What are the signs, symptoms, and workup for Plummer-Vinson syndrome?

Plummer-Vinson Syndrome: Signs, Symptoms, and Workup

Signs and Symptoms

Plummer-Vinson syndrome (PVS) is characterized by the classic triad of dysphagia, iron deficiency anemia, and upper esophageal web(s), primarily affecting middle-aged women. 1 The clinical presentation includes:

Primary Clinical Features

• Dysphagia:

  • Progressive difficulty swallowing, particularly with solid foods
  • Often described as food "getting stuck" in the upper throat
  • May be accompanied by occasional choking or aspiration episodes 2
  • Usually early-grade dysphagia that worsens over time 1

• Iron Deficiency Anemia:

  • Fatigue, weakness, and pallor
  • Shortness of breath
  • Conjunctival pallor
  • Tachycardia

• Esophageal Web:

  • Located in the post-cricoid region of the upper esophagus
  • May be single or multiple
  • Causes narrowing that can prevent passage of food or even endoscopes 3

Additional Clinical Manifestations

• Oropharyngeal Findings:

  • Glossitis (smooth, red, painful tongue)
  • Angular cheilitis (painful cracks at corners of mouth)
  • Atrophic oral mucosa

• Dermatological Signs:

  • Koilonychia (spoon-shaped nails)
  • Brittle nails
  • Pallor of skin and mucous membranes

• Other Symptoms:

  • Weight loss due to reduced food intake 3
  • Vomiting or regurgitation of undigested food
  • Malnutrition in advanced cases

Diagnostic Workup

Laboratory Tests

1. Complete Blood Count (CBC):

   • Microcytic hypochromic anemia
   • Low hemoglobin and hematocrit levels
   • Reduced mean corpuscular volume (MCV)

2. Iron Studies (essential for diagnosis):

   • Low serum iron
   • Low ferritin
   • Elevated total iron-binding capacity (TIBC)
   • Low transferrin saturation

3. Additional Blood Tests:

   • Vitamin B12 and folate levels (to rule out other causes of anemia)
   • Inflammatory markers (to exclude inflammatory causes of anemia)

Imaging Studies

1. Barium Swallow Esophagography:
   • Gold standard for visualizing esophageal webs
   • Shows characteristic web(s) in the post-cricoid region
   • May demonstrate narrowing of the upper esophageal lumen 2, 3

Endoscopic Evaluation

1. Upper Gastrointestinal Endoscopy:

   • Direct visualization of the esophageal web(s)
   • Assessment of web thickness and location
   • Evaluation of esophageal mucosa for other abnormalities
   • Opportunity for therapeutic intervention (dilation)
   • Critical for malignancy surveillance as PVS is associated with increased risk of upper GI malignancies (4.5% risk reported) 1

2. Videofluoroscopy:

   • Can be used to evaluate swallowing dynamics
   • Helps identify silent aspirations or other swallowing abnormalities

Additional Assessments

1. Nutritional Evaluation:

   • Assessment of nutritional status
   • Evaluation for malnutrition due to chronic dysphagia

2. Malignancy Screening:

   • Careful endoscopic examination of the hypopharynx, esophagus, and stomach
   • Biopsy of any suspicious lesions
   • Regular surveillance in long-term follow-up

Management Approach

The management of PVS focuses on two main aspects:

1. Iron Supplementation:

   • Oral or parenteral iron therapy based on severity
   • May lead to rapid improvement of dysphagia in some cases (improvement reported in as little as 2 weeks) 3
   • Continued until iron stores are replenished

2. Mechanical Dilation of Esophageal Webs:

   • Savary-Gilliard bougie dilation (successful in 90.7% of cases with a single session) 1
   • Endoscopic balloon dilation
   • May be required for immediate relief of significant dysphagia

Follow-up and Monitoring

• Regular monitoring of hemoglobin and iron studies
• Periodic endoscopic evaluation for recurrence of webs (9.3% recurrence rate reported) 1
• Long-term surveillance for development of upper GI malignancies

Special Considerations

• Although typically seen in middle-aged women, PVS can occur in other populations, including children 4
• The syndrome has become relatively rare in developed countries due to improved nutritional status 1
• Patients should be counseled about the importance of maintaining adequate iron intake and adherence to follow-up