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Pierre Robin sequence: A comprehensive narrative review of the literature over time.

Abstract：

:Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form. Because of the clinical importance of PRS, it's fundamental to describe the features of the Robin sequence to clearly define its primary and secondary clinical signs useful to diagnosis. A complete evaluation of the syndrome allows choosing the most appropriate therapeutic treatment, opting for conservative or surgical management, in order to improve the quality of life of the patient.

journal_name

J Stomatol Oral Maxillofac Surg

journal_title

Journal of stomatology, oral and maxillofacial surgery

authors

Giudice A,Barone S,Belhous K,Morice A,Soupre V,Bennardo F,Boddaert N,Vazquez MP,Abadie V,Picard A

doi

10.1016/j.jormas.2018.05.002

subject

Has Abstract

pub_date

2018-11-01 00:00:00

pages

419-428

issue

5

eissn

2468-8509

issn

2468-7855

pii

S2468-7855(18)30118-6

journal_volume

119

pub_type

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