Plummer Vinson Syndrome

About Plummer Vinson Syndrome

1. Plummer-Vinson Syndrome Madhu Vamsi G

2. But why?

3. Definition Plummer-Vinson syndrome (PVS) is a classical triad of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia . Identification and follow-up is relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx.

4. Also known as Plummer-Vinson syndrome (USA) : Named after Henry Stanley Plummer and Porter Paisley Vinson who were physicians on the staff of the Mayo Clinic. Paterson-Kelly / Paterson-Brown Kelly syndrome (UK) : Named after Donald Ross Paterson and Adam Brown-Kelly, both British laryngologists, who published their findings independently in 1919. Sideropenic dysphagia : Because the syndrome can occur with iron deficiency (sideropenia).

5. Risk Factors RACE: Whites & Scandinavians (Norway, Sweden, Finland) AGE: 40-70 years SEX: Women are at higher risk than men. Menstrual blood loss (as much as 200-500 cc per month) might explain the sex ratio (higher rate of sideropenic anemia among women), but Plummer-Vinson syndrome is more likley to occour postmenopausal women. So there might be also a different role of female hormones.

6. Pathophysiology Basically unclear. Proposed mechanisms include : iron deficiencies , causes may include GI blood loss, celiac sprue nutritional deficiency - vitamin B complex genetic predisposition autoimmunity , causes may include rheumatoid arthritis, pernicious anaemia, thyroiditis

7. Clinical Features Symptoms: Dysphagia, is typically intermittent and limited to solids. Odynophagia (painful swallowing, also called algiaphagia) Choking spells and aspiration may occur because of the proximal location of the web. Weakness, fatigue and dyspnea are secondary to iron deficiency anemia. Weight loss is uncommon.

8. Signs: Manifestations of iron deficiency (with or without anemia) include: Angular stomatitis Glossitis Koilonychia (spoon nails) Pallor Others include: Splenomegaly Edentia (loss of teeth) - due to esophagial relux Enlarged nodular thyroid glands Gastritis with Acholorhydia Post cricoid webs

9. Cheilitis (angular stomatitis)

10. Atrophic Glossitis burning sensation of the tongue, and atrophy of lingual papillae produces a smooth, shiny red tongue dorsum

11. Koilonychia (due to low O2 saturation)

12. Post cricoid webs on endoscopy (Pathognomonic)

13. Enlarged nodular thyroid glands

14. Differential Diagnosis C auses of dysphagia especially malignant tumors, benign strictures , esophageal rings or Zenkers diverticula M otility disorders such as achalasia, spastic motility disorders, scleroderma, diabetes mellitus, gastroesophageal reflux disease Heterotopic gastric mucosa (inlet patch) with stricture Hypopharyngeal bar (to differentiate from a web) Inflammatory conditions associated with web formation ( blistering skin diseases, chronic graft versus host disease)

15. Investigations C omplete blood cell (CBC) counts : d ecreased values of hemoglobin, hematocrit, mean corpuscular volume P eripheral blood smears : hypochromic microcytic anemia I ron studie s: decreased values of serum iron and ferritin, and increased total iron binding capacity

16. Peripheral Blood Smear

17. Histopathology esophageal webs appear smooth, thin, and gray with eccentric or central lumen characterized by one or more thin horizontal membranes consisting of squamous epithelium and submucosa occur in the proximal part of the esophagus protrude from the anterior wall, extending laterally but not to the posterior wall

18. Barium Oesophagraphy: Conventional barium swallow may detect the web, which characteristically appears as a thin projection off the postcricoid, anterior esophageal wall. On occasion, multiple webs can be observed. Videofluroscopy : If the conventional barium swallow findings are negative or equivocal and a high index of suspicion remains, a video swallow study is performed. The latter can distinguish subtle true webs from weblike formations (false webs) due to insignificant mucosal foldings and submucosal phenomena. Upper GI Endoscopy

19. Barium swallow x-ray on lateral view

20. Videofluoroscopy

21. Treatment

22. Treat iron deficiency and its underlying cause Iron replacement (Ferrous Sulphate) Address the cause of iron deficiency (celiac sprue, bleeding, malignancy) Treat dysphagia and the web Mechanical dilation by upper endoscopy Bougie (eg, Savary dilator) ND:YAG laser therapy Needle-knife electroincision Diet Eat slowly and chew thoroughly. Solid foods cut in small pieces, especially meats.

23. Upper GI Endoscopy

24. Bougie Dilatation

25. Long-term monitoring Due to increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely. A surveillance upper gastrointestinal endoscopy is recommended every year . F ollow-up complete blood cell counts (CBCs) and iron studies in 3 months, after initiation of iron replacement, to document resolution of sideropenia and anemia. Provide follow-up care for patients whose treatment has been initiated for the specific cause . Repeat esophageal dilation s those who have recurrent dysphagia.

26. Prognosis Prognosis of the Plummer-Vinson syndrome is excellent. D ysphagia and anemia can be treated effectively. Complications: In case of an associated squamous cell carcinoma of the hypopharynx or upper esophagus the prognosis worsens dramatically.

27. Therefore...

28. Henry Stanley Plummer Also known for Plummer's Nail (onchylosis - separation of nail from nailbed) in thyrotoxicosis.

29. It is in the nature of surgery that you are gonna screw up. You are gonna kill someone. If you can't handle that reality, pick another profession. Or finish medical school and teach. Thank You :)