Plummer vinson syndrome is a rare disease characterized by difficulty swallowing, irondeficiency anemia, glossitis, cheilosis and esophageal webs. Plummervinson syndrome has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. This condition is also associated with development of esophageal cancer 2, 3, 4. Brownpatersonkelly syndrome is associated with anemia, esophageal web, and additional features of angular cheilitis and glossitis. Plummervinson syndrome classically presents as a triad of ironde. Plummervinson syndrome classically presents as a triad of irondeficiency anaemia, postcricoid dysphagia and upper esophageal webs. Plummervinson syndrome pvs is a rare condition characterized by the triad of iron. Patients must be screened regularly for any development of upper gastrointestinal malignancy. It is still common in developing and under developed countries. This clinical triad of ida, esophageal web and dysphagia has been named variously as plummervinson syndrome pvs. Plummervinson syndrome radiology reference article. Plummervinson syndrome is a rare cause of dysphagia in children because of an upper esophageal web. The main clinical features of plummervinson syndrome are postcricoid dysphagia, upper esophageal webs and iron deficiency anemia. The patient illustrates the typical clinical features of plummervinson syndrome pvs.
Plummervinson syndrome with simultaneous midesophageal growth. Most of the patients are white middleaged women, in the fourth to seventh decade of life 8, 11 but the syndrome has also been described in children and adolescents 1214. Waldenstorm introduced the term sideropenic dysphagia because of absence of stainable iron in the bone marrow. Plummervinson syndrome with simultaneous midesophageal growth mukesh nasa1, gaurav patil 2, zubin sharma, rajesh puri3 abstract patterson brown kelly or plummervinson syndrome is characterized by dysphagia, iron deficiency anemia and postcricoidal esophageal web. Download download esophageal webs plummer vinson syndrome pdf read online read online esophageal webs plummer vinson syndrome pdf 10 nov 2005 plummer vinson syndrome is characterized by cervical dysphagia, iron deficiency anemia, and upper esophageal web or webs. Plummervinson syndrome with proximal esophageal web. Plummervinson syndrome pvs is a rare disorder characterized by iron deficiency anemia, post cricoid dysphagia and esophageal webs. Jun 01, 2003 plummervinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Brunton and eban 1 reported on 4 men with an irondeficiency. Esophageal webs plummer vinson syndrome pdf telegraph. Exact data about epidemiology of the syndrome are not available. Plummer vinson syndrome symptoms, diagnosis, treatment, causes. Plummervinson syndrome diseases of the esophagus oxford. Plummervinson syndrome heralded by postcricoid carcinoma.
Most of the patients are white middleaged women, in the fourth to seventh decade of life 8, 11 but the syndrome has also been described in children and adolescents 12 14. Plummervinson paterson, brownkelly syndrome refers to the association of irondeficiency anaemia with dysphagia secondary to a postcricoid web. Plummervinson syndrome gastrointestinal medbullets step 23. Waldenstorm introduced the term sideropenic dysphagia because of absence. Esophageal web on xray discussion in the past plummer vinson syndrome was common in the scandinavian population, especially in rural areas of sweden but nowadays it is rare over there 3. A 58yearold woman with iron deficiency anemia presents to her primary care physician complaining of difficulty swallowing. Pdf plummervinson syndrome presenting as squamous cell. Summary plummervinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. The literature describes relations between these webs and plummervinson syndrome, bullous dermatologic disorders, inlet patch, graftversushost disease and celiac disease. Plummervinson syndrome pvs is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures. The literature describes relations between these webs and plummer vinson syndrome, bullous dermatologic disorders, inlet patch, graftversushost disease and celiac disease. Plummervinson syndrome orphanet journal of rare diseases.
Clinical description the main clinical features of plummervinson syndrome are postcricoid dysphagia, upper esophageal webs and iron deficiency anemia. In adults, iron supplementation is frequently sufficient to control dysphagia. Plummervinson syndrome, diseases of the esophagus 10. Plummer vinson syndrome is defined by the classic triad of dysphagia, irondeficiency anemia and esophageal webs.
There is evidence that the web is a direct consequence of chronic iron deficiency in some cases at least. A case of plummervinson syndrome showing rapid improvement. Plummer vinson syndrome is a condition that can occur in people with longterm chronic iron deficiency anemia. Most of the patients are white middleaged women, in the fourth to seventh decade of life 8,11 but the syndrome has also been described in children and adolescents 1214. In adults, plummer vinson syndrome may be associated with iron deficiency or diverse inflammatory disorders. Patients with plummervinson syndrome have iron deficiency that is associated with difficulty in swallowing due to the presence of an esophageal web. This disorder has been associated with autoimmune disorders like celiac disease, sjogrens disease and also malignancies. Celiac disease presenting as the patersonbrown kelly. A 77yearold woman was admitted to our hospital with complaints of dysphagia, vomiting, shortness of breath and weight loss for 1 month. Pvs is relatively common among middleaged female caucasians in northern countries whereas in japan, pvs seems to be rare. Plummervinson syndrome is one of the names given to the constellation of dysphagia, irondeficiency anemia, and esophageal webbing. Although it is a very rare condition, an increased risk of esophageal squamous cell carcinoma makes. Plummervinson syndrome pvs is a rare entity characterized by upper esophageal webs and iron deficiency anemia.
To determine the role of esophagoscopy as a safe and costeffective procedure which can be used. Plummervinson syndrome and esophageal cancer in an. To report the epidemiological, clinical, paraclinical and histological characteristics of patients with plummervinson syndrome associated with esophageal cancer. Esophagoscopy in the early detection and treatment of plummer. A case of plummervinson syndrome esophageal web dysphagia treated by dilatation with. In recent years, however, reports of esophageal webs in men have appeared in the literature. Rarely, webs develop in patients with untreated severe iron deficiency anemia. Plummervinson syndrome with simultaneous midesophageal. Plummervinson syndrome is rarely seen as a fullfledged syndrome. Plummervinson syndrome with esophageal web formation in. We report a case of pvs whose esophageal web was rapidly improved by iron therapy. Plummervinson syndrome associated with celiac disease and complicated by postcricoid carcinoma and carcinoma of the tongue.
Plummer vinson syndrome pvs is a rare pathology combining dysphagia, an iron deficiency anemia and a cervical esophageal web. Presence of glossitis, angular cheilitis, and koilonychia. Plummervinson syndrome pvs, also called patersonbrownkelly syndrome, is a rare entity whose main clinical features are upper esophageal webs, dysphagia and iron deficiency anemia. Plummer vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. Treatment of the iron deficiency frequently relieves dysphagia though some patients may need endoscopic web dilatation. The main culprit is thought to be iron deficiency anemia 2. Plummervinson syndrome and esophageal cancer in an endoscopy. Plummer vinson syndrome is a rare cause of dysphagia in children because of an upper esophageal web. The patient was a 56yearold woman who complained of restricted dietary habit because of an upper esophageal circumferential web associated with plummervinson syndrome. A case of plummervinson syndrome showing rapid improvement of dysphagia and esophageal web after two weeks of iron therapy. Plummer vinson syndrome also known as paterson brownkelly syndrome is a. The syndrome is associated with an increased risk of postcricoid carcinoma, which usually presents at an advanced stage.
Treatment consists of iron supplementation, possibly dilation, and surveillance. Esophageal web gastrointestinal disorders merck manuals. The pathogenesis of plummervinson syndrome is not clear 2. The esophageal webs in pvs are thin mucosal folds, which are best seen. As part of the workup for unexplained iron deficiency anemia, a duodenal biopsy was taken that revealed moderate flattening of villi with. Plummervinson 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. Identification and followup is relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx. Mayada issa, md, linda green, md, jalil ahari, md, and kalai ayyanar, md, of prince georges hospital center, cheverly, md, diagnosed plummervinson syndrome pvs based on the following findings. The patient was a 63yearold woman with a clinical history of iron deficiency anemia and glossitis in her 20s to 40s and who had experienced swallowing difficulties for the past 20 years. An esophageal web is a thin mucosal membrane that grows across the lumen and may cause dysphagia.
A case of plummervinson syndrome associated with tongue. The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as plummervinson syndrome pvs in the united states and patersonbrown kelly syndrome in the united kingdom. Plummer vinson syndrome diagnosis the diagnosis of pvs is made on the confirmation of irondeficiency anemia as well as one 1 or even more esophageal webs in a patient with postcricoid difficulty in swallowing. Esophagoscopy in the early detection and treatment of plummer vinson syndrome muhammad aslam chaudhry, ashar alamgir. A 40yearold man presented with insidious onset dysphagia for both solids and liquids for 4 years with recurrent oral ulcerations. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. Plummer vinson syndrome pvs, also known as patersonkelly syndrome or sideropenic dysphagia, is a rare pathology combining dysphagia, iron deficiency anemia and cervical esophageal web.
There were no muscular atrophy, keratinization, or inflammatory changes observed as are seen in the classic plummervinson syndrome. Plummer vinson syndrome is a rare association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Patients esophagograms revealed the pre sence of webs in part of the post cricoid region. Being common in northern countries in the early twentieth century, it has become very rare nowadays. Iron deficiency anemia is the prime etiological factor and other probable factors include malnutrition, genetic predisposition, or autoimmune processes characterized by three distinctive features.
We report a case of pvs whose esophageal web was rapidly. Department of ent, rawalpindi general hospital and rawalpindi medical college, rawalpindi. Esophageal webs are more common in white individuals and in women with a ratio 2. On examination he was anemic, and barium swallow demonstrated a web in the postcricoid region.
In the past, the plummervinson syndrome, comprising a web in the postcricoid region of the upper esophagus, dysphagia, koilonychia, and an irondeficiency anemia, has been described as occurring predominantly in women. When a web is the cause of the symptoms, surgical management is rarely necessary. It is one of the risk factors of the esophageal cancer. Most of the patients are white middleaged women, in the fourth to seventh decade of life 8, 11 but the syndrome has also been described in children and adolescents 12, 14. Plummer vinson syndrome is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. Both patients presented with dysphagia, anemia, sideropenia, glossitis and cheilitis. Even though the syndrome is very rare nowadays, its recognition is important because it identifies a group of patients at increased risk of squamous cell carcinoma of the pharynx and the esophagus. Esophagoscopy in the early detection and treatment of. Jun 01, 2003 summary plummervinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Revisiting plummer vinson syndrome annals of medical and. The esophageal webs, which can also occur in the absence of anemia and plummervinson syndrome, are characterized by one or more thin horizontal membranes consisting of squamous epithelium and submucosa. This type of web is probably of no clinical significance as it. Plummervinson syndrome, patersonbrownkelly syndrome, esophageal web, dysphagia, iron deficiency anemia.
Plummervinson syndrome classically presents as a triad of irondeficiency anemia, postcricoid dysphagia and upper esophageal webs2. Patients esophagograms revealed the pre sence of webs in part of the postcricoid region. The patersonbrown kelly plummer vinson syndrome comprises dysphagia due to a postcricoid esophageal web and anemia. People with this condition have problems swallowing due to small, thin growths of tissue that partially block the upper food pipe esophagus. Data regarding this condition is limited to mostly case reports and a few small cohort studies. Plummer vinson syndrome also known as paterson brownkelly syndrome is a syndrome associated with the triad of symptoms comprising microcytic hypochromic anemia, oesophageal strictures, and dysphagia. Plummer vinson syndrome has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. It is characterized by dysphagia, iron deficiency and the presence of superior esophageal web. A case report of dysphagia and esophageal web in an anorexic child. In the present report, we describe our experience of a case of plummer. The plummervinson syndrome is characterized by dysphagia, irondeficiency anemia, and esophageal webs. A rare case report on plummervinson syndrome with a. The postcricoid web, once considered pathognomonic of the syndrome, was shown epidemiologically to occur in only 15% of females with dysphagia.
Plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal webs. Plummer vinson syndrome pvs is a rare entity characterized by upper esophageal webs and iron deficiency anemia. Plummervinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. Esophageal webs appear to be common in a hospital population. Two cases of this syndrome are reported in middleaged women, which were treated over the last eight years. Plummervinson syndrome a rare presentation in a child. It is one of the syndromes associated with iron deficiency anemia. Esophageal webs that occur in association with iron deficiency anemia, glossitis, and koilonychias are referred to as plummervinson or pattersonkelly syndrome in the united states and united kingdom, respectively. Jun 07, 2014 plummer vinson syndrome pvs is a rare entity characterized by upper esophageal webs and iron deficiency anemia. Learning points plummervinson syndrome classically presents as a triad of ironde. Only seven cases of plummervinson syndrome in children and adolescents between the ages of 14 and 19 have been reported in the world literature.
Sep 15, 2006 the main clinical features of plummervinson syndrome are postcricoid dysphagia, upper esophageal webs and iron deficiency anemia. Pvs is commonly found in women of middle age especially in the fourth and fifth decade of life and is rarely reported in males. The plummer vinson syndrome is characterized by dysphagia, irondeficiency anemia, and esophageal webs. In patients with postcricoid webs and plummervinson syndrome pvs, histologic findings of hyperplasia degeneration of mucosa have been reported and attributed to iron deficiency. Mayada issa, md, linda green, md, jalil ahari, md, and kalai ayyanar, md, of prince georges hospital center, cheverly, md, diagnosed plummer vinson syndrome pvs based on the following findings. Feb 14, 2019 the association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as plummervinson syndrome pvs in the united states and patersonbrown kelly syndrome in the united kingdom. Plummer vinson syndrome pvs is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures. While exact data about the epidemiology is unknown, this syndrome has become extremely rare. However the surgical indication and technique for the web have been controversial. Plummervinson syndrome with hyperplastic gastric polyp.
The term sideropenic dysphagia has also been used, since the syndrome can occur with iron deficiency sideropenia, but it is not as. Both patients were treated with dilation therapy and iron supplementation. Full text iron deficiency anemia and plummervinson syndrome. Definition plummervinson syndrome pvs is a classical triad of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. See also overview of esophageal and swallowing disorders. This chapter presents a summary of a rare disease, plummervinson syndrome pvs. Symptoms resulting from anemia predominates the clinical picture, apart from the additional features such as glossitis, angular cheilitis, and dysphagia. The operation of upper esophageal web in plummervinson. Virtually every manifestation of the syndrome is also seen as an isolated problem. The reduction in the prevalence of plummer vinson syndrome has been hypothesized to be the result of improvements in nutritional. Pdf plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, iron deficiency anemia and esophageal webs. Both patients were treated with esophageal bougienage or balloon. Plummer vinson syndrome, also known as the patersonbrownkelly syndrome, predisposes to hypopharyngeal squamous cell carcinoma and consists of four features.
Plummervinson syndrome pvs is a rare disorder characterized by iron deficiency anemia, post cricoid. Atypical celiac disease with iga deficiency presenting as. Three to 15 per cent of the patients with plummervinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer 15,16. Endoscopy confirmed the presence of esophageal web post cricoids web 1718 cm and also a ruptured web figure 4b. Iron deficiency state has been hypothesized to play an etiological role. History of painless dysphagia and the finding of an esophageal web. Plummervinson syndrome pvs, also called patersonbrownkelly syndrome, is a rare medical syndrome generally affecting middleaged women. Upper endoscopy revealed thickening of the esophageal mucosa and a web, which was ruptured with the scope. Revisiting plummer vinson syndrome gude d, bansal dp1, malu a internal medicine, 1pulmonology and critical care, medwin hospital, nampally, hyderabad, andhra pradesh, india abstract plummer vinson syndrome is a rare association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Plummervinson syndrome with hyperplastic gastric polyp kelly schrapp md, marvin crawford md, punita kaveti md, avantika chenna md department of internal medicine, morehouse school of medicine, atlanta, ga 303101495. Deficiency of irondependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs.
Three to 15 per cent of the patients with plummer vinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer 15,16. Plummer vinson syndrome is a condition where iron deficiency is associated with difficulty swallowing due to the presence of an esophageal web. Iron repletion often improves the dysphagia, although some patients require esophageal dilatation or bougienage. In addition, the syndrome is well known to be a high risk factor for postcricoid and cervical esophagus cancers, and a relationship with oral cancer has also been suggested.
Plummer vinson syndrome is a rare affection which affects mainly white women. The association between webs, iron and postcricoid. Dysphagiaplummervinson syndromeiron therapyesophageal. In adults, plummervinson syndrome may be associated with iron deficiency or diverse inflammatory disorders. Given that it can involve iron deficiency sideropenia without anemia, it has been also called sideropenic dysphagia.
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