Plummer-Vinson syndrome: a rare occurrence in paediatrics.

BACKGROUND: Plummer-Vinson syndrome (PVS) is characterized by a triad of symptoms consisting of microcytic hypochromic anaemia, oesophageal webs, and dysphagia. PVS is commonly found in women in the fourth and fifth decades of life and is rarely reported in the paediatric population. CASE PRESENTATION: We report the case of a 1-year-old male South Asian child who presented with dysphagia and anaemia for 4 months and frequent episodes of vomiting after ingesting semisolid and solid food. A complete blood analysis revealed microcytic hypochromic anaemia. An oesophagogram revealed circumferential narrowing of the upper thoracic oesophagus. Based on these findings, our suspicion was that the patient had an oesophageal web and vascular ring. Oesophageal dilation was performed with a Savary-Gilliard dilator; initially, 5 mm and 7 mm probes were used, and final dilation with a 9 mm probe was performed. CONCLUSION: Although rare in paediatric patients, a high suspicion of this syndrome is necessary in these patients to provide relief to the patient for better growth and development. Iron supplements increase the haemoglobin level but do not subside dysphagia, and oesophageal dilation is needed to open the blocked enteral pathway.

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.

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)

Plummer-Vinson Syndrome: A Rare Cause of Dysphagia in an Octogenarian.

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.

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%.

Iron Deficiency as Cause of Dysphagia and Burning Mouth (Plummer-Vinson or Kelly-Patterson Syndrome): a Case Report.

The clinical presentation of iron deficiency can be very heterogeneous, including various oral and other mucosal problems. Here, in this case, we report the patient with burning mouth and dysphagia symptoms where iron deficiency was found to be the underlying cause after several months of investigations. This clinical syndrome is called Plummer-Vinson syndrome. It is sporadic with an incidence less than 0.1% of patients suffering from iron deficiency anemia.

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 and Heart Failure: An Unusual Association in an African American Woman.

BACKGROUND Plummer-Vinson syndrome (PVS) is a rare disorder composed of the triad of dysphagia, iron-deficiency anemia (IDA), and esophageal webs. It is most prevalent in middle-aged white women, and the dysphagia often improves when the anemia is treated. It is well established that chronic hypertension can lead to congestive heart failure (CHF). While IDA is frequently found concomitantly with CHF, there have been no reported cases of new-onset CHF with anemia presenting as PVS. CASE REPORT We present the case of a 48-year-old African American woman with symptomatic anemia and new-onset congestive heart failure secondary to hypertension, who presented with the classic symptoms of PVS. CONCLUSIONS CHF with accompanying IDA may be an independent risk factor for the development of PVS. At the very least, there is an association between CHF-induced IDA and PVS. Patients presenting with CHF with symptoms of dysphagia should be considered at risk for the syndrome, and endoscopy may be warranted. Treatment for PVS includes iron replacement, and in some cases requires mechanical dilation.

Disfagia y anemia. Síndrome de Plummer-Vinson / Dysphagia and Anemia. Plummer-Vinson síndrome

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Síndrome de Plummer-Vinson: a propósito de un caso / Plummer-Vinson syndrome: a case study / Síndrome de Plummer-Vinson: relato de caso

Presentamos el caso de una paciente de sexo femenino de 40 años que consultó por astenia, adinamia, mareos y disnea progresiva de diez meses de evolución agregando en los últimos cuatro meses disfagia intermitente para sólidos. La radiografía esofágica con bario evidenció la presencia de una estenosis subcricoidea con buen pasaje distal y la videogastroscopía demostró inmediatamente por debajo del cricofaríngeo una membrana fibrosa estenosante. Se realizó tratamiento con hierro vía oral y reiteradas sesiones de dilatación con balón que lograron la resolución de los síntomas. El síndrome de Plummer-Vinson es una entidad poco frecuente caracterizada por la tríada anemia ferropénica, disfagia y membrana esofágica alta.

The study presents the case of a 40 year old female patient who consulted for asthenia, adynamia, dizziness and progressive dyspnea with 10 months of evolution, evidencing intermittent solid dysphagia in the last 4 months. Barium X-rays of the esophagus evidenced subcricoid stenosis with good distal passage and the gastroscopy immediately showed a stenosing fibrous membrane under the cricopharyngeal. Iron oral treatment was initiated and several sessions with a balloon catheter which solved the symptoms. Plummer-inson syndrome is a rare entity characterized by iron deficiency anemia, dysphagia and a high esophageal membrane.

Apresentamos o caso de uma paciente de sexo feminino de 40 anos que consultou por astenia, adinamia, tontura e dispneia progressiva com 10 meses de evolução e disfagia intermitente para sólidos nos últimos 4 meses. A radiografia esofágica com bário mostrou a presença de estenose abaixo da cartilagem cricoide com boa passagem distal e a endoscopia digestiva alta evidenciou membrana fibrosa estenosante logo abaixo do cricofaríngeo. Realizou-se tratamento com ferro por via oral e várias sessões de dilatação com balão com eliminação dos sintomas. A síndrome de Plummer-Vinson é uma doença rara caracterizada pela tríade ferropenia, disfagia e membrana esofágica alta.