Plummer Vinson Syndrome
Plummer–Vinson syndrome is a rare disease characterized by difficulty swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs.
Causes of Plummer Vinson Syndrome
The cause of Plummer Vinson syndrome is unknown, however, genetic factors and nutritional deficiencies may play a role. It is more common in women, particularly in middle age, with a peak age over 50 years.
Symptoms of Plummer Vinson Syndrome
Dysphagia (difficulty swallowing)
Pain
Weakness
Odynophagia (painful swallowing)
Atrophic glossitis
Angular cheilitis
Koilonychia (Abnormally thin nails, also called spoon nails)
Splenomegaly (an enlarged spleen).
Upper esophageal webs (post cricoid region contrasts with Schatzki rings found at the lower end of esophagus)
Diagnosis of Plummer Vinson Syndrome
The following clinical presentations may be used in the diagnosis of this condition.
Dizziness
Pallor of the conjunctiva and face
Erythematous oral mucosa with burning sensation
Breathlessness
Atrophic and smooth tongue
Peripheral rhagades around the oral cavity
Lab tests
Complete blood cell counts, peripheral blood smears, and iron studies (e.g., serum iron, total iron-binding capacity, ferritin, and saturation percentage) to confirm iron deficiency, either with or without hypochromic microcytic anemia
Imaging
Barium esophagography and video fluoroscopy will help to detect esophageal webs. Esophago-gastroduodenoscopy will enable visual confirmation of esophageal webs. The webs occur due to sub-epithelial fibrosis.
Treatment of Plummer Vinson Syndrome
Treatment is primarily aimed at correcting the iron-deficiency anemia.
Patients with Plummer–Vinson syndrome should receive iron supplementation in their diet.
This may improve dysphagia and pain.
If not, the web can be dilated with esophageal bougies during upper endoscopy to allow normal swallowing and passage of food.
There is risk of perforation of the esophagus with the use of dilators for treatment.