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

10. Cheilitis (angular stomatitis)

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

12. Koilonychia (due to low O2 saturation)

13. Post cricoid webs on endoscopy (Pathognomonic)

14. Enlarged nodular thyroid glands

16. Differential Diagnosis
• Causes of dysphagia especially malignant tumors, benign strictures,
esophageal rings or Zenkers diverticula
• Motility 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)

17. Investigations
Complete blood cell (CBC) counts: decreased values of hemoglobin,
hematocrit, mean corpuscular volume
Peripheral blood smears: hypochromic microcytic anemia
Iron studies: decreased values of serum iron and ferritin, and increased
total iron binding capacity

18. Peripheral Blood Smear

20. 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

21. 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

22. Barium swallow x-ray on lateral view

23. Videofluoroscopy

24. Treatment

25. 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.

26. Upper GI Endoscopy

27. Bougie Dilatation

28. 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.
• Follow-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 dilations those who have recurrent dysphagia.

29. Prognosis
• Prognosis of the Plummer-Vinson syndrome is excellent.
• Dysphagia 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.

30. Therefore...

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

32. 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 :)