P0485 - Plummer-Vinson or Paterson-Kelly Syndrome: Two Refractory Cases Requiring Pneumatic Dilation

Sean-Patrick Prince, MD, MPH¹, Qitan Huang, DO², Denisse Camille Dayto, MD², Andrew Sephien, MD², Lakshmipathi Reddi, MD, FACG^2
1University of Miami/Holy Cross Hospital, Pompano Beach, FL; ²HCA Healthcare/USF Morsani GME Consortium: HCA Florida Citrus Hospital, Inverness, FL

Introduction:
Plummer–Vinson syndrome (PVS), also known as Paterson-Kelly syndrome, is a rare clinical condition characterized by a triad of dysphagia, iron deficiency anemia and a post-cricoid esophageal web. We present two cases of dysphagia due to post-cricoid esophageal webs compatible with the diagnosis of PVS.

Case Description/Methods:
A 40-year-old caucasian female with a past medical history of iron deficiency anemia presented for elective esophagogastroduodenoscopy (EGD) due to progressive dysphagia and cachexia. Lab findings noted a hemoglobin of 9.5 g/dl and MCV 76 fL. EGD attempted one month prior was unsuccessful due to a cervical esophageal stricture.

On this presentation, EGD revealed multiple impassable post-cricoid esophageal membranous strictures; wire-guided dilation was performed; post-dilation evaluation revealed multiple disrupted cervical esophageal membranous strictures. Esophageal and duodenal biopsies for non-specific inflammation were sent for pathology and immunoperoxidase failed to reveal IgA or IgG positive plasma cells.
After multiple dilations, patient’s dysphagia and weight improved. Iron deficiency was treated parenterally and she was maintained on famotidine 40 mg twice daily. Pathology reports later confirmed active esophagitis without evidence of eosinophilic esophagitis or duodenal disease. Patient has remained asymptomatic on follow-up visits.

A 78-year-old caucasian male with past medical history of rheumatoid arthritis, iron deficiency anemia and previous history of esophageal web presented for EGD due to progressive dysphagia and cachexia. Lab values were consistent with iron deficiency and EGD revealed recurrent cervical esophageal webs, multiple disrupted esophageal strictures and a non-obstructing Schatzki’s ring at the gastroesophageal junction. Patient had multiple dilations of the recurrent esophageal web without complication. After an unremarkable post-op period, the patient was maintained on famotidine 20 mg twice daily and oral iron supplementation.

Other causes of iron deficiency anemia as well as iron malabsorption conditions were ruled out for both patients.

Discussion:
PVS remains a rare condition and diagnosis of exclusion. Other causes of iron deficiency must be ruled out including celiac disease, IBD and others. Some cases are refractory to iron supplementation such as the cases discussed above. In lieu of so much unknowns, further studies are warranted to create formalized guidelines for treatment, monitoring and surveillance.

Disclosures:


Sean-Patrick Prince, MD, MPH¹, Qitan Huang, DO², Denisse Camille Dayto, MD², Andrew Sephien, MD², Lakshmipathi Reddi, MD, FACG². P0485 - Plummer-Vinson or Paterson-Kelly Syndrome: Two Refractory Cases Requiring Pneumatic Dilation, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.