Plummer-Vinson Syndrome
Plummer-Vinson Syndrome

Video Endoscopic Sequence 1 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

The gastric cardia seen from an retrogade endoscopy from the hole of surgical gastrostomy performed 16 years previous, The gastrostomy tube is removed. A standard endoscope is passed retrograde to the upper esophageal sphinter.

This is the case of a 65 year-old female who had suffered from a mutilans rheumatoid arthritis since 19. She had difficulty to swallow, and therefore she had not been tasting flavors well during 16 years. She arrived for a second opinion, and on two different clinics they didn´t do an endoscopy becasue of Temporomandibular joint involvement. The only treatment they did was to place a gastrostomy tube to feed her.

Deformity of both hands is observed, the patient had suffered from 19 years of this rheumatoid arthritis,
There destruction of finger bones, due to arthritis of the temporomandibular joint, do not open her jaw, and impossible to do an upper endoscopy, the patient could not wander, comes in a wheelchair, there are deformation on feet, and two previous surgeries were placed prosthetic knees.

For the therapeutic management, we took a conservative approach because of her status and oxygen saturation. However we had intended to try an aggressive plan, like using a hydrostatic dilator and combine it with argon plasma.

Also, because of her arthritis, her temporo-mandibular joint is compromised so the endoscopy was not able to be done properly, so we were planning on doing it through the nasal airways.

The endoscopy was finally done via a gastrostomy fistula. When getting to the superior esophageal sphincter, the inferior part was surrounded with a membrane.


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images.



Endoscopy of Plummer-Vinson Syndrome

Video Endoscopic Sequence 2 of 26.

Endoscopy of Plummer-Vinson Syndrome

Endoscopy of Plummer-Vinson syndrome associated with longstanding and mutilans rheumatoid arthritis

Image and video clip of the upper gastroesophageal sphincter seen by retrograde endoscopy which is avanced from gastrostomy fistula.

Autoimmune Phenomenon
The syndrome has been associated with autoimmune conditions, such as rheumatoid arthritis,
pernicious anemia, celiac disease, and thyroiditis.

One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. The etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

The pathogenesis of PVS remains speculative, and the existence of the syndrome has been challenged. Postulated etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others.


The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-Brown Kelly syndrome in the United Kingdom. is a rare entity.



Plummer Vinson Syndrome

Video Endoscopic Sequence 3 of 26.

Image and Video clip of Plummer Vinson Syndrome


The upper esophageal sphincter, examined from the hole fistula percutaneous gastrostomy performed 16 years earlier.

Gastrointestinal endoscopy detected a web that prevents passage of the endoscope into the upper esophageal sphincter and thus out through the mouth, but

into the upper portion of the esophagus.

The prevalent iron deficiency theory remains controversial. Older reports have implicated iron deficiency in the pathogenesis of esophageal webs and dysphagia in predisposed individuals. The depletion of iron-dependent oxidative enzymes may produce myasthenic changes in muscles involved in the swallowing mechanism, atrophy of the esophageal mucosa, and formation of webs as epithelial complications.

The improvement in dysphagia after iron therapy provides evidence for an association between iron deficiency and postcricoid dysphagia. Anecdotal reports have also been made of patients with PVS exhibiting impaired esophageal motility (with dysphagia) that recovers following iron therapy. Moreover, the decline in PVS seems to parallel an improvement in nutritional status, including iron supplementation.

the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss.



Paterson–Brown–Kelly syndrome

Video Endoscopic Sequence 4 of 26.

Being celiac disease often associated recommend taking multiple biopsies in the duodenum during the procedure.

Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia, is a rare disease characterized by difficulty in swallowing, iron deficiency anemia, and esophageal webs. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome.

Nowadays, this syndrome has become extremely rare.[1] It generally occurs in postmenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx

 




Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 5 of 26.

Endoscopy of Plummer-Vinson Syndrome

We also find a hyperplastic pedunculated polyp in the duodenal bulb with a thick stalk, and multi-lobular surface.

Plummer-Vinson Syndrome: Since it is a premalignant condition association with some cancers of the digestive tract.

Pubmed

Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 6 of 26.

Endoscopy of Plummer-Vinson Syndrome

Hyperplastic pedunculated polyp in the duodenal bulb with a thick stalk, and multi-lobular surface.

In the image and video clip the polyp is seen on etroflexion maneuver in the duodenal bulb.


Endoscopy of Plummer-Vinson Syndrome

Video Endoscopic Sequence 7 of 26.

Endoscopy of Plummer-Vinson Syndrome

Endoscopy documented esophageal ring

in order to try to advance the endoscope some tissue are being manipulated.

Esophageal dysfunction can occur in a variety of musculoskeletal conditions, most notably the autoimmune connective tissue diseases. However, correlation between oesophageal symptoms and investigations is frequently poor. It is often uncertain whether symptoms are caused by the underlying disease or by its treatment. As the primary disease process may not be responsive to treatment, correctable iatrogenic causes should always be sought.

 

 

Plummer Vinson Syndrome

Video Endoscopic Sequence 8 of 26.

Plummer-Vinson Syndrome

Image and video clip of the upper gastroesophageal sphincter seen by retrograde endoscopy from gastrostomy fistula.

Upper gastrointestinal endoscopy showed a severe upper esophageal stricture caused by a smooth mucosal diaphragm.

Esophageal dysfunction can occur in a wide range of musculoskeletal conditions. Symptoms include dysphagia, odynophagia and heartburn.

The esophagus connects the pharynx opposite the sixth cervical vertebra to the cardia, slightly to the left of the eleventh thoracic vertebra. It is fixed above by the cricoid cartilage and below by the diaphragm, the upper end being closed by the pharyngo–esophageal sphincter and the lower end by the lower oesophageal sphincter (LOS). The muscular coat has outer longitudinal and inner circular layers with Auerbach's plexus in between. Both muscle layers are striated in the upper portion of the esophagus.

 





Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 9 of 26.

Endoscopy of Plummer-Vinson Syndrome

Plummer-Vinson (Paterson-Brown-Kelly) syndrome is uncommon nowadays. Although iron repletion improves its symptoms, endoscopic dilatation of associated esophageal webs is sometimes required.

Although dysfunction of the oesophagus is common in autoimmune and inflammatory rheumatic diseases, it is usually asymptomatic. Nevertheless, significant swallowing difficulties can occur and occasionally dysphagia is severe. Many different mechanisms can contribute to dysphagia in the musculoskeletal diseases. However, the cause of oesophageal symptoms is often obscure as they frequently correlate poorly with the findings of investigations.

In a survey of digestive symptoms in connective tissue diseases, one-third of the patients with RA reported dysphagia.



Plummer Vinson Syndrome

Video Endoscopic Sequence 10 of 26.

Plummer-Vinson Syndrome

With the forceps biopsy some membranes are removed

Prognosis of the Plummer-Vinson syndrome is excellent. As described above, dysphagia and anemia can be treated effectively. In case of an associated squamous cell carcinoma of the hypopharynx or upper esophagus the prognosis worsens dramatically.

Laryngeal involvement in patients with RA is invariably underdiagnosed early in the course of the disease in view of the subtle clinical findings. A high index of suspicion is often needed to recognize laryngeal involvement especially in the presence of confounding factors such as laryngopharyngeal reflux disease and allergy. The prevalence of the laryngeal manifestations of RA has been on the rise.


Plummer Vinson Syndrome

Video Endoscopic Sequence 11 of 26.

Plummer-Vinson Syndrome

Disease name and synonyms

Plummer-Vinson syndrome, PVS

Paterson-Kelly syndrome

Paterson-Brown Kelly syndrome

Sideropenic dysphagia



Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 12 of 26.

Plummer-Vinson Syndrome

Back side of the upper esophageal sphincter is observed.

Guia Savary-Gilliard utilizado para introducir un Dilatador Savary-Gilliard

It can move ahead and take it by mouth, a guide wire at its distal end with a flexible spring.

Although correction of iron deficiency may result in resolution of dysphagia and sometimes disappearance of the webs, dilation therapy is usually necessary to remove webs and relieve dysphagia.



Plummer Vinson Syndrome

Video Endoscopic Sequence 13 of 26.

Plummer-Vinson Syndrome

Back side of the upper esophageal sphincter is observed..

Endoscopy image and video clip obtained by endoscopic retrograde via gastrostomy fistula.

Differential diagnosis:

Since dysphagia is a main clinical feature of Plummer-Vinson syndrome, the differential diagnosis includes all other causes of dysphagia especially malignant tumors, benign strictures or esophageal rings. Other reasons for dysphagia are diverticula, motility disorders such as achalasia, spastic motility disorders, scleroderma, diabetes mellitus, gastroesophageal reflux disease, and neuromuscular and skeletal muscle disorders.




Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 14 of 26.

Plummer-Vinson Syndrome

The image and the video display, output savary wire through the mouth, which have passed through the working channel of the endoscope.

After these maneuvers the patient drinks liquids such as milk, We are waiting for he evolution, as due to oxygen saturation problems, we were quite conservative but if dysphagia is not correct, The next plan will use a hydrostatic balloon dilatation or a single or combined therapeutic procedure.

 

Download the video clips by clicking on the endoscopic
image

 

Plummer Vinson Syndrome

Video Endoscopic Sequence 15 of 26.

Plummer-Vinson Syndrome

The Savary guide which have been advanced, in the upper esophageal sphincter and has gone through the mouth.

In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web should be performed. After endoscopic placement of a guidewire, dilators with a diameter of up to 17 mm can be used. Usually only one dilation is enough to relieve dysphagia, but occasionally multiple sessions are required. Also successful balloon dilation has been described. Since the Plummer-Vinson syndrome is associated with an 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, even though the effectiveness of this recommendation is not definitively confirmed.

Endoscopy of Plummer Vinson Syndrome

Video Endoscopic Sequence 16 of 26.

Plummer-Vinson Syndrome

Gastrostomy fistula where retrograde endoscopy was performed

History

The syndrome eponym has been frequently discussed . The most used name is Plummer-Vinson syndrome, named after Henry Stanley Plummer (1874–1936) and Porter Paisley Vinson (1890–1959) who were physicians on the staff of the Mayo Clinic. In 1912, Plummer reported a series of patients with long-standing iron deficiency anemia, dysphagia and spasm of the upper esophagus without anatomic stenosis, which was described as hysterical. In 1919 Vinson reported another case of 'angulation' of the esophagus and attributed the first description of this entity to the earlier report of Plummer. Some years later he published a series of patients with dysphagia who were successfully treated with the passage of bougies; most of the patients were women.

Another term is Paterson-Kelly syndrome, named after Donald Ross Paterson (1863–1939) and Adam Brown-Kelly (1865–1941), both British laryngologists, who published their findings independently in 1919. They were the first to describe the characteristic clinical features of the syndrome. Paterson gave the fullest description but without reference to anemia. He was also the first to draw attention to an association with post-cricoid carcinoma. Brown-Kelly not only described the signs and symptoms of the condition, but also considered anemia.

 

 

Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 17 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

Deformity of both hands is observed, the patient had suffered from 19 years of this rheumatoid arthritis,
There destruction of finger bones, due to arthritis of the temporomandibular joint, do not open her jaw, and impossible to do an upper endoscopy, the patient could not wander, comes in a wheelchair, there are deformation on feet, and two previous surgeries were placed prosthetic knees.

Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology that primarily involves synovial joints. The arthritis is typically symmetrical, and usually leads, if uncontrolled, to destruction of joints due to erosion of cartilage and bone, causing joint deformities. The disease usually progresses from the periphery to more proximal joints and results in significant locomotor disability within 10 to 20 years in patients who do not fully respond to treatment.

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 18 of 26.

Mutilans Rheumatoid Arthritis

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

In Arthritis mutilans, a patient's fingers become shortened by arthritis, and the shortening may become severe enough that the hand looks paw-like, with the first deformity occurring at the interphalangeal and metacarpophalangeal joints. The excess skin from the shortening of the phalanx bones becomes folded transversely, as if retracted into one another like opera glasses, hence the description la main en lorgnette. As the condition worsens, luxation, phalangeal and metacarpal bone absorption, and skeletal architecture loss in the fingers occurs

Arthritis mutilans occurs mainly in patients with psoriasis and rarely advanced rheumatoid arthritis, but can occur independently.

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 19 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

The hallmark feature of rheumatoid arthritis (RA) is persistent symmetric polyarthritis (synovitis) that affects the hands and feet, although any joint lined by a synovial membrane may be involved. The severity of RA may fluctuate over time, but chronic RA most commonly results in the progressive development of various degrees of joint destruction, deformity, and a significant decline in functional status. Extra-articular involvement of organs such as the skin, heart, lungs, and eyes can also be significant.

 

 

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 20 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

Patients with RA may report difficulty performing activities of daily living (ADLs), such as dressing, standing, walking, personal hygiene, or use of hands. In addition to articular deterioration, constitutional symptoms (eg, fatigue, malaise, morning stiffness, weight loss, and low-grade fever) may be present.

In most patients, RA has an insidious onset. It may begin with systemic features (eg, fever, malaise, arthralgias, and weakness) before the appearance of overt joint inflammation and swelling. A small percentage (approximately 10%) of patients with this disease have an abrupt onset with the acute development of synovitis and extra-articular manifestations. Spontaneous remission is uncommon, especially after the first 3-6 months.

 

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 21 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

During the physical examination, it is important to assess the following:

  • Stiffness
  • Tenderness
  • Pain on motion
  • Swelling
  • Deformity
  • Limitation of motion
  • Extra-articular manifestations
  • Rheumatoid nodules

 

 

 

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 22 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

 

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 23 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

Joint involvement is the characteristic feature of RA. In general, the small joints of the hands and feet are affected in a relatively symmetric distribution. In decreasing frequency, the metacarpophalangeal (MCP), wrist, proximal interphalangeal (PIP), knee, metatarsophalangeal (MTP), shoulder, ankle, cervical spine, hip, elbow, and temporomandibular joints are most commonly affected.

Affected joints show inflammation with swelling, tenderness, warmth, and decreased range of motion (ROM). Atrophy of the interosseous muscles of the hands is a typical early finding. Joint and tendon destruction may lead to deformities such as ulnar deviation, boutonniere and swan-neck deformities, hammer toes, and, occasionally, joint ankylosis.

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 24 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

Other commonly observed musculoskeletal manifestations include the following:

Tenosynovitis (defined as inflammation of the tendon and its enveloping tendon sheath) and associated tendon rupture due to tendon and ligament involvement, most commonly involving the fourth and fifth digital extensor tendons at the wrist
Periarticular osteoporosis due to localized inflammation
Generalized osteoporosis due to systemic chronic inflammation, immobilization-related changes, or corticosteroid therapy
Carpal tunnel syndrome
Most patients with RA have muscle atrophy from disuse, which is often secondary to joint inflammation.

 

 

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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 25 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

The boutonniere deformity (see the image below) describes nonreducible flexion at the PIP joint along with hyperextension of the distal interphalangeal (DIP) joint of the finger.

Boutonniere_deformity

Boutonniere Deformity



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Plummer Vinson Syndrome associated with Mutilans Rheumatoid Arthritis

Video Endoscopic Sequence 26 of 26.

Plummer-Vinson Syndrome
associated with Mutilans Rheumatoid Arthritis

The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

 

 

To enlarge image in new window pressing on it.

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