Plummer-Vinson syndrome: Clinical, therapeutic and evolutionary aspects: a Moroccan case series

Abstract

Plummer Vinson syndrome (PVS) is a rare entity characterized mainly by upper dysphagia, irondeficiency anemia, and the presence of an esophageal ring on endoscopic examination. We conducted a retrospective study over a period of 18 years describing the epidemiological and clinical profile, endoscopic management and the medium and long-term evolution. A total of 96 patients were enrolled, with a median age of 41,5 years and a clear female predominance (19/77). dysphagia was present in almost all our patients (97,9%), and an anemic syndrome in half the cases (52%). Female gender was significantly associated with PVS occurrence (p=0.002). The frequency of the disease was significantly higher after the age of 30 years with a peak in frequency in the 4th decade (p<0.034). Upper endoscopy found a membranous ring located in the cervical area in all cases. 1 patient had, in addition to the plummer vinson ring, a lower esophageal membranous called Schatzki ring with a small sliding hiatal hernia. PVS was associated with other autoimmune diseases mostly represented by celiac disease and autoimmune thyroiditis. Treatment was based on iron supplementation followed by endoscopic dilatation with Savary dilators in 91,6% (n=88) and balloons for 8,3% of cases (n=8). long-term outcome was favorable in 79.1% of patients with no clinical recurrence. 20,8% patients relapsed after a median of 29,2 months [2-72 months] requiring a second dilation. PVS was complicated by squamous cell carcinoma in 5,2% (n=5).