Plummer-Vinson Syndrome

Snapshot

  • A 50-year-old woman presents to her primary care physician with problems with eating. She reports that over the past few months, she has experienced some difficulty swallowing food. She drinks liquids and soups normally, but reports difficulty swallowing particularly solid foods. On exam, she is noted to have atrophic glossitis and fissures in the bilateral oral commissure. An upper gastrointestinal barium swallow study reveals narrowing, and esophagogastroduodenoscopy shows thin web-like tissue growths in the upper esophagus.

Introduction

  • Clinical definition
    • characterized by atrophic glossitis, upper esophageal webs (eccentric and often anterior), and iron deficiency anemia
  • Epidemiology
    • most commonly observed in postmenopausal women
    • rare
  • Pathogenesis
    • unknown
  • Associated conditions
  • autoimmune disease

Presentation

  • Symptoms
    • dysphagia to solid foods
    • cough
    • choking
    • fatigue
    • nail changes
    • dizziness
  • Physical exam
    • atrophic glossitis 
    • angular cheilitis 
    • pallor
  • koilonychia 

Imaging

  • Upper gastrointestinal barium swallow radiography 
    • may show a thin projection post-cricoid
    • may show multiple esophageal webs
  • Video fluoroscopic swallowing exam
  • can help differentiate a true web from false webs such as mucosal folding

Studies

  • Serum labs
    • complete blood count
      • hypochromic and microcytic anemia
    • peripheral blood smear
      • hypochromic and microcytic anemia 
    • iron studies
      • iron deficiency
  • invasive studies
    • esophagogastroduodenoscopy
      • thin web-like tissue growth in the upper esophagus 
  • normal squamous mucosa and submucosa, possible atrophy and chronic inflammation on histopathological exam

Differential

  • Schatzki ring
    • distinguishing factor
      • distal esophageal web
  • Post-cricoid cancer
    • distinguishing factor
      • malignancy on histopathological exam
  • Achalasia 
    • distinguishing factor
  • dysphagia at the lower esophageal sphincter

Treatment

  • Medical
    • iron supplementation
    • treat the underlying cause of iron deficiency
  • Surgical
    • esophageal dilatation, most commonly via balloon method
      • can be performed concurrently with esophagogastroduodenoscopy
  • not required if iron supplementation improves dysphagia

Complications

  • Squamous cell carcinoma of the esophagus, oral cavity, or hypopharynx
  • Aspiration