2015 HSC Section 1 Book of Articles

Runyan et al

around 1:8,500, 3,4 there are approximately 500 new cases of PRS in the US annually, 30% of which may have airway obstruction severe enough to warrant operative intervention. 11 Our data suggest a $300,000 cost savings for each patient treated with mandibular distraction rather than tracheostomy, over 3 years. Hypothetically, performing MD on all patients with severe PRS would generate health care savings of $450,000,000 over a 10-year period, assuming those patients would otherwise receive a tracheostomy. This estimate does not include long-term and indirect costs, which include costs associated with scars, radiation exposure, and quality of life. Cost savings is one important consideration for selection of treatment of PRS and is the focus of this study. Of equal or greater importance though are the long-term airway, speech, nutrition, and developmental outcomes of patients treated with mandibular distraction compared to tracheost- omy. However, as described in a recent systematic review, outcomes of neonates with PRS are poorly understood due to wide variability of study inclusion criteria, lack of stan- dardized indications for interventions, and a general paucity of data. 5 Based on the present study and our clinical experi- ence, 10 we support the use of mandibular distraction for treatment of severe PRS but acknowledge that further study is necessary to standardize diagnostic and treatment criteria and to identify appropriate outcome measures. Acknowledgments We thank Dr David Billmire, Dr J. Paul Willging, Dr Evan Probst, and Patricia Bender for assistance with study design, data collec- tion, and analysis. Author Contributions Christopher M. Runyan , data collection, data analysis, majority of the writing; Armando Uribe-Rivera , data collection, data analysis, writing; Audrey Karlea , data collection, data analysis, early manu- script writing; Jareen Meinzen-Derr , statistical and data analyses; Dawn Rothchild , data collection, data analysis, early manuscript revi- sions; Howard Saal , study conception and design, data collection, data analysis, early manuscript revisions; Robert J. Hopkin , study concep- tion and design, data collection, data analysis, Institutional Review Board protocol, early manuscript draft and revisions; Christopher B. Gordon , study conception and design, data collection, data analysis. Disclosures Competing interests: None. Sponsorships: None. Funding source: Internal, we would like to acknowledge the Cincinnati Children’s Hospital Medical Center Divisions of Genetics and Plastic Surgery for funding this project. Supplemental Material Additional supporting information may be found at http://otojournal .org/supplemental. References 1. Robin P. La glossoptose. son diagnostic, ses consequences, son traitement. Journal de me´decine de Paris . 1923;43:235-237.

2. Schaefer RB, Gosain AK. Airway management in patients with isolated Pierre Robin sequence during the first year of life. J Craniofac Surg . 2003;14:462-467. 3. Sheffield LJ, Reiss JA, Strohm K, et al. A genetic follow-up study of 64 patients with the Pierre Robin complex. Am J Med Genet . 1987;28:25-36. 4. Brunner HG, van Beersum SE, Warman ML, et al. A stickler syndrome gene is linked to chromosome 6 near the COL11A2 gene. Hum Mol Genet . 1994;3:1561-1564. 5. Bookman LB, Melton KR, Pan BS, et al. Neonates with tongue-based airway obstruction: a systematic review. Otolaryngol Head Neck Surg . 2012;146:8-18. 6. Denny AD, Amm CA, Schaefer RB. Outcomes of tongue-lip adhesion for neonatal respiratory distress caused by Pierre Robin sequence. J Craniofac Surg . 2004;15:819-823. 7. Rogers GF, Murthy AS, LaBrie RA, Mulliken JB. The GILLS score: part I. Patient selection for tongue-lip adhesion in Robin sequence. Plast Reconstr Surg . 2011;128:243-251. 8. Bijnen CL, Don Griot PJ, Mulder WJ, Haumann TJ, Van Hagen AJ. Tongue-lip adhesion in the treatment of Pierre Robin sequence. J Craniofac Surg . 2009;20:315-320. 9. Denny A, Amm C. New technique for airway correction in neonates with severe Pierre Robin sequence. J Pediatr . 2005;147:97-101. 10. Lam DJ, Tabangin ME, Shikary TA, et al. Outcomes of mandib- ular distraction osteogenesis in the treatment of severe micro- gnathia. JAMA Otolaryngol Head Neck Surg . 2014;140:338-345. 11. Denny A, Kalantarian B. Mandibular distraction in neonates: a strategy to avoid tracheostomy. Plast Reconstr Surg . 2002; 109:896-904. 12. Jiang D, Morrison GA. The influence of long-term tracheost- omy on speech and language development in children. Int J Pediatr Otorhinolaryngol . 2003;67(suppl 1):S217-S220. 13. Denny AD. Distraction osteogenesis in Pierre Robin neonates with airway obstruction. Clin Plast Surg . 2004;31:221-229. 14. Steinbacher DM, Kaban LB, Troulis MJ. Mandibular advance- ment by distraction osteogenesis for tracheostomy-dependent children with severe micrognathia. J Oral Maxillofac Surg . 2005;63:1072-1079. 15. Zeitouni A, Manoukian J. Tracheotomy in the first year of life. J Otolaryngol . 1993;22:431-434. 16. McCarthy JG, Schreiber J, Karp N, et al. Lengthening the human mandible by gradual distraction. Plast Reconstr Surg . 1992;89:1-8. 17. Denny AD, Talisman R, Hanson PR, et al. Mandibular distrac- tion osteogenesis in very young patients to correct airway obstruction. Plast Reconstr Surg . 2001;108:302-311. 18. Sidman JD, Sampson D, Templeton B. Distraction osteogen- esis of the mandible for airway obstruction in children. Laryngoscope . 2001;111:1137-1146. 19. Monasterio FO, Drucker M, Molina F, et al. Distraction osteo- genesis in Pierre Robin sequence and related respiratory prob- lems in children. J Craniofac Surg . 2002;13:79-83. 20. Izadi K, Yellon R, Mandell DL, et al. Correction of upper airway obstruction in the newborn with internal mandibular distraction osteogenesis. J Craniofac Surg . 2003;14:493- 499.

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