PC1199: Plummer-vinson Syndrome as a Unique Trigger for Disordered Eating

Introduction: Plummer-Vinson syndrome (PVS) is a rare syndrome characterized by the classic triad of iron deficiency anemia (IDA), dysphagia and esophageal webs. Here, we describe a case of an East Asian female adolescent presenting with severe IDA and underweight status later diagnosed with PVS thought to have triggered restrictive eating.

Case Description: A 13-year-old perimenstrual female of East Asian descent was admitted to the pediatric hospital service due to severe anemia (hemoglobin 4 g/dl) during a routine laboratory screening at her wellness visit. Consultation with pediatric hematology was obtained for concerns of microcytic anemia, thrombocytopenia, and leukocytosis of unknown etiology. Bone marrow biopsy was negative for leukemia and patient received blood transfusion. Further history revealed chronically low weight and disordered eating (BMI 14.8 kg/m2). Adolescent medicine consultation conveyed concerns for a restrictive type of eating disorder. Patient was placed on the inpatient feeding protocol for closer monitoring of refeeding syndrome with increased nutritional intake. Additional history of pill dysphagia prompted pediatric gastroenterology consultation. Per their recommendations, work-up revealed negative celiac screen, inflammatory markers and fecal occult blood however a mildly elevated calprotectin was noted. With some weight gain and improved anemia, patient was discharged home hemodynamically stable with planned outpatient iron transfusions and esophagogastroduodenoscopy (EGD). A week later, EGD revealed an upper esophageal stricture and biopsies that were suggestive of mild reflux. A follow-up esophagogram confirmed a diagnosis of an esophageal web. With the classic triad of symptoms, a diagnosis of Plummer-Vinson syndrome was made. The patient underwent multiple balloon dilations leading to improvement of symptoms including weight recovery.

Discussion: PVS is a rare condition particularly in developed countries due to a low occurrence of severe IDA. Usual presentation is seen in middle-aged white women with rare cases seen in the pediatric population. Globally, the incidence is decreasing likely due to improved nutrition and iron fortified diet. Temporal bone marrow suppression from malnutrition is expected, however severe anemia is not common even in restrictive types of eating disorders. Iron deficiency is believed to be strongly associated with some hypotheses linking depletion of iron dependent oxidative enzymes causing esophageal mucosal changes. Constellation of signs and symptoms prompted the multidisciplinary approach to clinical evaluation and management.

Conclusion: This is a unique presentation of Plummer Vinson Syndrome in an adolescent female with severe anemia said to be multifactorial and esophageal webs with dysphagia resulting in avoidant/restrictive intake of food. Correction of anemia leads to resolution of dysphagia however younger patients tend to require esophageal dilation.