Plummer Vinson syndrome is defined by the classic triad of dysphagia, iron deficiency anemia, and an esophageal problem. Although very rare, its diagnosis is important because it identifies a group of patients at increased risk for squamous cell carcinoma of the pharynx and esophagus.
- Plummer-Vinson, PVS;
- Paterson-Kelly syndrome;
- Paterson-Brown Kelly;
- Sideropenic dysphagia.
Story
The most commonly used name is Plummer-Vinson syndrome. It is named after Henry Stanley Plummer (1874-1936) and Porter Paisley Vinson (1890-1959), who were physicians at the Mayo Clinic.
In 1912, Plummer reported a series of patients with years of iron deficiency anemia, dysphagia, spasm of the upper esophagus without anatomical stenosis, which was described as hysterical.
The synonym is Paterson-Kelly syndrome, named after Donald Ross Paterson (1863-1939) and Adam Brown-Kelly (1865-1941). British laryngologists who published their results independently in 1919.
They were the first to describe the characteristic clinical features of the syndrome. Paterson gave a full description, but without reference to anemia. He was also the first to draw attention to the association with postcricoid carcinoma. Brown-Kelly not only described the signs and symptoms of the condition, but also anemia.
Epidemiology
Precise data on the prevalence of the syndrome are not available. In the first half of the 20th century, Plummer-Vinson syndrome seemed common among northern Caucasians, especially among middle-aged women. It is currently extremely rare.
Of 1000 patients who underwent cineradography of the hypopharynx and cervical esophagus, linens were found in 5.5% of cases, but only six had dysphagia associated with Vinson's Syndrome.
The rapid decline in the prevalence of the syndrome correlates with improved nutrition and the disappearance of widespread iron deficiency in countries where it has previously been described. In Africa, where iron deficiency and malnutrition are common, the syndrome is very rare.
Clinical Description
The main clinical features of Plummer-Vinson syndrome are postcricoid dysphagia, upper esophageal networks, and iron deficiency anemia. Most patients are middle-aged white women in their fourth decade of life, but the syndrome also occurs in children and adolescents.
Dysphagia is usually painless, intermittent, progressive over many years, limited to solid particles, sometimes associated with weight loss.
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Symptoms resulting from anemia, such as weakness, pallor, fatigue, tachycardia, dominate the clinical picture. In addition, it is characterized by glossitis, angular cheilitis, koilonics (spoon-shaped nails). There is also an increase in the spleen, thyroid gland.
Plummer-Vinson syndrome is a risk factor for the development of squamous cell carcinoma of the upper gastrointestinal tract. It has been reported that 3 to 15 percent of patients with Plummer-Vinson syndrome, mostly women between the ages of 15 and 50, develop cancer of the esophagus or pharynx.
A decreasing trend towards overall incidence in hypopharyngeal cancer in women has been demonstrated, probably due to a decrease in the prevalence of the syndrome.
Case reports
An analysis of case histories published in the literature over 7 years (1999-2005) showed that 25 out of 28 adult patients with Plummer-Vinson syndrome were women (89%). The median age at presentation was 47 years (range 28-80 years).
All patients had iron deficiency anemia with a mean hemoglobin value of 8.2 g/dL. The most common reports were associated disease (n=6), increased menstrual blood loss (n=5), oral hernia, chronic gastrointestinal bleeding of unknown origin (n=3).
All patients were prescribed iron supplements. Most of them underwent dilatation of the esophagus (n = 20). In general, the prognosis was very good.
Patients did not receive dysphagia during the follow-up period. In two cases, squamous cell carcinoma was diagnosed, and in one case, gastric cancer was detected by endoscopy a year after the diagnosis of Plummer-Vinson syndrome. This malignancy is extremely rare, but has been described in two case reports from previous years.
Diagnostics
Diagnosis is based on evidence of iron deficiency anemia and one or more esophageal tissues in a patient with postcricoid dysphagia. Esophageal tissue can be seen with a barium x-ray, but video fluoroscopy is the best way to demonstrate. Symptoms are detected by endoscopy of the upper gastrointestinal tract.
They look smooth, thin, gray with an eccentric or central clearance. Meshes are commonly found in the proximal esophagus. May be missed and accidentally torn if the endoscope is not inserted under direct visualization.
Esophageal networks, which also occur in the absence of anemia and Plummer-Vinson syndrome, are characterized by one or more thin horizontal membranes composed of squamous epithelium and submucosa. Usually protrude from the front wall, extending in the transverse direction. But not to the back wall, rarely surround the lumen.
Laboratory tests
Laboratory studies usually reveal iron deficiency anemia with decreased hemoglobin, hematocrit, serum iron, ferritin, and increased total iron binding capacity. Other laboratory anomalies are not described.
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Differential Diagnosis
Since dysphagia is the main clinical feature of Plummer-Vinson syndrome, the differential diagnosis includes all other causes of dysphagia, especially malignancy, benign strictures, or oesophageal rings.
Other causes of dysphagia are diverticula, mobility disorders such as achalasia, spastic dysmotility, scleroderma, diabetes mellitus, neuromuscular, skeletal muscle disorders.
Etiology and pathogenesis
The pathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. This theory is based primarily on the fact that iron deficiency is part of the classic triad of Plummer-Vinson syndrome, dysphagia, and esophageal tissues, and that dysphagia is ameliorated by iron supplementation.
Indeed, impaired esophageal mobility is described in Plummer-Vinson syndrome and is compensated for by the addition of iron. Iron deficiency has been proven to precede dysphagia. On the other hand, the digestive tract is susceptible to iron deficiency.
Rapidly loses iron-dependent enzymes due to high cellular turnover. It causes mucosal degeneration and web formation. However, large clinical series have shown that for many patients iron deficiency is neither a necessary nor a sufficient cause of these formations.
Other etiological factors have been proposed, including malnutrition, genetic predisposition, autoimmune processes. The latter is based on the association between Plummer-Vinson syndrome and certain autoimmune disorders. For example, celiac disease, thyroid disease, rheumatoid arthritis.
Treatment
The first step in treating Plummer-Vinson syndrome is to find out the cause of the iron deficiency. To exclude active hemorrhage, malignancy, or celiac disease.
Plummer-Vinson syndrome is easily and effectively treated with iron supplementation and mechanical expansion. Iron alone can resolve dysphagia in many patients. However, in case of significant obstruction of the esophageal lumen or cavity and persistent dysphagia despite iron supplementation, ruptures and expansion of the tissue should be performed.
After endoscopic placement of the guidewire, dilators up to 17 mm in diameter are used. Usually one dilation is enough to relieve dysphagia, but sometimes multiple sessions are needed. Successful balloon dilatation has also been described.
Plummer disease or toxic nodular goiter, - Abnormal growth of the thyroid gland. The gland enlarges, nodules appear, which secrete additional hormones, thus causing hyperfunction of the thyroid gland.
This thyroid disease may be associated with chronic iodine deficiency or excessive iodine intake. Most often affects women aged 45-60 years. The level of the TSH hormone decreases, and the concentration of thyroid hormones is normal or increased.
Causes of Plummer's disease
Toxic nodular goiter, most often develops as a result of treatment of iodine deficiency. Large intake of iodine causes the development of nodules on the thyroid gland, which secrete thyroid hormones, regardless of the level of TSH. In medicine, such places are called ectopic sources.
Thyroid goiter...
In any case, the secretion of gland hormones is out of control. There is a sharp increase in the gland and other symptoms of hyperthyroidism develop. toxic thyroid nodules can also appear as a result of the intake of large doses of iodine contained in radiological protective equipment or as a result of therapy with drugs that have iodine atoms in their structure (amiodarone, some disinfectants).
Toxic goiter accounts for 10-50% of all cases hyperthyroidism. Plummer's disease can occur in people who have been deficient in iodine for a long time. Nowadays, however, it is a rare form of the disease due to the intake of iodine in the diet, mainly through iodized salt.
Symptoms of Plummer's disease
As a result of increased secretion of thyroid hormones, typical symptoms of hyperthyroidism appear.
These include:
- feeling of inner restlessness;
- increased sweating;
- heat intolerance;
- burst of nervous excitability;
- weakness, fatigue;
- labored breathing;
- increased appetite, with simultaneous weight loss;
- warm and moist skin;
- irregular menstruation;
- growth retardation;
In persons over 60, apathy appears, physical efficiency decreases and former interests are lost.
As a result of excessive production of thyroid hormones by the nodules, there is a decrease in the secretion of the hormone TSH - a thyroid-stimulating hormone secreted by the pituitary gland, and with the help of negative feedback, the production of thyroid hormones by the correct cells is slowed down.
Untreated nodules increase the risk of a so-called remodeling of the thyroid gland. Nodules are usually benign, but sometimes they can become cancerous.
Diagnosis and treatment of Plummer's disease
Diagnosis of Plummer's disease is based primarily on ultrasound of the thyroid gland, which shows the existence of toxic nodules. An isotope study of the thyroid gland reveals autoimmune nodules.
Sometimes a biopsy of thyroid nodules and their histological examination are performed. A biochemical study is also carried out, in which the concentration of the TSH hormone is checked.
Treatment consists of the use of antithyroid drugs, as well as radioactive iodine. Sometimes a surgical procedure is performed when the goiter grows to a significant size and can inhibit the work of other organs. Beta-blockers are used for heart rhythm disturbances.
(D.R. Paterson, 1883-1939, English otolaryngologist; A.B. Kelly, 1865-1941, Scottish otorhinolaryngologist)
see Plummer-Vinson syndrome.
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Synonyms for Plummer-Vinson Syndrome. S. Paterson. S. Paterson-Kelly. S. Kelly. S. Paterson-Kelly - Plummer - Vinson - Sjogren. S. Paterson-(Brown) Kelly. S. Kelly - Paterson. Sideropenic dysphagia.
Definition of Plummer-Vinson Syndrome. Dysphagia with atrophy of the mucous membrane of the mouth, throat and.
Author. Plummer Henry Stanley - American physician, Rochester, Minnesota, 1874-1937. Vinson Porter Paisley is a modern American physician. Paterson D. R. is a modern British otolaryngologist, Cardiff. Kelly Adam Brown - Scottish laryngologist, 1865 - 1941. The syndrome was first described by Plummer in 1908, Vinson in 1919, Paterson in 1906-1919. and Kelly in 1919.
Symptoms of Plummer-Vinson Syndrome:
1. Dysphagia (difficulty swallowing, burning tongue, functional spasm of the esophagus and cardiospasm).
2. Trophic changes in the mucous membranes (atrophy of the mucous membrane of the oral cavity, throat, esophagus, intestines), superficial glossitis.
3. Cracks in the corners of the mouth, cheilitis.
4. Nail dystrophies (glossy, flattening, hollow nail, koilonychia).
5. Seborrheic dermatitis of the face, hyperkeratosis of other skin areas.
6. Cracks in the corners of the eyes, chronic blepharoconjunctivitis, superficial punctate and guttate keratitis with surface vascularization, blurred vision at dusk.
7. Blood morphology: hypochromic anemia, anisocytosis, microcytosis, poikilocytosis. planocytosis; rarely hyperchromic anemia.
8. Blood biochemistry: decrease in serum iron.
9. Achlorhydria, chronic gastritis.
10. Porphyrinuria.
11. Almost exclusively women get sick.
Etiology and pathogenesis of Plummer-Vinson syndrome. Apparently, we are talking about the manifestation of complex hypovitaminosis, and the deficiency of lactoflavin and nicotinic acid amide is especially pronounced. Treatment with iron preparations often leads to an improvement in the process, however, apparently, there may be cases of the syndrome without iron deficiency and without anemia. Some authors believe that the syndrome may be the result of primary gastritis. Connection with S. Moeller-Hunter, with manifestations of S. Faber, and also with S. Sjogren.
Differential Diagnosis. ariboflavinosis syndrome. S. Sjogren (see). S. Biermer (see). S. Moeller-Hunter (see). S. Reichert (see).