How is Plummer-Vinson syndrome managed?

Management of Plummer-Vinson Syndrome

Iron supplementation combined with mechanical dilation of esophageal webs is the definitive treatment for Plummer-Vinson syndrome, with most patients experiencing complete resolution of symptoms after a single dilation session.

Clinical Presentation and Diagnosis

Plummer-Vinson syndrome (PVS) is characterized by a classic triad of:

• Dysphagia (usually painless, intermittent, and progressive over years)
• Iron deficiency anemia
• Esophageal web in the post-cricoid region

Additional clinical features include:

• Glossitis (inflammation of the tongue)
• Angular cheilitis (cracks at the corners of the mouth)
• Koilonychia (spoon-shaped nails)
• Symptoms of anemia (weakness, pallor, fatigue, tachycardia)

Diagnostic Workup

1. Complete blood count to confirm iron deficiency anemia
2. Iron studies (serum iron, ferritin, TIBC)
3. Barium swallow to identify esophageal webs (best seen in lateral views)
4. Upper endoscopy to visualize the webs and rule out malignancy

Treatment Algorithm

First-Line Treatment

1. Iron Supplementation

   • Oral iron therapy (ferrous sulfate 325 mg three times daily)
   • Continue until anemia resolves and iron stores are replenished
   • May take 3-6 months for complete correction

2. Mechanical Dilation

   • Savary-Gilliard bougie dilation or balloon dilation of esophageal webs
   • Single session is successful in approximately 90% of cases 1
   • Performed under endoscopic guidance

Treatment Response

• Most patients experience significant improvement in dysphagia after a single dilation session
• Iron supplementation helps resolve other symptoms related to iron deficiency
• Approximately 9.3% of patients may develop recurrence requiring repeated dilations 1

Follow-up and Monitoring

Short-term Follow-up

• Clinical evaluation 2-4 weeks after initiation of therapy
• Monitor hemoglobin, iron studies until normalized
• Assess dysphagia symptoms

Long-term Follow-up

• Regular surveillance endoscopy every 1-3 years
• Monitoring for recurrence of anemia with annual CBC and iron studies
• Prompt evaluation of any new or worsening dysphagia

Special Considerations

Risk of Malignancy

• PVS is associated with an increased risk of upper gastrointestinal malignancies
• Overall risk of developing upper GI malignancy is approximately 4.5% 1
• Squamous cell carcinoma of the hypopharynx and esophagus are most common
• Regular endoscopic surveillance is essential

Demographics

• While traditionally considered a disease of middle-aged women, recent evidence shows it can affect both genders
• In a recent study of 132 PVS cases, 85.6% were women with a mean age of 43.5 years 1
• Male cases are increasingly recognized 2

Prognosis

With appropriate treatment, the prognosis for PVS is excellent:

• Complete resolution of dysphagia in most patients after a single dilation session
• Correction of iron deficiency typically resolves other symptoms
• Long-term outcomes are good with appropriate surveillance for malignancy

Pitfalls to Avoid

1. Failing to provide adequate iron supplementation after mechanical dilation
2. Neglecting regular surveillance for upper GI malignancies
3. Missing the diagnosis in male patients (the condition is not exclusive to women)
4. Overlooking other causes of iron deficiency that may coexist with PVS
5. Delaying treatment, which may lead to increased risk of complications

Remember that PVS is rare but treatable, with excellent outcomes when properly managed with the combination of iron supplementation and mechanical dilation.