Plummer Vinson Syndrome (Pattern Brown Kelly Syndrome)

 

Mr. D. Muthukumaran¹, Dr. Danasu², Dr. Sridevi³

¹Assistant Professor, Department of Medical Surgical Nursing,

Sri Manakula Vinayagar Nursing College, Puducherry.

²Principal, Sri Manakula Vinayagar Nursing College, Puducherry.

³Associate Professor, Department of Medical Surgical Nursing,

Sri Manakula Vinayagar Nursing College, Puducherry.

 

ABSTRACT:

Pattern brown Kelly syndrome is characterized by web formation in the region of cricopharynx the prevalence of post cricoid webs was 0.3%-1.1% in women overall. It is characterized by iron deficiency anemia and dysphagia. Patients usually have koilonychias, atrophic gastritis and glossitis. The cause of disease is unknown, genetic, nutritional deficiency, its gradually lead to thinning of mucosa followed by atrophy of the mucosa and subepithelial fibrosis, which may responsible for web formation the symptoms may be difficulty in swallowing initially for solids followed by for liquids too, difficulty in breathing, tachycardia, stomatitis, glossitis. It can be diagnosed through Hemogram, serum iron, barium swallow and oesophagoscopy to rule out the malignancy. That should be treated with supplemental irons and vitamins and excision of web.

 

 

INTRODUCTION:

Plummer Vinson syndrome is characterized by web formation in the region of cricopharynx associated with progressive hypochromic microcytic anemia, angular stomatitis and superficial glossitis commonly seen in females. [5]

 

EPIDEMIOLOGY:

Epidemiological data about incidence and prevalence for Plummer-Vinson syndrome are not widely available. A single population-based study conducted in the 1960s in South Wales assessed the prevalence of post-cricoid webs was 0.3%-1.1% in women overall, and 8.4%-22.4% in women who had preexisting dysphagia, none of the men were found to have the post-cricoid web. [2]

 

DEFINITION:

This premalignant condition is the disease of females. It is characterized by iron deficiency anemia and dysphagia. Patients usually have koilonychias, atrophic gastritis and glossitis. [1]

 

CAUSES:

1.     Unknown

2.     Genetic

3.     Nutritional deficiencies

 

 

PATHOLOGY:

A chronic superficial pharyngoesophageitis with sub-epithelial inflammation

 

Gradually lead to thinning of mucosa followed by atrophy of the mucosa and subepithelial fibrosis, which may responsible for web formation

 

At an early stage there is some spasm of the cricopharyngeous sphincter, but stenosis occur later

 

Thinning of the mucosa of the upper alimentary tract with the loss of the rete pegs and reduction or absence of glycogen in the cells

 

This leads to stomatitis, glossitis [5]

 

SYMPTOMS:

1.     Difficulty in swallowing initially for solids followed by for liquids too.

2.     Difficulty in breathing

3.     Tachycardia

 

GENERAL EXAMINATION:

1.     Patient will be pale

2.     Koilonychias (spoon – shaped nails)

 

EXAMINATION OF THE ORAL CAVITY:

1.     Angular stomatitis

2.     Superficial glossitis

3.     Ulceration in the oral cavity

 

EXAMINATION OF THE HYPOPHARYNX:

1.     Pooling of saliva in the pyriform fossa

2.     Associated with post cricoid malignancy                 [5]

INVESTIGATION:

1.     Hemogram reveals hypochromic, microcytic anemia

2.     Serum iron level is reduced

3.     Low mean corpuscular haemoglobulin concerntration (MCHC)

4.     Total iron binding capacity is raised in 50% of patients

5.     Vitamin B12 absorption is reduced in 20% of the patients

6.     Barium swallow demonstrates web formation in the cricopharyngeal region

7.     Flexible oesophagosopy demonstrates the web formation and to rule out the presence of malignancy.

8.     CT scan with contrast in suspected cases of post cricoid malignancy.

 

 

OTHER METABOLIC ABNORMALITIES PRESENT ARE:

1.     Pernicious anemia

2.     Pyridoxine deficiency

3.     Abnormal tryptophan metabolism                            [5]

 

DIFFERENTIAL DIAGNOSIS:

1.     Post cricoid neoplasm to be excluded

2.     Functional dysphagia (globus pharyngeus)

3.     Gastroesophageal reflux disease

4.     Achalasia

5.     Tracheo oesophageal fistula                                       [3]

 

TREATMENT:

1.     Correct anemia with iron supplemental with vitamins (150mg – 200mg). If the haemoglobin level is less than 8gm, injectable iron is preferred and if the level is more than 8g then oral iron tablets are preferred. B12 and B6 deficiency is also corrected.

2.     Excision of the web followed by biopsy by rigid esophagoscopy. In case of reccurent dysphagia, bougie dilatation is performed periodically till the symptoms improve. [5]

 

COMPLICATIONS:

Squamous cell cancer of oesophagus, obstruction of lumen and persistent oesophagus [3]

 

NURSING MANAGEMENT:

1.     Assess the nutritional pattern to rule out anemia.

2.     Provide fowler position to reduce the level of dyspnea.

3.     Assess the swallowing pattern.

4.     Administer iron as per the physician order.

5.     Advice the patient to come for the regular checkup.

 

REFRENCES:

1.     PL. Dhingra, Textbook of diseases of Ear, nose, and throat and head and neck surgery, 7^th edition, Elsevier publications pg.no 354.

2.     Elwood pc, jacobs a, pitman rg, entwistle cc. Epidemiology of the paterson-kelly syndrome. Lancet. 1964 oct 03;2(7362):716-20. PubMed

3.     Messmann H. Squamous cell cancer of the oesophagus. Best Practice Res Clinical Gastroenterology. 2001 Apr; 15(2):249-65. PUBMED

4.     Mohan bansal, Textbook of diseases of Ear, Noseand throat, jaypee publications, page.no. 388-389.

5.     Zakir hussain, Textbook of otorhinolaryngology, 4^th edition, paras medical publisher, page.no. 832-833.

 

Received on 06.01.2020          Modified on 29.02.2020

Accepted on 19.04.2020     © AandV Publications all right reserved