Abstract

Plummer-Vinson syndrome (PVS) is characterized by dysphagia, iron deficiency anemia and upper esophageal web. It is predominantly a disease of middle-aged females and very rarely seen in childhood. To date only nine cases of PVS have been reported in the English-language literature in children and adolescents. We report a case of this uncommon syndrome with an uncommon presentation. An 18-year-old male presented with complains of easy fatigability, palpitation and under-developed secondary sexual characters. On examination he was severely pale, with kylonichia, easy pluckable hair and delayed secondary sexual characters. Investigations for cause of anemia revealed microcytic hypochromic anemia in peripheral smear with Hb 5 g/dl, TC 5300 cells/mm3, Platelet 9 lakh/mm3, Thyroid profile was normal, serum ferritin levels were 13.8 ng/ml. Not able to find a definitive cause for iron deficiency, anemia history was revisited and the patient complained of mild dysphagia since age 9 years for solids greater than liquids. Upper gastrointestinal endoscopy was done which showed upper esophageal web with the endoscope not able to pass through the web. Blood transfusion and symptomatic treatment was given and patient symptoms improved. On follow-up, repeat blood picture showed hemoglobin of 12 g/dl and patient is doing well with iron supplements. Esophageal web (plummer vinson syndrome) is usually seen in women and is rarely documented in adolescent male. A careful history-taking and knowledge of occurrence of this rare disorder will benefit patients. Delayed secondary sexual characters may be due to chronic malnutrition due to the dysphagia caused by the web in the esophagus.

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