Interesting case Chest 14

Salient findings:

• Significantly distended oesophagus which is fluid and debris filled back to its origin.

• No gross oesophageal wall thickening.

• No significant abnormality identified at the gastro-oesophageal junction.

• Diffuse bilateral central ground glass opacification within the lungs

• Patchy tree-in-bud changes

• No dense consolidation

• Chest X ray confirms the dilated oesophagus. There is also subtle increased central hazy nodular opacification which represents the inflammation of the alveoli seen in pneumonitis.

Principle Diagnosis:

Achalasia with complication of acute aspiration pneumonitis

Learning points:

• Achalasia v Pseudoachalasia – Primary or secondary?

  • Achalasia is a functional abnormality where the Lower Oesophageal Sphincter fails to relax due to degeneration or loss of inhibitory neurons within the myenteric plexus.

  • Pseudoachalasia results in lower oesophageal obstruction as a result of a non-functional organic aetiology.

• Potential causes of Pseudoachalasia:

  • Oesophageal malignancy

  • Gastric adenocarcinoma at the gastroesophageal junction

  • Scleroderma

  • Metastatic disease to the distal oesophagus, most likely culprits being lymphoma, breast or lung ca.

  • Amyloidosis infiltration of the brainstem

  • Sarcoidosis

  • Brainstem infarcts resulting in neuropathy and denervation

  • Peptic strictures and inflammation at the cardia/GOJ

  • Post- fundoplication

• How is primary achalasia diagnosed?

  • Manometry which assesses the pressure across the lower oesophageal sphincter as well as the absence of normal peristalsis

  • Secondary confirmatory tests such as barium swallow and endoscopy are often adjuncts to the investigation.

• Complications of achalasia:

  • Oesophageal carcinoma

    - Mid to upper oesophagus

    - More likely to be a squamous cell carcinoma

    - Result of food and debris stasis and resulting chronic inflammation resulting in statis oesophagitis.

  • Aspiration pneumonitis

  • Aspiration pneumonia with infection and consolidation

  • Candida oesophagitis

  • Oesophageal perforation

  • Gastroesophageal reflux disease

• Usefulness of radiology?

  • Adjunctive tests such as barium swallow in the initial diagnosis

    - ‘Bird’s beak’ oesophagus

  • Assessing for underlying secondary causes of Pseudoachalasia

  • Assessment of complications