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

Journal of Stomatology, Oral and Maxillofacial Surgery
A GiudiceA Picard

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.

Citations

Nov 15, 2019·The Journal of Craniofacial Surgery·Fatma Nihal Durmus KocaaslanÖzhan Çelebiler
Jul 16, 2020·Birth Defects Research·Kurt ReynoldsChengji J Zhou
Jul 14, 2019·Disease Models & Mechanisms·Regina M FriedlLisa L Sandell
Nov 11, 2020·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Floriane RemyLionel Thollon
Jan 3, 2021·NeoReviews·Stefanie RiddlePaul Kingma
Sep 9, 2020·Journal of Stomatology, Oral and Maxillofacial Surgery·Selene BaroneAmerigo Giudice
Jun 17, 2021·Paediatric and Perinatal Epidemiology·Michele SantoroEster Garne
Jul 14, 2021·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·Austin S LamJohn P Dahl

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