Plummer-Vinson Syndrome and Role of Endoscopic Balloon Dilatation in a 4-Year-Old Child.

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.

Could Plummer-Vinson Syndrome Be Associated with Celiac Disease?

A 16-year-old female presented to our hospital clinic with a main complaint of difficulty swallowing. She reported mild dizziness and frequent fatigue and denied weight loss, fever, joint pain, or a history of diarrhea. Lab and physical results showed low weight; low hemoglobin, ferritin, and vitamin D levels; and a low red blood cell count. Swallowing assessment showed esophageal webs and swallowing difficulty, especially in the pharyngeal stage, and aspiration. It was initially suspected that clinical manifestations, including esophageal webs, iron deficiency anemia (IDA), and swallowing difficulty, were related to Plummer-Vinson syndrome (PVS). However, further investigations and pathological findings revealed several gastrointestinal manifestations consistent with celiac disease (CD). Based on this finding, the patient began a gluten-free diet for the management of CD. Afterward, she began to gain weight, followed by a resolution of swallowing difficulty. Therefore, clinicians should be familiar with the symptoms of CD when conducting a thorough clinical examination and maintain a high level of suspicion to rule out other causes and reach an accurate diagnosis. It is also recommended to screen all patients presenting with IDA, esophageal web, and dysphagia for CD even in the absence of diarrhea.

Endoscopic Glucocorticoid Injection for the Treatment of a Refractory Benign Esophageal Stenosis in a Patient With Plummer-Vinson Syndrome.

Plummer-Vinson syndrome (PVS) or Paterson-Brown-Kelly syndrome is a rare clinical condition characterized by the triad of esophageal webs/stenoses, iron-deficiency anemia, and progressively worsening dysphagia. It occurs mostly in Caucasian women in the fourth to seventh decades, particularly in northern countries. Esophageal webs and stenoses can be encountered during endoscopic evaluation for the patient's complaint of dysphagia. Esophageal stenoses are characterized as simple or complex. A stenosis should be considered refractory once the patient has undergone several sequential dilatations within short intervals, optimized treatment for potential underlying causes (eosinophilic esophagitis or acid reflux), and after neuromuscular causes have been excluded. Glucocorticoid injection into a stenosis during an endoscopic dilation session has been proven to be beneficial as the initial treatment modality of refractory nonmalignant esophageal stenoses. We present a case of a 39-year-old woman with refractory esophageal stenosis in the setting of PVS which was successfully treated with serial endoscopic glucocorticoid injections while she received oral iron supplementation. To our knowledge, there are no previous cases of esophageal stenoses associated with PVS in the literature requiring endoscopic glucocorticoid injection for successful resolution.

A Case of Plummer-Vinson Syndrome in an Omani Woman.

Plummer-Vinson syndrome (PVS) is the triad of iron-deficiency anemia, esophageal webs and dysphagia. This article discusses the first reported case of PVS from Oman. A female patient in her 40s presented with a one-year history of dysphagia and odynophagia and a known background of untreated iron-deficiency anemia. After an elaborate workup, a diagnosis of PVS was made following visualization of the esophageal web with barium swallow study and esophagogastroduodenoscopy (EGD). A prompt and accurate diagnosis of PVS, although rare, is crucial, given that it is, at times, a precancerous condition. The treatment with iron therapy is the cornerstone of management, and it corrects the anemia as well as the dysphagia. If dysphagia persists, endoscopic dilation can be carried out.

Clinical and therapeutic features of Plummer-Vinson syndrome in a Tunisian population: a case series.

Plummer Vinson syndrome (PVS) is a rare entity and most publications are case or series of cases. Thus, we report a series from southern Tunisia. Our aim was to analyse the epidemiological and clinical characteristics, the therapeutic modalities as well as the evolution of this pathology. Thus we carried out a retrospective study from 2009 until 2019. For each patient with PVS, we collected the epidemiological, clinical, paraclinical data and therapeutic modalities. A total of 23 patients were enrolled with a median age of 49.52 years [18-82 years] and a clear female predominance (M/F=2/21). The median duration of dysphagia was 42 months [4-92 months]. Moderate microcytic hypochromic anemia was noted in 16 patients. The anemia was without obvious cause in 60.8% (n=14) of cases. The main endoscopic finding was a diaphragm in the cervical area. Treatment was based on iron supplementation followed by endoscopic dilatation with Savary dilators in 90.9% (n=20) and balloons for 9.1% of patients (n=2). Dysphagia recurred in 5 patients after a median of 26.6 months [2-60 months]. Three cases of PVS were complicated by esophageal squamous cell carcinoma. In conclusion, our series confirms that PVS affects mostly women. Anemia is frequently noted in these patients. Treatment is based on endoscopic dilatation which is often an easy and risk-free procedure and iron supplementation.

Esophageal squamous cell cancer in Plummer-Vinson syndrome: Is lichen planus a missing link?

This retrospective study analyzed the occurrence of esophageal squamous cell cancer (ESCC) in a cohort of patients with Plummer-Vinson syndrome (PVS) and mucosal lichen planus (LP). ESCC occurred in 6.2% cases of PVS, more than half of whom had associated oral LP. Mucosal LP and PVS together may increase the predisposition to ESCC.

Plummer-Vinson syndrome in primary Sjögren syndrome: a case-based review.

This study aimed to describe a patient with Sjögren syndrome who developed Plummer-Vinson syndrome, and to review the literature and describe shared aspects of this rare association. A systematic screening of articles was conducted in PubMed/MEDLINE, LILACS, SciELO, Scopus, Web of Science, and Cochrane, dating 1940 to 2020. All the articles included the association between Sjögren syndrome and Plummer-Vinson syndrome. No language restriction was applied. The following terms were used: "Sjögren syndrome" or "sicca syndrome" and "Plummer-Vinson syndrome" or "Paterson-Kelly syndrome." We performed our analysis by adding our present case, with a total of 4 cases. Three out of four were female (75%), age varied from 56 to 58 years old. In 2 cases, Sjögren syndrome preceded Plummer-Vinson syndrome diagnosis, and in 1 report, Plummer-Vinson syndrome appeared before Sjögren syndrome. Disease duration varied from 7 to 20 years. In two cases, autoantibodies were available, and antinuclear antibodies and anti-Ro/SS-A were positive in both, and anti-La/SS-B in one of them was associated with anti-dsDNA; however, no data regarding lupus was available in the article. Treatment involved iron supplementation in 3/3. Two out of three received parenteral iron supplementation, and in these two cases, mechanical esophageal dilatation was needless. In the other case, an additional endoscopic esophageal dilatation was necessary to receive the oral iron supplement. All 3 cases had a good outcome. This case illustrates a patient with Sjögren syndrome who developed the rare Plummer-Vinson syndrome. In Sjögren syndrome, the presence of iron-deficiency anemia, dysphagia, and weight loss should alert the physician to search for associated Plummer-Vinson syndrome.

Plummer-Vinson Syndrome in an African-American Woman.

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.

Síndrome de Plummer-Vinson / Plummer-Vinson syndrome

Introducción: El síndrome de Plummer-Vinson es una entidad rara caracterizada por la tríada anemia ferropénica, disfagia y membrana esofágica. Descrito en la segunda década del siglo pasado, en la actualidad la mayoría de los datos que se obtienen provienen de presentaciones de casos o pequeñas series de estudios prospectivos. Objetivo: Hacer la revisión de la bibliografía disponible a propósito de un caso portador de síndrome de Plummer-Vinson. Caso clínico: Paciente femenina de 35 años de edad con anemia desde la adolescencia remitida por presentar disfagia de 8 años de evolución. Se realizaron complementarios de laboratorio donde se constata anemia ferropénica y estudio contrastado del tracto digestivo superior que reveló imagen sugestiva de membrana en esófago cervical. Conclusiones: A pesar de su baja frecuencia en la actualidad debemos mantenernos alertas ante la aparición de síntomas sugestivos del síndrome de Plummer-Vinson fundamentalmente en mujeres con cuadros de anemia(AU)

Introduction: Plummer-Vinson syndrome is a rare entity characterized by the triad of iron deficiency anemia, dysphagia and esophageal membrane. Described in the second decade of the last century, today most of the data obtained comes from case presentations or small series of prospective studies. Objective: To review the available bibliography regarding a case with Plummer-Vinson syndrome. Clinical case: 35-year-old female patient with anemia since adolescence, referred to surgery clinic for presenting dysphagia of 8 years of evolution. Additional laboratory tests were carried out where iron deficiency anemia was found. A contrasted study of the upper digestive tract revealed a suggestive image of a membrane in the cervical esophagus. Conclusions: Despite its low frequency we must be alert to the appearance of symptoms suggestive of Plummer-Vinson syndrome, mainly in women with anemia. Its association with esophageal cancer indicates this(AU)

Anemia ferropénica crónica: síndrome de Plummer-Vinson (Paterson-Brown-Kelly) / Chronic Iron Deficiency Anemia: Plummer- Vinson (Paterson-Brown-Kelly) Syndrome. A Case Report

Resumen El síndrome de Plummer-Vinson (Paterson-Brown-Kelly) es una entidad rara, caracterizada por disfagia, formación de membranas esofágicas y anemia por deficiencia de hierro. Presentamos el caso de una mujer de 46 años con antecedentes clínicos de anemia ferropénica de larga evolución, que posteriormente presentó disfagia y odinofagia. Se encontró una membrana subcricoidea que fue rota exitosamente con el endoscopio. Los médicos de primer contacto deben estar familiarizados de los síntomas del síndrome de Plummer- Vinson, y tenerlos en cuenta a la hora de abordar un paciente con la tríada clásica. Como el síndrome es una condición pre- cancerosa con alto potencial maligno, el diagnóstico precoz y tratamiento oportuno es de suma importancia.

Abstract Plummer-Vinson syndrome (Paterson-Brown-Kelly) is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. We present the case of a 46-year-old woman with a clinical history of iron deficiency anemia who subsequently presents dysphagia and odynophagia. A subcricoid web that was successfully broken with the endoscope was found. First contact doctors should be familiar with the symptoms of Plummer-Vinson syndrome, and take them into account, when addressing a patient with the classic triad. Since the syndrome is a precancerous condition with high malignant potential, early diagnosis and treatment is of utmost importance for better prognosis.

Plummer-Vinson syndrome: A decade's experience of 132 cases from a single center.

BACKGROUND AND AIM: Plummer-Vinson syndrome (PVS) comprises triad of iron deficiency anemia, dysphagia, and post-cricoid esophageal web. PVS is rare nowadays due to improved nutritional status. However, we encountered patients with PVS regularly at our center. Data regarding PVS are limited; hence, we aimed to study the clinical features, treatment outcomes, and development of complications in patients with PVS. METHODS: The study was conducted over a 10-year period (January 2008 to January 2018) in a medical college setting. All adults with dysphagia, anemia, and post-cricoid web or those with iron deficiency anemia and post-cricoids web were included in the study. Patients were treated with iron supplementation and Savary-Gilliard bougie dilation of the web. Patients were followed-up for the recurrence of dysphagia and development of complications. RESULTS: Overall, 153 patients exhibited esophageal web, of which 132 (86.27%) patients had concomitant PVS and 21 (13.7%) patients did not. The mean age was 43.50 years (range 16-76) and 113 (85.6%) were women. Single session of Savary-Gilliard bougie dilation was successful in 90.7% of patients in relieving dysphagia and 9.3% developed recurrence, requiring repeated dilations. Four patients had concomitant squamous cell carcinoma of esophagus along with PVS and two developed upper gastrointestinal malignancy during follow-up. CONCLUSION: Plummer-Vinson syndrome is predominantly seen in middle aged women and present with symptoms of iron deficiency anemia and early grade dysphagia. Single session of Savary-Gilliard bougie dilation was successful in majority of patients in relieving dysphagia. Overall risk of developing upper gastrointestinal malignancy was 4.5%.

Plummer-Vinson Syndrome in a Crohn's disease patient.

Plummer Vinson Syndrome (PVS), a rare condition complicating iron deficiency anaemia, manifests as a classic triad of dysphagia, anaemia and oesophageal web. Iron deficiency is the commonest systematic complication of Crohn's disease with only one described case of a Crohn's disease patient combined with PVS in the literature. We present a case of PVS complicating Crohn's disease in a 38-years-old female patient under treatment with the biologic agent Ustekinumab (human interleukin 12/23 monoclonal antibody) suffering from a progressively worsening dysphagia.

A Holistic Approach to Diagnosing and Treating Dysphagia.

We report a 39-year-old Native American female with an almost 20-year history of dysphagia that had increased in the 6 months prior to the initial evaluation. Investigation revealed a number of distinct esophageal disorders including Plummer-Vinson syndrome, gastroesophageal reflux disease with esophagitis, distal esophageal stricture, esophageal intramural pseudo-diverticulosis, and recurrent esophageal Candida infections. Although prolonged therapy with proton pump inhibitors, fluconazole, nystatin, and repeated esophageal balloon dilations relieved her symptoms, her prognosis remains uncertain.

Plummer-Vinson Syndrome With Concomitant Factor VII Deficiency.

Background: Plummer-Vinson syndrome (PVS), a rare disorder characterized by dysphagia, iron deficiency anemia, and esophageal webs, has principally been described in middle-aged women. This disorder is uncommon in the 21st century because of the abundance of iron-fortified foods. Clotting factor deficiencies are also rare. Factor VII deficiency is a bleeding disorder characterized by the absence of a critical protein in the coagulation cascade. Case Report: We present a case of PVS associated with factor VII deficiency in a 26-year-old African American female. The patient had a history of anemia that was repeatedly attributed to menstrual bleeding and dysphagia for 10 years. She presented with symptomatic anemia requiring transfusion. She reported a history of food getting stuck in her chest, and workup revealed esophageal webs with no evidence of overt luminal gastrointestinal bleeding. Coagulation laboratory tests revealed the incidental finding of a borderline increased prothrombin time. Hematologic studies confirmed the presence of factor VII deficiency. Conclusion: To our knowledge, no case has been published about a patient diagnosed with PVS and concomitant factor VII deficiency. Our case illustrates several learning points: (1) PVS is an uncommon disorder that may still be diagnosed in a developed country in the 21st century; (2) PVS requires close follow-up and esophageal surveillance because of the increased risk of esophageal cancer; (3) factor VII exhibits a high degree of phenotypic variability; (4) phenotype in factor VII deficiency does not always correlate with factor VII activity, although life-threatening spontaneous bleeding is not expected with levels >2%.

A Rare Case of Plummer-Vinson Syndrome.

Plummer-Vinson syndrome (PVS) is characterized by a triad of symptoms comprising microcytic hypochromic anemia, esophageal webs, and dysphagia. PVS is commonly found in women of middle age especially in the fourth and fifth decade of life and is rarely reported in males. We report a case of a 39-year-old female patient who had a classic presentation of PVS. PVS is precancerous with high malignant potential; early diagnosis is of utmost importance for better prognosis and surveillance endoscopy is recommended. Iron repletion oftentimes improves the dysphagia; seldom esophageal dilatation is used to provide symptomatic relief.

Plummer-Vinson Syndrome with Esophageal Web Formation in which Detailed Endoscopic Images Were Obtained.

Plummer-Vinson syndrome is a rare entity, characterized by dysphagia, esophageal web formation, and iron deficiency anemia. The patient was a 63-year-old 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. A membranous stricture was found in the cervical esophagus during a fluoroscopic examination. An endoscopic examination conducted under general anesthesia revealed an oblique linear scar on the proximal surface of the stricture. Sequential balloon dilation was performed successfully. We suggest that the esophageal web formation might have been related to the healing of an esophageal ulcer.

Plummer-vinson syndrome associated with lower esophageal ring (Schatzki's Ring)

We report a case of Plummer-Vinson syndrome (PVS) and lower esophageal ring with a small sliding hiatal hernia. PVS is a rare entity formed by the combination of dysphagia, cervical esophageal web and iron deficiency anemia. It occurs mainly in middle-aged women1,2,3. A lower esophageal ring and a small sliding hiatal hernia were also observed in this case. We documented clinical manifestations of iron deficiency anemia through images and esophageal abnormalities through barium esophagogram. (AU)

Plummer-Vinson syndrome complicated with hypopharyngeal carcinoma: A case report and literature review / 吉林大学学报(医学版)

Objective: To observe the clinical manifestations of cervical esophageal webbed stenosis in the patients with Plummer-Vinson syndrome(PVS) and analyze its relationship with hypopharyngeal carcinoma, and to raise the clinicians' awareness of the disease. Methods: The diagnosis and treatment process of one patient with PVS complicated with hypopharyngeal carcinoma was summarized, and its clinical characteristics were analyzed in combination with relevant literatures.Results: A 39-year-old patient with PVS as a complaint by "dysphagia" was diagnosed as hypopharyngeal carcinoma by electronic laryngoscope and pathological biopsy. A lot of intraepithelial papillary capillary loops (IPCL) were seen on narrow band imaging(NBI) of the mucosa extending downward from the posterior and lateral walls of the hypopharynx to the upper part of the esophageal web. The hypopharyngeal mucosa with dense IPCL was found to have moderate to severe dysplasia. After 2 courses of induction chemotherapy for hypopharyngeal carcinoma, the symptoms disappeared and the tumor volume was significantly decreased.Conclusion: The occurrence of hypopharyngeal carcinoma is related to the mucosal changes of PVS. NBI technique is helpful for early diagnosis of hypopharyngeal and esophageal mucosal degeneration in the PVS patients.