The Plummer-Vinson syndrome: a Moroccan single center experience of 96 patients

Aims The aim of our study is to describe the clinical profile, treatment characteristics and outcomes, while exploring the associations with certain autoimmune diseases .

Methods This is a retrospective single center study which collated all cases of PVS diagnosed and treated in our ​​department of Hepato-Gastroenterology C at Ibn Sina hospital in Rabat over 18 years (from 2005 to 2023).

Results There were a total of 96 patients, with an average age of 41.6 years and extremes ranging from 17 to 90 years. A clear female predominance was observed, accounting for 80% (n=77).

Clinically, 97.9% (n=94)of patients presented dysphagia as the main symptom, associated with a clinical anemia syndrome in 51% (n=49), weight loss in 17.7% (n=17), and odynophagia in 3% (n=3).

Biological tests revealed an iron-deficiency anemia in all patients (n=96).All patients underwent oeso-gastro-duodenal fibroscopy, which revealed a membranous ring localized below the cricopharyngeal muscle in 44 cases , and the upper third of the esophagus in 34 cases .

The ring was impassable in more than half of our patients 56.25% (n=54).

All patients underwent dilatation that has been performed by Savary-Gilliard candles or hydrostatic balloons associated with a martial supplementation.

The majority, 87.5% (n=84) had a favorable outcome with a clinical improvement on short and medium term, with a median follow-up of 9 months.

One single ring was found in 98.9% (n=95)of patients , while one patient had an association of 2 rings with a lower esophageal ring with a small sliding hiatal hernia corresponding to a schatzki ring.

However, 26% (n=25) of patients presented a recurrence of dysphagia, requiring a second dilatation session, the average time to clinical recurrence was 7 months+/-.

Autoimmune associations were identified, including celiac disease in 4 patients, Crohn's disease in 2 patients, autoimmune bullous dermatosis in 1 patient and type 1 diabetes in 1 patient.

Five patients developed squamous cell carcinoma. Three of them were discovered at the time of diagnosis of the ring, and 2 were diagnosed during endoscopic follow up after 8 years

Conclusions PVS is relatively rare, affecting mainly female individuals. Although endoscopic dilation has proven effective in most cases, in our study,the recurrence interested less than a third of patients (n=25). Regular monitoring is important because of the risk of degeneration and the high frequency of associated autoimmune diseases. However, correction of the iron-deficiency anemia remains crucial to prevent any relapse of the disease.

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Artikel online veröffentlicht:
15. April 2024