Achalasia

Question 1

What is it?

Answer 1

Achalasia is a primary motility disorder of the oesophagus, characterised by a failure of relaxation of the lower oesophageal sphincter and the absence of peristalsis along the oesophageal body.

Question 2

Clinical features?

Answer 2

Patients with achalasia will classically present with progressive dysphagia when ingesting both solids and liquids, as well as regurgitation of food. The symptom severity frequently varies day-to-day.

Other symptoms include respiratory complications (either a nocturnal cough or aspiration), chest pain, dyspepsia, and weight loss. Symptoms are often non-specific, therefore there is often a substantial delay to diagnosis.

On examination, there are rarely any obvious signs of note, except for visible weight loss in longstanding cases, secondary to a reduced oral intake.

Question 3

Investigation?

Answer 3

In any patient presenting with dysphagia, an upper GI endoscopy (OGD) is essential in order to exclude a cancer as the cause of symptoms; in severe disease, endoscopy might show a dilated oesophagus with retained food and increased resistance at the GOJ.

The gold standard in the diagnosis* of motility disorders is oesophageal manometry (Fig 3); this procedure involves a pressure sensitive probe inserted into the oesophagus (approximately 5cm proximal to the LOS), which measures the pressure of the sphincter and the surrounding muscle. In achalasia, the three key features on manometry are:

Absence of oesophageal peristalsis
Failure of relaxation of the lower oesophageal sphincter
High resting lower oesophageal sphincter tone

Question 4

How do we classify achalasia?

Answer 4

Type I = classical achalasia, no evidence of pressurisation
Type II = achalasia with compression or compartmentalisation in the distal oesophagus >30mmHg
Type III = two or more spastic contractions

Question 5

How do we manage achalasia?

Answer 5

The management of achalasia can be divided into medical and surgical management, all aiming to reduce the LOS pressure and relieve the outflow obstruction.

All patients should be given advice including sleeping with multiple pillows to minimise regurgitation, eating slowly and chewing food thoroughly, and taking plenty of fluids with meals.

Pharmacological options include the use of calcium channel blockers (typically sublingual Nifedipine) to inhibit LOS muscle contraction, however their benefit is often short lived. Botox injections into the lower oesophageal sphincter via endoscopy can be trialled and are often effective, however their effect only lasts for a few months at most.

Surgical Interventions

The main surgical treatments for achalasia are:

Laparoscopic Heller Myotomy* – the division of the specific fibres of the lower oesophageal sphincter which fail to relax (Fig. 4), often seen as the
A long-term improvement in swallowing is seen in ~85% of patients, with lower side-effect profile compared to endoscopic treatment
Per Oral Endoscopic Myotomy (POEM) – a cardiomyotomy at the LOS is performed from the inside of the oesophageal lumen, through a submucosal tunnel
Early clinical trials have suggested POEM provides a very good clinical response, especially in patients with a type III pattern of disease, although rates of post-operative GORD are high
Endoscopic Balloon Dilatation – insertion of a balloon into the lower oesophageal sphincter, which is dilated to stretch the muscle fibres
It provides a good response in ~75% of patients but carries the risks of perforation (~5%) and the need for further intervention, therefore best reserved for well patients with type II pattern by HRM
Those with end-stage refractory achalasia may eventually require an oesophagectomy.