Unmasking Plummer–Vinson Syndrome: A Rare Case Report

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 53-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 (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.

Introduction Plummer Vinson syndrome also known as Paterson Brown-Kelly syndrome is a syndrome associated with the triad of symptoms comprising microcytic hypochromic anemia, oesophageal strictures, 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. Case Report The authors report a case of 43-year-old male patient who presented with the classic symptoms of Plummer Vinson syndrome. Conclusion Dentists have to be familiar with symptoms of PVS and a thorough clinical examination of the patient is necessary for early diagnosis and treatment. As PVS is a precancerous condition with high malignant potential, early diagnosis is of utmost importance for better prognosis. Clinical Significance Mutual interaction of systemic and oral health has largely been underestimated by many patients in the developing countries and hence this report includes a note on importance of adequate medical history taking and its relevance to the dental health and treatment.

Plummer-Vinson syndrome (PVS) is characterized by the classic triad of post-cricoid dysphagia, iron-deficiency anemia and esophageal webs. PVS is commonly found in women of middle age especially in the fourth and fifth decade of life. The prevalence of PVS has decreased due to early detection of iron deficiency and repletion of iron stores. We report a case of a 81-year-old female patient who had a classic presentation of PVS, treated successfully with endoscopic procedure. To our knowledge, the current case is the fourth case of dysphagia related to Plummer-Vinson syndrome reported in an octogenarian in the literature so far. Iron supplementation can resolve dysphagia in many patients, but dilation of esophageal webs may sometimes be required. PVS should be part of the differential diagnosis of sideropenic dysphagia, especially due the risk of pharyngeal and esophageal epidermoid neoplasia.

Dysphagia in an Adolescent with Iron Deficiency Anemia.

Plummer-Vinson syndrome is a rare disorder that consists of dysphagia, iron deficiency anemia and esophageal web. It tends to be rare in black population. It is also known to be a predisposing factor for upper alimentary cancers and even gastric cancer. We report a 54 year old black female patient that presented with characteristic symptoms and signs of this rare syndrome. She had been complaining of dysphagia and also had evidence of iron deficiency anemia. Our suspicions were confirmed by a barium swallow study that showed an esophageal web. She underwent an endoscopic work up that also showed an esophageal web and this led to a successful esophageal dilation procedure. Although Plummer-Vinson is rare in black population, we should still include this syndrome on our differential list for iron deficiency anemia. INTRODUCTION Plummer-Vinson syndrome consists of a triad of iron deficiency, anemia, dysphagia which usually improves after iron replacement and esophageal webbing 1 . It has been associated with increased risk of upper alimentary tract cancers 2 . It is rare in black population 3 . We report a black female who presented with the characteristic triad. CASE REPORT The patient is a 53 year old black female with no significant medical history. She presented with a one week history of progressive dysphagia with associated sore throat and voice changes. She initially was having difficulty swallowing solid foods which then progressed to swallowing difficulty with liquids. The patient also noted episodes of bright red blood per rectum in the past. She denied any melena and weight loss. Her only medication use was occasional use of naproxen. Her vital signs were unremarkable. Her physical exam was unremarkable including a negative guaic exam. Her laboratory findings revealed hemoglobin of 5.7 g/dl and MCV of 61.6 fl. Her ferritin levels were also low at 2.1 ng/ml. The patient's folate and vitamin b12 levels were also low at 5.8 ng/ml and 134 pg/ml respectively. She was admitted for work up of hypochromic microcytic anemia and was given four units of packed red blood cells with clinical improvement. She was also given intravenous iron. She subsequently underwent a barium swallow test which revealed persistent narrowing of esophagus at the levels of 6 th and 7 th cervical vertebrae as well as a small Zenker's diverticulum. (Figure 1a ) Figure 1 Figure 1: A Barium swallow showing Zenker's diverticulum Plummer-Vinson syndrome in a black female 2 of 3 Figure 2 Figure 2: Images from the Barium swallow showing the area of Zenker's Diverticulum Her upper endoscopy showed an upper esophageal stricture that was dilated with Maloney dilators. The patient felt better after iron therapy, blood transfusion and endoscopic dilation. DISCUSSION The pathogenesis of Plummer-Vinson syndrome is not clear 2 .The main culprit is thought to be iron deficiency anemia 2 . This condition is also associated with development of esophageal cancer 2,3,4 . as well as rare incidence of gastric cancer 5 . There have also been several case reports linking celiac disease to Plummer-Vinson syndrome 6, 7 . We also advise clinicians to remember this rare association and work these patients up for celiac disease. The treatment of this condition includes iron replacement, blood transfusions and endoscopic dilation techniques to remove webs. Surveillance follow up for development of upper alimentary tract cancers is also crucial. It also tends to be rare in black population 3 . In a prior case study, Audrey et al. 3 described three Senegalese female patients with Plummer-Vinson syndrome. Plummer-Vinson can present with a multitude of findings including dysphagia, iron deficiency anemia and esophageal web. Plummer-Vinson can be rare in black population therefore it can easily be overlooked. It is also crucial to follow up these patients since this condition is associated with gastrointestinal malignancy. CORRESPONDENCE TO Dr. Askin Uysal. Veterans Hospital Overton Brooks Medical Center, Department of Medicine, 501 East Stoner Avenue, Shreveport, Louisiana 71104 Email: askinuysal2003@yahoo.com

Patient: Female, 88-year-oldFinal Diagnosis: Esophageal web • iron deficiency • Plummer-Vinson syndromeSymptoms: DysphagiaMedication:—Clinical Procedure: EsophagogastroduodenoscopySpecialty: Gastroenterology and HepatologyObjective:Rare co-existance of disease or pathologyBackground: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 re-flux 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 esophagogastroduodenos-copy. 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 (PVS), also known as Paterson-Brown-Kelly syndrome, is a rare clinical condition characterized by a triad of iron deficiency anemia, esophageal webs, and dysphagia. PVS has been rarely reported in African American females. In this case, however, we report a 26-year-old African American female with a 10-year history of iron deficiency anemia, who presented with severe fatigue and dysphagia. Her work-up revealed severe iron deficiency anemia, and upper endoscopy showed two esophageal rings. A 26-year-old African American female presented with severe fatigue, headache, and dysphagia for 5 months. The dysphagia was predominantly to solid foods compared to liquids. Patient also has a 10-year history of iron deficiency anemia and has stopped taking iron supplementations for the last 2 years. Physical examination revealed severe conjunctival pallor but was otherwise normal. Routine investigations revealed severe microcytic anemia with hemoglobin of 4.2 g/dL, mean corpuscular volume of 62.5 fL, and a ferritin level of 2 ng/dL. A modified barium swallow study showed two esophageal impressions in the cervical region, which were concerning for esophageal webs. Patient later underwent an esophagogastroduodenoscopy (EGD) which showed two upper esophageal rings; one semi-circumferential at 15 cm and a second circumferential at 17 cm from the incisors. A pediatric size scope was used to bypass the rings. She was initiated on iron replacement therapy with no symptomatic improvement of her dysphagia after 6 months of treatment. She then had a follow up EGD with wire-guided savary dilation with 8 mm dilator which resulted in an immediate improvement of her symptoms. Patient remained symptom free in her 2 months follow-up. PVS is characterized by iron deficiency anemia, esophageal webs, and dysphagia. It is predominantly seen in middle aged Caucasian females. Our case is unique because only a few cases of PVS have been reported in African American females. PVS is also a precancerous condition. Therefore, patients with this syndrome are at increased risk of developing gastrointestinal squamous cell carcinomas. Since nutritional deficiency is one of the major causes of iron deficiency anemia, the improvement in nutritional status of populations worldwide has led to a decreased incidence of PVS. Dysphagia in PVS usually responds to iron supplementation. However, resistant cases may require endoscopic dilatation of the webs as in our patient.1776_A Figure 1. EGD image Showing the first upper esophageal web, 15 cm from the incisors.1776_B Figure 2. EGD image Showing the second esophageal web, 17 cm from the incisors.1776_C Figure 3. Esophageal ring post wire-guided savary dilation with a satisfactory mucosal tear shown.

Post-cricoid web is an uncommon cause for dysphagia and is most frequently reported in middle-aged women. Triad of web, iron deficiency anemia (IDA), and dysphagia is known as Plummer-Vinson syndrome (PVS). Literature on PVS is very limited. Here we report the first prospective study of PVS with predefined diagnostic criteria and management plan. Adults with dysphagia or those incidentally found to have esophageal web were prospectively enrolled between July 2011 and June 2013. Participants were evaluated with hemogram, barium swallow, and esophagogastroduodenoscopy. PVS was diagnosed if a person had IDA and a post-cricoid web in barium swallow and/or endoscopy. Patients were managed with dilation using through-the-scope controlled radial expansion balloon followed by oral iron and folic acid supplementation. Thirty-seven patients (age, median [range] 40 [19-65] years; 32 [86%] women) were enrolled. Thirty-one symptomatic patients had dysphagia grade 1 (n = 12, 39%), 2 (n = 13, 42%), and 3 (n = 6, 19%) for a median (range) duration of 24 (4-324) months. Barium swallow, done in 29, showed web in 25 which were either circumferential or anterior in position. Twenty-nine (29/31, 94%) patients had complete and two had partial response after the first session of endoscopic dilatation without any complication. Dysphagia recurred in three (10%) of the 30 patients who were followed for a median (range) of 10 (1-24) months. Esophageal-web related dysphagia in patients with PVS responds favorably after single session of endoscopic dilation.

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. Keywords: Plummer-Vinson syndrome; chronic iron deficiency anemia; esophageal membrane; squamous cell esophageal cancer; Paterson-Brown-Kelly syndrome.

Endocarditis prophylaxis for esophageal dilation: A confusing issue?

BackgroundPlummer–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 presentationWe 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.ConclusionAlthough 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.

Steroid-responsive esophageal obstruction in a child with chronic granulomatous disease (CGD).

Introduction: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and cervical esophageal web. Celiac disease and Plummer-Vinson syndrome are both known to be associated with iron deficiency. There are very few reported cases of both being seen in the same patient. We report a case of a female found to have Plummer-Vinson syndrome along with celiac disase. Case report: We present a case of a 48 year old female with a long standing history of iron deficiency anemia. She had been following up with her primary care physician for a number of years for iron deficiency anemia and had extensive workup done. She required intermittent blood transfusions and was on B12 and Iron. She had been seen by gastroenterology 5 years prior to this visit, at that time it was recommended she have an upper endoscopy and colonoscopy which she refused due to being pregnant at the time. She was told to follow up with gastroenterology for an upper endoscopy and colonoscopy after her pregnancy was over. Years later she re-presented with anemia and dysphagia. An upper endoscopy and colonoscopy were scheduled. Upper endoscopy demonstrated a benign appearing intrinsic stenosis 24 cm from the incisors. A pediatric endoscope was required to traverse the web-like stenosis in the proximal esophagus. Duodenal biopsies were taken to check for celiac disease. Colonoscopy showed the entire colon to be normal. Duodenal biopsies were consistent with immune-mediated injury such as gluten sensitivity. She was thought to have Plummer-Vinson syndrome in the setting of iron deficiency anemia secondary to celiac disease. It was recommended that the patient adhere to a gluten free diet. The patient was scheduled for upper endoscopy for esophageal dilation but was again lost to follow-up. Conclusion: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and a cervical esophageal web. Celiac disease and Plummer Vinson syndrome are rarely seen coinciding together in the same patient. Important point to remember is that if a patient is thought to have Plummer-Vinson syndrome they should be worked up for celiac disease as a cause of iron deficiency anemia. This case demonstrates this importance of keeping in mind that Plummer-Vinson syndrome and celiac disease can coexist in the same patient.

Introduction: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and cervical esophageal web. Celiac disease and Plummer-Vinson syndrome are both known to be associated with iron deficiency. There are very few reported cases of both being seen in the same patient. We report a case of a female found to have Plummer-Vinson syndrome along with celiac disase. Case report: We present a case of a 48 year old female with a long standing history of iron deficiency anemia. She had been following up with her primary care physician for a number of years for iron deficiency anemia and had extensive workup done. She required intermittent blood transfusions and was on B12 and Iron. She had been seen by gastroenterology 5 years prior to this visit, at that time it was recommended she have an upper endoscopy and colonoscopy which she refused due to being pregnant at the time. She was told to follow up with gastroenterology for an upper endoscopy and colonoscopy after her pregnancy was over. Years later she re-presented with anemia and dysphagia. An upper endoscopy and colonoscopy were scheduled. Upper endoscopy demonstrated a benign appearing intrinsic stenosis 24 cm from the incisors. A pediatric endoscope was required to traverse the web-like stenosis in the proximal esophagus. Duodenal biopsies were taken to check for celiac disease. Colonoscopy showed the entire colon to be normal. Duodenal biopsies were consistent with immune-mediated injury such as gluten sensitivity. She was thought to have Plummer-Vinson syndrome in the setting of iron deficiency anemia secondary to celiac disease. It was recommended that the patient adhere to a gluten free diet. The patient was scheduled for upper endoscopy for esophageal dilation but was again lost to follow-up. Conclusion: Plummer-Vinson syndrome is a triad of iron deficiency anemia, dysphagia, and a cervical esophageal web. Celiac disease and Plummer Vinson syndrome are rarely seen coinciding together in the same patient. Important point to remember is that if a patient is thought to have Plummer-Vinson syndrome they should be worked up for celiac disease as a cause of iron deficiency anemia. This case demonstrates this importance of keeping in mind that Plummer-Vinson syndrome and celiac disease can coexist in the same patient.