“Counterclockwise Craniofacial Distraction Osteogenesis (C3DO) for tracheostomy-dependent children with Treacher Collins syndrome”

Background: The craniofacial rotation deformity in Treacher Collins syndrome (TCS) results in airway compression that is not adequately addressed by isolated mandibular distraction osteogenesis (MDO). The purpose of this work is to present a novel surgical technique, counterclockwise craniofacial distraction osteogenesis (C3DO), to improve the airway morphology and occlusion in tracheostomy-dependent patients with TCS. Methods: This is a prospective study of tracheostomy-dependent children with TCS who underwent C3DO. All patients underwent subcranial LeFort II osteotomies with simultaneous mandibular osteotomies, followed by coordinated maxillomandibular distraction with counterclockwise rotation. All patients had pre-, post-, and end-treatment lateral cephalograms. Airway changes were assessed using polysomnography (PSG), sleep endoscopy, and direct laryngoscopy. Bivariate statistics were computed to compare pre- and post-treatment measures. Results: Five subjects (age: 4.5 - 12.1 years) underwent C3DO; 3 had previously undergone mandibular distraction (MDO). The average palatal plane rotation was 17 degrees, the effective mandible length increase was 18 mm, and the facial plane relative to skull base rotation forward was 14 degrees. There was a symmetric 30% relapse of rotation with maintained occlusion in the first nine months of follow-up that then stabilized. Four patients were successfully decannulated following C3DO following PSG. Sleep endoscopy available on two patients demonstrated resolution of the upper airway obstruction. Conclusion: C3DO provided greater palatal rotation than previous techniques in the literature. The resulting improvement in airway anatomy allowed for decannulation in four out of five tracheotomized patients. Stability of the counterclockwise rotation is comparable to related orthognathic operations, despite substantially greater magnitude. Financial Disclosure Statement: Dr. Hopper is an inventor on a patented nasal molding device licensed for distribution to KLS Martin LLP. Drs. Kapadia, Bly and Johnson have nothing to disclose. No funding was received for this article. Presented at: American Cleft lip and Palate Association (ACPA) 2015, Palm Springs, CA; International Society of Craniofacial Surgery (ISCFS) 2015, Tokyo, Japan; 13th International Cleft Conference 2017, Chennai, India; the British Association of Oral and Maxillofacial Surgeons 2017, Birmingham, UK; and the 36th Congreso Nacional de Cirugia Plastica SCCP 2017, Cartagena, Columbia. Authorship contributions: Dr. Richard Hopper reviewed and summarized the data and wrote the manuscript. Dr. Hitesh Kapadia performed the cephalometric analysis and reviewed the manuscript. Dr. Susarla performed the statistical analysis and reviewed the manuscript. Drs. Bly and Johnson compiled the airway analysis data and reviewed the manuscript. The study was approved by the Institutional Review Board of Seattle Children's Hospital and conformed to the Declaration of Helsinki. Acknowledgments: The authors would like to acknowledge Drs. Joseph Losee and Jesse Goldstein for providing images of their patient (Case #3) for this article, and for coordinating and acting as co-surgeons, with the primary author (RAH), at Pittsburgh Children's Hospital. Corresponding Author: Richard A Hopper MD, Surgical Director, Craniofacial Center, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105 USA. Richard.hopper@seattlechildrens.org ©2018American Society of Plastic Surgeons

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