title: Pierre Robin sequence: review of diagnostic and treatment challenges.
abstract: Pierre Robin sequence is not a rare condition and paediatric specialists caring for respiratory related issues are likely to encounter cases in their practice. There have been a few recent reviews on the topic, mostly focusing on the surgical interventions performed for cases with severe airway obstruction. In the present review, we will highlight the different challenges that remain today in the global evaluation of infants afflicted with this condition through a thorough review of the medical literature, giving the clinician a full scope of the disease and of the various management options. The need for an improved objective evaluation of airway obstruction and for a better classification will be emphasized. We are therefore proposing a novel classification scheme that will better account for respiratory and feeding difficulties in these infants. Finally, many knowledge gaps persist regarding this condition, underlining the necessity for further research both in the genetic field and regarding the outcome of therapy.
authors.affiliation: Respiratory Medicine Division, The Montreal Children's Hospital, Montreal, Canada. Electronic address: aurore.cote@muhc.mcgill.ca.
authors.forename: Aurore
authors.initials: A
authors.lastname: Côté
authors.affiliation: Otolaryngology - Head and Neck Surgery Division, The Montreal Children's Hospital, Montreal, Canada.
authors.forename: Amanda
authors.initials: A
authors.lastname: Fanous
authors.affiliation: Respiratory Medicine Division, The Montreal Children's Hospital, Montreal, Canada.
authors.forename: Athari
authors.initials: A
authors.lastname: Almajed
authors.affiliation: Otolaryngology - Head and Neck Surgery Division, The Montreal Children's Hospital, Montreal, Canada.
authors.forename: Yolène
authors.initials: Y
authors.lastname: Lacroix
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000628:therapy; D002648:Child; D006801:Humans; D007223:Infant; D008046:Lip / Q000002:abnormalities / Q000601:surgery; D008297:Male; D019857:Osteogenesis, Distraction; D010855:Pierre Robin Syndrome / Q000175:diagnosis* / Q000628:therapy*; D017286:Polysomnography; D014059:Tongue / Q000002:abnormalities / Q000601:surgery
keywords: Airway obstruction; Classification; Mandibular distraction; Nasopharyngeal airway; Polysomnography; Tongue–lip adhesion