1754 Plummer-Vinson Syndrome: A Time for a Redefinition and New Perspective

Abstract

INTRODUCTION: Plummer-Vinson syndrome (PVS), or Kelly-Patterson syndrome, is a rare condition defined by the classic triad of dysphagia, iron-deficiency anemia, and esophageal webs, and dominated by Caucasian females. The pathophysiology remains unknown although iron deficiency is one theory as well as part of an autoimmune spectrum. We present two patients seen recently meeting criteria for PVS in whom new observations were made. CASE DESCRIPTION/METHODS: A 46-year-old Hispanic female with history of hypothyroidism presented with a Hgb of 3 and a ferritin of 16. She reported 2 weeks of fatigue, dyspnea, and constipation without overt bleeding, preceded by several months of dysphagia to solids. She was severely hypothyroid and placed on IV levothyroxine. Barium swallow suggested an esophageal web in the upper esophagus. At esophagogastroduodenoscopy (EGD) both adult and neonatal scopes could not be passed through a tight stenosis at the cricopharyngeus (Figure 1). This was dilated with Savary dilators to 8 mm. She was discharged home on iron supplements and follow up EGD with dilations scheduled. A 55-year-old Hispanic female with history of lupus, a positive ANA, Raynaud's, prior partial thyroidectomy, and chronic anemia was referred to GI clinic for evaluation of chronic dysphagia which had required multiple dilations at an outside facility over the past 18 months without improvement. She had a hgb of 6.7 with iron deficiency without any overt GI bleeding. EGD was performed and showed two benign appearing areas of intrinsic stenosis 15 and 20 cm from the incisors that were smooth and not ulcerated and could not be passed with the adult or pediatric endoscopes (Figure 2). Savary dilators were used to dilate to 11 mm diameter. Patient was placed on iron replacement and a fo llow up EGD was scheduled. DISCUSSION: Why PVS is only in females with accompanying iron deficiency anemia and a personal/family history of autoimmune phenomena and now is decreasing in recognition are not clear. The resolution of dysphagia with iron supplementation, could also be explained by the accompanying esophageal dilations restoring oral intake and hence improving iron absorption. Our cases highlight that PVS: 1) is not a true “esophageal web” but rather a tight fibrotic stricture at the level of the upper esophageal sphincter requiring aggressive dilation approach 2) occurs also in Hispanic females presenting with iron deficiency, dysphagia, and an autoimmune picture.