Achalasia🎥 {Free Video}

Achalasia

Introduction

Achalasia is a primary oesophageal motility disorder caused by loss of inhibitory ganglion cells in Auerbach’s (myenteric) plexus. The result is absent peristalsis and failure of the lower oesophageal sphincter (LOS) to relax on swallowing.

Epidemiology

  • Incidence peaks between 30 and 60 years of age.
  • Affects both sexes equally and occurs worldwide.

Pathophysiology

  • Degeneration of nitrergic inhibitory neurons prevents LOS relaxation.
  • Oesophageal body becomes aperistaltic; food stasis leads to progressive dilatation (“mega-oesophagus”).
  • Chicago Classification (v4.0) sub-types:
    • Type I (classic) – minimal pressurisation.
    • Type II – panesophageal pressurisation after each swallow.
    • Type III (spastic) – premature or spastic contractions.
  • Understanding the sub-type guides choice between pneumatic dilatation and myotomy.

Symptoms

  • Progressive dysphagia to both solids and liquids.
  • Nocturnal or post-prandial regurgitation of undigested food.
  • Cough, aspiration or recurrent chest infections.
  • Retrosternal chest pain or heartburn-like discomfort.
  • Unintentional weight loss and malnutrition in advanced disease.

Signs

  • Usually unremarkable; may see halitosis or signs of aspiration pneumonia.

Investigations

Test Typical Findings
High-resolution manometry (gold standard) Aperistalsis plus elevated integrated relaxation pressure; classify Type I–III.
Barium swallow ‘Bird-beak’ tapering, dilated fluid-filled oesophagus, possible “corkscrew” in spastic cases.
Upper GI endoscopy Excludes malignancy (“pseudo-achalasia”) and candida; retained food often seen.
Chest X-ray / CT Widened, air-fluid oesophageal column; rules out extrinsic compression.

Complications

  • Aspiration pneumonia and chronic lung disease.
  • Oesophageal ulceration or candida oesophagitis.
  • Progressive mega-oesophagus and risk of perforation.
  • Small but definite increase in squamous cell carcinoma risk after > 10 years.

Management

First-line options (curative intent)

  • Pneumatic (balloon) dilatation – endoscopic graded dilatation; effective for Type II, may need repeats.
  • Laparoscopic (Heller) myotomy with fundoplication – durable symptom relief; preferred for young patients or Type III disease.
  • Per-oral endoscopic myotomy (POEM) – minimally invasive endoscopic alternative, especially for spastic Type III.

Second-line / palliative

  • Botulinum-toxin injection into LOS – temporary (< 6 months); useful in frail or high-risk surgical patients.
  • Pharmacotherapy (limited role): sublingual nifedipine, isosorbide dinitrate or sildenafil taken before meals.

Supportive measures

  • Small, frequent meals; avoid late-night eating.
  • Sleep with head of bed elevated to reduce nocturnal regurgitation.
  • Proton pump inhibitor after myotomy or POEM to prevent reflux.

Follow-up

  • Repeat manometry or timed barium swallow if symptoms recur.

  • Consider 3- to 5-yearly surveillance endoscopy where long-standing disease or mega-oesophagus is present (practice varies).

FAQ from our users

What causes achalasia?
  • The exact aetiology is largely still unknown.
Why does the dysphagia often get worst over time?
  • There is a degenerative loss of ganglia in the smooth muscle layer affecting the auerbach’s plexus. The degenerative nature of this is why the onset of dysphagia worsens with time.
What is a barium swallow?

The patient swallows barium sulfate, which when exposed to x-rays can be used to visualise the GI tract.

How do botox injections work?

An endoscope is used to access the LOS & botulinum toxin is injected. This relaxes the sphincter & relieves the pressure.

How does pneumatic dilatation work?

An endoscope passes a balloon into the oesophagus which helps to stretch the LOS. The patient is sedated during this. This does however come at a small risk of tearing the oesophagus.

Common pitfalls in a clinical setting

Common pitfalls in a clinical setting
  • The level of dysphagia amongst patients can vary widely according to the stage of disease.
  • Do not forget to exclude oesophageal cancer in patients presenting with symptoms of achalasia.
  • Please note that that there is no definitive cure, but treatment aims to relieve symptoms.
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