Open AccessThe Early Diagnosis of the Marfan Syndrome: What is the Role of the Pediatric Dentist?
Author(s) -
chayma ben arbia,
Chalbi Manel,
Zaroui Jihène,
azi Imene,
Chemli Med ALI
Publication year - 2022
Publication title -
eas journal of dentistry and oral medicine
Language(s) - English
Resource type - Journals
eISSN - 2663-7324
pISSN - 2663-1849
DOI - 10.36349/easjdom.2022.v04i01.007
Subject(s) - pathognomonic , marfan syndrome , medicine , nosology , pediatrics , genetic disorder , dermatology , disease , intensive care medicine , pathology , surgery
Marfan syndrome (MIM 154700) is a variable, autosomal dominant disorder of connective tissue whose cardinal features affect the cardiovascular system, eyes and skeleton. The diagnosis of Marfan syndrome (MFS) relies on defined clinical criteria (Ghent nosology), outlined by international expert opinion to facilitate accurate recognition of this genetic aneurysm syndrome. The patient with MFS has multiple oral decrease that may be diagnosed and treated on time to increase the life quality of the patient. Treatment planning is related to the age of the patient, the type and severity of the disorder, and the oral health of the patient. AIM: The aim of this article was to describe through two cases the orofacial manifestations of Marfan syndrome and to demonstrate the role of the pediatric dentist in the early diagnosis of this genetic defect from the oral pathognomonic signs of this syndrome and and the therapeutic management of those children.