title: Pierre Robin sequence: Management of respiratory and feeding complications during the first year of life in a tertiary referral centre.
abstract: OBJECTIVE
  To review early clinical manifestations of Pierre Robin sequence (PRS) and their management during the first year of life in the University Hospitals Leuven.
  
  
  METHODS
  Retrospective series of 48 patients with PRS born between 2001 and 2011 and treated at a tertiary referral hospital. Review of the current literature about management of respiratory and breathing difficulties in the early life of PRS patients.
  
  
  RESULTS
  Of our cleft palate patients 15.3% presented with PRS. A syndrome was diagnosed in 14.6%, associated anomalies without a syndromic diagnosis in 56.3% and isolated PRS in 29.2% of the cases. Mortality rate directly related to PRS was 2.1%. Respiratory difficulties were observed in 83.3% and feeding difficulties in 95.6% of the patients. Respiratory problems were addressed in a conservative way in 75%, in a non-surgical invasive way in 42.5% and in a surgical way in 12.5%. A statistically significant relationship between the association of a syndrome or other anomalies, and a higher need for resuscitation and invasive treatment were found (chi-square test, p-values=0.019 and 0.034). Feeding difficulties were managed conservatively in 91.3%, invasively in 80.4% and surgically in 15.2%.
  
  
  CONCLUSIONS
  PRS is frequently associated with other abnormalities or syndromes. Therefore routine screening for associated anomalies in neonates with PRS is recommendable. Respiratory and feeding complications are highly frequent and possibly severe, particularly in patients with associated anomalies or syndromes, and should be recognized and addressed appropriately in an early stage. There is a potential role for the nasopharyngeal airway in reducing the need for the more traditional surgical interventions for respiratory problems.
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, Kapucijnenvoer 33, 3000 Leuven, Belgium. Electronic address: maaike.rathe@uzleuven.be.
authors.forename: M
authors.initials: M
authors.lastname: Rathé
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Department of Neonatology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: M
authors.initials: M
authors.lastname: Rayyan
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Department of Oromaxillofacial Surgery, University Hospitals Leuven, Leuven, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Schoenaers
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Department of Oromaxillofacial Surgery, University Hospitals Leuven, Leuven, Belgium.
authors.forename: J T
authors.initials: JT
authors.lastname: Dormaar
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Department of Speech and Language Therapy, University Hospitals Leuven, Leuven, Belgium.
authors.forename: M
authors.initials: M
authors.lastname: Breuls
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Department of Oral Health Sciences, University Hospitals Leuven, Leuven, Belgium.
authors.forename: A
authors.initials: A
authors.lastname: Verdonck
authors.affiliation: Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Centre for Human Genetics, University Hospitals Leuven, Leuven, Belgium.
authors.forename: K
authors.initials: K
authors.lastname: Devriendt
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, Kapucijnenvoer 33, 3000 Leuven, Belgium; Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium.
authors.forename: V Vander
authors.initials: VV
authors.lastname: Poorten
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, Kapucijnenvoer 33, 3000 Leuven, Belgium; Multidisciplinary Cleft Lip and Palate Team, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium.
authors.forename: G
authors.initials: G
authors.lastname: Hens
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000628:therapy*; D002972:Cleft Palate / Q000453:epidemiology; D003680:Deglutition Disorders / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010855:Pierre Robin Syndrome / Q000150:complications* / Q000453:epidemiology*; D015995:Prevalence; D012189:Retrospective Studies; D062606:Tertiary Care Centers
keywords: Cleft palate; Feeding difficulties; Micrognathia; Nasopharyngeal airway; Pierre Robin sequence; Respiratory problems