title: Robin sequence: from diagnosis to development of an effective management plan.
abstract: The triad of micrognathia, glossoptosis, and resultant airway obstruction is known as Robin sequence (RS). Although RS is a well-recognized clinical entity, there is wide variability in the diagnosis and care of children born with RS. Systematic evaluations of treatments and clinical outcomes for children with RS are lacking despite the advances in clinical care over the past 20 years. We explore the pathogenesis, developmental and genetic models, morphology, and syndromes and malformations associated with RS. Current classification systems for RS do not account for the heterogeneity among infants with RS, and they do not allow for prediction of the optimal management course for an individual child. Although upper airway obstruction for some infants with RS can be treated adequately with positioning, other children may require a tracheostomy. Care must be customized for each patient with RS, and health care providers must understand the anatomy and mechanism of airway obstruction to develop an individualized treatment plan to improve breathing and achieve optimal growth and development. In this article we provide a comprehensive overview of evaluation strategies and therapeutic options for children born with RS. We also propose a conceptual treatment protocol to guide the provider who is caring for a child with RS.
authors.affiliation: Children's Craniofacial Center, Seattle Children's Hospital, Box 5371, M/S W7847, Seattle, WA 98145, USA. kelly.evans@seattlechildrens.org
authors.forename: Kelly N
authors.initials: KN
authors.lastname: Evans
authors.forename: Kathleen C
authors.initials: KC
authors.lastname: Sie
authors.forename: Richard A
authors.initials: RA
authors.lastname: Hopper
authors.forename: Robin P
authors.initials: RP
authors.lastname: Glass
authors.forename: Anne V
authors.initials: AV
authors.lastname: Hing
authors.forename: Michael L
authors.initials: ML
authors.lastname: Cunningham
mesh_terms: D000402:Airway Obstruction / Q000175:diagnosis / Q000628:therapy; D019090:Case Management / Q000458:organization & administration*; D002657:Child Development / Q000502:physiology; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008844:Micrognathism / Q000175:diagnosis / Q000628:therapy; D019647:Oral Surgical Procedures / Q000379:methods; D010348:Patient Care Team; D010855:Pierre Robin Syndrome / Q000175:diagnosis* / Q000601:surgery / Q000628:therapy*; D011379:Prognosis; D018570:Risk Assessment; D014060:Tongue Diseases / Q000175:diagnosis / Q000628:therapy; D014140:Tracheotomy / Q000379:methods; D016896:Treatment Outcome