PEDIATRIC/CRANIOFACIAL Counterclockwise Craniofacial Distraction Osteogenesis for Tracheostomy-Dependent Children with Treacher Collins Syndrome Richard A. Hopper, M.D., Background: The craniofacial rotation deformity in Treacher Collins syndrome M.Sc. results in airway compression that is not addressed by isolated mandibular Hitesh Kapadia, D.D.S., distraction osteogenesis. Our purpose is to present a surgical technique— Ph.D. counterclockwise craniofacial distraction osteogenesis—that improves airway Srinivas Susarla, D.M.D., morphology and occlusal rotation in tracheostomy-dependent patients with M.D., M.P.H. this condition. Randall Bly, M.D. Methods: All patients underwent subcranial Le Fort II osteotomies with simultane- Kaalan Johnson, M.D. ous mandibular osteotomies, followed by coordinated maxillomandibular distrac- Seattle, Wash. tion with counterclockwise rotation. We reviewed pretreatment, posttreatment, and end-treatment cephalograms. Airway changes were assessed using polysom- nography, sleep endoscopy, and direct laryngoscopy. Bivariate statistics were com- puted to compare pretreatment and posttreatment measures. Results: Five subjects (age range, 4.5 to 12.1 years) underwent this new pro- cedure; 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 SUPPLEMENTAL DIGITAL CONTENT IS AVAIL- first 9 months of follow-up that then stabilized. Four patients were successfully ABLE IN THE TEXT. decannulated following counterclockwise craniofacial distraction osteogenesis following polysomnography. Sleep endoscopy available on two patients demon- strated 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 pa- tients. Stability of the counterclockwise rotation is comparable to that of relat- ed orthognathic operations, despite substantially greater magnitude. (Plast. Reconstr. Surg. 142: 447, 2018.) CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV. reacher Collins syndrome is an autosomal minimal medical intervention to severe anomalies dominant condition1–3 affecting the first requiring multiple procedures.10–14 Severe presen- and second branchial arches and occur- tations of Treacher Collins syndrome have a clock- T 4–6 ring in one in 50,000 live births. The pheno- wise rotation of a hypoplastic maxillomandibular type7–9 ranges from mild presentations requiring complex. These manifest clinically as an anterior From the Division of Plastic Surgery and the Department of United Kingdom, June 28 through 30, 2017; and the 36th Otolaryngology, University of Washington; and the Cranio- Congreso Nacional de Cirugia Plastica SCCP 2017, in Carta- facial Center, Seattle Children’s Hospital. gena, Colombia, September 13 through 16, 2017. Received for publication September 19, 2017; accepted Copyright © 2018 by the American Society of Plastic Surgeons February 9, 2018. Presented at 72nd Annual Meeting of the American Cleft DOI: 10.1097/PRS.0000000000004606 Palate-Craniofacial Association, in Palm Springs, Califor- nia, April 20 through 25, 2015; the 16th Biennial Congress Disclosure: Dr. Hopper is an inventor on a patent- of the International Society of Craniofacial Surgery, in To- ed nasal molding device licensed for distribution to kyo, Japan, April 14 through 18, 2015; the 13th Interna- KLS Martin LLP. Dr. Susarla owns stock in Polarity tional Congress of Cleft Lip and Palate and Related Cranio- TE, Inc. Drs. Kapadia, Bly, and Johnson have no fi- facial Anomalies, in Chennai, India, February 8 through nancial interest to disclose. No funding was received 11, 2017; the British Association of Oral and Maxillofa- for this article. cial Surgeons Annual Scientific Meeting, in Birmingham, www.PRSJournal.com 447 Copyright © 2018 American Society of Plastic Surgeons. Unauthorized reproduction of this article is prohibited. Plastic and Reconstructive Surgery • August 2018 open bite; steep facial, occlusal, and mandibular grafting in one or two stages.30 Although a novel plane angles; long anterior face height; and air- approach, there were multiple shortcomings that way obstruction from both retrognathia and com- prevented widespread acceptance of the tech- pression.15–18 The prevalence of obstructive sleep nique: a high rate of relapse of mandibular posi- apnea associated with Treacher Collins syndrome tion in the immature skeleton (as early as 1 year dysmorphology has been reported to be as high as after surgery), instability of the maxillary occlu- 95 percent,19,20 with a tracheostomy rate of 6 to 41 sal rotation, the surgical challenge of overcom- percent.19–21 ing high soft-tissue resistance to achieve en bloc The goals of surgical reconstruction of the cra- bony movement, and the need for extensive bone niofacial skeleton in Treacher Collins syndrome grafting.22,30 are multifold: expansion of the upper airway, In this report, we present a novel surgical tech- restoration of functional occlusion, and improve- nique that revisits subcranial rotation in Treacher ment in facial aesthetics.15 Achieving these goals Collins syndrome patients through simultaneous in complex Treacher Collins syndrome cases Le Fort II and mandibular distraction around a remains elusive, with a high skeletal relapse rate nasofrontal pivot. We hypothesized that counter- after orthognathic surgery and mandibular dis- clockwise craniofacial distraction osteogenesis traction osteogenesis,22 and an inability to treat (C3DO) would create a normalizing rotation of obstructive sleep apnea or remove the need for a the palatal plane and result in favorable changes tracheostomy despite multiple surgical attempts in along the entire upper airway. With regard to this 21 many patients. In addition, there is no evidence hypothesis, our specific aims were to (1) assess in the literature for a reduction in the severity and the cephalometric changes that occur as a result prevalence of airway obstruction in Treacher Col- of the counterclockwise craniofacial distraction 10 lins syndrome with age, emphasizing the need osteogenesis technique and (2) evaluate sleep for successful early treatment. study and airway data following counterclockwise Airway volume analysis of Treacher Collins craniofacial distraction osteogenesis to assess air- syndrome patients has demonstrated that both way improvement. maxillomandibular hypoplasia and clockwise rotation contribute to the degree of obstruction.23 Although the importance of the clockwise rota- PATIENTS AND METHODS tion deformity is well recognized, the current Study Design/Sample standard treatment of airway compromise in the immature skeleton is isolated mandibular surgery This was a prospective study of children with using distraction lengthening24,25 or genioglos- a complex phenotype of Treacher Collins syn- sus advancement by means of genioplasty.26 Both drome who underwent counterclockwise cranio- techniques are significantly limited in their ability facial distraction osteogenesis performed by the to address the maxillomandibular rotation defor- primary author (R.A.H.) for treatment of severe mity, as they offer little to no significant counter- airway obstruction. All patients already had tra- clockwise rotation. cheostomies in place at the time of surgery. Three Counterclockwise rotation will not only cor- patients had gastrotomy tubes before surgery. rect the occlusal deformity, but is known to The other two had previous gastrotomy tubes that increase posterior airway dimensions,27–29 and were replaced at the time of surgery to help with should be a primary treatment goal. Tulasne and nutrition after surgery. All patients had preop- Tessier recognized the importance of this and, in erative, end-distraction, end-consolidation, and 1986, proposed the “procedure integral,” which end-treatment (at a minimum of 6 months after attempted a correction of the rotation deformity consolidation) lateral cephalograms. The study with subcranial osteotomies, impaction, and bone was approved by the Institutional Review Board of Seattle Children’s Hospital and conformed to the Supplemental digital content is available for Declaration of Helsinki. this article. Direct URL citations appear in the text; simply type the URL address into any Web Surgical Technique browser to access this content. Clickable links We performed a Le Fort II subcranial separa- to the material are provided in the HTML text tion through a coronal incision. After removing of this article on the Journal’s website (www. 5 mm of bone below the nasofrontal osteotomy, PRSJournal.com). we created a fixed point of rotation for the subcra- nial en bloc rotation movement during distraction 448 Copyright © 2018 American Society of Plastic Surgeons. Unauthorized reproduction of this article is prohibited. Volume 142, Number 2 • Treacher Collins Rotation Distraction with a 26-gauge wire hinge across the nasofrontal device traction on the maxillomandibular fixa- osteotomy. Through preexisting Risdon incision tion splint exerted a counterclockwise rotational scars, we used custom virtual surgical planning cut- pull on the face (Fig. 1). The mandible distraction ting guides (VSP; 3D Systems, Littleton, Colo.)