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Severe midface hypoplasia is often managed by Le Fort III distraction. Le Fort II distraction with zygomatic repositioning is a modification of the Le Fort III distraction operation aimed to correct abnormal facial ratios of patients with greater central than lateral midface deficiency. The operation starts with Le Fort III osteotomies and is followed by separation and fixation of bilateral zygomas. The central nasomaxillary Le Fort II segment is then distracted to achieve independent movements of the central and lateral midface. The Le Fort II zygomatic repositioning operation has become our procedure of choice for patients with Apert facial dysmorphology.

Background Tissue expansion in the pediatric population can be complicated by high rates of infection and extrusion. The aim of this study was to examine the impact of postoperative antibiotic prophylaxis on infectious complications. Methods A retrospective study of all pediatric patients who underwent tissue expander insertion at a children's hospital over a 12-year period was performed. Predictor variables included age, sex, race, indication, anatomical location, number of expanders inserted, serial expansion, history of infection or extrusion, and postoperative antibiotics. Outcome variables included infection and extrusion. Bivariate and multivariate analyses were performed to identify factors associated with infection and/or extrusion. Results A total of 180 patients who underwent 317 operations for tissue expander insertion were included in this study. Postoperative infection and/or extrusion occurred after 73 operations (23 percent). Postoperative prophylactic antibiotics were prescribed after 232 operations (75 percent), and only perioperative (≤24 hours) antibiotics were administered in 85 cases (25 percent). There were no significant differences in the rate of infection (12.1 percent versus 8.9 percent; p = 0.46), extrusion (16.8 percent versus 17.7 percent; p = 0.88), or infection and/or extrusion (23.7 percent versus 24.1 percent; p = 0.95) between these two groups. Multivariate analysis revealed that postoperative antibiotics did not have a significant association with infection and/or extrusion (OR, 0.84; 95 percent CI, 0.44 to 1.63; p = 0.61). Conclusions The rates of infection/extrusion were similar between pediatric patients who received only perioperative antibiotics (≤24 hours) and those who were prescribed a course of postoperative antibiotics. Based on these results, a course of postoperative prophylactic antibiotics may be unnecessary after insertion of tissue expanders in pediatric patients. Clinical question/level of evidence Therapeutic, III.

Anatomic studies have identified that patients with Treacher Collins syndrome and some cases of bilateral craniofacial microsomia are characterized by multilevel airway obstruction as a result of hypoplasia and clockwise rotation of the maxillomandibular complex. Patients often remain tracheostomy-dependent despite multiple airway surgeries. Counterclockwise craniofacial distraction osteogenesis aims to correct the facial skeletal deformity and expand the upper airway volume by rotating the subcranial complex en bloc around the nasofrontal junction. Early results have demonstrated significant increases in the nasopharyngeal and oropharyngeal airway volumes with successful decannulation in a majority of patients who have undergone this operation.

Background Tissue expansion is used for soft-tissue reconstruction in pediatric patients. The expansion process can be complicated by infection and extrusion, leading to premature expander removal. The aim of this study was to identify risk factors associated with premature expander removal caused by infection or extrusion in pediatric patients. Methods A retrospective study of pediatric patients who underwent tissue expansion performed by the senior author (R.J.R.) over a 12-year period was performed. Predictor variables included age, sex, race, indication, anatomical location, number of expanders, serial expansion, and expander size. Bivariate and multivariate analyses were performed to identify risk factors for premature expander removal. Results A total of 139 patients with 472 expanders were included in this study. Complications occurred with 78 expanders (16.5 percent). Premature expander removal caused by infection or exposure occurred with 51 expanders (10.8 percent). In terms of location, the highest rates of premature removal occurred in the lower extremity (20.0 percent) and scalp (16.3 percent). Multivariate analysis identified younger age (0 to 6 years compared with 13 to 17 years; OR, 3.98; 95 percent CI, 1.13 to 14.08; p = 0.03), greater number of expanders (OR, 1.45; 95 percent CI, 1.03 to 2.03; p = 0.03), and lower extremity location (OR, 4.27; 95 percent CI, 1.45 to 12.53; p = 0.008) were associated with an increased odds of premature expander removal. Conclusions Expander removal occurred in approximately 10 percent of tissue expanders. Odds of premature removal is increased with younger age, greater number of expanders, and lower extremity location. Clinical question/level of evidence Risk, III.

Fibrous dysplasia of bone (FD) is a mosaic disease caused by mutations in GNAS. Constitutive activation of the α-subunit of the Gs stimulatory protein (Gαs) leads to dysregulated proliferation of bone marrow stromal cells (BMSCs), generating expansile lesions of fibrotic tissue and abnormal bone. Local bone remodeling regulation by BMSCs is also altered, and FD tissue is characterized by abundant osteoclast-like cells that may be essential for lesion expansion. Animal models show local expression of RANKL in bone lesions, and treatment with the RANKL neutralizing antibody denosumab decreased lesion expansion rate in a patient with aggressive FD. However, the role of RANKL/osteoprotegerin (OPG) in FD pathophysiology is not yet understood. We measured serum levels of RANKL, OPG, and inactive RANKL-OPG complexes in FD patients of known disease burden and in healthy volunteers (HVs). RANK, RANKL, and Ki67 immunohistochemistry were assessed in FD tissue. Cultured FD and HV BMSCs were stimulated with prostaglandin E2 (PGE2 ) and 1,25 vitamin D3 to increase RANKL expression, and media levels of RANKL and OPG were measured. Osteoclastogenic induction by FD or HV BMSCs was assessed in co-cultures with HV peripheral monocytes. FD patients showed a 16-fold increase in serum RANKL compared to HVs. OPG was moderately increased (24%), although RANKL/OPG ratio was 12-fold higher in FD patients than in HVs. These measurements were positively correlated with the skeletal burden score (SBS), a validated marker of overall FD burden. No differences in serum inactive RANKL-OPG complexes were observed. In FD tissue, RANKL+ and Ki67+ fibroblastic cells were observed near RANK+ osteoclasts. High levels of RANKL were released by FD BMSCs cultures, but were undetectable in HV cultures. FD BMSC released less OPG than HV BMSCs. FD, but not HV BMSCs, induced osteoclastogenesis in monocyte co-cultures, which was prevented by denosumab addition. These data are consistent with the role of RANKL as a driver in FD-induced osteoclastogenesis. © 2018 American Society for Bone and Mineral Research.

Purpose Nonunion is an uncommon complication after mandibular fractures. The purpose of this investigation was to compare outcomes of patients with mandibular fracture nonunion who were treated with a 1- versus 2-stage approach and propose a pragmatic treatment algorithm for surgical management based on preoperative characteristics. Materials and Methods The authors conducted a retrospective study consisting of patients who presented to 2 level 1 trauma centers for the management of mandibular fracture nonunion over a 10-year period. The primary predictor variable was 1- versus 2-stage treatment. Outcomes were examined to propose a treatment algorithm. Results Eighteen patients were included in the study. The sample's mean age was 44.0 ± 19.3 years and most were men (88.9%). Mandibular angle and body accounted for 77.8% of cases. A single-stage approach was used in 13 patients (72.2%). Bone grafts or vascularized bone flaps were required in 13 patients (72.2%). Patients who required 2-stage treatments had intraoral soft tissue defects. Mean length of follow-up was 13.3 ± 20.4 months. All patients achieved bony union, with complications occurring in 5 patients (27.8%). The authors' 10-year experience was used to formulate a treatment algorithm based on bony defect size and soft tissue status, which can be used to inform optimal surgical management. Conclusions Nonunion of mandibular fractures is an infrequent and complex condition requiring careful and deliberate surgical management. A single-stage approach is appropriate in most cases and does not negatively affect outcomes. Bony defect size and soft tissue status are essential parameters for determining the approach and timing of reconstruction.

Background Currently, there is a paucity of information on the presentation and proper management of pediatric nasoorbitoethmoid fractures. The purpose of this study was to examine the incidence, cause, associated injuries, and management of these fractures. Furthermore, the authors sought to assess outcomes after transnasal wiring or suture canthopexy for type III nasoorbitoethmoid fractures. Methods A retrospective cohort review was performed of all patients with nasoorbitoethmoid fractures who presented to a Level I trauma center from 1990 to 2010. Charts and computed tomographic imaging were reviewed, and nasoorbitoethmoid fractures were labeled based on the Markowitz-Manson classification system. Patient fracture patterns, demographics, characteristics, and outcomes were recorded. Univariate and multivariate methods were used to compare groups. Results A total of 63 pediatric patients were identified in the study period. The sample's mean age was 8.78 ± 4.08 years, and 28.6 percent were girls. The sample included 18 type I injuries, 28 type II injuries, and 17 type III injuries. No significant demographic differences were found between patients with type I, II, and III fractures (p > 0.05). Operative intervention was pursued in 16.7, 46.4, and 82.4 percent of type I, II, and III nasoorbitoethmoid fractures, respectively. In patients with type III nasoorbitoethmoid fractures, no patients with transnasal wiring developed telecanthus. Conclusions Pediatric nasoorbitoethmoid fractures are uncommon injuries. Type I fracture can often be treated with close observation. However, type II and III injury patterns should be evaluated for operative intervention. Transnasal wiring is an effective method to prevent traumatic telecanthus deformity in type III fracture patterns.

Denosumab is a humanized monoclonal antibody to receptor activator of nuclear factor-κB ligand used primarily for postmenopausal osteoporosis and skeletal-related events from metastatic cancer. Its safety in children has not been established.The objective of the study was to investigate the effects of denosumab treatment on skeletal growth and histology.This was an observational case report with radiological and histopathological analyses.The study was conducted at a clinical research center.A 9-year-old boy with fibrous dysplasia treated with a 7-month course of denosumab participated in the study.Histological analyses were performed and compared on growth plates from limbs that had been amputated before and 17 months after denosumab treatment.Skeletal radiographs and bone histopathology from before and after treatment were measured.After initiating denosumab, sclerotic metaphyseal bands appeared on radiographs. Posttreatment radiographs revealed migration of the bands away from the growth plates, consistent with continued linear growth. Histologically, the bands were composed of horizontally arranged trabeculae containing calcified cartilage. This cartilage appeared to derive from unresorbed primary spongiosa as a result of osteoclast inhibition by denosumab, similar to what has been observed with bisphosphonates. By 17 months after treatment, active bone resorption and formation had returned, as evidenced by the presence of active osteoclasts in resorption pits and osteoid surfaces.Further studies are needed to determine the safety of denosumab on the growing skeleton. However, in this child there was continued epiphyseal activity both during and after treatment and reversal of bone turnover suppression after treatment discontinuation, suggesting that denosumab did not have significant adverse effects on growth.

Background: As of July of 2013, 27 facial vascularized composite allotransplantations have been performed. The authors developed a classification system and treatment algorithm that is practical and surgically applicable. Methods: The majority of the transplants have been described in the surgical literature and the media, and a review of the data was performed. A classification system and a treatment algorithm were designed. Skeletal defects were defined by craniofacial osteotomies and soft-tissue defects by aesthetic facial subunits. The soft-tissue defect was subdivided into the following subunits: oral-nasal (type 1), oronasal-orbital (type 2), and full facial (type 3). The bony defects were subdivided into mandibular involvement (M), Le Fort 1 (A), Le Fort 3 (B), and monobloc (C). Results: The mechanisms of injury included trauma (n = 13), burns (n = 8), congenital deformity (n = 3), oncologic resection (n = 1), and unreported (n = 2). According to the proposed classification system: one was type 1; one was type 1-M; one was type 1-MB; two were type 2; two were type 2-B; two were type 2-MB; six were type 3; one was type 3-B; and three were type 3-MB; eight could not be classified due to a lack of data. The treatment algorithm designed a vascularized composite allotransplantation that addressed the bony and soft-tissue components. Conclusions: Patient selection for these complicated procedures, currently dependent on lifelong immunosuppression, is crucial to their success. The authors describe a classification system and treatment algorithm for facial defects that may be ideally suited for facial transplantation. The proposed classification and algorithm may help centers define indications and ideally improve patient outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Improvements in surgical techniques have allowed us to achieve primary closure in a high percentage of large abdominal hernia repairs. However, postoperative wound complications remain common. The benefits of negative-pressure wound therapy (NPWT) in the management of open abdominal wounds are well described in the literature. Our study investigates the effects of incisional NPWT after primary closure of the abdominal wall.A retrospective chart review was performed for the period between September 2008 and May 2011 to analyze the outcomes of patients treated postoperatively with incisional NPWT versus conventional dry gauze dressings. Patient information collected included history of abdominal surgeries, smoking status, and body mass index. Postoperative complications were analyzed using χ exact test and logistic regression analysis.Fifty-six patients were included in this study; of them, 23 were treated with incisional NPWT, whereas 33 received conventional dressings. The rates of overall wound complications in groups I and II were 22% and 63.6%, respectively (P = 0.020). The rates of skin dehiscence were 9% and 39%, respectively (P = 0.014). Both outcomes achieved statistical significance. Rates of infection, skin and fat necrosis, seroma, and hernia recurrence were 4%, 9%, 0%, and 4% for group I and 6%, 18%, 12%, 9% for group II, respectively.This study suggests that incisional NPWT following abdominal wall reconstruction significantly improves rates of wound complication and skin dehiscence when compared with conventional dressings. Prospective, randomized, controlled studies are needed to further characterize the potential benefits of this therapy on wound healing after abdominal wall reconstruction.

Background. Components separation has been proposed as a means to close large ventral hernia without undue tension. We report a modification on open components separation that allows for the incorporation of onlaid noncrosslinked porcine acellular dermal matrix (Strattice, LifeCell Corp, Branchburg, NJ) as a load-sharing structure. Methods. This was a retrospective case series including all cases using Strattice from July 2008 through December 2009. Data evaluated included patient demographics, comorbidities associated with risk of recurrence, hernia grade, and postoperative complications. The primary outcomes were hernia recurrence and surgical site occurrences. Results. There were 58 patients; 60.8% presented with a recurrent incisional hernia. Average length of follow-up was 384 days. There were 4 hernia recurrences (7.9%). Complications included surgical site infection (20.7%), seroma (15.5%), and hematoma (5%) requiring intervention. Four deaths occurred in the series due to causes unrelated to the hernia repair, only 1 within 30 days of operation. Conclusions. This series demonstrates that components separation reinforced with noncrosslinked porcine acellular dermal matrix onlay is an efficacious, single-stage repair with a low rate of recurrence and surgical site occurrences.