What is Plummer-Vinson syndrome?

Plummer-Vinson Syndrome

Plummer-Vinson syndrome is a rare condition characterized by the triad of dysphagia, iron deficiency anemia, and esophageal webs in the post-cricoid region, which requires both iron supplementation and often endoscopic dilation for effective treatment.

Clinical Features

Plummer-Vinson syndrome (PVS) presents with several characteristic features:

• Classic triad:

  • Dysphagia (difficulty swallowing, particularly solids)
  • Iron deficiency anemia
  • Esophageal webs in the post-cricoid region

• Additional manifestations:

  • Glossitis (inflammation of the tongue)
  • Koilonychia (spoon-shaped nails)
  • Occasional choking and aspiration episodes during eating 1
  • Weight loss due to decreased appetite and difficulty eating 2

Epidemiology

• Most commonly affects middle-aged white women 3
• Becoming increasingly rare worldwide, likely due to improvements in nutrition and early detection of iron deficiency 3
• Can occur in various age groups:
  • Predominantly affects women in their 40s-70s
  • Rarely reported in the elderly population 2
  • Can occasionally affect children 4
  • Clear female predominance (M/F ratio of approximately 1:10) 5

Pathophysiology

The exact cause and pathogenesis remain unclear, but several factors have been implicated:

• Iron deficiency: Considered the primary etiological factor
• Nutritional deficiencies: Beyond just iron
• Genetic predisposition: May play a role in susceptibility
• Autoimmunity: Potentially involved in web formation 3

Diagnosis

Diagnosis of PVS requires:

1. Clinical evaluation:

   • History of dysphagia
   • Signs of iron deficiency anemia

2. Laboratory tests:

   • Complete blood count showing microcytic hypochromic anemia
   • Iron studies confirming iron deficiency

3. Imaging:

   • Barium swallow: Can reveal narrowing in the upper esophagus and characteristic webs
   • Best seen in lateral views 3

4. Endoscopy:

   • Visualization of esophageal webs
   • Webs are typically thin mucosal folds
   • Usually semilunar or crescentic
   • Most commonly located along the anterior esophageal wall
   • Can occasionally be concentric 3

Treatment

Management of PVS involves a two-pronged approach:

1. Iron supplementation:

   • Correction of iron deficiency is essential
   • May lead to resolution of dysphagia in some cases
   • Can potentially reverse mucosal changes 3

2. Mechanical dilation:

   • Often necessary despite iron replacement 2
   • Methods include:
     • Esophageal bougienage
     • Balloon dilation
   • Typically provides immediate relief of dysphagia 1, 5

Prognosis and Follow-up

• Recurrence: Dysphagia may recur in approximately 20% of cases, necessitating repeat dilation 5
• Malignancy risk: PVS is associated with an increased risk of hypopharyngeal and esophageal squamous cell carcinoma 3, 5
• Follow-up: Regular endoscopic surveillance is recommended due to the increased cancer risk

Special Considerations

• Elderly patients: Though rare in octogenarians, PVS should be considered in elderly patients with dysphagia and iron deficiency anemia 2
• Pediatric patients: Though uncommon, PVS can affect children and should be considered in the differential diagnosis of pediatric dysphagia with iron deficiency 4
• Cancer surveillance: Given the increased risk of malignancy, patients with PVS require long-term follow-up and monitoring

Treatment Algorithm

1. Confirm diagnosis:

   • Document the triad of dysphagia, iron deficiency anemia, and esophageal webs

2. Initial treatment:

   • Begin iron supplementation immediately
   • Monitor response to iron therapy

3. Endoscopic intervention:

   • Proceed with endoscopic dilation if:
     • Dysphagia persists despite iron supplementation
     • Severe dysphagia is present at diagnosis
     • Food impaction occurs

4. Follow-up:

   • Regular monitoring of iron status
   • Periodic endoscopic evaluation to assess for:
     • Recurrence of webs
     • Development of malignancy