Faculty Bibliography

Background/Purpose: Despite the increasing trend toward ambulatory surgery rate in general, for cleft lip repair, 72.1% of patients in the United States are still hospitalized. Multiple centers have been studying this and with the very recent publishing of 2 large volume studies published in Plastic and Reconstructive Surgery supporting the safety of outpatient cleft lip surgery, it has become a high-profile debatable topic in cleft management. Last year at ACPA in the plastic surgery breakout session there was a heated debate on the topic between surgeons. And just this summer, one of the authors articles was featured as the PRS Journal Club article of the month via Social media, where there were 68 comments, 11 shares, and 40 likes, which placed the article in the TOP 15 most viewed article in PRS Journal's website. So this is a topic that more and more surgeons and craniofacial teams are thinking about and considering changing practice management but that deserves being brought into the spotlight to discuss all the pros and cons. The goal of the presentation are to present a literature review up to date on inpatient versus outpatient cleft lip surgery and have members of the panel share their and data behind their approach to postoperative cleft lip management. We would like to focus on what has been studied and reported versus what people practice because "that is how they were trained" or that is how they "historically" have always done it. Methods/Description: We will start with an overview of the literature surrounding inpatient versus outpatient cleft lip surgery and epidemiology about the numbers of institutions that do inpatients versus outpatient. If possible, we would like to include an audience poll to get an idea of practice patterns in the room. Then based on the recent published studies, each panelist will share different protocols that have been successful for outpatient cleft lip management and how that could be implemented if a center wants to consider outpatient cleft lip surgery. This includes: preoperative education that can be done by the team or nurse managers, perioperative management before, during, and after the operation, and postoperative care after they go home. All are very key components to a successful outpatient cleft lip management. We will also discuss the barriers to doing outpatient cleft lip surgery as well as having a panelist who consistently does inpatient cleft lip surgery to present the rationale behind their practice management and also their barriers to outpatient cleft lip surgery. I have reached out to a few people and will be discussing with them at the upcoming ASPS meeting to see who may be interested in joining the panel to discuss that. We will discuss the economic impact overall of practice changes to a predominantly outpatient surgery and also potential insurance policy impact. We think this will be a very useful topic for any team member participating in the care of a cleft lip patient

Background/Purpose: Alveolar cleft surgery is the most common bone reconstruction performed in patients with a cleft. Osteogenic agents such as BMP-2 have been used to restore the bony cleft without the morbidity of bone graft, but concerns remain regarding premature fusion of sutures, exuberant bone formation, and malignant degeneration. We have recently demonstrated that dipyridamole-coated, 3D printed bio-ceramic (3DPBC) scaffolds generate comparable bone amounts to BMP2 and significantly greater bone compared to negative controls in short-term growing animal model studies. No detrimental effects to growth sutures were noted in any animals. This study investigates the long-term osteogenic properties, degradation kinetics, and effects on facial growth of these tissue engineering constructs in growing animal models. Methods/Description: Twenty-two 1-month-old (immature) New Zealand white rabbits underwent creation of unilateral 3.5 x 3.5 mm alveolar defects. Each alveolar defect was repaired with either 3DPBC scaffolds coated with 1000 muM dipyridamole (n = 14) or with autogenous bone graft from the radius (n = 8). Six rabbits from the 3DPBC scaffold group were sacrificed at 8 weeks. The remaining rabbits (n = 8 each group) were euthanized following completion of craniofacial growth (6 months). Bone regeneration, scaffold degradation, and maxillary suture patency were calculated using CT images reconstructed and analyzed in Amira software. Facial symmetry was evaluated using dense-surface 3D modeling and validated with bilateral cephalometric measurements of maxillary projection. Bone growth and suture patency were qualitatively evaluated through histologic analysis.
Result(s): After 6 months, animals with defects repaired with 3DPBC scaffolds regenerated an average of 52.9% +/- 3.3% bone (mean +/- SEM), compared to 40.7%+/-4.0% in defects repaired with bone graft (P = .02). This is compared to unoperated alveolus occupied by 39.3% +/- 1.6% bone. Scaffolds showed significant degradation at 6 months (6.7% +/- 1.6%) compared to at 8 weeks (27.1% +/- 1.9%; P >= .001). Morphometric analysis using dense surface modeling showed similar symmetry indices of 55.0 +/- 3.3 for scaffold animals and 61.7% +/- 1.6% for bone graft animals (P = .10). Comparative measurements of operated and unoperated sides showed no significant differences in asymmetry between scaffold and bone graft animals (P = .86). Histologic analysis of scaffold samples revealed vascularized, organized bone within scaffold interstices without evidence of ectopic bone, excess inflammatory cells, or suture fusion.
Conclusion(s): In a growing animal model, dipyridamole-coated 3DPBC scaffolds can regenerate bone comparable to autogenous bone graft by radiographic and histologic analysis. Over 6 months, scaffolds show significant, favorable degradation and do not result in premature suture fusion or disruption of facial growth compared to bone graft. These results support long-term safety and efficacy of this tissue engineering strategy in the repair of alveolar cleft defects

OBJECTIVE/UNASSIGNED:Assess the weight and contribution of each of the parameters of the Asher-McDade Scale to overall subjective assessment of nasolabial aesthetics following cleft lip repair. DESIGN/UNASSIGNED:Retrospective cohort evaluation. SETTING/UNASSIGNED:Cleft and craniofacial center. PARTICIPANTS/UNASSIGNED:Forty-one patients who underwent unilateral cleft lip repair. INTERVENTIONS/UNASSIGNED:Unilateral cleft lip repair. MAIN OUTCOME MEASURES/UNASSIGNED:Nasolabial rating using the Asher-McDade scale and overall subjective assessment of nasolabial aesthetics using a rank score following unilateral cleft lip repair. RESULTS/UNASSIGNED:= .69; P < .001). CONCLUSION/UNASSIGNED:The parameters evaluated in the Asher-McDade scale have different weights and contribute differently to overall subjective assessment of nasolabial aesthetic outcomes following cleft lip repair. Adjusting for their weights results in a modified score that demonstrates superior correlation with overall subjective assessment of nasolabial aesthetic outcomes.

BACKGROUND:Alveolar clefts are traditionally treated with secondary bone grafting, but this is associated with morbidity and graft resorption. Although recombinant human bone morphogenetic protein-2 (rhBMP-2) is under investigation for alveolar cleft repair, safety concerns remain. Dipyridamole is an adenosine receptor indirect agonist with known osteogenic potential. This study compared dipyridamole to rhBMP-2 at alveolar cleft defects delivered using bioceramic scaffolds. METHODS:Skeletally immature New Zealand White rabbits underwent unilateral, 3.5 × 3.5-mm alveolar resection adjacent to the growing suture. Five served as negative controls. The remaining defects were reconstructed with three-dimensionally printed bioceramic scaffolds coated with 1000 μm of dipyridamole (n = 6), 10,000 μm of dipyridamole (n = 7), or 0.2 mg/ml of rhBMP-2 (n = 5). At 8 weeks, new bone was quantified. Nondecalcified histologic evaluation was performed, and new bone was evaluated mechanically. Statistical analysis was performed using a generalized linear mixed model and the Wilcoxon rank sum test. RESULTS:Negative controls did not heal, whereas new bone formation bridged all three-dimensionally printed bioceramic treatment groups. The 1000-μm dipyridamole scaffolds regenerated 28.03 ± 7.38 percent, 10,000-μm dipyridamole scaffolds regenerated 36.18 ± 6.83 percent (1000 μm versus 10,000 μm dipyridamole; p = 0.104), and rhBMP-2-coated scaffolds regenerated 37.17 ± 16.69 percent bone (p = 0.124 versus 1000 μm dipyridamole, and p = 0.938 versus 10,000 μm dipyridamole). On histology/electron microscopy, no changes in suture biology were evident for dipyridamole, whereas rhBMP-2 demonstrated early signs of suture fusion. Healing was highly cellular and vascularized across all groups. No statistical differences in mechanical properties were observed between either dipyridamole or rhBMP-2 compared with native bone. CONCLUSION/CONCLUSIONS:Dipyridamole generates new bone without osteolysis and early suture fusion associated with rhBMP-2 in skeletally immature bone defects.

BACKGROUND:Facial transplantation introduced a paradigm shift in the reconstruction of extensive facial defects. Although the feasibility of the procedure is well established, new challenges face the field in its second decade. METHODS:The authors' team has successfully treated patients with extensive thermal and ballistic facial injuries with allotransplantation. The authors further validate facial transplantation as a reconstructive solution for irreparable facial injuries. Following informed consent and institutional review board approval, a partial face and double jaw transplantation was performed in a 25-year-old man who sustained ballistic facial trauma. Extensive team preparations, thorough patient evaluation, preoperative diagnostic imaging, three-dimensional printing technology, intraoperative surgical navigation, and the use of dual induction immunosuppression contributed to the success of the procedure. RESULTS:The procedure was performed on January 5 and 6, 2018, and lasted nearly 25 hours. The patient underwent hyoid and genioglossus advancement for floor-of-mouth dehiscence, and palate wound dehiscence repair on postoperative day 11. Open reduction and internal fixation of left mandibular nonunion were performed on postoperative day 108. Nearly 1 year postoperatively, the patient demonstrates excellent aesthetic outcomes, intelligible speech, and is tolerating an oral diet. He remains free from acute rejection. CONCLUSIONS:The authors validate facial transplantation as the modern answer to the classic reconstructive challenge imposed by extensive facial defects resulting from ballistic injury. Relying on a multidisciplinary collaborative approach, coupled with innovative emerging technologies and immunosuppression protocols, can overcome significant challenges in facial transplantation and reinforce its position as the highest rung on the reconstructive ladder. CLINICAL QUESTION/LEVEL OF EVIDENCE/METHODS:Therapeutic, V.

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.

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.

BACKGROUND:Single-stage primary cleft lip and palate (PCLP) repair is controversial in the United States, and most patients are treated with a staged approach. In this study, early postoperative complications of the single-stage approach as compared to primary cleft lip (PCL) or primary cleft palate (PCP) alone were evaluated. This study represents the largest cohort of patients undergoing combined cleft lip and palate repair. METHODS:The American College of Surgeons National Surgical Quality Improvement Program-Pediatric database was used to identify patients undergoing single-stage PCLP, PCL, or PCP repairs. Preoperative factors and postoperative outcomes were compared between the 3 groups, as well as within the PCLP group between patients with and without complications. Univariate and multivariate analyses were performed. RESULTS:A TOTAL OF:: 181 patients were included in the single-stage PCLP group, 1007 in the PCP group and 783 in the PCL group. There was no difference in the rates of early complications between the 3 groups. Within the PCLP group, cardiac risk factors (β = 35.19; 95% confidence interval [CI] 7.88-75.21; P = 0.04) and complications (β = 77.31; 95% CI 35.82-118.79; P < 0.001) were significant risk factors for longer operative time. CONCLUSION/CONCLUSIONS:Analysis of a national database showed that single-stage PCLP repair is not associated with increased risk of early postoperative complications as compared to primary lip or palate repair alone. In-depth long-term analyses of craniofacial morphology, fistulae rate, speech, and dental outcomes are essential for a comprehensive assessment of the effects of combined cleft lip and palate repair.

Nasal trauma is a common complication of nasal continuous positive airway pressure (NCPAP) and may range from erythema, edema, and skin breakdown to columellar necrosis. Although rare, columellar necrosis can be a devastating complication following NCPAP, and surgical repair remains challenging due to contour and color-match difficulties, tenuous vascularity, and limited available adjacent skin. In addition, because operative site protection is critical to a successful repair, many surgeons opt to delay surgical intervention from infancy until a later age so that the patient does not inadvertently injure and compromise the graft during the early postoperative period. Here, we present a case of composite nasal reconstruction in an infant following columellar necrosis secondary to NCPAP, along with the design and implementation of a simple, inexpensive, and protective nasal splint that allows for early repair at the time of infancy.

OBJECTIVE/UNASSIGNED:Informed decision-making relies on available information, including online resources. We evaluated the content and readability of websites published by American Cleft Palate-Craniofacial Association (ACPA)-approved cleft lip and/or palate (CLP) teams in the United States. DESIGN/UNASSIGNED:Team websites were reviewed, and teams with no accessible website or <30 sentences of content were excluded. Website content was scored by presence/absence of 20 variables derived from ACPA approval standards. Readability was evaluated with 8 scales. Readability was then compared to American Medical Association (AMA) recommendations. The relationship between website content and readability was assessed. MAIN OUTCOME MEASURE(S)/UNASSIGNED:Content and readability of team websites. RESULTS/UNASSIGNED:From 167 reviewed teams, 47 (28.1%) had nonfunctional links, 17 (10.2%) had no accessible website, and 39 (23.4%) had <30 sentences. The average content score for all 111 team websites included was 14.5 (2.6) of 20. The combined average reading level across all scales (10.7 [1.9]) exceeded the AMA-recommended sixth-grade reading level; this finding held true for each individual website. Children's Hospital-affiliated teams (n = 86) had a significantly higher content score (14.8 vs 13.5; P = .03) and better readability as evidenced by lower reading grade level (10.5 vs 11.4; P = .04). On linear regression, a higher content score significantly predicted better readability (β = -0.226; P < .001). CONCLUSIONS/UNASSIGNED:Websites published by ACPA-approved CLP teams vary in accessibility and content and exceed the recommended reading level. These findings could inform future efforts to improve patient-oriented resources.