A Simple Guide to Plummer-Vinson Syndrome, Diagnosis, Treatment and Related Conditions

This book describes Plummer-Vinson Syndrome, Diagnosis and Treatment and Related Diseases

Plummer-Vinson syndrome (PVS) is a rare disorder featured by the conventional triad of:

1. dysphagia,
2. iron-deficiency anemia, and
3. esophageal web.

The significance of Plummer-Vinson Syndrome is the relationship of long-term (chronic) iron deficiency anemia with dysphagia.

The incidence of Plummer-Vinson Syndrome has declined due to improvement of nutrition world wide.

The precise cause of Plummer-Vinson syndrome is not known.

While genetic predispositions and several other mechanisms have been suggested, the evidence is weak, even though iron deficiency seems to consistently have an important part to play.

This is partly related to studies that have documented an improvement in dysphagia with iron supplements, while iron deficiency is suspected of inducing mucositis leading to web production.

Since patients with Plummer Vinson syndrome may also experience malnutrition, deficiency of vitamin B has also been indicated as a cause, even though the evidence is weak and inconclusive.

Other disorders documented to be linked with Plummer Vinson Syndrome are:

1. Celiac disease,
2. Crohn disease,
3. Rheumatoid arthritis, and
4. Thyroid disease

This raises the possibility that immune dysregulation may be affected in its pathogenesis, even though this has not been proven

It is a rare disorder that can be linked to cancers of the esophagus (gullet) and pharynx (throat).

The precise pathogenesis of PVS and the production of the esophageal web are not well known.

It has been suggested that iron deficiency causes iron-dependent enzyme dysfunction, resulting in oxidative stress and DNA injuries in the epithelia of the esophageal mucosa.

Recurring injury to epithelia due to iron deficiency causes atrophy of mucosa and degradation of pharyngeal muscles, resulting in the development of esophageal webs.

The esophageal web is sited below the cricopharyngeal muscle and is asymmetrically connected to the anterior esophageal wall.

The esophageal web forms a thin mucous membrane on the wall, composed of squamous epithelia.

Upon biopsy, no inflammatory infiltrates are detected.

Iron deficiency has been reported to induce a decrease in contraction amplitude of esophageal muscle.

The part of mucosal inflammation and atrophy particularly in the post-cricoid region has been proposed as a factor for the pathogenesis of PVS.

The post-cricoid region endures maximum trauma during swallowing of the solid bolus, resulting in a higher risk of web production.

Long-standing iron deficiency anemia can manifest as:

1. Dyspnea or difficulty breathing,
2. Tachycardia,
3. Weakness,
4. Pallor, and
5. Koilonychia or spoon nails.

Dysphagia is normally painless and slowly progressing, beginning with solid foods and difficulty swallowing liquids after years of first onset.

Dysphagia shows symptoms only when the luminal diameter in the region of the esophageal web is less than 12 mm.

Hematological testing is done to establish the source of iron deficiency and the severity of anemia.

Video-fluoroscopy is normally more dependable for the demonstration of esophageal webs.

It is a dynamic X-ray evaluation that appraises swallowing while the barium bolus travels to the esophagus from the mouth.

Fiber optic endoscopy is the safest and most reliable method for esophageal examination.

Esophagoscopy, endoscopic examination of the esophagus, has the benefit of being both diagnostic and therapeutic at the same time.

Iron replacement and dilation of esophageal webs are the main treatments of Plummer-Vinson Syndrome.

TABLE OF CONTENT
Introduction
Chapter 1 Plummer-Vinson Syndrome

Dettagli