Original Research Cleft Palates and Occlusal Outcomes in Pierre Robin Sequence Otolaryngology– Head and Neck Surgery 1–9 Ó American Academy of Otolaryngology–Head and Neck Surgery Foundation 2018 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599818807918 http://otojournal.org Julie Bao Anh Do, MDCM, FRCS(c) 1 , Audrey Bellerive, DMD, MSc, FRCD(c) 2 , Anne-Sophie Julien, MSc 3 , and Jacques E. Leclerc, MD, FRCS(c) 4 No sponsorships or competing interests have been disclosed for this article. Abstract Objective. To assess the dental class occlusion and lateral cephalometry of children with conservatively treated Pierre Robin sequence (PRS) and to identify associations between these findings and prepalatoplasty cleft palate measurements. Study Design. Retrospective cohort study. Subjects and Methods. Among 22 patients with PRS, the fol- lowing data were prospectively collected: demographics and preoperative cleft palate measurements. After patients reached age 6 years, an orthodontist assessed dental occlusion class and performed a lateral cephalometric analysis. PRS cephalo- metric data were compared with reference population values. Bivariate logistic regression was used to test the association with malocclusion class. Results are presented as odds ratios with 95% profile likelihood confidence intervals. The associa- tion between cleft measurements and cephalometric para- meters was tested with Spearman’s correlation (r s ). Results. All 22 patients had bimaxillary hypoplasia and were prone to hyperdivergency, with a 41% rate of dental class III malocclusion. An increased anterior growth of the still retru- sive mandible mostly accounts for the occurrence of the class III malocclusion in PRS (class II SNB = 74.3° vs class III SNB = 77.6°, P = .04). A larger cleft at the time of the cleft repair (mean, 11 months) was associated with increased mandibular retrusion (smaller SNB angle, r s = 20.5, P = .02). Conclusions. The 41% rate of class III malocclusion among these conservatively treated patients needs to be consid- ered in the choice of the initial airway approach. The future impact of early mandibular advancement will have to be determined. Keywords Pierre Robin sequence, cleft palate, malocclusion, maxillary retrusion, mandibular retrusion, orthognathic surgery, maxil- lary advancement, mandibular advancement. Received April 18, 2018; revised August 30, 2018; accepted September 28, 2018. P ierre Robin sequence (PRS) is defined as mandibular micrognathia, glossoptosis, and cleft palate. It is associ- ated with breathing and feeding difficulties. Various ini- tial airway management plans 1,2 include prone positioning, orthodontic appliances, 3 tongue-lip adhesion, 4 nasopharyngeal intubation, and more invasive options, such as mandibular dis- traction osteogenesis 5,6 and tracheotomy. Only a few studies have discussed the evolution of craniofacial features of PRS. 7-11 In our institution, patients with PRS are tested at birth in the prone position without any nasopharyngeal airway support. If the result is unsatisfactory, a nasopharyngeal airway is added, and the babies are evaluated in the prone and supine positions. Adjunctive measures, such as oxygen therapy, con- tinuous positive airway pressure, nasogastric feeding, and lan- soprazole (1.0-1.7 mg/kg), are added as required in more severe cases. Endotracheal intubation and tracheotomy are rarely performed. The ultimate goal is the removal of the naso- pharyngeal support with maintenance of adequate saturation and no CO 2 retention. The approach is considered a failure if no major improvement is noted after a 6- to 8-week trial. The objectives of our study are twofold: (1) to assess the growth of the maxilla, the mandible, and the occlusal outcome among patients with PRS and (2) to identify associations between pre- operative anatomic cleft palate measurements and malocclu- sion dental class/cephalometric measurements. Materials and Methods Study Design After obtaining ethics approval from the Comite ´ d’E ´ thique de la Recherche of CHU de Que ´bec, we performed a 1 Department of Otolaryngology–Head and Neck Surgery, Universite ´ Laval, Quebec City, Quebec, Canada 2 Faculty of Dentistry, Universite ´ Laval, Quebec City, Quebec, Canada 3 Clinical Research Platform, CHU de Que ´bec–Universite ´ Laval Research Centre, Quebec City, Quebec, Canada 4 Department of Otolaryngology–Head and Neck Surgery, Centre Hospitalier Universitaire de Que ´bec, Quebec City, Quebec, Canada This article was presented at the meeting of the International Federation of Oto-Rhino-Laryngological Societies; June 2017; Paris, France. Corresponding Author: Jacques E. Leclerc, MD, FRCS(c), Department of Otolaryngology–Head and Neck Surgery, Centre Hospitalier Universitaire de Que ´bec, 2705 Boul Laurier, Quebec City, QC G1V 4G2, Canada. Email: jeleclerc@ccapcable.com