Plummer-Vinson Syndrome

Plummer-Vinson syndrome is a classical triad of dysphagia, iron-deficiency anemia and esophageal webs, Plummer-Vinson or Paterson-Kelly syndrome occurs. There is no exact data on the epidemiology of the condition; the syndrome is extremely rare. Most of the patients are middle-aged white women, but the syndrome has also been identified in children and adolescents in the fourth to seventh decades of life. Over the years, dysphagia is typically painless and sporadic or incremental, restricted to solids and often related to weight loss. The association with upper alimentary tract cancers is one of the most significant clinical aspects of Plummer-Vinson syndrome. Etiopathogenesis is unknown due to Plummer-Vinson syndrome.

OROFACIAL MANIFESTATIONS OF HEMATOLOGICAL DISORDERS

Various anemic disorders which shows different orofacial manifestations are iron deciency anemia,plummer Vinson syndrome,megaloblastic anemia,sickle cell anemia,thalassemia and aplastic anemia.These orofacial manifestations are facial pallor,atrophic glossitis,angular stomatitis,magenta tongue,midface growth etc. Spontaneous & post traumatic hemorrhage,prolong bleeding after tooth extraction are sign of different hemostatic disorder such as VonWillebrand disease or Hemophilia. Oral manifestations of most of the hematological diseases are nonspecic. In this article I have reviewed various literatures to identify orofacial manifestations of various hematological disorders.

Plummer-Vinson Syndrome in an African-American Woman

Plummer-Vinson syndrome (PVS) presents with the classic triad of iron-deficiency anemia, dysphagia, and esophageal webs. The mainstay treatment of PVS is iron supplementation and the dysphagia usually responds to iron supplementation before the hematologic abnormalities are corrected. This syndrome classically affects middle-aged Caucasian women and very few cases have been reported in African Americans. We present a rare case of PVS in an African-American woman. A 63-year-old woman presented with shortness of breath, dysphagia, and lightheadedness for several weeks. Chest X-ray was negative for any acute abnormalities. Initial hemoglobin was 7.0 g/dL, which improved to 7.5 g/dL after 1 unit of packed red blood cells. She had a mean corpuscular volume of 62 fL, a ferritin level of 6 ng/mL, and an iron level of 12 μg/dL. Fecal occult blood test was negative and barium swallow revealed a proximal esophageal web. Her dysphagia did not significantly improve despite intravenous iron supplementation and esophageal web dilation. Video-fluoroscopic swallow study revealed esophageal and pharyngeal phase dysphagia with food entrapment. She was discharged with plans to follow up with a primary care physician and repeat esophagogastroduodenoscopy in 1 year. This case report highlights a rare case of PVS in an African-American woman and emphasizes the importance of maintaining a comprehensive and broad differential diagnosis.

Síndrome de Plummer-Vinson en un paciente con enfermedad celíaca. Reporte de un caso

Plummer-Vinson syndrome is infrequent and is more common in middle-aged women. There are few reports of its association with celiac disease. Both entities can be linked to hypochromic anemia and esophageal carcinoma. We report the case of a 47-year-old female patient who, since the age of six, presented frequent episodes of iron deficiency anaemia, having required blood transfusions Eight months before the doctor’s appointment, when she swallowed a tablet, she presented a choking episode due to obstruction. Since then, she has been eating only soft foods. She had iron deficiency anemia, with low ferritin levels. A video swallow performed three months earlier showed a lack of fillong of 2 mm by 7 mm, which obliterated 75% of the esophageal lumen, and another similar image, 2 cm below the first one, arranged in the opposite direction, which obliterated the lumen by 50%. A video endoscopy was performed, which showed two membranes causing stenosis of the lumen in the cricopharyngeal region; they were removed. In the duodenal bulb and second portion, a flattened mucosa was observed, compatible with celiac disease. Biopsies were performed and the anatomopathological diagnosis was highly compatible with celiac disease, Marsh Oberhuber 3B. Conclusion. This observation demonstrates that celiac disease can coexist with Plummer Vinson syndrome. Therefore, it should be investigated in the context of this syndrome, with which it shares the relationship with iron deficiency anemia, and also taking into account the possibility of development of esophageal carcinoma in both entities.

ASSOCIATION OF IRON DEFICIENCY ANEMIAAND POST CRICOID WEBS – A PROSPECTIVE STUDY

Iron deciency anemia, post-cricoidal esophageal web and dysphagia are characteristics of Patterson Brown Kelly or Plummer-Vinson syndrome. Sideropenic dysphagia was coined by Waldenstorm. There has been a debate regarding the existence and frequency of esophageal webs in patients with iron deciency anemia.In, literature there is no study that correlates the severity of anemia and severity of the web or stricture.This is a prospective study of 50 patients over a period of 3 years. Through our study we would like to analyse if there is any corelationship especially in developing countries like ours were this study was conducted.