Plummer-Vinson Syndrome

Madhu Vamsi G

But why?

DefinitionPlummer-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.

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).

Risk FactorsRACE: 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.

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

Clinical FeaturesSymptoms:

• 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.

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

Cheilitis (angular stomatitis)

Atrophic Glossitisburning sensation of the tongue, and

atrophy of lingual papillae produces a smooth, shiny red tongue dorsum

Koilonychia (due to low O2 saturation)

Post cricoid webs on endoscopy (Pathognomonic)

Enlarged nodular thyroid glands

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)

InvestigationsComplete 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

Peripheral Blood Smear

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

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

Barium swallow x-ray on lateral view

Videofluoroscopy

Treatment

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.

Upper GI Endoscopy

Bougie Dilatation

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.

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.

Therefore...

Henry Stanley PlummerAlso known for Plummer's Nail (onchylosis - separation of nail from nailbed) in

thyrotoxicosis.

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 :)