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Ear, Nose, & Throat Journal Jun 2019
Topics: Anemia, Iron-Deficiency; Dyspnea; Fatigue; Female; Humans; Middle Aged; Plummer-Vinson Syndrome
PubMed: 31208220
DOI: 10.1177/0145561319850412 -
Journal of Blood Medicine 2017Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region.... (Review)
Review
Plummer-Vinson syndrome (PVS), a rare clinical condition, is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web in the post-cricoid region. It was first described over a century ago. However, literature on this condition remains scanty, and its prevalence appears to be declining worldwide, possibly due to improvements in nutrition over time. The condition has been reported most commonly in thin-built, middle-aged, white women. The esophageal webs in PVS are thin mucosal folds, which are best seen either in lateral views at barium swallow or at esophagoscopy. These are usually semilunar or crescentic, being located most often along the anterior esophageal wall, but can be concentric. The exact cause and pathogenesis of PVS remain unclear, though iron and other nutritional deficiencies, genetic predisposition and autoimmunity have all been implicated in formation of the webs. Treatment includes correction of iron deficiency and endoscopic dilation of the esophageal webs to relieve dysphagia. PVS is associated with an increased risk of hypopharyngeal and esophageal malignancies. Correction of iron deficiency may arrest and reverse the mucosal changes and possibly reduces this risk.
PubMed: 29089792
DOI: 10.2147/JBM.S127801 -
Indian Journal of Dental Research :... 2011The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of... (Review)
Review
The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial signs and symptoms include iron deficiency anemia, Plummer-Vinson syndrome, megaloblastic anemia, sickle cell anemia, thalassaemia and aplastic anemia. The manifestations include conjunctiva and facial pallor, atrophic glossitis, angular stomatitis, dysphagia, magenta tongue, midfacial overgrowth, osteoclerosis, osteomyelitis and paraesthesia/anesthesia of the mental nerve. Orofacial petechiae, conjunctivae hemorrhage, nose-bleeding, spontaneous and post-traumatic gingival hemorrhage and prolonged post-extraction bleeding are common orofacial manifestations of inherited hemostatic disorders such as von Willebrand's disease and hemophilia. A wide array of anemic and hemostatic disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself.
Topics: Anemia; Facies; Hemostatic Disorders; Humans; Mouth Diseases
PubMed: 22048588
DOI: 10.4103/0970-9290.87070 -
Orphanet Journal of Rare Diseases Sep 2006Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the... (Review)
Review
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.
Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Deglutition Disorders; Diagnosis, Differential; Female; Global Health; Humans; Iron; Male; Middle Aged; Plummer-Vinson Syndrome; Prevalence; Prognosis; Rare Diseases; Sex Distribution; Young Adult
PubMed: 16978405
DOI: 10.1186/1750-1172-1-36 -
BMJ Case Reports Jul 2012
Topics: Adult; Anemia, Iron-Deficiency; Barium Sulfate; Cheilitis; Deglutition Disorders; Endoscopy, Gastrointestinal; Fatigue; Female; Humans; Iron, Dietary; Mouth Mucosa; Plummer-Vinson Syndrome; Treatment Outcome
PubMed: 22843759
DOI: 10.1136/bcr-2012-006403 -
Case Reports in Gastroenterology 2021Plummer-Vinson syndrome (PVS) presents with the classic triad of iron-deficiency anemia, dysphagia, and esophageal webs. The mainstay treatment of PVS is iron...
Plummer-Vinson syndrome (PVS) presents with the classic triad of iron-deficiency anemia, dysphagia, and esophageal webs. The mainstay treatment of PVS is iron supplementation and the dysphagia usually responds to iron supplementation before the hematologic abnormalities are corrected. This syndrome classically affects middle-aged Caucasian women and very few cases have been reported in African Americans. We present a rare case of PVS in an African-American woman. A 63-year-old woman presented with shortness of breath, dysphagia, and lightheadedness for several weeks. Chest X-ray was negative for any acute abnormalities. Initial hemoglobin was 7.0 g/dL, which improved to 7.5 g/dL after 1 unit of packed red blood cells. She had a mean corpuscular volume of 62 fL, a ferritin level of 6 ng/mL, and an iron level of 12 μg/dL. Fecal occult blood test was negative and barium swallow revealed a proximal esophageal web. Her dysphagia did not significantly improve despite intravenous iron supplementation and esophageal web dilation. Video-fluoroscopic swallow study revealed esophageal and pharyngeal phase dysphagia with food entrapment. She was discharged with plans to follow up with a primary care physician and repeat esophagogastroduodenoscopy in 1 year. This case report highlights a rare case of PVS in an African-American woman and emphasizes the importance of maintaining a comprehensive and broad differential diagnosis.
PubMed: 34616256
DOI: 10.1159/000516937 -
JPGN Reports Nov 2023Plummer-Vinson syndrome (PVS), also called Patterson-Kelly-Brown syndrome, is a rare cause of dysphagia in children. This syndrome is associated with single or multiple...
Plummer-Vinson syndrome (PVS), also called Patterson-Kelly-Brown syndrome, is a rare cause of dysphagia in children. This syndrome is associated with single or multiple webs in the upper esophagus with frequent iron deficiency. PVS usually occurs in adults, particularly in Caucasian middle-aged women, in the fourth to seventh decade of life, and is rare in childhood. There are various theories about what causes PVS. One theory suggests that iron deficiency plays a crucial role in its development. Iron repletion often improves dysphagia, although some patients require esophageal dilatation or bougienage. Herein, we describe the case of a 4-year-old male child, having complaints of difficulty in swallowing solid food, diagnosed with PVS.
PubMed: 38034443
DOI: 10.1097/PG9.0000000000000375 -
Anales de Pediatria May 2019
Topics: Anemia, Iron-Deficiency; Child; Deglutition Disorders; Female; Humans; Plummer-Vinson Syndrome
PubMed: 29773525
DOI: 10.1016/j.anpedi.2018.03.019 -
The American Journal of Case Reports Apr 2021BACKGROUND Plummer-Vinson syndrome is a rare disease that presents with iron-deficiency anemia, dysphagia, and esophageal webs. It usually occurs in middle-aged White...
BACKGROUND Plummer-Vinson syndrome is a rare disease that presents with iron-deficiency anemia, dysphagia, and esophageal webs. It usually occurs in middle-aged White women, and it increases the risk for esophageal cancer. The prevalence of Plummer-Vinson syndrome has decreased due to early detection of iron deficiency and repletion of iron stores. Although Plummer-Vinson syndrome has also been commonly described in children and adolescents, it is seldom reported in the elderly population. CASE REPORT An 88-year-old women with a history of mild cognitive impairment, allergic rhinitis, and gastroesophageal reflux disease presented with difficulty in swallowing solid foods. She had a decreased appetite, along with a 4.5-kg weight loss in the last 1 year. She was also found to have severe iron deficiency and mild anemia. Her dysphagia continued to progress even after starting iron supplementation for her iron deficiency. She eventually had a food bolus trapped in her cervical esophagus that required removal via esophagogastroduodenoscopy. A barium swallow revealed a narrowing in the upper esophagus. A repeat esophagogastroduodenoscopy revealed an esophageal web that was dilated, resulting in relief of symptoms. CONCLUSIONS Dysphagia is reported in up to 10% of the elderly population. It commonly causes malnutrition and is associated with increased mortality. The usual etiologies include cognitive dysfunction, neurological disorders, and/or esophageal dysmotility or narrowing. Although the incidence of Plummer-Vinson syndrome has decreased over time, the possibility of its presence should not be overlooked. To our knowledge, the current case is the third case of dysphagia related to Plummer-Vinson syndrome reported in an octogenarian in the literature so far. Iron replacement can help resolve dysphagia in Plummer-Vinson syndrome but dilation of esophageal webs may sometimes be required.
Topics: Adolescent; Aged; Aged, 80 and over; Child; Deglutition; Deglutition Disorders; Female; Humans; Iron; Middle Aged; Plummer-Vinson Syndrome
PubMed: 33895769
DOI: 10.12659/AJCR.929899