Plummer-Vinson syndrome

Introduction

Plummer-Vinson syndrome is a rare disorder characterised by the classic triad of:

• Oesophageal webs.
• Iron-deficiency anaemia.
• Glossitis.

It is considered a pre-malignant condition, associated with an increased risk of squamous cell carcinoma of the pharynx and proximal oesophagus. The condition predominantly affects middle-aged women.

Peak Incidence

• Most commonly affects individuals between the ages of 40 and 70, with a female predominance.

Pathophysiology

• Iron deficiency leads to mucosal atrophy, impairing epithelial regeneration and resulting in the formation of oesophageal webs.
• The exact mechanism remains unclear, but chronic iron deficiency is thought to affect muscle and epithelial function in the upper GI tract.
• Mucosal changes may also predispose to dysplasia and carcinoma.

Symptoms

• Dysphagia – Typically painless and intermittent, initially for solids and potentially progressing to liquids.
• Glossitis – Red, smooth, painful tongue.
• Fatigue and weakness – Due to iron-deficiency anaemia.
• Pica – Craving for non-nutritive substances (e.g. ice, dirt), commonly seen in iron deficiency.

Signs

• Pallor – Due to anaemia.
• Smooth tongue – Indicative of glossitis.
• Koilonychia – Spoon-shaped nails, a classic sign of severe iron deficiency.
• Angular cheilitis – Cracking or inflammation at the corners of the mouth.

Diagnosis

Gold Standard

• Upper GI endoscopy – Reveals oesophageal webs, typically in the proximal oesophagus.

Additional Investigations

• Full blood count and iron studies – To confirm iron-deficiency anaemia (low Hb, low ferritin, low serum iron, raised TIBC).
• Barium swallow – May demonstrate narrowing or webs in the cervical oesophagus.
• Biopsy – Performed if suspicious lesions are noted, to exclude malignancy.

Complications

• Squamous cell carcinoma of the upper oesophagus or pharynx – Due to chronic mucosal irritation and atrophy.
• Recurrent dysphagia – Oesophageal webs may recur after treatment.
• Severe iron-deficiency anaemia – Can lead to:
  • Fatigue.
  • Poor wound healing.
  • Cardiac complications such as high-output heart failure.

Management

• Iron supplementation:

  • Oral iron is first-line if haemoglobin is >70 g/L and tolerated.
  • Intravenous (IV) iron if haemoglobin <70 g/L, if symptoms are severe, or if oral iron is ineffective or not tolerated.

• Endoscopic treatment:

  • Balloon dilatation of oesophageal webs to relieve dysphagia.

• Surveillance:

  • Patients should undergo regular monitoring for early signs of upper GI malignancy, particularly squamous cell carcinoma.