Pediatric/Craniofacial

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 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 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 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. (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,

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 subcranial en bloc rotation movement during 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.) to devices were used to maintain the contact of the mark inverted-L ramus osteotomies and guide two condyles with the skull base during the rotation, pairs of parallel transfacial 0.078-inch steel pins but care was taken to ensure the maximum ten- on each side of the osteotomy. This allowed accu- sion was on the midface device wires and not on rate placement in these hypoplastic mandibles the mandibular pins to avoid undue compression relative to surrounding teeth and nerve. Paired at the temporomandibular joint. multivector external mandible distraction devices Patients remained in the intensive care unit (KLS Martin, Jacksonville, Fla.) were attached to for 48 hours with heavy sedation. Activation of the pins before completing the osteotomy. We the mandible and midface distraction devices was placed the patient in maxillomandibular fixation started on postoperative day 5 at 1.5 mm/day. around a custom acrylic occlusal splint with extra- Patients were followed with lateral cephalograms oral traction posts from an embedded facebow. until the palatal plane approached a normal value The maxillomandibular fixation was maintained relative to the sella-nasion plane of 7 degrees. In with transpalatal and circummandibular 26-gauge patients 4 and 5, rotation was stopped short of this wires secured to the acrylic splint. After closing goal because of developing enophthalmos. the incisions, we applied an external midface dis- After completion of activation, the patients traction device (DePuy Synthes CMF, Paoli, Pa.) returned to the operating room for removal of the and wired the activation arms to the splint trac- maxillomandibular fixation wires to allow inde- tion posts with a 45-degree upward vector relative pendent mandibular movement during consoli- to the Frankfort horizontal. dation. In cases 2 through 5, at the time of device At the end of the operation, the subcranial and splint removal, we performed simultaneous facial skeleton had been mobilized and wired zygomatic reconstruction with full-thickness pari- together as one unit with a rotation point at the etal cranial grafts using virtual surgical planning nasion. We performed lateral canthopexies to (Fig. 2). (See Video, Supplemental Digital Con- the lateral orbital rims. The midface distraction tent 1, which demonstrates presurgical virtual

Fig. 1. Counterclockwise craniofacial distraction osteogenesis. (Left) The Treacher Collins syndrome dysmorphology includes a clockwise rotation of the occlusal plane with associated upper airway compression. (Right) The subcranial skeleton is separated from its connection to the skull base through a Le Fort II and bilateral mandible osteotomies. A wire hinge is placed at the nasofrontal osteotomy and the patient is placed in maxillomandibular fixation. A midface distractor is attached to the maxillomandibular fixation splint, and an external mandible distractor is placed with transfacial pins. The upward traction of the midface device creates a rotational force on the face and the mandible devices keep the mandible condyle in position. During consolidation, the maxillomandibular fixation is released to allow mouth opening.

Fig. 2. Computed tomographic scan of case 2 before (left) and after (right) counterclockwise craniofacial distraction osteogenesis surgery and after consolidation zygoma reconstruction. In cases 2 through 5 (adolescent), full-thickness zygoma reconstruction across the facial clefts was performed using presurgical virtual planning. Split calvarial grafts were used to repair the craniotomy donor sites.

planning, available in the “Related Videos” sec- cephalometric analysis before surgery (preopera- tion of the full-text article on PRSJournal.com or, tively, T0), at the time of device removal (T1, end- for Ovid users, available at http://links.lww.com/ consolidation), and 5 to 9 months after device PRS/C864.) removal (T2, end-treatment).

Cephalometric Analysis Standardized Direct Airway Analysis Low-dose protocol computed tomographic Sleep endoscopy and direct laryngoscopy were images and lateral cephalograms were analyzed performed on cases 3 and 5 immediately before using Dolphin image analysis software (Dolphin surgery and at the time of device removal. Stan- Imaging & Management Solutions, Chatsworth, dardized views were captured at the oropharynx Calif.) for lateral two-dimensional superimposition (with and without jaw thrust) using flexible sleep endoscopy under light total intravenous general anesthesia to preserve airway dynamics. Airways before and after counterclockwise craniofacial distraction osteogenesis were graded using the Chan-Parikh sleep endoscopy obstruction scale (0 to 3 scale, with 3 indicating complete obstruction) and the Cormack-Lehane laryngoscope grading scale (1 to 4 scale, with 1 being the easiest exposure and grade 4 being inability to visualize any laryngeal structures).31,32

Polysomnography Polysomnography with capped tracheostomy was performed during the patient’s habitual sleep period in accordance with standards established by the American Academy of Sleep Medicine. Standard definitions of polysomnographic events and indices were used as described previously.31 Video. Supplemental Digital Content 1 demonstrates presurgi- Preoperative polysomnographs were obtained cal virtual planning, available in the “Related Videos” section of only in those patients able to tolerate capping of the full-text article on PRSJournal.com or, for Ovid users, avail- their tracheostomy. Post–counterclockwise cranio- able at http://links.lww.com/PRS/C864. facial distraction osteogenesis polysomnography

was performed with the tracheostomy capped a at our center. Cases 3, 4, and 5 had received their minimum of 3 months after removal of the dis- previous care outside our institution. Active distraction device. traction ranged from 23 to 34 days. All cases had consolidation of the distracted mandible osteoto- Statistical Analysis mies at the time of device removal (Fig. 3). Cases Pooled data were iteratively entered into a sta- 2 through 5 (adolescents with postremoval malar tistical database program (IBM SPSS Version 24.0; reconstruction) had consolidation of the midface IBM Corp., Armonk, N.Y.) for analysis. Descriptive osteotomies, whereas case 1 (preadolescent with statistics were computed to provide an overview of the sample. Paired samples analyses were used to no malar grafts) demonstrated rotational relapse compare preoperative and postoperative cephalo- of the maxilla and the need for surgical reposition- metric data. Nonparametric methods were used, ing and plate fixation. No patients have required given the small sample and lack of confirmed nor- or are scheduled for revision mandible or maxil- mality within the data set. For all analyses, a value lary surgery at last follow-up. Cases 2 through 5 of p ≤ 0.05 was considered significant. were all successfully decannulated based on airway examination and polysomnographic results. RESULTS Case 1 has not yet been decannulated, pending Five subjects (age range, 4.5 to 12.1 years) with planned tongue base reduction surgery. Interin- Treacher Collins syndrome underwent the counter- cisal opening was comparable to the preoperative clockwise craniofacial distraction osteogenesis pro- state. Complications associated with each opera- cedure. Cases 1 and 2 had been treated previously tion are listed in Table 1.

Fig. 3. Oblique view photographs of cases 2, 3, and 5 (left to right) before (above) and after (below) counterclockwise craniofacial distraction osteogenesis and autogenous zygoma reconstruction at device removal. No ancillary techniques such as fat grafting or lower eyelid reconstruction were performed. The periorbital changes were solely secondary to the counterclockwise craniofacial distraction osteogenesis and zygoma grafting procedures. Consent for use of photographs was not available for cases 1 and 4.

Table 1. Patient Demographics and Surgical History Patient

1 2 3 4 5 Sex F F M M M Age at surgery, yr 4.5 8.8 12.1 9.2 7.2 Previous airway Tracheostomy Tracheostomy; Tracheostomy; MDO Tracheostomy; Tracheostomy choana operations MDO (3×); tongue MDO; atresia repair; suspension; choana palatoplasty; posterior septectomy; dilation (10×) pharyngeal flap inferior turbinectomy Activation, days 26 24 34 28 25 Zygoma reconstruction No Yes Yes Yes Yes at device removal Perioperative Cheek abscess None Cheek abscess None None complications (resolved with (resolved with drainage); drainage); orbital midface relapse abscess (resolved needing ORIF with removal of implant) F, female; M, male; MDO, mandibular distraction osteogenesis.

Cephalometric Analysis Case 1 was unable to tolerate polysomnography Cephalometric analysis results before and after preoperatively. Postoperatively, case 1 (preadoles- counterclockwise craniofacial distraction osteo- cent) had improvement of airway on endoscopy, genesis surgery are listed in Table 2. Following but was not considered a candidate for decan- counterclockwise craniofacial distraction osteo- nulation until further airway interventions (e.g., genesis, there were statistically significant changes tongue base reduction) could be performed. noted (p ≤ 0.04). The mean facial plane rotation was 14 degrees forward, with an average relapse DISCUSSION of 5 degrees at end-treatment. The mean counter- Isolated mandible distraction osteogenesis is clockwise palatal plane rotation was 17 degrees, currently the standard treatment of immature chil- with an average relapse of 5 degrees. Mandibu- dren with Treacher Collins syndrome and severe lar length increased an average of 18 mm, with airway compromise.10 Although mandibular distrac- a 7-mm relapse. The maxillomandibular relation tion osteogenesis can correct the linear deficiency was maintained with a stable A point–nasion–B of Treacher Collins syndrome micrognathia, it does point angle (p > 0.16). not correct the underlying clockwise rotational deformity that is fundamental to the complex airway Sleep Endoscopy Changes compression.33 As such, although it can improve the Cases 3 and 5 were scheduled for pre–coun- airway anatomy in some children, in many cases it terclockwise craniofacial distraction osteogenesis is not sufficient to normalize severely compromised and 3-month post–device removal sleep endoscopy airways, such as those in tracheostomy-dependent and graded using the Chan-Parikh classification of children.21 In this report, we describe counter- pediatric airway obstruction31 and the Cormack- clockwise craniofacial distraction osteogenesis to Lehane grading of direct laryngoscopy view.32 On address the subcranial rotational deformity in five the Chan-Parikh scale at the tongue base, both Treacher Collins syndrome patients. The four ado- cases went from a grade 3 (complete obstruction) lescent patients (cases 2 through 5) all underwent to a grade 1 (0 to 50 percent obstructed), and from successful decannulation following expansion of a grade 3 Cormack-Lehane laryngoscope exposure their airway. In three of these patients, prior man- (epiglottis visible only) to a normal grade 1 expo- dibular distraction osteogenesis had not been suc- sure (anterior commissure visible) (Fig. 4). cessful in removing tracheostomy dependence until counterclockwise craniofacial distraction osteogen- Polysomnographic Changes esis was performed. This is consistent with recent Available polysomnography study results are literature in adult obstructive sleep apnea patients shown in Table 3. The post–counterclockwise that identified occlusal rotational correction as the craniofacial distraction osteogenesis studies were most predictive factor in the clinical improvement performed at least 3 months after device removal of obstructive sleep apnea.27,29 These data make a and with the tracheostomy capped and uncapped. compelling argument for correction of not only the

sagittal maxillomandibular position, but also the 6.5 4.5 1.5

∆

-4.75 −8 −0.5 −5.5 −12 19.25 n/a n/a facial and palatal plane rotation. −27.5 T2-NV McCarthy et al. introduced the technique 2 7 of distraction lengthening of the human man-

82 81 77

50 60

NV 105 100 n/a n/a 110 dible,34 and others later described its use in advancing the retruded midface.35–38 Since that

% time, the recognized benefits of distraction −33 −38 −50 −36 −29 −39 −14 −50 −30 −100 −151 T1-T2

Relapse include the ability to overcome strong soft-tissue forces through gradual movement. In counter-

clockwise craniofacial distraction osteogenesis, 0.07 0.07 0.16 0.07 0.07 0.20 0.59 1.00 0.07 0.46 1.44 0.07 T1-T2 we used combined mandible and midface dis- p traction to exert a rotational force against the

strong soft-tissue resistance of the Treacher Col- 0.04* 0.04* 0.5 0.04* 0.04* 0.04* 0.04* 0.89 0.08 0.23 0.04* 0.23 T0-T1 lins syndrome face. Because of the known risk of 8

84 76 75

74 33 38 61

condylar resorption following mandibular dis- T2 105 108 106 traction osteogenesis against high resistance,39–41 6 5

85 79 77

89 33 34 67

T1 we used transfacial pins to use the adjacent soft 110 109 tissue to stabilize the thin distal mandible seg- 8

74 67 64

73 26 42 61

T0 ments from rotation and temporomandibular 108 122 108 joint compression. We also initiated midface

T2 — — — — — — — — — — — — device activation first to minimize the strain on the mandible device. We have performed coun-

77 66 10 63

81 27 36 74

4 T1 114 122 102 terclockwise craniofacial distraction osteogen-

8 esis using internal mandible distraction devices

68 60 58

72 23 35 67

97 T0 109 164 in two patients with craniofacial microsomia. 7 8 As this procedure evolves and is applied to less

72 65 65

80 35 36 68

T2 111 132 102 severe Treacher Collins syndrome phenotypes, 7 5 internal mandibular distraction osteogenesis 3

76 69 68

91 36 34 67

T1 109 120 123 could also be considered. There are few data on Subject 6

63 57 the stability of isolated mandibular distraction 56

70 26 41 68

T0 116 134 130 osteogenesis in Treacher Collins syndrome, but

86 75 11 75

81 30 39 58

one small series demonstrated a 21 to 33 percent

T2 102 129 100 decrease in effective mandible length and a 14 to 9 8 2

91 82 83

77 33 38 56

46 percent retrusion in pogonion position 1 year T1 100 107 117 after mandibular distraction osteogenesis, which

78 67 10 66

60 25 41 59

is comparable to our observations with counter- T0 106 139 108 clockwise craniofacial distraction osteogenesis.42 8

84 76 74

75 37 39 59

T2 In addition, compared with the procedure inte- 103 108 110 gral in which mandible relapse was greater than 8 3 1

89 81 79

79 35 32 65

T1 the midface, all counterclockwise craniofacial 104 110 112 distraction osteogenesis patients maintained

77 61 16 56

50 28 89 37 53

99 T0 their occlusal relationship as noted by a stable A 146 point–nasion–B point angle measure. We were unable to find other reports in the literature of a case series with an average palatal plane rotation of 17 degrees; thus, it is diffi- cult to place the degree of relapse we observed into context. After counterclockwise craniofacial distraction osteogenesis, we observed a 27 percent palatal plane rotation, 35 percent mandible length, and 32 percentage sella–

Cephalometric Measures before and after Counterclockwise Craniofacial Distraction Osteogenesis Compared to Age-Matched Normal Values Age-Matched to Normal Distraction Compared Osteogenesis Craniofacial and after Counterclockwise before Measures Cephalometric nasion–A point angle (maxillary advancement) relapse in the first 5 to 9 months after device

(Sn-Gn ′ -C), deg removal (Table 2). In case 2, for which cepha- Facial plane to SN (SN-NPog), deg Anterior facial height (N-Me), mm Upper face height (N-ANS), mm Lower face height (ANS-Me), mm 5 mo after halo removal); NV, age-matched normative value; —, not performed; N/A, not available. T0, preoperative; T1, end-consolidation; T2, end-treatment ( > 5 mo after halo removal); NV, *Statistically significant ( p < 0.05). Table 2. Table Maxillomandibular relationship SNA, deg SNB, deg ANB, deg

Maxillary rotation SN-palatal plane, deg Relative facial height Mandible unit length (Co-Pog) PNS-sella, mm

Neck changes Lower face-throat angle Palatoalveolar changes U1-palatal plane, deg lometric data past 1 year after device removal

Fig. 4. Case 3 sleep endoscopy views before (above) and after (below) counterclockwise craniofacial distraction osteogenesis. (Left) visualization of the retroglossal area from the nasopharynx. (Center) visualization of the retroglossal area from the nasopharynx with jaw thrust. (Right) visualization of the vocal cords on laryngoscopy. The patient went from a Chan-Parikh grade 3 (complete obstruction) and Cormack-Lehane grade 3 (epiglottis visible only) to grade 1on both scales (essentially normal airway). Case 5 also had standardized endoscopy with similar findings. Pixilation of images is secondary to magnification of the fiberoptic view through the 3-mm endoscope.

were available, the early changes we observed and mandible position.47,48 Although greater stabilized and did not continue to change on rotational changes are achieved with counter- later follow-up (Fig. 5). The counterclockwise clockwise craniofacial distraction osteogenesis, rotation movement achieved with traditional the stability appears to be comparable to simi- Le Fort I impaction and bilateral sagittal split lar patient groups undergoing much smaller mandible advancement in non–Treacher Col- counterclockwise rotation with conventional lins syndrome patients has been generally orthognathic approaches. considered to be unstable.43,44 Although there Two possible causes of the changes observed are data to suggest that relatively small rota- following counterclockwise craniofacial dis- tional movements are stable in the context of traction osteogenesis are the forces of the sur- a healthy normal temporomandibular joint,45,46 rounding musculocutaneous environment, or a Treacher Collins syndrome patients have abnor- change in centric relation of the mandible from mal temporomandibular joint morphology. temporomandibular joint instability. For future The population of Treacher Collins syndrome counterclockwise craniofacial distraction osteo- patients would be more comparable to orthog- genesis cases we are considering the following nathic surgical patients with conditions such modifications: (1) surgical release of the geniohy- as articular disk displacement. In this latter oid muscles; (2) temporary paralysis of the ptery- group, small counterclockwise rotations of 5 to gomasseteric sling using botulinum toxin; (3) 7 degrees have been described to result in a 36 extending the consolidation phase to 16 weeks; percent relapse in both occlusal plane rotation and (4) ensuring the condyles are well seated

against the skull base before placement of the transfacial pins. 0 1 99 96 97 In case 1, we performed counterclockwise cra- 0.5 niofacial distraction osteogenesis at 4.5 years of age and it did not result in decannulation. There Postoperatively was a dramatic visible improvement in the upper

Case 5 airway, but the retroglossal area remained too nar- row. In this patient, we did not perform zygoma reconstruction at device removal because of the 32.9 97 77 58 58 76 patient’s young age, and we observed early midface

Preoperatively rotational relapse that required open treatment with plate fixation for stabilization. It is unclear whether this relapse contributed to the less favorable airway result, or whether the patient had a 0.6 7 7.5 more complex airway than the other patients. 95 87 90 In summary, we describe a procedure that revisits the large counterclockwise rotation move- Postoperatively ment in Treacher Collins syndrome patients envi-

Case 4 sioned by Tessier and Tulasne three decades ago, but has achieved increased stability through the use of coordinated subcranial maxillomandibular n/a n/a n/a n/a n/a n/a‡ distraction. We have been able to decannulate four

Preoperatively tracheostomy-dependent adolescent Treacher Collins syndrome patients with this technique. All four of these patients had undergone prior mandible distraction(s) and/or choana operations without successful decannulation before 98 94 90 1.9 5.8 6.7 counterclockwise craniofacial distraction osteogenesis. Besides the airway benefit of the coun- Postoperatively terclockwise craniofacial distraction osteogenesis

Case 3 procedure, the occlusal plane rotation and airway improvement achieved appears to be stable after the first year, and the potential favorable impact n/a n/a n/a n/a n/a n/a† on orthognathic surgery at maturity remains to be seen. No patients have required or are scheduled Preoperatively for revisional maxillary or mandibular surgery at last follow-up, which is different from the high revision rate described following the procedure , oxygen saturation; C3DO, counterclockwise craniofacial distraction osteogenesis; PSG, polysomnography. 2 3 6 6.9 integral. We anticipate that all patients will still o 98 93 98 require a traditional linear double-jaw operation at the Le Fort I level at maturity for occlusion and Postoperatively continued improvement in their airway. Case 2 CONCLUSIONS 31 96 84 45 48 81 Based on our early favorable experience, counterclockwise craniofacial distraction osteo- operatively Pre genesis (C3DO) has replaced mandibular distraction osteogenesis as the first operation in tracheostomy-dependent Treacher Collins syndrome patients with a rotational deformity. We , % , % Polysomnographic Measures before and after Counterclockwise Craniofacial Distraction Osteogenesis* Craniofacial and after Counterclockwise before Measures Polysomnographic 2 2 o

o recommend that it be performed after the age of 7 years for the maximal chance at stability and airway resolution, and that it include zygoma recon- Nadir Sa Desaturation index Mean Sa N/A, not applicable; AHI, apnea-hypopnea index; Sa Table 3. Table Measure *Pre-C3DO PSG studies were performed after all operations such as MDO that preceded C3DO. Post-C3DO PSG studies were performed with the capped tracheostomy tube in place at least case 1 had improvement of airway on endoscopy but was not considered a candidate for Postoperatively, 3 months after device removal. Case 1 was unable to tolerate a PSG preoperatively. decannulation until further airway operations could be performed. †Case 3 was unable to tolerate capped pre-C3DO PSG. ‡Case 4 did not have pre-C3DO PSG performed. Obstructive AHI AHI Sleep efficiency, % Sleep efficiency, struction at the time of device removal.

Fig. 5. Lateral cephalogram superimpositions of cases 2 (left), 3 (center), and 5 (right). Represented time points are pre–counterclockwise craniofacial distraction osteogenesis (black lines), device removal (green lines), and last follow-up (red lines). We observed a bimaxillary retrusion in the first year after device removal that then stabilized. Relative maxillomandibular relationship was maintained and the counterclockwise rotation was preserved. The majority of changes between device removal and last follow-up were not in bone position such as anterior nasal spine or palatal plane, but were instead central incisor angle changes relative to the palate. We hypothesize that the occlusal splint traction during distraction caused a proclination of the central incisors within the alveolar bone, which then reverted to more normal upper incisor angulation at last follow-up.

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