A GIRL WITH DYSPHAGIA.
INTRODUCTION Dysphagia is an agonizing symptom for the patient, which requires thorough clinical evaluation and treatment. We present one such case of dysphagia managed at our hospital. CASE REPORT A 22-year-old girl presented in medical out patient department with history of difficulty in swallowing for both solids and liquids. Her illness started about 4 years earlier when she noticed stickiness of food in the region of lower neck .This symptom was observed intermittently over the ensuing 5-6 months. Occasionally she would require sips of water to engulf the food properly. After 6 months the symptom progressed in severity and she would require a glass of water each time while taking solid food. As the symptom worsened further, gradually, she would keep the morsel of food in her mouth chewing it for prolonged periods reducing its thickness, thus making it as much edible as possible. Even then she would require a glass of water with every morsel of food that she would eat.
Subsequently she developed hesitation for food and her appetite reduced to half a piece of bread along with difficulty in swallowing of liquids as well. Off and on she would develop soreness at the corner of her mouth which would improve either spontaneously or with medication. There was no history of abdominal pain, nasal regurgitation of liquids, joint pains, rash on body, cough, loose stools or menstrual irregularities. Her first Barium Swallow was performed 3 months after the onset of symptoms but it did not reveal any abnormality. She was evaluated with other routine investigations and offered some treatment but it did not improve her symptoms. Her examination at the time of current visit revealed pallor, koilonychia and angular stomatitis. Her indirect larryngoscopy performed by ENT specialist did not reveal any abnormality. Her investigations showed a Hemoglobin value of 8.9 g/dL with MCV of 64.1fl and hypochromic microcytic blood picture and low ferritin level.
Rest of the biochemical profile and stool examination was normal. Her repeat Barium swallow with 250% v/w barium sulphate showed a shelf like infolding of mucosa protruding in the lumen from the wall of upper part of cervical oesophagus suggestive of oesophageal web, rest of mid and lower oesophagus was normal .Her upper gastrointestinal endoscopy was performed which confirmed the finding of Oesophageal web. A combination of angular stomatitis, oesophageal web and iron deficiency anemia established the diagnosis of Plummer Vinson Syndrome. An oesophageal dilatation was performed during the same procedure. Her symptoms improved considerably following the procedure and she was able to eat comfortably. She was started on oral iron supplements and her hemoglobin improved to 11.1g/dL one month after the therapy. She was advised regular follow up and continuation of iron supplements till the iron stores are replenished as monitored with serum ferritin levels.
DISCUSSION The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-Brown Kelly syndrome in the United Kingdom. The term sideropenic dysphagia has also been used, since the syndrome can occur with iron deficiency (sideropenia), but it is not associated with anemia.1 The pathogenesis of PVS remains speculative. Postulated aetiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others. The prevalent iron deficiency theory remains controversial. The depletion of iron-dependent oxidative enzymes may produce myasthenic changes in muscles involved in the swallowing mechanism, atrophy of the esophageal mucosa, and formation of webs as epithelial complications. PVS has also been viewed as an autoimmune phenomenon.
The syndrome has been associated with autoimmune conditions, such as rheumatoid arthritis, pernicious anemia, celiac disease, and thyroiditis. In one study, a significantly higher proportion of patients with PVS had thyroid cytoplasmic autoimmune antibodies compared to controls with iron deficiency. The autoimmune theory, however, has gained little acceptance to date1. Differential diagnosis includes oesophageal cancer, hypopharyngeal cancer, pill induced stricture and inflammatory conditions associated with webs e.g. blistering skin diseases. Conventional barium radiographs remain the diagnostic investigation of choice, in doubtful cases video swallow study can be performed which can distinguish between subtle true webs and web like formations due to insignificant mucosal foldings and submucosal phenomena.2,3. A thin web can be easily ruptured and can go unnoticed during passage of the endoscope into the upper esophagus.
If webbing is suspected, the endoscope should be advanced carefully under direct vision through the upper esophageal sphincter. The web typically appears as a thin mucosal membrane covered by normal squamous epithelium. Most webs are located along the anterior esophageal wall in the shape of a crescent, a few are concentric. Upper esophagus should be carefully examined for presence of an inlet patch. The differential diagnosis at endoscopy should also include inflammatory or infectious stricture, postcricoid cancer, or extrinsic compression from a submucosal venous plexus. Potential causes of iron deficiency anemia may be found, including Cameron erosions within a large diaphragmatic hernia or angioectasias. It is also prudent to obtain small bowel biopsies at the time of endoscopy to exclude villous atrophy from celiac sprue as the cause of iron deficiency anemia4. Iron replacement is necessary to correct the anemia, if present, and to resolve most of the physical signs of iron deficiency.
The necessity for continued iron treatment is doubtful other than for anemia correction. Dysphagia may improve with iron replacement alone, particularly in patients whose webs are not substantially obstructive. Dysphagia caused by more advanced webs is unlikely to respond to iron replacement alone and, thus, is managed with mechanical dilation5.
1. Atmatzidis K, Papaziogas B, Pavlidis T, Mirelis Ch, Papaziogas T. Plummer-Vinson syndrome. Dis Esophagus. 2003;16: 2: 154-7.
2. Bredenkamp JK, Castro DJ, Mickel RA. Importance of iron repletion in the management of Plummer-Vinson syndrome. Ann Otol Rhinol Laryngol. Jan 1990;99: 1: 51-4.
3. Chen TS, Chen PS. Rise and fall of the Plummer-Vinson syndrome. J Gastroenterol Hepatol. Nov-Dec 1994;9: 6: 654-8.
4. Dantas RO, Villanova MG. Esophageal motility impairment in Plummer-Vinson syndrome. Correction by iron treatment. Dig Dis Sci.May 1993; 38: 5: 968-71.
5. Plummer Vinson Syndrome by Louis Michel Wong Kee Song (updated April 2008). Accessed August 12, 2008, at http://www.emedicine.org.com.
6. Slater SD. The Brown Kelly-Paterson or Plummer-Vinson syndrome: an old score finally settled. J R Coll Physicians Lond. 1991;25: 3: 257-8.
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|Publication:||Pakistan Armed Forces Medical Journal|
|Article Type:||Clinical report|
|Date:||Sep 30, 2010|
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