Faculty Bibliography

Background/Purpose: Treacher Collins Syndrome (TCS) produces complex deformities of the maxilla, mandible, nasal airway and palate, all of which may impact speech. Clinical outcomes of cleft palate repair in TCS have not been well-defined. Methods/Descriptions: A 35 year, single-institution retrospective review of all patients with TCS and cleft palate was performed. Variables evaluated included Veau-type palate classification, age and technique of repair, Pruzansky-type mandible classification, history of tracheostomy and age of decannulation, history of mandibular distraction, bone grafting, or orthognathic surgery, palatoplasty complications or readmissions, and presence of palatal fistula. A craniofacial speech pathologist assessed velopharyngeal dysfunction, resonance and sound production long-term. Results: 58 patients with TCS were identified of whom 25 (43%) had a cleft palate and 9 (15.5%) underwent palatoplasty at our institution. Veau-type palate classifications included Veau 1 (1/9), Veau 2 (5/9), Veau 3 (1/9), and Veau 4 (2/9). Pruzansky-type mandible classifications included IIA 1/9; IIB 6/9; III 2/9. Mean age of palatoplasty was 24.4 months (13-80), average length of stay 2.2 days (1-5), and followup time of 12.2 years (2.2-24.3). There were no peri-operative complications or unplanned re-admissions. Post-operative palatal fistula rate was 33%, including both of the Veau 4 patients. Seven patients had tracheostomies, of which three were ultimately decannulated. Seven patients had long-term follow-up with a speech pathologist 6.9-22.4 years after surgery. Three (42%) presented with sound errors related to cleft palate (2/3 had an oronasal fistula, while one presented with structurally-based velopharyngeal dysfunction requiring surgical management). Six (86%) presented with articulatory- based VPD. All seven patients presented with sound errors and resonance quality consistent with the anatomical dental-skeletal anomalies associated with TCS. Conclusions: Patients with TCS and cleft palate undergo palatoplasty at an older age and have higher incidences of palatal fistula and longterm sound and resonance disorders compared to patients with isolated cleft palate. Highest risk patients had Veau 4 cleft palates and Pruzansky-type III mandibles. Given the high incidence of articulatory- based velopharyngeal dysfunction, comprehensive speech therapy should be a critical component of long-term care in this patient population

Background/Purpose: Septal deviation is a common finding in skeletally mature patients with cleft lip and palate (CL/P), however the contribution of the cartilaginous and bony septum to airway obstruction is poorly defined. This study characterizes the septal and airway anatomy in skeletally mature patients with CL/P utilizing cone beam computed tomography (CBCT) and will help guide airway management of this patient population at the time of definitive rhinoplasty. Methods/Descriptions: This is a retrospective single institution review of all CL/P patients over the age of 15 who have undergone CBCT analysis. Septal deviation was measured in coronal sections of CBCT scans at the cartilaginous septum [anterior nasal spine (ANS)], and bony septum [posterior nasal spine (PNS) and midpoint between the ANS and PNS (MID)]. Airway obstruction was defined as the smallest linear distance between nasal septum and adjacent turbinate and was similarly measured at all three points. Superior (perpendicular plate of ethmoid) and inferior (vomer) bony septal displacement was measured as an angle from vertical at the coronal slice of maximal septal deviation. CL/P patients were compared to age-matched controls using Student's t-test. Stepwise multivariable linear regression was used to compare septal deviation to obstruction. Measurements were performed by two separate raters and interrater reliability was assessed using Pearson's r coeffecient. Statistical significance was held at p<0.05. Results: 24 CL/P patients and 16 age-matched controls were identified for the study. Interrater reliability for 210 independent measurements was r=0.94 (p<0.0001). Results are reported as CL/P versus control. Septal deviation was significantly increased at the ANS (2.1+/-2.2 mm vs 0.7+/-1.0 mm, p=0.03), MID (4.6+/-3.1 mm vs 2.2+/-1.2 mm, p=0.01), and PNS (2.9+/-1.8 mm vs 1.0+/-0.6 mm, p=0.0002). The airway was significantly obstructed at the ANS (1.8+/-0.8 mm vs 2.3+/-0.6 mm, p=0.03). Maximal septal deviation occurred at the bony septum in 39 of 40 patients. Both the perpendicular plate of the ethmoid (14+/-7.8degree vs 8.0+/-5.4degree, p=0.01) and vomer (25+/-15degree vs 9.0+/-7.9degree, p=0.0006) were significantly displaced from vertical. Midpoint bony septal deviation was a good predictor of anterior nasal airway obstruction (r=-0.525, p=0.008). Conclusions: Skeletally mature patients with a cleft demonstrate severe septal deviation which includes both cartilage and bone. Resection of the bony and cartilaginous septum should be considered at time of definitive rhinoplasty in CL/P patients

Background/Purpose: Treacher Collins (TC) and Robin Sequence (RS) are both associated with a retrognathic mandible, glossoptosis, and airway obstruction. TC is associated with aberrations of the lower jaw, upper jaw and nasal airway in a manner distinct from RS. Although in both TC and RS, airway obstructions in severe cases may benefit from mandibular distraction (MDO), the different pathophysiology suggests MDO to be less successful in TC compared to the current literature on RS. The purpose of this study is to report on the clinical outcomes of tracheostomy removal utilizing MDO in the TC patient population. Methods/Descriptions: A single center, twenty-year retrospective review (1991-2010) was conducted of all patients with TC treated with MDO. Recorded variables included: age of MDO, number of MDO procedures, presence of tracheostomy and complications. Literature review of clinical outcomes of MDO in the RS population demonstrates age of MDO of under one year, average on distraction per patient and avoidance of tracheostomy in over 90% of patients. Results: 24 patients with TC who underwent MDO were included in our analysis. The follow up time was 9.2 years (range1.7-17 years). The mean age of the first MDO was 4.97 years. The mean number of distractions was 1.42 with 46% of patients who had more than onedistraction attempted. The distraction devices used were external in 67% and internal in 33% of cases. 19 patients (79%) had a tracheostomy prior to MDO and only 9 (47%) patients were decannulated within one year of distraction. An additional 5 patients were decannulated several years later after further distraction and other airway procedures. Complications were divided into major (ankylosis, device failure) moderate and minor (pin infection, hypertrophic scar). Overall, 67% of TC patients had at least one complication with 41% having major complications. There was a 20% incidence of TMJ ankylosis. Conclusions: Compared to the RS population, TC patients undergo MDO at a later age, require more distraction and have less successful decannulation. Further surgery is required to effectively treat airway obstruction. The incidence of major complications

Background/Purpose: Craniofacial surgery remains a challenging field to learn and master. We present a freely-available internet-based multimedia simulator for craniofacial surgery designed as a resource of surgeons in craniofacial fellowship training. Methods/Descriptions: Previously constructed digital animations of craniofacial surgery were upgraded in Maya 10 (Autodesk, San Rapheal, CA) in preparation for web-based simulation. These animations were exported into an internet cloud-based, surgical simulator produced by BioDigital Systems Inc. (New York, NY). High-definition intra-operative video recordings of all procedures were edited in Adobe Premiere CS5.5 (Adobe, San Jose, CA) and exported into the simulator with the addition of voiceover. Test questions were produced for each surgical module. Results: Nine craniofacial surgery procedures are demonstrated in this interactive surgical simulator. Through a customized digital interface the user can manipulate the 3D simulations in real-time including the ability to alter perspective, pace and order of the virtual operation. High-definition intra-operative video footage compliments the critical steps of each procedure demonstrated in the simulation. A voiceover and text guides the user through each tutorial. A test is included at the end of each simulation. As the simulator is internet-based, there is no need for specialized software or downloads and simulator upgrades are immediately available to all users. Access is zero cost and the tutorial can be viewed on a modern laptop or desktop computer with a current web-browser. Conclusions: We present the first internet-based surgical simulator of craniofacial surgery. This freely available resource capitalizes on recent improvements in internet capability to produce an interactive virtual surgical environment for students and teachers of craniofacial surgery. This free simulator is designed as an educational resource for the next generation of craniofacial surgeons

BACKGROUND: Cleft palate is present in one-third of patients with Treacher-Collins syndrome. The authors present long-term speech and surgical outcomes of palatoplasty in this challenging patient population. METHODS: A retrospective review of all patients with Treacher-Collins syndrome and cleft palate was conducted over a 35-year period at a single institution. Demographics, palatal, mandibular, airway, and surgical outcomes were recorded. Speech outcomes were assessed by the same craniofacial speech pathologist. RESULTS: Fifty-eight patients with Treacher-Collins syndrome were identified: 43% (25) had a cleft palate and 16% (9) underwent palatoplasty at our institution. Cleft palate types included 1 Veau I, 5 Veau II, 1 Veau III, and 2 Veau IV. Mean age at the time of palatoplasty was 2.0 years (range, 1.0-6.7 years). Three patients had fistulas (33%) and underwent repairs. Pruzansky classifications included 1 type IIA, 6 type IIB, and 2 type III. Seven patients completed long-term speech evaluations. Mean age at follow-up was 13.9 years (range 2.2-24.3 years). Six patients had articulatory velopharyngeal dysfunction related to Treacher-Collins syndrome. Two patients had structural velopharyngeal dysfunction and required further palatal/pharyngeal surgery. CONCLUSIONS: Cleft palate repair in patients with Treacher-Collins syndrome has a high incidence of velopharyngeal dysfunction. However, the majority of patients are articulatory-based in whom further surgery would not provide benefit. Patients with Treacher-Collins syndrome and cleft palate require close evaluation by a speech pathologist as the incidence of articulatory dysfunction is high.

PURPOSE: Alveolar bone graft (ABG) has traditionally been performed with a postoperative inpatient stay secondary to donor site pain. Upon transitioning from an open iliac bone harvesting technique to an Acumed trephine, the authors observed that donor site pain was reduced eliminating an inpatient stay. This study examines the cost savings associated with outpatient ABG surgery. METHODS: A retrospective single-institution review was conducted on all patients who had an ABG performed from 2012 to 2015. Patients were categorized based upon hospital stay: inpatient, observation (23-hour), or outpatient. Cost data reported included: total direct cost, total variable direct cost, fixed direct cost, and the sum of total direct costs for both medical/surgical supplies and operating room costs. T tests were used to determine differences in various cost categories between groups of patients. RESULTS: Sixty-two procedures were performed: 7 procedures were inpatient, 16 observation, and 39 outpatient. The total direct costs averaged $4536 for inpatients, $3222 for the observation group, and $3340 for the outpatient group. Inpatient and outpatient costs were significantly different (P <0.01). Total variable direct costs (P <0.05) and fixed direct costs (P <0.01) were significantly lower in the outpatient/observation group. All costs for the observation group were significantly lower than inpatient costs, but were not significantly different than outpatient costs. There were no readmissions reported. CONCLUSIONS: Cost of an inpatient stay is significantly higher than outpatient or 23-hour observation for ABG procedures. The Acumed trephine technique allows for same-day discharge. In the face of declining reimbursement, safe and cost-efficient treatments are an appealing option.

BACKGROUND: Reconstruction of extensive facial and scalp burns can be increasingly challenging, especially in patients that have undergone multiple procedures with less than ideal outcomes resulting in restricting neck and oral contractures, eyelid dysfunction, and suboptimal aesthetic appearance. METHODS: To establish a reconstructive solution for this challenging deformity, a multidisciplinary team was assembled to develop the foundation to a facial vascularized composite allotransplantation program. The strategy of developing and executing a clinical transplant was derived on the basis of fostering a cohesive and supportive institutional clinical environment, implementing computer software and advanced technology, establishing a cadaveric transplant model, performing a research facial procurement, and selecting an optimal candidate with the aforementioned burn defect who was well informed and had the desire to undergo face transplantation. RESULTS: Approval from the institutional review board and organ procurement organization enabled our face transplant team to successfully perform a total face, eyelids, ears, scalp, and skeletal subunit transplant in a 41-year-old man with a full face and total scalp burn. CONCLUSIONS: The culmination of knowledge attained from previous experiences continues to influence the progression of facial vascularized composite allotransplantation. This surgical endeavor methodically and effectively synchronized the fundamental principles of aesthetic, craniofacial, and microvascular surgery to restore appearance and function to a patient suffering from failed conventional surgery for full face and total scalp burns. This procedure represents the most extensive soft-tissue clinical face transplant performed to date. CLINICAL QUESTION/LEVEL OF EVIDEMCE: Therapeutic, V.

Importance: Septal deviation commonly occurs in patients with cleft lip and palate (CLP); however, the contribution of the cartilaginous and bony septum to airway obstruction in skeletally mature patients is poorly understood. Objectives: To describe the internal nasal airway anatomy of skeletally mature patients with CLP and to determine the contributors to airway obstruction. Design, Setting, and Participants: This single-center retrospective review included patients undergoing cone-beam computed tomography (CBCT) from November 1, 2011, to July 6, 2015, at the cleft lip and palate division of a major academic tertiary referral center. Patients met inclusion criteria for the study if they were at least 15 years old at the time of CBCT, and images were used only if they were obtained before Le Fort I osteotomy and/or formal septorhinoplasty. Twenty-four skeletally mature patients with CLP and 16 age-matched control individuals were identified for the study. Main Outcomes and Measures: Septal deviation and airway stenosis were measured in the following 3 coronal sections: at the cartilaginous septum (anterior nasal spine), bony septum (posterior nasal spine), and midpoint between the anterior and posterior nasal spine. The perpendicular plate of the ethmoid bone and vomer displacement were measured as angles from the vertical plane at the coronal section of maximal septal deviation. The site of maximal septal deviation was identified. Results: Among the 40 study participants, 26 were male. The mean (SD) age was 21 (5) and 23 (6) years for patients with CLP and controls, respectively. Septal deviation in patients with CLP was significantly worse than that of controls at the anterior nasal spine (2.1 [0.5] vs 0.8 [0.2] mm; P < .05) and posterior nasal spine (2.9 [0.5] vs 1.0 [0.3] mm; P < .01) and most severe at the midpoint (mean [SD], 4.4 [0.6] vs 2.1 [0.3] mm; P < .01). The point of maximal septal deviation occurred in the bony posterior half of the nasal airway in 27 of 40 patients (68%). The CLP bony angular deviation from the vertical plane was significant in the CLP group compared with the control group (perpendicular plate of the ethmoid bone, 14 degrees [2 degrees ] vs 8 degrees [1 degrees ]; vomer, 34 degrees [5 degrees ] vs 13 degrees [2 degrees ]; P < .05 for both), and vomer deviation was significantly associated with anterior nasal airway stenosis (r = -0.61; P < .01). Conclusions and Relevance: Skeletally mature patients with CLP have significant septal deviation involving bone and cartilage. Resection of the bony and cartilaginous septum should be considered at the time of definitive cleft rhinoplasty. Level of Evidence: NA.

BACKGROUND: The application of aesthetic, craniofacial, and microsurgical principles in the execution of face transplantation may improve outcomes. Optimal soft-tissue face transplantation can be achieved by incorporating subunit facial skeletal replacement and subsequent tissue resuspension. The purpose of this study was to establish a reconstructive solution for a full face and scalp burn and to evaluate outcome precision and consistency. METHODS: Seven mock face transplants (14 cadavers) were completed in the span of 1 year. Components of the vascularized composite allograft included the eyelids, nose, lips, facial muscles, oral mucosa, total scalp, and ears; and skeletal subunits of the zygoma, nasal bone, and genial segment. Virtual surgical planning was used for osteotomy selection, and to evaluate postoperative precision of hard- and soft-tissue elements. RESULTS: Each transplant experience decreased each subsequent transplant surgical time. Prefabricated cutting guides facilitated a faster dissection of both donor and recipient tissue, requiring minimal alteration to the allograft for proper fixation of bony segments during inset. Regardless of donor-to-recipient size discrepancy, ample soft tissue was available to achieve tension-free allograft inset. Differences between virtual transplant simulation and posttransplant measurements were minimal or insignificant, supporting replicable and precise outcomes. CONCLUSIONS: This facial transplant model was designed to optimize reconstruction of extensive soft-tissue defects of the craniofacial region representative of electrical, thermal, and chemical burns, by incorporating skeletal subunits within the allograft. The implementation of aesthetic, craniofacial, and microsurgical principles and computer-assisted technology improves surgical precision, decreases operative time, and may optimize function.