Esophageal cancer histology squamous cell adenocarcinoma anatomic location proximal and midportion distal third and gastroesophageal junction incidence decreasing increasing african american caucasian caucasian african american risk factors tobacco and alcohol tobacco hpv obesity ingestion of corrosives gerd plummervinson syndrome barrett. Plummer vinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal webs. Pvs is commonly found in women of middle age especially in the fourth and fifth decade of life and is rarely reported in males. In case of an associated squamous cell carcinoma of the hypopharynx or upper esophagus the prognosis worsens dramatically. Plummervinson syndrome is defined by the classic triad of dysphagia, irondeficiency anemia and esophageal webs. Plummervinson or patersonkelly syndrome presents as a classical. A case of plummervinson syndrome showing rapid improvement. Plummervinson syndrome, if left untreated, carries an increased risk of developing squamous cell carcinoma of the upper alimentary tract. The main clinical features of plummervinson syndrome are postcricoid dysphagia, upper esophageal webs and iron deficiency anemia. Plummer vinson or patersonkelly syndrome presents as a classical triad of dysphagia.
Plummervinson syndrome is characterized by a triad of dysphagia, iron. One of the main characteristics of pvs is the significant dysphagia swallowing disorder that occurs due to the presence of esophageal webs. Plummervinson syndrome pvs, also called patersonbrownkelly syndrome or sideropenic dysphagia, is a rare disease characterized by difficulty in. While this syndrome is known as plummer vinson syndrome in the united states, it is known as patersonbrown kelly syndrome in the united kingdom. Cost effectiveness of plummervinson syndrome group5 clinical trials involving plummervinson syndrome list5 ongoing trials on plummervinson syndrome at clinical trials. Sideropenic dysphagia is more commonly known as plummervinson syndrome pvs in the united states. The incidence of the plummer vinson syndrome has recently decreased because of improved nutrition and health care especially for pregnant women. If you have problems viewing pdf files, download the latest version of adobe reader. Plummervinson syndromea rare presentation in a child. Plummervinson syndrome definition of plummervinson.
Plummervinson syndrome is a condition where iron deficiency is associated with difficulty swallowing due to the presence of an esophageal web. Iron deficiency anemia, esophageal webs, and dysphagia difficulty swallowing. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Plummervinson syndrome, plummers nails, treatment of goiters with iodine, plummers disease secondmost common cause of.
It has been known since the beginning of the 20th century. Plummer vinson syndrome in a male and his chromosomal. 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. Pdf plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, iron deficiency anemia and esophageal webs. Plummervinson syndrome with mutilans rheumatoid arthritis. Plummer became a partner in the clinic practice in 1901, and william mayo would later quip that hiring plummer was the best days work he had ever done plummers work in internal medicine and endocrinology led to several important advances in the specialty, including.
It is a rare disorder consisting of the clinical triad of dysphagia, an esophageal web or membrane and iron deficiency anemia. 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. It is known as pattersonkelly syndrome in the united kingdom. View abstract jessner w, vogelsang h, puspok a, et al. Plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal webs. Plummer vinson syndrome is characterized by cervical dysphagia, iron deficiency anemia, and upper esophageal web or webs. Deficiency of irondependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs. Plummervinson syndrome associated with celiac disease and complicated by postcricoid carcinoma and carcinoma of the tongue. 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. Pvs is discussed in terms of its history, etiology, epidemiology, clinical presentation, diagnosis, treatment and the significant.
Plummervinson syndrome lupus tion and stenosis have included diphthe ria,5v2 rhinoscleroma,5 tuberculosis,2,3 and scarlet fever. Plummervinson syndrome article about plummervinson. Gastroesophageal or laryngotracheal reflux postcricoid. Prognosis of the plummervinson syndrome is excellent. Sideropenic dysphagia pdf patersonbrownkelly syndrome, is a rare disease characterised by dysphagia. Plummervinson syndrome pvs is a triad of microcytic hypochromic anemia. The exact etiology of pvs remains controversial but it has been associated with nutritional deficiency, autoimmune disorders, hereditary factors and remarkable high female predominance. Although it is a very rare condition, an increased risk of esophageal squamous cell. While this syndrome is known as plummervinson syndrome in the united states, it is known as patersonbrown kelly syndrome in the united kingdom. For language access assistance, contact the ncats public information officer. Dental management of medically compromised patients. People with this condition have problems swallowing due to small, thin growths of tissue that partially block the upper food pipe esophagus. Identification and followup is relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx.
Plummervinson syndrome with simultaneous midesophageal. Patients suspected of plummervinson syndrome should be tested with complete blood count cbc, iron studies, peripheral smear, stool test for occult blood, blood lead levels and bone marrow biopsy for stainable iron. One of the most important clinical aspects of plummervinson syndrome is the association with upper alimentary tract cancers. Pvs is also termed as patersonkelly syndrome, named after donald ross paterson and adam brownkelly, who first described the characteristic clinical features of the syndrome and published their findings independently in 1919 4. Most researchers believe that iron therapy reverses the dysphagia. Deficiency anemia can be caused by lack of iron, vitamin b12 or folate. Plummervinson or patersonbrownkelly syndrome pvs is characterised by a triad of cervical dysphagia, upper oesophageal web and iron deficiency anaemia. A syndrome of dysphagia with irondeficiency anemia that is due to congenital anomalies in the esophagus such as cervical esophageal webs. Plummervinsonpattersonkelly syndrome description of plummervinsonpattersonkelly syndrome.
With the forceps biopsy some membranes are removed. Hoarseness indicates that the nerve to the larynx is affected. A 56yearold woman has had dysphagia and odynophagia for 2months. Squamous cell carcinoma in the tongue and plummervinson. It triggers the growth of weblike membranes in the throat which cause dysphagia.
This doesnt happen to everyone who is diagnosed with plummervinson syndrome, but for those who do experience this type of bone pathology, iron deficiency is believed to be a very important contributing factor. Plummervinson syndrome is characterized by cervical dysphagia, iron deficiency anemia, and upper esophageal web or webs. Plummervinson syndrome pvs presents as a classical triad of dysphagia. It is important to have in mind that the approach to a patient with cheilitis includes, apart from dermatologic conditions, other related diseases such as some granulomatous diseases crohns disease, sarcoidosis and melkerssonrosenthal syndrome, nutritional deficiencies, and gastrointestinal diseases e. 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 syndrome has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. Exhibit upper esophageal web, iron deficiency anemia and dysphagia.
The dysphagia is usually painless and intermittent or progressive over years. This syndrome represents the combination of dysphagia, iron deficiency anemia, and hypopharyngeal and esophageal webs. For the past 5months, she has felt extreme fatigue, shortness of breath, and palpitations on exertion. This is the syndrome when associated with associated with mutilans rheumatoid arthritis. Postulated etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others. Reconstruction of a hypopharyngeal defect with a palatal. As described above, dysphagia and anemia can be treated effectively. Laboratory findings consistent with the diagnosis of plummervinson syndrome include presence of iron deficiency anemia. The patients were examined periodi cally for two years after the initial treatment and found to be in good general condition. Dental management of medically compromised patients deficiency anemia anemia is a decrease in the level of circulating hemoglobin below the normal reference range for a patients age and sex appendix j, page 148. Plummer vinson syndrome was more common in caucasians in northern countries, particulary amongst middle aged women. The iron deficiency is typically chronic and severe. A 58yearold woman with iron deficiency anemia presents to her primary care physician complaining of difficulty swallowing.
Plummervinson syndrome pvs is a rare entity characterized by upper esophageal webs and iron deficiency. Other articles where plummervinson syndrome is discussed. Exact data about epidemiology of the syndrome is not available, but the. First described in 1912, pvs affects mostly women of caucasian origin age group of fourth to seventh decade. The disorder is also known as pattersonbrownkelly syndrome in the uk. The recognition of this malady is important because it identifies a group of patients. It is a precancerous condition the esophageal squamous cell carcinoma risk s increased in which there is a severe spasm of the circular muscle fibres at the cricopharyngeal sphincter level or. Plummervinson syndrome is a condition that can occur in people with longterm chronic iron deficiency anemia. Exact data about epidemiology of the syndrome are not available. Plummervinson syndrome pvs was first described by patterson and kelly in 1919. Plummervinson syndrome classically presents as a triad of irondeficiency anaemia, postcricoid dysphagia and upper esophageal webs. She has difficulty with initiation of swallowing and pain in the throat.
Sideropenic dysphagia, hashimotos thyroiditis and chronic gastritis. Plummervinson syndrome laboratory findings wikidoc. Plummervinson syndrome with proximal esophageal web. Pdf plummervinson or pattersonkelly syndrome is a rare disease characterized by the triad intermittent dysphagia, irondeficiency anemia and. Jie deng orthobullets team orthobullets team 0 % topic.
It is also termed as patersonbrownkelly syndrome after two british. Plummer vinson syndrome pvs is a triad of iron deficiency anemia, esophageal web and dysphagia. Definition plummervinson syndrome pvs is a classical triad of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Most of the patients are white middleaged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. Treatment is iron supplementation and, if need be, dilation of the web to permit normal swallowing and the passage. 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. The pathogenesis of plummervinson syndrome pvs remains speculative, and the existence of the syndrome has been challenged. Plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal. A previous y fit and well 14 weeks primigravida in one of her bouts of. Plummervinson syndrome gastrointestinal medbullets. Dworkinvalenti department of otolaryngology, detroit medical center, usa. Plummer vinson syndrome article about plummer vinson. Plummer vinson syndrome also called patersonbrownkelly syndrome pvs is a rare entity comprising upper esophageal webs and iron deficiency anemia. The plummervinson syndrome, called sideropenic dysphagia, is characterized by.
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