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Plummer-Vinson Syndrome in Children.
Mathilde Butori1, Sana Mahmoudi, Emmanuelle Dugelay-Ecochard
1*Departments of Pediatric Digestive and Respiratory Diseases †Radiology, Robert-Debré Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Journal of Pediatric Gastroenterology and Nutrition
|October 27, 2015
Summary
Plummer-Vinson syndrome (PVS) in children often presents with iron deficiency and dysphagia. Unlike adults, pediatric PVS typically requires endoscopic dilation for effective treatment.
Area of Science:
- Pediatric Gastroenterology
- Rare Diseases
- Esophageal Disorders
Background:
- Plummer-Vinson syndrome (PVS), or Kelly-Paterson syndrome, is a rare condition causing dysphagia in children.
- It is characterized by upper esophageal webs and associated iron deficiency.
Observation:
- A review of 17 pediatric PVS cases revealed universal iron-deficiency anemia.
- The male/female ratio was 1:1.9, with most cases occurring in adolescents.
- Unlike adults, iron supplementation alone was often insufficient to resolve dysphagia.
Findings:
- Endoscopic dilation was frequently required and usually successful in a single session for pediatric PVS.
- No cases of immunological disease were reported in children with PVS.
- One pediatric case later developed esophageal cancer in adulthood.
Implications:
- Swallow barium exploration is recommended for pediatric dysphagia to identify PVS.
- Endoscopic dilation is a key intervention for managing dysphagia in pediatric PVS.
- Early diagnosis and intervention are crucial for managing PVS and its potential long-term complications.
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