry

This is the gold standard investigation for achalasia. A thin tube is passed via the nose and a series of pressure measurements are made whilst the patient swallows small sips of water to assess the function of the gullet (oesophagus).

What are the treatment options?

Current recommendations are that graded pneumatic dilatation or surgical myotomy are recommended as initial treatment for those fit and willing to undergo these procedures. Injection of botulinum toxin is recommended for those patients not suitable for dilatation or surgery. Medication is recommended for those not suitable for dilatation or surgery and in whom botulinum toxin injection has failed.

Each of these treatments is outlined below:-

Pneumatic Dilatation (stretch muscle with balloon)

Under sedation or a full general anaesthetic, a balloon (3-4cm in diameter) is passed into the gullet (oesophagus) via the mouth. Under x-ray guidance it is inflated to stretch and disrupt the muscles of the valve at the lower end of the gullet. This may need to be repeated after one or more years. There is a risk of rupture of the gullet that may require surgery (around 1 in 50 patients).

Surgery (Heller’s cardiomyotomy & Dor fundoplication)

Before surgery

Once a decision is made to go ahead with surgery, you will need to attend the pre-assessment clinic, at which a number of routine checks and blood tests will be performed.

The operation

Surgery is performed with keyhole (laparoscopic) surgery. Five keyhole incisions are made in the upper part of the tummy (abdomen). The operation involves dividing the muscles (myotomy) of the valve at the lower end of the gullet that fails to relax. The top section of the stomach (fundus) is then stitched across this divided valve to prevent acid or food regurgitating back from the stomach into the gullet. This is known as a ‘fundoplication’ or ‘wrap’. The procedure normally takes 1-2 hours.

After surgery

Patients are normally ready to be discharged the following day after surgery. You will be discharged with painkillers and written post-operative instructions. All stitches are dissolvable. The waterproof dressings are suitable for the shower and can be removed after 7 days.

You are advised to drink fluids only for the initial 24 hours after surgery before commencing a mashed diet for the following 3 weeks. More solid foods can then be gradually reintroduced as tolerated.

Recovery can take around two weeks however you are advised to avoid heavy lifting for up to 6 weeks after surgery.

What are the risks of surgery?

• Shoulder tip pain – This is common after keyhole surgery and normally resolves within 24 hours. It is due to gas used to inflate the abdomen during surgery
• Bleeding – This can occur with any operation and on occasions may require a blood transfusion (unless the patient expresses a wish not to). It is more common in patients taking blood thinning medications (e.g. warfarin, clopidogrel, apixaban, rivaroxaban)
• Infection – This may affect the wounds, lungs or site of intravenous lines.
• Blood clots in the legs or lungs (deep vein thrombosis or pulmonary embolism) – Patients wear antiembolism stockings and may receive heparin injections to reduce this risk
• Damage to nearby structures – this is uncommon
• Bloating – this can affect upto 1 in 3 patients and patients may find it diffciult to belch
• Flatulence – due to bloating and inability to belch in some patiens this can lead to increase flatulence
• Diarrhoea – some patients develop diarrhoea following surgery, the cause for which is not always clear. This may require management with medication to control.
• Dysphagia – difficulty swallowing is relatively common in the initial post-operative period but normally improves by 6 weeks. In rare circumstances it may persist longer than 6 months and may require further intervention.
• Recurrence – around 20% of patients may require additional treatment for recurrent symptoms within 5 years

Medication

The muscle at the lower end of the gullet (oesophagus) can be relaxed using medications such as nitrates or calcium channel blocker drugs (e.g. nifedipine). These may provide temporary relief and are not effective in all aptients.

Botulinum toxin (Botox)

Botulinum toxin is injected painlessly into the muscle of the valve at the lower end of the gullet (oesophagus) via endoscopy. This can be effective for a few months to years.