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Plummer-Vinson Syndrome

Plummer Vinson Syndrome

Plummer-Vinson syndrome is a condition that affects mostly middle-aged caucasian women and is defined by the triad of iron deficiency anemia, upper esophageal webs and postcricoid dysphagia. Given that it can involve iron deficiency (sideropenia) without anemia, it has been also called sideropenic dysphagia.


Presentation

Dysphagia is the most prominent symptom in patients with PVS and is usually painless, intermittent to progressive, limited to solid food and sometimes associated with weight loss [18]. The presence of esophageal webs can also manifest with severe choking spells or aspiration. Iron deficiency is exhibited with symptoms typical for anemia, such as weakness, fatigue, dyspnea and tachycardia. Other manifestations of iron deficiency anemia are angular cheilitis, glossitis, koilonychia (spooned nails) and pallor [3] [19]. Patients with PVS can also present very uncommonly with splenomegaly, loss of teeth and an enlarged, nodular thyroid [3].

Palpable Spleen
  • On examination the child had severe pallor, koilonychias, angular cheilitis and a just palpable spleen. Child’s weight was 36kgs (centile) and height 148cms (centile). BMI was 16.8.[crimsonpublishers.com]
Anemia
  • Iron therapy is the cornerstone for the treatment of iron deficiency anemia and is necessary for the resolution of anemia symptoms that accompany the disorder.[symptoma.com]
  • Crohn's disease is usually accompanied by malnutrition and iron deficiency anemia; however, no case of concomitant Crohn's disease and Plummer-Vinson syndrome with aggravated malnutrition and anemia has been previously reported.[ncbi.nlm.nih.gov]
Fatigue
  • METHODS: Five males presented to us with dysphagia and fatigue of various durations, from April to August, 2012.[ncbi.nlm.nih.gov]
  • Iron deficiency is exhibited with symptoms typical for anemia, such as weakness, fatigue, dyspnea and tachycardia. Other manifestations of iron deficiency anemia are angular cheilitis, glossitis, koilonychia (spooned nails) and pallor.[symptoma.com]
  • Symptoms resulting from anemia such as pallor, fatigue and weakness may dominate the clinical picture. In our case pallor, fatigue and dysphagia were the presenting symptoms past 2 years.[crimsonpublishers.com]
  • This is a case of a middle aged lady who presented with fatigue and was diagnosed to have iron deficiency anaemia. She did not have any complaints of dysphagia. On endoscopic evaluation she was found to have post cricoid web and haemorrhoids.[nepjol.info]
Nail Abnormality
  • Symptoms Difficulty swallowing Weakness Signs and tests You may develop skin and nail abnormalities thatyour doctor can see during an exam. Upper GI series or upper endoscopy may show the abnormal tissue in the food pipe.[indiatoday.in]
Pharyngitis
  • Deficiency of iron-dependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs.[ncbi.nlm.nih.gov]
  • Patients need to be on annual surveillance EGD due to risk for esophageal or pharyngeal malignancy.[insights.ovid.com]
  • Key words: Anemia, Dysphagia, Esophageal and pharyngeal carcinoma Advertisement American Journal of Preventive Medicine and Public Health SUBMIT YOUR ARTICLE NOW[scopemed.org]
  • Recognition of Plummer-Vinson syndrome is important because it is a risk factor for developing esophageal or pharyngeal squamous cell carcinoma.[freemedicalvideos.com]
Orthopnea
  • Patient denied any cough, chest pain, light-headedness, palpitation, orthopnea or PND. She reported difficulty swallowing solid food for 3 months. She had no difficulty swallowing liquids and she would either blend her food or eat soft food.[article.sapub.org]
Odynophagia
  • She complained of dysphagia and odynophagia and had cheilitis, glossitis, and hypochromic anemia. An esophageal motility study showed low amplitude of contraction and high intrabolus pressure in the esophageal body.[ncbi.nlm.nih.gov]
  • Some may also have weight loss, odynophagia, retrosternal pain,dyspnea and hoarseness.The physical examination is usually unremarkable.[explainmedicine.com]
  • . • Odynophagia. • Tachycardia. • Weight loss. • Underlying loose of connective tissue. • Atrophic glossitis.[drthindhomeopathy.com]
  • Symptoms include: Dysphagia (difficulty swallowing) Pain Weakness Odynophagia (painful swallowing) Atrophic glossitis Angular stomatitis Serial contrasted gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal the web in the esophagus[en.wikipedia.org]
Intermittent Dysphagia
  • The clinical features include intermittent dysphagia for solid foods (rarely or never for liquid). Symptoms and signs of anaemia (weakness, pallor, fatigue, tachycardia, glossitis, koilonychia and cheilitis) may predominate.[apamedcentral.org]
  • Presentation [ 3, 4 ] The presentation is usually with painless, intermittent dysphagia. It tends to be with solid foods but, if untreated, may progress to soft foods and even liquids.[patient.info]
Deglutition Disorder
  • Key words: Plummer-Vinson syndrome; Iron deficiency; Anemia; Deglutition disorders; Men's health RESUMO Contexto A síndrome de Plummer-Vinson é caracterizada por uma tríade composta por disfagia, anemia por deficiência de ferro e anel esofágico.[scielo.br]
Rectal Pain
Premature Loss of Teeth
  • IDA may be associated with deficiencies of other nutrients, in particular, vitamins, which could manifest as soreness of mouth, glossitis, angular cheilitis, atrophic glossitis ( Figure 1 ), premature loss of teeth, koilonychia (spoon-shaped finger nails[dovepress.com]
Koilonychia
  • Plummer Vinson syndrome is a constellation of postcricoid esophageal webs, iron deficiency anemia, dysphagia and koilonychia.[ncbi.nlm.nih.gov]
  • Links: dysphagia iron deficiency anaemia (IDA) glossitis koilonychia angular stomatitis barium swallow[gpnotebook.co.uk]
  • Vinson, American physician, 1890-1959 a rare disorder associated with severe and chronic iron deficiency anemia, characterized by glossitis, koilonychia, and dysphagia caused by esophageal webs at the level of the cricoid cartilage.[medical-dictionary.thefreedictionary.com]

Workup

Workup in patients suspected to have PVS starts with diagnostic tests to confirm iron-deficiency anemia. These include a complete blood count (CBC), a peripheral blood smear and iron studies, such as serum iron, ferritin levels, total iron binding capacity (TIBC) and saturation percentage. Other tests are added, if needed.

Esophageal webs are usually detected with esophagograms or videofluoroscopy [3] [20]. A regular barium swallow can detect esophageal webs and is usually best performed with a lateral view. It would show thin projections of the anterior esophageal wall and the postcricoid. Sometimes these webs can be multiple. In case the barium swallow is negative or equivocal and the physician still maintains a high index of suspicion for PVS, a video swallow study is usually the next adequate diagnostic test. The video swallow test has the ability to distinguish true webs from false webs, which are usually caused by mucosal foldings and submucosal phenomena. Esophagogastroduodenoscopy is another potential diagnostic modality. It needs to be done very carefully, so as not to rupture the web. Otherwise, esophageal webs would go undetected. The web usually consists of a smooth, thin, gray mucosal membrane covered by squamous epithelium. They are present on the anterior esophageal walls of the proximal esophagus and extend laterally, but not to the posterior wall and are mostly crescent-shaped, although some are concentric [20].

Diagnosis is established by the confirmation of iron deficiency anemia in addition to the presence of dysphagia and one or multiple esophageal webs.  

Esophageal Motility Disorder
  • We report the case of a 41-year-old woman with Plummer-Vinson syndrome and an esophageal motility disorder. She complained of dysphagia and odynophagia and had cheilitis, glossitis, and hypochromic anemia.[ncbi.nlm.nih.gov]
  • motility disorder, motor disorders of the esophagus 食道炎 esophagitis アルカリ性逆流性食道炎 alkaline reflux esophagitis 逆流性食道炎 reflux esophagitis 腐食性食道炎 corrossive esophagitis 食道癌 carcinoma of the esophagus 食道胃接合部癌 cancer of esophagogastric junction 進行食道癌 advanced[jsge.or.jp]
  • Esophageal motility disorders such as scleroderma, achalasia cardia, diffuse esophageal spasm and nutcracker esophagus can also present with dysphagia, and the web may be an incidental finding.[dovepress.com]
Enlargement of the Spleen
  • 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.[ncbi.nlm.nih.gov]
  • 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.[orpha.net]
Microcytic Anemia
  • Blood tests demonstrate a hypochromic microcytic anemia that is consistent with an iron-deficiency anemia. Biopsy of involved mucosa typically reveals epithelial atrophy (shrinking) and varying amounts of submucosal chronic inflammation.[en.wikipedia.org]
Gastric Lesion
  • Four explanations arose to account for the pathogenesis: iron deficiency; nutritional deficits; autoimmunity; and gastric lesion.[ncbi.nlm.nih.gov]

Treatment

Treatment of PVS mostly occurs in the outpatient department. Iron therapy is the cornerstone for the treatment of iron deficiency anemia and is necessary for the resolution of anemia symptoms that accompany the disorder. Continuous iron supplementation beyond what is needed for the treatment of anemia is usually not required and has not proven effective [21].

Dysphagia can sometimes resolve with iron supplementation alone if not particularly severe, but obstructive dysphagia resulting from more advanced webs is better managed with mechanical dilation [22]. The passing of an endoscope can sometimes be sufficient in disrupting the web structure. Mechanical dilation is performed with the passage of a bougie (for example a Savary dilator) with a diameter of up to 17 mm after the endoscopic placement of a guidewire [23]. It is generally more appropriate to insert a single large dilator than to repeat the process more than once. Endoscopy is rarely performed under fluoroscopic guidance, unless the mechanical obstruction is so severe that it prevents the passage of the endoscope. Balloon dilation is not adequate, given the proximal location of the web, although reports of its use have been described when performed under fluoroscopic guidance [24] [25] [26]. Laser therapy and needle-knife electroincision are other potential therapeutic modalities, but their use is rarely required [27] [28] [23]. Surgery is reserved only for recalcitrant webs or those associated with Zenker diverticulum.

A change in dietary habits is important in the treatment of the disease. Solid food, especially meat, should be cut in small pieces and patients need to be advised to chew thoroughly and eat slowly.

Finally, because of the significant association of PVS with upper gastrointestinal carcinomas, patients need to be followed closely. A yearly surveillance with upper gastrointestinal endoscopy is recommended, although the effectiveness of this measure has not been confirmed [22].

Prognosis

Prognosis of PVS is excellent when it is not complicated by hypopharyngeal or esophageal carcinoma [14] [15]. The disease responds very well to a combination of iron supplementation, dietary changes and esophageal dilation. Morbidity is mostly related to dietary changes and repeated esophageal dilation in patients with recurrent dysphagia, with an increased risk of esophageal perforation. Mortality figures are uncertain, given the rarity of the disease, although it is thought that PVS has very low mortality.

Around 3 to 15% of patients with PVS ultimately develop cancers of the upper gastrointestinal tract  (pharynx and esophagus) [12] [16]. These relatively high rates necessitate frequent follow-up and supervision of patients with PVS. Recent data demonstrate a decrease in the rate of cancer of the hypopharynx among women, probably because of a decrease in the prevalence of PVS and better follow-up [17]. Prognosis for the disease worsens significantly when cancer is diagnosed.

Etiology

The cause of Plummer-Vinson syndrome remains unknown, although several factors have been proposed, including iron and other nutritional deficiencies, a genetic predisposition and autoimmune mechanisms [7]. The most prominent theory attributes the syndrome to iron deficiency, given its involvement in the classic defining triad of the syndrome and the positive response of dysphagia and esophageal webs to iron supplementation.

Epidemiology

The prevalence of PVS was considerably more elevated in the first half of the 20th century and then displayed a steep decline with the concomitant improvements in nutritional status, particularly with the addition of iron supplements to flour [8]. Nowadays, it is mostly found in northern European countries among middle-aged white women in the age range of 40 to 70 years, although cases involving children and adolescents have also been reported [9] [10]. It is worth noting that despite widespread malnutrition and iron deficiency in African populations, the disease remains very rare amongst this population[8].

Sex distribution
Age distribution

Pathophysiology

The pathophysiology of PVS is unknown, although several theories have been proposed. They mostly involve iron deficiency, malnutrition, genetic predisposition and autoimmunity [7] [11].

The most prominent theory identifies iron deficiency as the causative mechanism and postulates that such a deficiency leads to a depletion of critical oxidative enzymes, ultimately resulting in atrophy of the esophageal mucosa, formation of webs and disturbances in muscle fiber involved in swallowing. The validity of this theory remains unaccepted and is based mostly on studies showing an improvement of dysphagia with iron supplementation, in addition to sparse and anecdotal reports of a positive response of impaired esophageal motility in patients with PVS to iron therapy [6] [12]. Nonetheless, other scientific work has cast doubt on this mechanism. Large population-based studies failed to find any association between dysphagia and anemia or sideropenia, while other work demonstrated that patients with esophageal webs are just as likely to be iron deficient as control subjects and that esophageal webs can be commonly found in patients without iron deficiency or dysphagia [6].

On the other hand, the association of PVS with several autoimmune conditions such as rheumatoid arthritis, pernicious anemia, celiac disease and thyroiditis has fueled speculation that PVS is an autoimmune disorder [7] [13]. One study reported that patients with PVS had significantly elevated levels of thyroid cytoplasmic autoimmune antibodies, when compared to control subjects with iron deficiency [7]. Overall, however, the autoimmune theory is not widely accepted.

Prevention

Iron supplementation, either through dietary changes or the administration of ferrous preparations, can prevent the development of the disease in patients with or without iron deficiency anemia. It can also resolve the condition if malignancy has not yet developed.

Summary

Plummer-Vinson syndrome (PVS) is a rare disorder characterized by a triad of dysphagia, esophageal webs and iron deficiency anemia [1] [2] [3] [4] [5]. In the United States, it is also known as Paterson-Brown-Kelly syndrome. It is also associated with a significantly increased risk of squamous cell carcinoma of the pharynx and the esophagus. PVS is mostly prevalent among Caucasian middle-aged women with a history of chronic iron deficiency anemia [6]. The cause of PVS has not been established, but it is believed that a deficiency in iron leads to depletion of important oxidative enzymes and the development of myasthenic phenomena that are displayed by muscles that mediate swallowing, as well as mucosal and submucosal atrophic alterations. Patients with PVS usually present with dysphagia and symptoms of anemia, such as fatigue, weakness and dyspnea. Diagnosis is established by the presence of dysphagia and independent confirmation of iron deficiency anemia and esophageal webs. The latter is most commonly identified with barium swallow, X-Rays or videofluoroscopy. PVS usually responds to iron therapy although esophageal obstruction necessitates endoscopy and dilation. Close follow-up and periodic upper gastrointestinal endoscopy are recommended to monitor any development of cancer. PVS can be prevented in patients with chronic iron deficiency through iron supplementation and prognosis is excellent if the disease is not complicated by the development of esophageal or pharyngeal cancer.

Patient Information

Plummer-Vinson syndrome (PVS) is a rare disorder that manifests mostly among middle-aged white women suffering from chronic iron deficiency anemia. Patients with this condition typically develop webs of thin tissue that can block the upper third of the alimentary tract known as the esophagus, leading to problems in swallowing food.

The exact cause of this disorder is unknown but scientists suspect that a deficiency in iron (as well as other nutrients) and genetic causes play a major role. Iron is an important component of many biochemical reactions in our body and its deficiency is thought to impair the muscles involved in normal swallowing, as well as the tissue that lines the esophagus (esophageal mucosa). 

Patients with PVS most commonly complain of a difficulty in swallowing (dysphagia), usually regarding solid food, that is not associated with pain, as well as symptoms of anemia such as fatigue, weakness, pallor and shortness of breath. In order to diagnose the disease, the doctor will perform a number of blood tests to confirm iron deficiency anemia. Esophageal webs are best identified with a barium swallow X-Ray or a video swallow test that enables the doctor to visualize any webs of tissue limiting the patency of the esophagus.

PVS is most commonly treated with iron supplementation, although severe obstruction of the esophagus requires mechanical intervention. This is done by mechanically rupturing the esophageal webs using a dilator or an endoscope. Patients with PVS usually respond very well to treatment. Nonetheless, the major risk of PVS is the development of cancer of the esophagus and throat, which illustrates the importance of regular follow up with a doctor if diagnosed. General recommendations suggest the performance of a yearly endoscopy of the upper gastrointestinal tract to detect the development of any possible cancer. The development of PVS in patients who are iron deficient can be prevented with iron supplementation.

References

Article

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Last updated: 2019-07-11 20:46