Plummer-Vinson Syndrome and Postcricoid Carcinoma: Late Complications of Unrecognized Celiac Disease

ArticleinThe American Journal of Gastroenterology 94(7):1991 · August 1999with 6 Reads
Abstract
The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
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  • Article
    Full-text available
    Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.
  • Article
    Full-text available
    Plummer-Vinson syndrome (PVS) is characterized by the presence of postcricoid dysphagia, iron deficiency anemia, and upper esophageal web. This syndrome is now a rare condition because of the improvement in nutritional status and increased awareness regarding iron deficiency anemia and the early diagnosis and easy treatment of this anemia or underlying causes. In this presentation, we report two middle-aged female patients with PVS and briefly review the literature.
  • Article
    Esophageal webs and rings have controversial definitions and poorly understood etiologies. Upper esophageal lesions are less common than lesions at the gastroesophageal junction. Most commonly, these lesions are Schatzki's rings and likely form as a result of gastroesophageal reflux. Luminal diameter correlates with the likelihood of developing symptoms, which in most cases include dysphagia to solid food. Food impaction can result in lesions regardless of luminal restriction. Treatment for upper and lower lesions often involves dilation. Recurrence is common, especially in lower lesions.
  • Article
    Contrary to early beliefs, celiac disease (CD) is relatively common; however, it still remains underdiagnosed since most cases are atypical, with few or no gastrointestinal symptoms and predominance of extraintestinal manifestations. As a consequence, the diagnosis of the disorder often requires a multidisciplinary approach. Also some oral ailments have been described in celiac patients. In this study, we review the papers that have reported oral manifestations in subjects with CD. A comprehensive literature search was conducted in Medline and Embase databases using appropriate key words. Additional papers were selected by cross-referencing from the retrieved articles. Dental enamel defects are the oral lesions most closely related to CD. There are conflicting data on the association between CD and recurrent aphthous stomatitis. A correlation of CD with atrophic glossitis has been reported, although robust evidence in support of it is lacking. Patients with CD have caries indexes seemingly lower than healthy individuals, but they may experience delay in tooth eruption. Occurrence of other oral mucosal lesions in CD subjects is likely occasional. Patients with systematic dental enamel defects should be screened for CD even in the absence of gastrointestinal symptoms. CD screening tests for patients with oral aphthae or idiopathic atrophic glossitis should be selectively considered during a medical evaluation that focuses on all aspects of the patient's status.
  • Article
    Plummer-Vinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Two cases of this syndrome are reported in middle-aged women, which were treated over the last eight years. Both patients presented with dysphagia, anemia, sideropenia, glossitis and cheilitis. Radiological examination of the pharynx showed the presence of webs in both cases. The patients were treated with iron supplementation, which resulted in elimination of the symptoms. Both patients remain in good general condition and without any dysphagic complaints, 5 and 8 years after the diagnosis, respectively.
  • Article
    Plummer-Vinson syndrome is known as the association of postcricoid dysphagia, upper esophageal web, and iron deficiency anemia. Although correction of iron deficiency may result in resolution of dysphagia and sometimes disappearance of the webs, dilation therapy is usually necessary to remove webs and relieve dysphagia. We report two cases of Plummer-Vinson syndrome. Both patients presented with significant and longstanding dysphagia, sideropenia, glossitis and koilonychia. Our two patients had occasional choking and aspiration episodes at eating and endoscope did not pass through at the level of the upper esophagus. Patients' esophagograms revealed the presence of webs in part of the post-cricoid region. Both patients were treated with esophageal bougienage or balloon dilation, and iron supplementation. The patients were examined periodically for two years after the initial treatment and found to be in good general condition.
  • Article
    The dramatic improvement in knowledge concerning celiac disease (CD) has disclosed the pattern of the associated clinical manifestations and the often atypical or silent presentation of this disease, which makes clinical diagnosis difficult. Also oral manifestations, mostly recurrent apthous stomatitis (RAS) and dental enamel hypoplasia, are atypical signs of CD. Our opinion about the possibility of performing mass-screening to reveal atypical or silent CD is in agreement whit who is asserting that a sistematical case-finding is, at present, the most suitable epidemiological approach. So, we think that patients affected by RAS, or dental enamel hypoplasia, should be considered, even in the absence of any gastrointestinal symptom, at-risk subjects, and should therefore undergo diagnostic procedure for CD.
  • Article
    The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
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  • Article
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    Full-text available
    The objective of the study was to determine the salivary Ferritin levels in children with iron deficiency anemia and its reliability as a predictive marker of the disease and whether these levels could give a new hypothetical insight on the oral epithelial changes seen in patients with iron deficiency anemia. Study The study comprised of 60 children of which the study group comprised of 30 individuals; aged 8-14 years with iron deficiency anemia. Venous blood was collected and hematological examination was performed to determine the hemoglobin and serum ferritin levels to confirm the diagnosis. Saliva was then collected from 30 children with iron deficiency anemia and an equal number of controls. The ferritin levels in saliva were then analyzed using solid phase ELISA. The mean value of salivary ferritin in iron deficient cases was 153.24 +/- 46.58 microg/dl and the mean ferritin levels in control subject were 93.87 +/- 30.15 microg/dl. Thus the salivary ferritin was found to be significantly higher in iron deficient subjects compared to the controls. The result of our research affirms the fact that the expression of ferritin in saliva of iron deficiency anemia may be due to the enzymatic functions in the saliva and the endocytosis of ferritin which can possibly elevate the salivary ferritin. The diminished level of cytochrome oxidase, together with its relatively high Ferritin content, depicts the association of oral epithelial changes and ferritin occurring in iron deficiency anemia.
  • Article
    Plummer-Vinson syndrome (PVS) comprises a triad of iron deficiency anemia, dysphagia and esophageal webs. Its occurrence in association with celiac disease which presents as iron deficiency anemia, has rarely been reported [1, 2]. We describe a 59-year-old female with PVS whose iron-deficiency anemia was due to celiac disease.
  • Article
    Aim The aim of the study was to describe the characteristic features (epidemiological, clinical, paraclinical, therapeutic and evolutive) of the Plummer-Vinson syndrome. Methods A retrospective and descriptive study was conducted from May 1997 to May 2010 including every patient hospitalized in the medical departments of the “hôpital Principal de Dakar” for dysphagia, iron deficiency anemia and upper esophageal web or stricture. Results Fifty patients were included. The mean age was 35.6 years and the sex-ratio 0.14. Laboratory tests showed an iron deficiency anemia in all patients with mean values of haemoglobin of 7.36 g/dl, serum iron 0.26 mg/l and of ferritin 6.65 μg/l. Esophagoscopy showed an upper esophageal web or stricture without web. The cause of iron deficiency was found in 16 patients (32%) who presented uterin myomatose with menorrhage (n = 5), increased menstrual blood loss (n = 10) and grade III esophageal varices (n = 1). The treatment included iron supplementation in all patients. An accidental web disruption occurred in 6 cases (12%) during the first endoscopic session. Savary Gilliard dilatation was performed in 36 patients (72%). In short term followup (0–6 months), the outcome was favourable with an improvement of dysphagia and complete resolution of biological abnormalities. Conclusion The Plummer-Vinson syndrome affects mainly reproductive women. The incidence is still rare in a context where iron deficiency is very common. The treatment includes iron supplementation and mechanical dilatation. The outcome is always favourable in short term follow-up.
  • Article
    Background/objectives: Plummer-Vinson syndrome also known as sideropenic dysphagia is a disease characterized by chroniciron-deficiency anemia, dysphagia and esophageal web. It commonly affects white female in the 4th to 7th decade. Most of the dysphagiaand iron deficiency can be treated by iron supplementation and rarely web dilatation is needed.Setting: Department of ENT, Head and Neck Surgery and Anesthesia, KVG Medical College, Sullia, Karnataka, India.Case report: A 36-year-old female with dysphagia of 10 months and iron-deficiency anemia with a small upper esophageal web seen onupper GI endoscopy and barium swallow.Intervention: Conservative line of management with blood transfusion and dilatation of the web with cuffed endotracheal tube.Results: A good symptomatic and radiological improvement was seen after blood transfusion and web dilatation with cuffed endotrachealtube.Conclusion: Cuffed endotracheal tube dilatation is a better way of managing upper esophageal webs with minimal complications undergeneral anesthesia.
  • There is paucity of prospective data on association between cervical esophageal webs and celiac disease. It is not clear whether all patients with cervical esophageal web need screening for celiac disease. Hence, the present study was carried out to determine the association of cervical esophageal web with celiac disease. This prospective study included consecutive patients with symptomatic cervical esophageal web diagnosed over a period of 4.5 years. Tissue transglutaminase antibody was measured in serum of each patient. Patients with elevated tissue transglutaminase antibody titer were subjected to esophagogastroduodenoscopy and biopsies were obtained from the descending duodenum to look for histological changes of celiac disease. Esophageal web was treated with bougie dilatation. Celiac disease was diagnosed on the basis of elevated tissue transglutaminase antibody and suggestive duodenal histology. Twenty one patients were diagnosed to have cervical esophageal web. Eighteen (85.7%) had evidence of iron deficiency. Five (23.8%) patients with cervical esophageal web fulfilled criteria for diagnosis of celiac disease. All five had evidence of iron deficiency. None of these patients gave a history of chronic diarrhea. All patients were treated with bougie dilatation. Patients with celiac disease were advised of a gluten-free diet. All five celiac disease patients are free of dysphagia without recurrence after a mean follow up of 10 months (range: 3 to 16 months). There is association between cervical esophageal web and celiac disease. All adult patients with cervical esophageal web and iron deficiency need screening for celiac disease even in the absence of chronic diarrhea.
  • Section of Gastroenterology, Penn State Geisinger Medical Center
    • M Komar
    Reprint requests and correspondence: M. Komar, M.D., F.A.C.G., Section of Gastroenterology, Penn State Geisinger Medical Center, Danville, PA 17822-2111. Received Mar. 15, 1999; accepted Mar. 18, 1999.
  • Article
    The association of iron deficiency anaemia, post-cricoid webs and carcinoma is controversial. The main findings in a recent study which re-examined this problem are presented here. It was shown that the majority of patients with a post-cricoid web have evidence of iron deficiency but that only about 10% of patients with iron deficiency develop post-cricoid webs. Factors which might be important in the pathogenesis of a post-cricoid web have been looked for and a few significant points noted. The main findings were an increased frequency of angular stomatitis and edentia, thyroid disease and thyroid cytoplasmic antibodies in patients with webs compared with patients with iron deficiency anaemia without a web.Follow-up studies in patients with a post-cricoid web or Paterson-Kelly syndrome show an increased frequency of post-cricoid carcinoma ranging from 4 to 16% in different series and the association is even more striking in patients presenting with post-cricoid carcinoma. It is concluded that the available evidence confirms the association between iron deficiency, postcricoid webs and carcinoma but the mechanism of the production of these changes is not yet understood.
  • Article
    The Plummer-Vinson syndrome is characterized by dysphagia, iron-deficiency anemia, and esophageal webs. The webs are best diagnosed by cineradiography. Iron repletion often improves the dysphagia, although some patients require esophageal dilatation or bougienage. The syndrome is associated with an increased incidence of postcricoid carcinoma, and surveillance endoscopy is recommended.
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  • Article
    We describe two patients with Paterson-Brown Kelly (Plummer-Vinson) syndrome whose iron deficiency anemia was due to celiac disease. They presented with dysphagia 13 and 9 yr, respectively, before celiac disease was diagnosed. Neither had gastrointestinal symptoms suggestive of malabsorption. Celiac disease is a recognized cause of chronic iron deficiency and should be considered as an etiological factor for sideropenic dysphagia.