Plummer Vinson Syndrome: A Case Report And Literature Review

摘要

Plummer-Vinson also known as sideropenic dysphagia is a disease that is characterized by chronic iron deficiency anemia, dysphagia and esophageal web. It is known to affect white female mainly but cases have been reported from other ethnic group in the literature. Treatment management is very promising when carcinoma is not involved. Introduction Plummer-Vinson Syndrome (PVS) was first described by Patterson and Kelly in 1919.1 The syndrome consist of dysphagia, atrophic oral mucosa, glossitis and anemia, and most of the patient affected are post meno-pausal women. Other presenting symptoms may include cracks or fissure at the corners of the mouth along with painful tongue. Koilonychia (spoon shaped finger nails) or nails that are brittle and break easily.2,3 In approximately 10% of cases , this uncommon syndrome is associated with hypopharyngeal, esophageal or oral cavity carcinoma which emanates from the degenerative changes in the mucosae of the oral cavity, pharynx, and esophagus. 4,5,6 The aim of this report is to highlight the unique presentation of almost all the clinical features attributable to this syndrome along with a concurrent tongue carcinoma in one patient, and to share our experience in the diagnosis and management of this case. Case Report A 27 year old Saudi female presented to the Emergency Department of the King Fahad Hospital at Al-Baha , Saudi Arabia on the 12th of June 1995 with a six weeks history of gradual difficulty in swallowing liquids and a tongue ulcer. The patient had an associated history of mental retardation, speech impairment and easy fatiquability. Prior to the onset of the illness, the patient was reported to be ambulant.Clinical Examination Patient physically looked emaciated, severely dehydrated, pale and lethargic. She weighed 29 kg and her temperature (oral) was 39.8°C. Blood pressure was 90/60 mmHg; Pulse 120/min and respiration was 22/minute. There was bilateral angular stomatitis with epithelial crust on the lips.The skin appeared generally dry and the finger nails were spoon shaped (figs 1 &2 ). There was a small discrete jugulodigastric node palpable on the right side of the neck. Chest auscultation revealed a normal heart sounds and bilateral basal crepitation in the lungs. The abdomen was soft, non tender and the liver, spleen and kidneys were not palpably enlarged. Intra-orally the oral mucosa was dry with thick patches of saliva. The tongue demonstrated a crater-like ulcer on the right side extending from the anterior third to the base.(fig 3)Laboratory And X-RayAdmitting laboratory values revealed a WBC of 10.5 x 109/L., RBC 4.34; Hemoglobin 7.6 gm/dL., hematocrit 23.8%, MCV 54.8; MCH 17.5 and serum ferritin of 38mg/dL. Blood chemistry revealed an elevated urea at 1.7 mmol/L., and creatinine of 117 mmoL/L. Chest x-ray showed scattered bronchopneumonic changes in the mid-and lower zones.The patient was admitted to the Oral & Maxillofacial service with a diagnosis of severe dehydration, dysphagia, and queried advance carcinoma of the tongueHospital Course On the first day of admission a rehydration measure was commenced with close monitoring of fluid imput and output. The on-call internist reviewed the patient and place her on combination of parentheral Benzyl Penicillin 2 gm qid, ceftazidime 1 gm q6h, and ferrous sulphate supension 300mg twice daily. Twenty four hour fluid intake was 240°Cc and the output stood at 50°Cc.Patient was transferred to the internal medicine service after 48 hour and was followed by the oral surgery unit. Culture swab from the tongue was taken and sent for AFB, mycology and routine culture and sensitivity. All were negative. Attempt to pass a NG tube to facilitate feeding and instillation of contrast medium for esophagograph failed ( fig 3 ) On the fourth day of admission 2 units of packed cell was transfused to boost the hemoglobin. General condition seemed to improve as patient was becoming more responsive to verbal dialogue and was also able to s