Faculty Bibliography

Background/Purpose: LeFort III distraction osteogenesis is commonly recommended for children with syndromic craniosynostosis to reduce exorbitism, improve airway function, and decrease dysmorphism. This purpose of this study is to report on the long-term clinical outcomes of patients with syndromic craniosynostosis patients who have undergone early primary subcranial LeFort III distraction osteogenesis and who have been followed longitudinally through skeletal maturity. Methods/Description: Retrospective review of all patients who underwent LeFort III distraction osteogenesis between the ages of 3 and 11 years and were followed throughout development with longitudinal dental, medical, radiographic, and photographic evaluations conducted through skeletal maturity and beyond. Inclusion criteria entailed having preoperative medical photographs and cephalometric studies at 6 months and 1, 5, and 10 years postoperatively after the primary LeFort III distraction osteogenesis as well as cephalometric documentation 6 months and 1 year after the secondary midface advancement after skeletal maturity.
Result(s): Seventeen patients fulfilled inclusion criteria, with a mean age of 5.7 years at the time of initial LeFort III distraction. The mean advancement of point A was 14.9 mm anteriorly and 2.7 mm inferiorly along the x- and y-axis, respectively. Orbitale moved 10.5 mm anteriorly and 2.2 mm inferiorly along the x- and y-axis, respectively. At 10 years postoperatively, point A moved 3.4 mm anterior along the xaxis and 4.7 mm inferiorly along the y-axis, while orbitale moved 0.4 mm posteriorly and 3 mm inferiorly along the x- and y-axis, respectively. At the time of skeletal maturity, there was a return of occlusal disharmony from normal mandibular growth and a return of proptosis owing to remodeling of orbitale inferiorly, and the lateral orbital rim posteriorly, while the globe continued to grow in the anterior vector. All but 1 study patient underwent or is scheduled to undergo a secondary midface advancement at the LeFort III and LeFort I level after skeletal maturity was attained.
Conclusion(s): The data demonstrate that patients who undergo early LeFort III distraction osteogenesis before the age of mixed dentition will still most likely need a secondary midface advancement after skeletal maturity is reached given that there is a small degree of anterior growth at the level of the maxilla and no anterior growth at orbitale over time

Background/Purpose: Financial constraints are driving hospitals toward shortening patient stay and favoring outpatient surgery when appropriate. This study compares perioperative complications between the outpatient and inpatient settings in patients undergoing primary cleft lip surgery (PCLS) and identifies risk factors associated with complications and longer lengths of stay. Methods/Description: The American College of Surgeons National Surgical Quality Improvement Program Pediatric database (ACS NSQIP-Pediatric) was reviewed from 2012 to 2015 using Current Procedure Terminology (CPT) codes for PCLS. Patients older than 5 years or undergoing concurrent cleft palate surgery were excluded. The objective of our study was to compare perioperative complications following outpatient vs inpatient PCLS. Statistical analyses were performed using SPSS (version 21.0; IBM Corp, Armonk, NY).
Result(s): We identified 3142 (1721 inpatient vs 1421 outpatient) eligible patients. The majority of patients were males (63.0%) and underwent unilateral PCLS (78.5%). Plastic surgeons were the most frequent providers (85.1%) performing these procedures followed by otolaryngologists (14.0%). The most commonly performed concurrent procedures were cleft lip rhinoplasty (24.2%) and tympanostomy tube insertion (4.7%). Tissue grafting and gingivoperiosteoplasty were each performed in 1.2% of patients. Mean age in days and weight in kilograms at surgery were 200.8+/-223.3 and 7.0+/-3.2, respectively. Mean age (222.6+/-258.7 vs 182.9+/-187.7; P < .001) and weight (7.1 +/- 3.2 vs 6.9 +/- 3.5; P = .03) were significantly higher in the outpatient group. The inpatient group included a significantly higher proportion of patients with cardiac risk factors (12.9% vs 9.4%; P = .002) and oxygen dependence (1.1% vs 0.4%; P = .02). Rates of surgical site infections, wound dehiscence, reoperation, readmission, 30-day mortality, cardiac arrest, transfusion requirements, reintubation and operative time were comparable between groups on univariate analysis. Multivariate regression showed that an underlying structural pulmonary abnormality was significantly associated with a longer hospital length of stay (B = 4.94, P = .001, 95% CI [2.21,7.66]). No other significant associations were identified on multivariate analysis.
Conclusion(s): Surgical site infections, wound dehiscence, reoperation, readmission, 30-day mortality, and other perioperative complications are comparable in patients undergoing outpatient and inpatient PCLS. Patient selection remains the cornerstone for safe practice. Increasing health care fiscal constraints warrant considering outpatient PCLS for appropriate candidates

Background/Purpose: Skeletal Class III malocclusion with maxillary hypoplasia results in anterior-posterior discrepancy of the upper and lower incisors position and lip incompetence. This affects the ability to achieve appropriate placement of tongue and management of the intraoral air pressure for sound production, resulting in perceived nasal emission and abnormal articulatory gestures. The aim of this study is to investigate the relationship between occlusal disharmony, velopharyngeal competence, and speech outcomes in patients with skeletal Class III malocclusion undergoing orthognathic surgery. Methods/Description: Seventy-five consecutive patients between 2015 and 2017 who underwent orthognathic surgery secondary to maxillary hypoplasia were evaluated on type of orthognathic procedure (LeFort I only; LeFort I and III; LeFort I and BSSO); amount of anterior and vertical advancement achieved, severity of skeletal Class III malocclusion, and lip incompetence. Patients were divided into 3 groups: cleft lip and palate, syndromic, and those with noncleft/nonsyndromic skeletal deformity. Each group received speech assessments preoperatively and 3 months postoperatively (velopharyngeal competence, resonance and articulatory integrity, and the Pittsburgh Weighted Speech Score [PWSS]).
Result(s): Following exclusionary analysis, 58 patients were included in the study; cleft lip and palate (n = 28), syndromic (n = 15), and noncleft/nonsyndromic skeletal (n =15). Preoperatively, the cleft palate and syndromic groups with increased mean skeletal discrepancy and lip incompetence values presented with higher total mean PWSS scores, and mean nasal emission values than the noncleft/nonsyndromic group (P <= .001). Postoperatively, the cleft group that underwent LFI only presented with insignificantly improved total PWSS (P <= .99) and nasality (P <= .28) scores. The syndromic patients who had the most severe skeletal discrepancy preoperatively and who underwent an LFI and LFIII combined procedure continued to present with significantly higher mean nasal emission values postoperatively than cleft and noncleft/ nonsyndromic patients undergoing LFI only or LFI and BSSO (P < .001). There was no significant difference in the amount of anterior advancement achieved and degree of velopharyngeal competence.
Conclusion(s): Our results show that orthognathic surgery to correct skeletal disharmony does not provide increased compromise to the velopharynx. However, the severity of the skeletal Class III malocclusion and lip position, especially those with a syndromic diagnosis, increases presence of nasal emission pre- and postoperatively. This study suggests that severity of skeletal Class III malocclusion can contribute to the perception of nasality post orthognathic surgery. Severity of skeletal discrepancy should be considered as an additional iatrogenic factor related to perceived velopharyngeal competence in orthognathic surgery

OBJECTIVE:/UNASSIGNED:To describe the conduct of the first multidisciplinary simulation-based workshop in the Middle East/North Africa region and evaluate participant satisfaction. DESIGN:/UNASSIGNED:Cross-sectional survey-based evaluation. SETTING:/UNASSIGNED:Educational comprehensive multidisciplinary simulation-based cleft care workshop. PARTICIPANTS:/UNASSIGNED:Total of 93 workshop participants from over 20 countries. INTERVENTIONS:/UNASSIGNED:Three-day educational comprehensive multidisciplinary simulation-based cleft care workshop. MAIN OUTCOME MEASURES:/UNASSIGNED:Number of workshop participants, number of participants stratified by specialty, satisfaction with workshop, number of workshop staff, and number of workshop staff stratified by specialty. RESULTS:/UNASSIGNED:The workshop included 93 participants from over 20 countries. The response rate was 47.3%, and participants reported high satisfaction with all aspects of the workshop. All participants reported they would recommend it to colleagues (100.0%) and participate again (100.0%). No significant difference was detected based on participant specialty or years of experience. The majority were unaware of other cleft practitioners in their countries (68.2%). CONCLUSION:/UNASSIGNED:Multidisciplinary simulation-based cleft care workshops are well received by cleft practitioners in developing countries, serve as a platform for intellectual exchange, and are only possible through strong collaborations. Advocates of international cleft surgery education should translate these successes from the regional to the global arena in order to contribute to sustainable cleft care through education.

Background/UNASSIGNED:In the subunit principle of nasal reconstruction, the valleys and low ridges of the nose are designated as topographic subunits. Surgical scars can be located at the borders of subunits to hide their appearance. Case Report/UNASSIGNED:A 30-month-old female presented with an obstructing nasal glial heterotopia (nasal glioma). Using the nasal subunit approach, the mass was exposed using an incision along the subunit borders of the nose. The nasal glioma was completely resected, and the internal nasal valve and the deformed lower lateral cartilages were reconstructed through the subunit approach access incision. The final scar was placed along the subunit borders of the nose. At 6-month follow-up, the patient demonstrated no airway obstruction, adequate nasal contour, and an esthetic nasal scar. Conclusion/UNASSIGNED:The subunit approach for a large, obstructing nasal glial heterotopia allows direct exposure for tumor resection, framework reconstruction, placement of the incision in an esthetic location, and excision of the expanded skin for recontouring of the skin envelope.

Amniotic band sequence is a complex congenital anomaly in which infants with typically no known genetic mutation have bands of maternal amniotic tissue wrapped around body parts, most commonly the limbs and digits. The authors report a novel variation on this presentation in 3 patients from 2 centers with complex craniofacial clefting and amniotic band sequence. They presented with hypertelorism, different forms of complex craniofacial clefting, and bands connecting ipsilateral hands to facial clefts, with digital-facial translocation in 2 cases. These findings support a model in which complex craniofacial clefts result in areas of exposed, sticky, and temporally and spatially coincident mesenchyme within the embryo that are susceptible to adherence of ipsilateral fetal hands. This strongly supports the intrinsic and adhesion theories of the etiology of amniotic band syndrome.

PURPOSE/OBJECTIVE:Surgical residencies have increasingly incorporated both digital and mannequin simulation into their training programs. The aim of our review was to identify all digital and mannequin maxillofacial simulators available for education and training, highlight their benefit, and critically assess the evidence in support of these educational resources. MATERIALS AND METHODS/METHODS:We performed a comprehensive literature review of all peer-reviewed publications of digital and mannequin simulators that met the inclusion criteria, defined as any simulator used in education or training. All simulators used in surgical planning were excluded. Before the query, it was hypothesized that most studies would be descriptive in nature and supported by low levels of evidence. Literature search strategies included the use of multiple combinations of key search terms, review of titles and abstracts, and precise identification of the use of the simulator described. All statistics were descriptive. RESULTS:The primary search yielded 259 results, from which 22 total simulators published on from 2001 to 2016 were identified using the inclusion and exclusion criteria: 10 virtual reality haptic-based simulators, 6 physical model simulators, and 6 Web-based simulators used for a variety of procedures such as dental skills, instrument handling, orthognathic surgery (Le Fort I osteotomy, vertical ramus osteotomy, bilateral sagittal split ramus osteotomy), genioplasty, bone grafting, sinus surgery, cleft lip repair, orbital floor repair, and oral biopsy. Only 9 formalized studies were completed; these were classified as low-level evidence-based cohort studies (Levels IV and V). All other simulator reports were descriptive in nature. There were no studies with high levels of evidence completed (Level I to III). CONCLUSIONS:The results of this review suggest that, although seemingly beneficial to the trainee in maxillofacial surgery, simulation in education in this field is an underused commodity because of the significant lack of scientific and validated study designs reported on in the literature thus far. The maxillofacial and simulation communities would benefit from studies on utility and efficacy with higher levels of evidence.

BACKGROUND:Single-stage cleft lip and palate repair is a debated surgical approach. While some studies have described favorable outcomes, concerns include the effect on craniomaxillofacial growth and increased risk of complications. To this date, there has not been a comprehensive appraisal of available data following combined cleft lip and palate repair. METHODS:An extensive literature review was performed to identify all relevant articles. The level of evidence of these articles was graded according to the Oxford Centre for Evidence-Based Medicine Levels of Evidence Scale. RESULTS:A total of 22 relevant articles were identified, all of which were retrospective in nature. Patient age at the time of surgery ranged from 1 month to 10 years, the longest duration of follow-up was 18 years, and the largest study included 106 patients. Review of the literature shows that overall surgical outcomes following combined cleft lip and palate repair are encouraging. An increased rate of postoperative fistulas with associated speech abnormalities in some studies is noteworthy. Importantly, there is no evidence to suggest an impact on craniomaxillofacial growth, and psychosocial outcomes and parental satisfaction seem to be improved with single-stage surgery as compared with the staged approach. CONCLUSIONS:Our review shows overall favorable outcomes associated with combined cleft lip and palate repair. The limited follow-up time or nature of evaluated outcomes in some studies may underrepresent the true rate of adverse events, and highlights the need for additional long-term studies with standardized follow-up. To our knowledge, our review is the first to evaluate existing data regarding outcomes following combined cleft lip and palate repair.

INTRODUCTION/BACKGROUND:Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. MATERIALS AND METHODS/METHODS:A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. RESULTS:Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients (P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. CONCLUSIONS:Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.

Craniomaxillofacial congenital anomalies comprise approximately one third of all congenital birth defects and include deformities such as alveolar clefts, craniosynostosis, and microtia. Current surgical treatments commonly require the use of autogenous graft material which are difficult to shape, limited in supply, associated with donor site morbidity and cannot grow with a maturing skeleton. Our group has demonstrated that 3D printed bio-ceramic scaffolds can generate vascularized bone within large, critical-sized defects (defects too large to heal spontaneously) of the craniomaxillofacial skeleton. Furthermore, these scaffolds are also able to function as a delivery vehicle for a new osteogenic agent with a well-established safety profile. The same 3D printers and imaging software platforms have been leveraged by our team to create sterilizable patient-specific intraoperative models for craniofacial reconstruction. For microtia repair, the current standard of care surgical guide is a two-dimensional drawing taken from the contralateral ear. Our laboratory has used 3D printers and open source software platforms to design personalized microtia surgical models. In this review, we report on the advancements in tissue engineering principles, digital imaging software platforms and 3D printing that have culminated in the application of this technology to repair large bone defects in skeletally immature transitional models and provide in-house manufactured, sterilizable patient-specific models for craniofacial reconstruction.