Plummer Vinson Syndrome - Article Example

Plummer Vinson Syndrome Abstract Plummer Vinson Syndrome is characterized by trio of symptoms – dysphagia, iron deficiency anemia and esophageal web. It is commonly seen in adults, more prevalent in females and rarely seen in children or adolescent. Patient demonstrates symptoms such as pallor, fatigue, weakness tachycardia with difficulty in swallowing solid food. Esophageal web is painless. As exact etiology for this syndrome is not clear, iron deficiency anemia is considered to be the primary cause for web formation. These cases are treated first with iron replacement therapy and mechanical dilatation of the occluded esophagus and if required curetting is done. Plummer Vinson Syndrome is considered to be a risk factor for cancer formation. It is a progressive condition and if not intervened in time, it may lead to malignancy pertaining to upper gastrointestinal tract. Introduction Plummer Vinson Syndrome or PVS, also known as Paterson Brown Kelly Syndrome, is a rare disease condition characterized by classical trio of symptoms such as dysphagia i.e. difficulty in swallowing, iron deficiency anemia and esophageal web (Novacek 1-36). It is also called as “sideropenic dysphagia”. PVS was more prevalent in initial decades of 20th century. Now, worldwide the incidences of this syndrome have been reduced drastically due to improved socio economic and dietary conditions but in developing countries the cases have still been reported. Distinct causative pathology of Plummer Vinson Syndrome is not yet confirmed. Some conditions are considered to be the possible predisposing factors. They are listed as malnutrition, iron deficiency anemia, genetics and auto-immune diseases. PVS is predominantly seen in adults of middle age group but incidences are enormously higher in females. Incidence rate is 10 times higher in females living in under developed communities (Dinler, Tander, Kalayc, et al. 384-386). “Plummer Vinson Syndrome” – this name was coined after series of cases reported by the two physicians of Mayo clinic. Plummer was the first person in 1912 to come across few patients having similar set of complaints such as dysphagia, prolonged anemia and narrowing of the esophagus. He could not confirm this clinical picture and thought them hysterical. Later on in 1919, Vinson published similar cases of “esophageal angulations” and available clinical data was confirmed (Chaudhary, Alamgir, and Obaid 37-40). This syndrome is also called as “Patterson Kelly syndrome” after the successful contribution of two British Laryngologist Patterson and Kelly in 1919. They had not only classified the syndrome but also had distinguished its relationship with post cricoids carcinomas (Novacek 1-36). Patients with Plummer Vinson Syndrome report difficulty in swallowing solids associated with weight loss. They also reflect anemic symptoms such as with pallor, fatigue, weakness and sometimes tachycardia. Dysphagia is often painless but progressive (Novacek 1-36). Due to web formation of atrophied mucosa oesophageal lumen gets obstructed giving trouble in swallowing. Web is usually 2-3 mm wide and typically found in upper part of oesophagus. This patient can experience cheilitis, Glossitis and Koilonychia too (Chaudhary, Alamgir, and Obaid 37-40).Sometimes on physical examination thyroid and spleen enlargement may also be found. (Novacek 1-36). In annals of gastroenterology journal, it has been mentioned that Plummer Vinson Syndrome could be precancerous due to mucosal degeneration. Incidence of squamous cell carcinoma of oral cavity or pharynx or oesophagus in such condition is seen in almost 10% of the affected cases of PVS. (Sawalakhe, Nistala, Sasidharan, et al. 52-55). Disease prognosis is very good if treated in time with iron replacement therapy and mechanical dilatation of the esophagus. Patient needs to be followed up regularly in order to control development of carcinomas in such cases. Discussion Incidences of Plummer Vinson Syndrome have been dramatically reduced worldwide but still not very uncommon in developing countries where people especially females who lack in adequate nutrition and suffer from blood loss due menstrual problems .They do not receive appropriate treatment for the same. (Chaudhary, Alamgir, and Obaid 37-40). Proper aetiology and pathology of PVS has not been understood. Only few cases have been reported in some medical journals. (Dinler, Tander, Kalayc, et al. 384-386). It is accepted that iron deficiency anaemia is principle cause and responsible for the changes in the epithelial lining of oesophagus and hypo pharynx. The epithelial cells of upper GI tract are vulnerable to the change in iron content of the body. However due to their “high cellular exchange mechanism”, they eventually lack in “Iron dependant enzymes”. These unnatural epithelial changes result in oral cavity affections such as Angular Glossitis, Cheilitis (Donohue-Cornejo, Guzman Gastelam, Constandase-Cortez, et al. 189-192) and create degeneration of mucosa forming web like structure. There are some cases reported in some journals where web has also been found along with autoimmune diseases such as Celiac disease or Rheumatoid Arthritis or Thyroid, etc. (Novacek 1-36) but pathological relation is still unclear. It has been widely accepted that Plummer Vinson Syndrome is a definite risk factor for causation of squamous cell carcinoma of the oral cavity. Hence it is a must for any dental professional or hygienist to look thoroughly for any oral or lingual lesion to rule out carcinoma (Donohue-Cornejo, Guzman Gastelam, Constandase-Cortez, et al 189-192). Some journals have described patients with PVS and squamous cell carcinoma of hypo pharynx and upper oesophagus. Some patients are also observed having gastric cancers with predominant Plummer Vinson Syndrome (Chaudhary, Alamgir, and Obaid 37-40) & (Sawalakhe, Nistala, Sasidharan, et al. 52-55).Relationship in these pathologies has not yet been established. It could be incidental. But, overall it has been distinctly noted that mucosa of upper GI tract is sensitive to prolong iron deficiency anaemia leads to major atrophic changes increasing chances of cancer (Sawalakhe, Nistala, Sasidharan, et al. 52-55). Diagnosis and treatment PVS is confirmed by having common blood count with hemoglobin and other haematocrit values and barium swallow x- ray. If required “Video Fluoroscopy” or “Upper endoscopy” may also be performed (Novacek 1-36).To begin with the treatment, iron supplements are administered. Increase in iron levels usually help in reversing the condition in most of the cases but not all. Hence, it highlights existence of other causative factors such as auto immune diseases (Donohue-Cornejo, Guzman Gastelam, Constandase-Cortez, et al 189-192). In complicated web formation, “Mechanical dilatation” under endoscopic guidance is preferred. “Endoscopy dilatation is the safest and cost effective method of treatment for oesophageal web and strictures with low complication rate.” (Chaudhary, Alamgir, and Obaid 37-40). PVS has good prognosis if treated in time. It has the potential to grow and result in carcinoma due to affinity towards squamous cells; this fact cannot be overlooked. Regular follow-up with close monitoring is mandatory. Conclusion Esophagoscopy has been proven to be the safest treatment so far rendering help in clearing webs and early detection of carcinomas. Since the first Plummer Vinson Syndrome case was recorded, experts have come across various cases demonstrating time synchronisation of Plummer Vinson Syndrome with other incidental malignancies of upper GI tract, oral cavity or gastric mucosa. But the causative factors of this syndrome and patho-physiological co-relationship between them are still not very evident and require further research. Work cited Chaudhary, Muhammad Aslam, Ashar Alamgir, and Amjad Obaid. "Esophagoscopy in the early detection and treatment of Plummer Vinson Syndrome." Journal of Rawalpindi Medical College. 12.2 (2008): 37-40. Web. 3 Dec.2011. . Dinler, Gonul, Burak Tander, Ayhan Gazi Kalayc, and Riza Rizalar. "Plummer-Vinson syndrome in a 15-year-old boy." Turkish Journal of Pediatrics. 51. (2009): 384-386. Web. 4 Dec. 2011. . Donohue-Cornejo, Alejandro, Dalia Abril Guzman Gastelam, Daniel Constandase-Cortez, Luis Alberto Gaitam-Cepeda, and Carlos Escalera. "Squamous cell carcinoma in the tongue and Plummer-Vinson Syndrome. A case report" Revista Odontológica Mexicana. 15.3 (2011): 189-192. Web. 3 Dec.2011. . Novacek, Gottfried. "Plummer-Vinson Syndrome." Orphanet Journal Of Rare Diseases. (2006): 1-36. Web. 3 Dec. 2011. . Sawalakhe, N.R., S.U Nistala, M Sasidharan, R.T Narendran, A.D Amrapurkar, R.M Joshi, and P.M Rathi. "Synchronous Gastric Carcinoid and Gastric Adenocarcinoma with Plummer Vinson Syndrome: A Case Report and Literature Review." Annals of Gastroenterology. 22.1 (2009): 52-55. Web. 3 Dec. 2011. . Read More