Faculty Bibliography

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.

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.

Background/Purpose: Patients with cleft lip and palate commonly require an alveolar bone graft (ABG) at approximately 8 years old to allow for tooth descent in the location of the alveolar cleft. This procedure was previously performed with at least one post-operative overnight stay due to hip pain. Upon transitioning from an open iliac crest bone harvesting technique to an Acumed drill, we observed that most patients had limited hip pain and were treatable as outpatients. We report feasibility and cost analysis of performed ABG surgery as an outpatient using the Acumed drill technique. Methods/Descriptions: Under IRB-approval, a 3-year retrospective review was conducted of all subjects born with a cleft lip/palate (CLP) and who had an alveolar bone graft performed between 2012 and 2015. Patients were categorized based upon hospital stay: inpatient (overnight stay), observation (23-hour), or outpatient. Prior to April 2013, patients underwent ABG surgery as a planned inpatient procedure (Group 1). After April 2013, patients underwent ABG surgery as a planned outpatient procedure (Group 2). Cost data acquired included: total direct cost (costs associated directly with patient care), total variable direct cost (costs which vary according to the needs of each patient), fixed direct cost (charges that do not fluctuate depending on what is done), 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: 58 patients were enrolled with a total of 62 procedures (4 patients had 2 ABGs), and with a mean age of 10 years at the time of surgery. 7 procedures were inpatient, 16 were observation, and 39 were outpatient. 7 of 14 Group 1 patients (50%) were admitted as inpatients, whereas 0 of 48 Group 2 patients were inpatients. The total direct costs averaged $4,536 for inpatients, $3,222 for the observation group, and $3,340 for the outpatient group. These inpatient and outpatient costs were significantly different (p<.001). There were significantly reduced total variable direct costs (p<0.05) and fixed direct costs (p<0.001) when comparing the outpatient to inpatient groups. Costs for the observation group were significantly lower in all three cost categories than inpatient costs, but were not significantly different than outpatient costs. Of the 39 outpatient procedures, no patients visited urgent care within 30 days postoperatively, and observation group had two patients visit urgent care. None were readmitted in any group. Conclusions: In ABG surgery done on patients born with a CLP, the costs for an inpatient stay are significantly higher than those of patients discharged the same day or who stay in the hospital for less than 23 hours on observational status. The Acumed drill technique results in improved pain control with no appreciable harms to the outpatient group, allowing for same day discharge

Background/Purpose: The complicated nature of craniofacial procedures can be difficult for patients and families to understand. Patients and families undergoing treatment for facial differences may, understandably, find the process overwhelming, as consultations are often scheduled with multiple members of the Craniofacial Team. Furthermore, a plethora of online (mis)information, commonly turns the consultation into a clarification session, rather than an opportunity to educate and address concerns. In order to provide a dependable resource for patients and families, we introduce a multi-media education portal for craniofacial surgical procedures. Methods/Descriptions: Postoperative interviews of adult-aged patients and parents of school-aged patients were performed to determine specific information that would have been useful upon consultation regarding their craniofacial surgery and the recovery process. Based on interview responses, the authors developed a free, multimedia educational portal. Previously constructed craniofacial digital animations were edited and upgraded in Maya 10 (Autodesk, San Rapheal, CA) and uploaded into the cloud-based multimedia portal. Voice-over, text and video were also created based on patient and parent instruction. Patient feedback was obtained on preliminary versions to further refine the portal. Results: An online portal has been designed to describe six common and complex craniofacial surgery procedures: Le Fort I, Le Fort III, Bilateral Sagittal Split Osteotomy, Monobloc, Genioplasty, Fronto- Orbital Advancement with Cranial Vault Remodeling. For each procedure, a brief text/voice-over description of the objectives of the surgery is followed by a schematic, yet anatomically accurate, threedimensional animation of the operation with voice-over narration. Then, a detailed description of the recovery process precedes video interviews of patients and families discussing their experiences. A portable document format (PDF) file is available for download with more detaied reading material related to each procedure. Conclusions: We introduce an online multimedia education portal containing scrutinized, sound, and procedure-specific information for patients and families undergoing craniofacial surgery. This freely available resource is intended to supplement preoperative consultation with the craniofacial team and may prove a powerful tool for patients and families undergoing the stressful process of facial reconstruction

Background/Purpose: Multidisciplinary Cleft and Craniofacial Centers require significant investment and maintenance by medical schools and/or departments, and yet the variables contributing to their academic productivity remain unknown. This study characterizes the elements that result in high academic productivity in Cleft and Craniofacial Centers. Methods/Descriptions: All cleft and craniofacial centers accredited by American Cleft Palate-Craniofacial Association were included. Vari-ables such as university affiliation (UNI); resident training (RES); number of active surgical and orthodontic faculty (FAC); and investment in a craniofacial surgery (CF) or craniofacial orthodontics (CO) fellowship program, or both (CF+CO), were obtained for each center. All craniofacial and cleft-related research published between July 2005 and June 2015 was identified using the National Library of Medicine database; for each article, journal of publication and impact factor were also recorded. A stepwise multivariable linear regression analysis was performed on the listed variables to outcome measures of total publications, summative impact factor, and basic science publications. Results: A total of 160 centers were identified, comprising 690 active faculty, 29 craniofacial fellowships and 9 orthodontic fellowships; 2,093 articles were published in 199 journals within the study period. Variables most positively associated to a high number of publications were, in order: CF+CO (beta, CF+CO = 0.555, p < 0.001), CF (beta, CF= 0.248, p < 0.001), RES (beta, RES = 0.198, p = 0.003). Variables most positively associated to a high summative impact factor are, in order: CF+CO (beta, CF+CO = 0.551, p < 0.001), CF (beta, CF = 0.313, p < 0.001), FAC (beta, FAC = 0.183, p = 0.006). Variables most positively associated to basic science publications are, in order: CF+CO (beta, CF+CO=0.491, p < 0.001), CF (beta, CF=0.322, p < 0.001), and RES (beta, RES = 0.164, p = 0.032). Conclusions: Participation in both craniofacial surgery and orthodontic fellowships demonstrate the strongest association with academic success; craniofacial fellowship alone, residency programs and number of active faculty are also predictive. Cleft and Craniofacial Centers interested in academic performance should allocate funds and resources into these variables, particularly interdisciplinary partnerships between surgery and dentistry

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