How is Plummer-Vinson Syndrome (PVS) managed?

Management of Plummer-Vinson Syndrome

Primary Treatment Strategy

The cornerstone of Plummer-Vinson syndrome management is iron supplementation combined with endoscopic dilation of esophageal webs when dysphagia persists despite correction of iron deficiency. 1, 2

Iron Replacement Therapy

• Iron supplementation is the first-line treatment for all patients with PVS, addressing the underlying iron deficiency anemia that characterizes this condition 1, 3
• Correction of iron deficiency may arrest and reverse mucosal changes, potentially reducing the risk of malignant transformation 3
• Iron therapy alone can sometimes result in resolution of dysphagia and disappearance of webs, though this is less common in practice 2

Endoscopic Dilation

• Savary-Gilliard bougie dilation or balloon dilation is typically necessary to relieve dysphagia, as iron supplementation alone often fails to resolve symptoms 1, 2
• A single session of dilation is successful in approximately 90% of patients in relieving dysphagia 1
• Only 9.3% of patients develop recurrent dysphagia requiring repeated dilations 1
• In pediatric patients, endoscopic dilation is more frequently necessary compared to adults, as dysphagia often resists iron supplementation alone 4

Treatment Algorithm

1. Initiate iron supplementation immediately upon diagnosis to correct anemia and address nutritional deficiency 1, 3
2. Perform endoscopic dilation if dysphagia persists after initiating iron therapy or if symptoms are severe 1, 2
3. Monitor for recurrence of dysphagia, which occurs in <10% of cases and requires repeat dilation 1

Surveillance for Malignancy

• PVS carries a 4.5% overall risk of developing upper gastrointestinal malignancy, including squamous cell carcinoma of the esophagus and pharynx 1, 3, 5
• Periodic endoscopic surveillance is warranted given the increased malignancy risk, though specific intervals are not well-defined in the literature 3, 5
• Correction of iron deficiency may reduce malignancy risk by reversing mucosal changes 3

Special Populations

Pediatric Patients

• Endoscopic dilation is more frequently required in children compared to adults, as dysphagia typically resists iron supplementation alone 4
• A single dilation is usually sufficient in pediatric cases 4
• Long-term follow-up is important, as one pediatric case developed esophageal cancer in adulthood 4

Clinical Pitfalls

• Do not delay endoscopic dilation waiting for iron supplementation to resolve dysphagia, as most patients require mechanical intervention 1, 2
• Lateral views during barium swallow are essential for detecting the thin, semilunar or crescentic webs typically located along the anterior esophageal wall 3
• Maintain high clinical suspicion for malignancy during follow-up, particularly in patients with persistent or recurrent symptoms 1, 3, 5