Faculty Bibliography

Background/UNASSIGNED:Facial transplantation (FT) experience has grown but success in this innovative and complex field has yet to be defined. The purpose of this study is to determine attitudes regarding the failures in FT and the appropriate management of these failures. Methods/UNASSIGNED:An anonymous, 20-question survey elicited opinions regarding FT failure management. This survey was administered to attendees of 2 FT-focused national meetings. Demographics included sex, age, and personal/institutional FT experience. Attitudes related to FT recipient education, definition of FT failure, and management of complications were gathered. Results/UNASSIGNED:Eighty of 271 attendees completed the survey (29.5%). Respondents were predominantly male (81.3%) and 50 years of age or younger (80.5%). Thirty-eight percentage previously performed an FT and 53.8% were a part of an institution with a vascularized composite allotransplantation (VCA)-related Institutional Review Board (IRB). Respondents almost unanimously agreed it was "absolutely essential" to discuss possibility of FT failure (93.8%), mortality (91.1%), and treatment for chronic rejection (78.8%). However, uncertainty of failure rate existed, with 56.4% citing failure rate as unknown, 25.6% citing <25% and 18.0% citing >25%. 51.2% of those with direct FT experience lacked clear criteria for defining FT success or an institutional protocol for managing chronic rejection. 78.8% believed failed FT patients should be considered for retransplantation, but only about 25% cited functional concerns or esthetic dissatisfaction as appropriate indications. Conclusion/UNASSIGNED:There is a lack of consensus regarding definition of FT failure and rates mortality amongst experts. Even institutions with FT experience lack protocols for managing chronic rejection. Expert consensus and institutional regulations surrounding these issues are warranted.

Craniosynostosis (CS) is a frequent congenital anomaly featuring the premature fusion of 1 or more sutures of the cranial vault. Syndromic cases, featuring additional congenital anomalies, make up 15% of CS. While many genes underlying syndromic CS have been identified, the cause of many syndromic cases remains unknown. We performed exome sequencing of 12 syndromic CS cases and their parents, in whom previous genetic evaluations were unrevealing. Damaging de novo or transmitted loss of function (LOF) mutations were found in 8 genes that are highly intolerant to LOF mutation (P = 4.0 × 10^-8); additionally, a rare damaging mutation in SOX11, which has a lower level of intolerance, was identified. Four probands had rare damaging mutations (2 de novo) in TFAP2B, a transcription factor that orchestrates neural crest cell migration and differentiation; this mutation burden is highly significant (P = 8.2 × 10^-12). Three probands had rare damaging mutations in GLI2, SOX11, or GPC4, which function in the Hedgehog, BMP, and Wnt signaling pathways; other genes in these pathways have previously been implicated in syndromic CS. Similarly, damaging de novo mutations were identified in genes encoding the chromatin modifier KAT6A, and CTNNA1, encoding catenin α-1. These findings establish TFAP2B as a CS gene, have implications for assessing risk to subsequent children in these families, and provide evidence implicating other genes in syndromic CS. This high yield indicates the value of performing exome sequencing of syndromic CS patients when sequencing of known disease loci is unrevealing.

INTRODUCTION/BACKGROUND:Ongoing combat operations in Iraq, Afghanistan, and other theaters have led to an increase in high energy craniomaxillofacial (CMF) wounds. These challenging injuries are typically associated with complex tissue deficiencies, evolving areas of necrosis, and bony comminution with bone and ballistic fragment sequestrum. Restoring form and function in these combat-sustained CMF injuries is challenging, and frequently requires local and distant tissue transfers. War injuries are different than the isolated trauma seen in the civilian sector. Donor sites are limited on patients with blast injuries and they may have preferences or functional reasons for the decisions to choose flaps from the available donor sites. METHODS:A case series of patients who sustained severe combat-related CMF injury and were treated at Walter Reed National Military Medical Center (WRNMMC) is presented. Our study was exempt from Institutional Review Board review, and appropriate written consent was obtained from all patients included in the study for the use of representative clinical images. RESULTS:Four patients treated by the CMF team at Walter Reed National Military Medical Center are presented. In this study, we highlight their surgical management by the CMF team at WRNMMC, detail their postoperative course, and illustrate the outcomes achieved using representative patient clinical images. We also supplement this case series demonstrating military approaches to complex CMF injuries with CMF reconstructive algorithms utilized by the senior author (EDR) in the management of civilian complex avulsive injuries of the upper, mid, and lower face are thoroughly reviewed. CONCLUSION/CONCLUSIONS:While the epidemiology and characteristics of military CMF injuries have been well described, their management remains poorly defined and creates an opportunity for reconstructive principles proven in the civilian sector to be applied in the care of severely wounded service members. The War on Terror marks the first time that microsurgery has been used extensively to reconstruct combat sustained wounds of the CMF region. Our manuscript reviews various options to reconstruct these devastating CMF injuries and emphasizes the need for steady communication between the civilian and military surgical communities to establish the best care for these complex patients.

Operative experience for the contemporary trainee has become exceedingly more challenging in the setting of more stringent hospital regulations. Surgical training is thus shifting toward more self-directed, independent learning to maximize operative opportunities as they become available; yet, this can prove difficult for complex surgeries like craniofacial procedures. The intricate anatomy and fine reconstructive techniques employed cannot be readily depicted onto a two-dimensional page. To address this educational gap, the Craniofacial Interactive Virtual Assistant-Pro Edition (CIVA-Pro) was developed as a web-based surgical simulator to aid learners with conceptualizing the surgical principles utilized in these cases. The current work reviews the Bilateral Sagittal Split Osteotomy module of CIVA-Pro, providing detailed narratives for each chapter with expert commentary on broadened indications and future directions.

INTRODUCTION/BACKGROUND:The number of patients who may benefit from evaluation for face transplantation in the United States (US) remains largely unknown. The goal of our study was to better delineate the pool of patients who might benefit from face transplant evaluation based on the characteristics and mechanisms of injury of previously reported face transplant recipients. METHODS:The authors utilized data from the National Electronic Injury Surveillance System-All Injury Program in this study. The US Census Bureau data were used for population estimates. Inclusion and exclusion criteria were determined based on the characteristics of face transplant recipients to date, and the mechanisms of injury they sustained ultimately necessitating face transplantation. Statistical significance was reached if P <0.05. RESULTS:The estimated annual incidence of preventable craniofacial injuries from firearms (44,266-58,299; 31.7% increase), burns (5712-19,433; 240.2% increase), and animal attacks (5355-14,666; 173.9% increase) increased from 2005 to 2014, whereas the estimated annual incidence of craniofacial injuries from machinery (3927-2933; 25.3% decrease) decreased between 2005 and 2014. The authors estimate the annual incidence rate to fall between 32.1 per 100,000 and 58.1 per 100,000 among individuals aged 20 to 64 in the US. CONCLUSION/CONCLUSIONS:In this study, the authors estimate the annual incidence rate of individuals aged 20 to 64 in the US who may benefit from face transplant evaluation and believe that this quantification has the potential to initiate actionable discussions regarding geographical and financial factors affecting access to care in this patient population.

INTRODUCTION/BACKGROUND:Online resources have become a major source of medical information for the general public. To date, there has not been an assessment of patient-oriented online resources for face and upper extremity transplantation candidates and patients. The goal of this study is to perform a comprehensive assessment of these resources. METHODS:Our analysis relied on 2 dimensions: comprehensiveness and readability. Comprehensiveness was evaluated using 14 predetermined variables. Readability was evaluated using 8 different readability scales through the Readability Studio Professional Edition Software (Oleander Software, Ltd, Vandalia, Ohio). Data were also collected from solid organ transplantation (SOT), specifically kidney and liver, programs for comparison. RESULTS:Face and upper extremity transplantation programs were significantly more likely to list exclusion criteria (73.9% vs 41.2%; P = 0.02), the need for life-long immunosuppression (87.0% vs 58.8%; P = 0.02), and benefits of transplantation (91.3% vs 61.8%; P = 0.01) compared with SOT programs. The average readability level of online resources by all face and upper extremity transplantation programs exceeded the sixth grade reading level recommended by the National Institutes of Health and the American Medical Association. The average reading grade level of online resources by these programs was also significantly higher than those of SOT with both exceeding the recommended reading level (13.95 ± 1.55 vs 12.60 ± 1.65; P = 0.003). CONCLUSIONS:Future efforts in face and upper extremity transplantation should be directed toward developing standardized, comprehensive, and intelligible resources with high-quality content and simple language.

INTRODUCTION/BACKGROUND:Orthognathic surgery plays an important role in restoring aesthetic facial contour, correcting dental malocclusion, and the surgical treatment of obstructive sleep apnea. However, the rate of complications following bimaxillary as compared with single-jaw orthognathic surgery remains unclear. The authors therefore sought to evaluate complication rates following bimaxillary as compared with single-jaw orthognathic surgery MATERIALS AND METHODS:: The American College of Surgeons National Surgical Quality Improvement Program database was used to identify comparison groups. Preoperative characteristics and postoperative outcomes were compared between groups. The listed procedures have different operating times and characteristics with longer time expected in the bimaxillary osteotomies group. Regression analyses were performed to control for potential confounders. RESULTS:The 3 groups of interest included patients who underwent mandibular osteotomies (n = 126), LeFort I osteotomy (n = 194), and bimaxillary osteotomies (n = 190). These procedures have different operating times, with a longer time expected with bimaxillary osteotomies. Patients undergoing bimaxillary osteotomies had significantly higher rates of early wound complications, overall complications, longer mean operative time, and mean hospital length of stay. Performing bimaxillary osteotomies in the outpatient setting was an independent risk factor for wound complications (OR = 12.58; 95% CI: 1.66-95.20; P = 0.01), while an ASA class of 3 or more was an independent risk factor for overall complications (OR = 3.61; 95% CI: 1.02-12.75; P = 0.04) and longer hospital length of stay (β = 4.96; 95% CI: 2.64 - 7.29; P < 0.001). CONCLUSIONS:Surgery in the outpatient setting as well as patient American Society of Anesthesiology physical status class 3 or higher were independent factors for postoperative adverse events in patients undergoing bimaxillary surgery. Our findings highlight the importance of addressing modifiable risk factors preoperatively and the need for closer postoperative monitoring in this patient population for optimal outcomes.

PURPOSE/OBJECTIVE:To highlight the challenges and lessons learned in tooth-bearing maxillomandibular facial allotransplantation. PATIENTS AND METHODS/METHODS:Two patients with ballistic composite facial injury underwent tooth-bearing maxillomandibular facial transplantation (FT) after informed consent and institutional review board approval. Patient 1 had undergone total face, double jaw, teeth, and tongue transplantation in March 2012. Patient 2 had undergone partial face, double jaw, and teeth transplantation in January 2018. Le Fort III and bilateral sagittal split skeletal osteotomies were performed in both transplants. Computerized surgical planning was used in both cases, and the allografts were transferred in intermaxillary fixation (IMF) with prefabricated dental splints before rigid skeletal fixation. RESULTS:Normal class I occlusion was achieved at the conclusion of each surgery. Patient 1 had developed a 2 × 2-mm palatal fistula in the early postoperative period and had also gradually developed class III malocclusion. Orthodontic treatment was started at 5 months after transplantation but failed. A Le Fort III advancement was performed 1 month later with successful restoration of class I occlusion. The palatal fistula was successfully repaired at 9 postoperative months. Patient 2 developed a postoperative palate and floor of mouth dehiscence, requiring palatal repair and hyoid and genioglossus advancement on postoperative day (POD) 11. Orthodontic treatment was initiated for Class II malocclusion. On POD 108, left mandibular nonunion was diagnosed. Left coronoidectomy, open reduction, and internal fixation were performed. IMF was maintained for 2 weeks. Orthodontic treatment was then resumed, with normalization of the occlusion by 10 months after FT. CONCLUSIONS:Maxillomandibular transplantation is a viable reconstructive solution for composite midface defects not amenable to autologous reconstruction. Improvement of functional outcomes and prevention of major complications rely on close attention to occlusal relationships, temporomandibular joint dynamics, dental health, and the intraoral donor-recipient soft tissue interface.

Facial transplant (FT) is a viable option for patients with severe craniomaxillofacial deformities. Transplant imaging requires coordination between radiologists and surgeons and an understanding of the merits and limitations of imaging modalities. Digital subtraction angiography and CT angiography are critical to mapping vascular anatomy, while volume-rendered CT allows evaluation of osseous defects and landmarks used for surgical cutting guides. This article highlights the components of successful FT imaging at two institutions and in two index cases. A deliberate stepwise approach to performance and interpretation of preoperative FT imaging, which consists of the modalities and protocols described here, is essential to seamless integration of the multidisciplinary FT team. ^©RSNA, 2019 See discussion on this article by Lincoln .