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Counterclockwise Craniofacial Distraction Osteogenesis for Tracheostomy-Dependent Children with Treacher Collins Syndrome

Hopper, Richard A., M.D., M.Sc.; Kapadia, Hitesh, D.D.S., Ph.D.; Susarla, Srinivas, D.M.D., M.D., M.P.H.; Bly, Randall, M.D.; Johnson, Kaalan, M.D.

Plastic and Reconstructive Surgery: August 2018 - Volume 142 - Issue 2 - p 447-457
doi: 10.1097/PRS.0000000000004606
Pediatric/Craniofacial: Original Articles

Background: The craniofacial rotation deformity in Treacher Collins syndrome results in airway compression that is not addressed by isolated mandibular distraction osteogenesis. Our purpose is to present a surgical technique—counterclockwise craniofacial distraction osteogenesis—that improves airway morphology and occlusal rotation in tracheostomy-dependent patients with this condition.

Methods: All patients underwent subcranial Le Fort II osteotomies with simultaneous mandibular osteotomies, followed by coordinated maxillomandibular distraction with counterclockwise rotation. We reviewed pretreatment, posttreatment, and end-treatment cephalograms. Airway changes were assessed using polysomnography, sleep endoscopy, and direct laryngoscopy. Bivariate statistics were computed to compare pretreatment and posttreatment measures.

Results: Five subjects (age range, 4.5 to 12.1 years) underwent this new procedure; three had previously undergone mandibular distraction. 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 was 14 degrees. There was a symmetric 30 percent relapse of rotation with maintained occlusion in the first 9 months of follow-up that then stabilized. Four patients were successfully decannulated following counterclockwise craniofacial distraction osteogenesis following polysomnography. Sleep endoscopy available on two patients demonstrated resolution of the upper airway obstruction.

Conclusions: Counterclockwise craniofacial distraction osteogenesis provided greater palatal rotation than previous techniques. The resulting improvement in airway anatomy allowed for decannulation in four of five tracheotomized patients. Stability of the counterclockwise rotation is comparable to that of related orthognathic operations, despite substantially greater magnitude.


Seattle, Wash.

From the Division of Plastic Surgery and the Department of Otolaryngology, University of Washington; and the Craniofacial Center, Seattle Children’s Hospital.

Received for publication September 19, 2017; accepted February 9, 2018.

Presented at 72nd Annual Meeting of the American Cleft Palate-Craniofacial Association, in Palm Springs, California, April 20 through 25, 2015; the 16th Biennial Congress of the International Society of Craniofacial Surgery, in Tokyo, Japan, April 14 through 18, 2015; the 13th International Congress of Cleft Lip and Palate and Related Craniofacial Anomalies, in Chennai, India, February 8 through 11, 2017; the British Association of Oral and Maxillofacial Surgeons Annual Scientific Meeting, in Birmingham, United Kingdom, June 28 through 30, 2017; and the 36th Congreso Nacional de Cirugia Plastica SCCP 2017, in Cartagena, Colombia, September 13 through 16, 2017.

Disclosure:Dr. Hopper is an inventor on a patented nasal molding device licensed for distribution to KLS Martin LLP. Dr. Susarla owns stock in Polarity TE, Inc. Drs. Kapadia, Bly, and Johnson have no financial interest to disclose. No funding was received for this article.

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Richard A. Hopper, M.D., M.Sc., Craniofacial Center, Seattle Children’s Hospital, 4800 Sand Point Way NE, Seattle, Wash. 98105,

Copyright © 2018 by the American Society of Plastic Surgeons