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Purpose Intraoral scanners (IOSs), which create digital “impressions” of dental arches, have become popular for prosthetic and orthodontic applications. Adoption in oral and maxillofacial surgery (OMS) practices has been slower, likely because of high implementation costs and low-volume use. The purpose of this study was to evaluate costs for introduction of an IOS into an OMS practice. The authors hypothesized that digital impressions would be more efficient in time and cost compared with conventional impressions and that implementation costs would be offset within 1 year. Materials and Methods This was a prospective study that included patients who had digital impressions during the first year after introduction of an IOS to the practice. Conventional alginate impressions obtained at the same visit were included for comparison. Variables included time for each step in each impression process, IOS experience of the operator obtaining the impression, and associated costs. Per-arch costs for each technique were calculated using time-driven activity-based costing methodology. Results Sixty-three digital impressions and 31 conventional impressions were included. Mean total times for digital and conventional impressions were 14.1 ± 1.3 and 19.4 ± 4.0 minutes per arch, respectively. On a per-patient basis (2 arches for digital impressions and 4 arches for conventional impressions because of the inability to create duplicate stone models from each alginate impression), total impression times were 24.8 ± 2.7 minutes for digital and 67.2 ± 14.8 minutes for conventional impressions. Total calculated costs for digital and conventional impressions were $21.42 and $29.40 per arch and $37.66 and $102.10 per patient, respectively. In a practice with 2 patients for impressions per working day (500 per year), it would take 1.04 years to offset the purchase of the IOS; with 5 sets of impressions per day (1,250 per year), it would take 5 months. Conclusion Digital impressions are more efficient and cost effective than standard impressions, and implementation costs can be offset within the first year.

Purpose Obstructive sleep apnea (OSA) is a common problem in patients with craniofacial microsomia (CFM); however, the exact pathophysiology in patients with CFM remains unclear. The first aim of this study was to evaluate upper airway volume and morphology in patients with CFM. The second aim was to identify risk factors for the presence of OSA. Materials and Methods A cross-sectional study was set up and 3 study groups were identified: 1) CFM with OSA, 2) CFM without OSA, and 3) control. Computed tomographic (CT) scans of the head and neck were included and used to create 3-dimensional models. The age-matched control group consisted of patients evaluated for traumatic head injury or epilepsy. Volumetric and morphologic parameters were measured. The results of patients with CFM were compared among the 3 study groups. Descriptive statistics were computed using the Pearson χ2 test for categorical variables and nonparametric tests for continuous variables. A multiple variable regression model was used to identify risk factors for OSA. Results In total, 79 patients with CFM were included, of which 25 patients were diagnosed with OSA. A total of 145 CT scans could be analyzed. In addition, a control population of 88 patients was identified. Oropharynx volume, mean cross-sectional area (CSA), minimal CSA, and minimal retropalatal area were found to be markedly smaller in patients with CFM compared with the control population. In contrast, in patients with CFM and OSA, minimal retroglossal area, sphericity, and uniformity markedly differed from those in patients without OSA. Sphericity was identified as the main predicting variable of OSA in patients with CFM. Conclusions The upper airway of patients with CFM is markedly smaller and puts them at risk for developing OSA. Patients with CFM diagnosed with OSA have a markedly smaller CSA behind the base of the tongue and a difference in sphericity.

Regeneration of large bones remains a challenge in surgery. Recent developmental engineering efforts aim to recapitulate endochondral ossification (EO), a critical step in bone formation. However, this process entails the condensation of mesenchymal stem cells (MSCs) into cartilaginous templates, which requires long-term cultures and is challenging to scale up. Here, we develop a biomimetic scaffold that allows rapid and self-sustained EO without initial hypertrophic chondrogenesis. The design comprises a porous chondroitin sulfate (CS) cryogel decorated with whitlockite calcium phosphate nanoparticles, and a soft hydrogel occupying the porous space. This composite scaffold enables human endothelial colony-forming cells (ECFCs) and MSCs to rapidly assemble into osteovascular niches in immunodeficient mice. These niches contain ECFC-lined blood vessels and perivascular MSCs that differentiate into RUNX2(+)OSX(+) pre-osteoblasts after one week in vivo. Subsequently, multiple ossification centers are formed, leading to de novo bone tissue formation by eight weeks, including mature human OCN(+)OPN(+) osteoblasts, collagen-rich mineralized extracellular matrix, hydroxyapatite, osteoclast activity, and gradual mechanical competence. The early establishment of blood vessels are essential, and grafts that do not contain ECFCs fail to produce osteovascular niches and ossification centers. Our findings suggest a novel bioengineering approach to recapitulate EO in the context of human bone regeneration.

Purpose Chronic nonbacterial osteomyelitis (CNO) is a focal sterile inflammatory osteitis in children that most commonly develops in the long bones, but can occur in any bone. The disease course is variable, ranging from acute and self-resolving isolated lesions to chronic recurrent multifocal osteomyelitis (CRMO), which is frequently associated with extraosseous inflammatory disease. The purpose of this study was to present our clinical experience with CNO of the mandible in children. The specific aims were to 1) document the clinical characteristics, radiographic findings, and histologic features of CNO and 2) determine the percentage of our sample with multifocal disease (CRMO). Materials and Methods This is a retrospective case series of patients with mandibular CNO. To be included, patients had to have a mandibular lesion radiographically consistent with osteomyelitis without infection, onset before aged 18 years, and complete records. Medical records were reviewed for history, clinical features, imaging, and pathology. Descriptive data were summarized. Results The sample included 22 patients (13 female and 9 male patients) with disease onset at a mean age of 9.05 ± 2.4 years. On presentation, all patients reported mandibular pain and swelling, and 45% had trismus. All had clinical and/or radiographic findings of multifocal intraosseous disease and/or extraosseous inflammatory lesions. Of the patients, 12 (54%) had a documented family history of autoimmune or autoinflammatory disease and 15 (68%) had elevated erythrocyte sedimentation rates during a flare. Computed tomography scans typically showed expansion of the affected mandible with sclerosis of the medullary space, small foci of poorly defined lytic destruction with a lamellated periosteal reaction, and swollen muscles of mastication. Four distinct histologic features were noted including parallel and interconnected osteoid seams, atypical osteoid, areas of woven bone and hypocellular fibroblastic stroma resembling fibrous dysplasia, and patchy nodular fibrosis. Conclusion Pediatric CNO of the mandible has characteristic radiographic and pathologic features and is usually found as one of multiple disease foci in CRMO rather than as an isolated lesion.

To determine the effects on time, cost, and complication rates of integrating physician assistants (PAs) into the procedural components of an outpatient oral and maxillofacial surgery practice.This is a prospective cohort study of patients from the Department of Plastic and Oral Surgery at Boston Children's Hospital who underwent removal of 4 impacted third molars with intravenous sedation in our outpatient facility. Patients were separated into the "no PA group" and PA group. Process maps were created to capture all activities from room preparation to patient discharge, and all activities were timed for each case. A time-driven activity-based costing method was used to calculate the average times and costs from the provider's perspective for each group. Complication rates were calculated during the periods for both groups. Descriptive statistics were calculated, and significance was set at P.05.The total process time did not differ significantly between groups, but the average total procedure cost decreased by $75.08 after the introduction of PAs (P.001). The time that the oral and maxillofacial surgeon was directly involved in the procedure decreased by an average of 19.2 minutes after the introduction of PAs (P.001). No significant differences in postoperative complications were found.The addition of PAs into the procedural components of an outpatient oral and maxillofacial surgery practice resulted in decreased costs whereas complication rates remained constant. The increased availability of the oral and maxillofacial surgeon after the incorporation of PAs allows for more patients to be seen during a clinic session, which has the potential to further increase efficiency and revenue.

The major limitation of the rigid external devices currently used for midfacial distraction after subcranial Le Fort III osteotomies is the ductile wire that connects the midface to the device, which makes it difficult to control the vector and force during distraction. The authors describe a novel external appliance that addresses this and other problems of contemporary devices, and application of a custom cranial template that facilitates precise placement of the device to achieve the planned vector of distraction.

The goal of this study was to describe the clinical characteristics and treatment outcomes of patients with hemifacial microsomia (HFM) and cleft lip/palate (CL/P), and to compare them to a historic cohort of patients with non-syndromic CL/P treated at the same centre. A retrospective review of patients with HFM and CL/P was performed; the main outcome measures assessed were cleft type/side, surgical outcome, midfacial retrusion, and speech. Twenty-six patients (13 male, 13 female; mean age 22.7±14.9, range 1-52 years) with cleft lip with/without cleft palate (CL±P) were identified: three with cleft lip (12%), two with cleft lip and alveolus and an intact secondary palate (8%), and 21 with cleft lip and palate (CLP) (81%; 15 unilateral and six bilateral). Four patients (19%) had a palatal fistula after palatoplasty. Twelve of 22 patients aged >5 years (55%) had midfacial retrusion and two (9%) required a pharyngeal flap for velopharyngeal insufficiency (VPI). Fisher's exact test demonstrated a higher frequency of complete labial clefting (P=0.004), CLP (P=0.009), midfacial retrusion (P=0.0009), and postoperative palatal fistula (P=0.03) in HFM compared to non-syndromic CL±P. There was no difference in VPI prevalence. This study revealed that patients with HFM and CL±P have more severe forms of orofacial clefting than patients with non-syndromic CL±P. Patients with HFM and CL±P have more severe midfacial retrusion and a higher palatal fistula rate compared to patients with non-syndromic CL±P.

Purpose Patients with unilateral complete cleft lip and palate (UCLP) have a characteristic bilateral septal deformity, and septal deviation can be associated with turbinate hyperplasia, leading to paradoxical nasal obstruction. The purpose of the present study was to measure and compare the bony and mucosal dimensions of the inferior turbinate on the cleft and non-cleft sides in patients with UCLP. Patients and Methods We implemented a retrospective cohort study of patients with UCLP who had undergone computed tomography (CT) scan between 2002 to 2013. Subjects who had undergone nasal revision, septoplasty, turbinectomy, or Le Fort I osteotomy before the imaging date were excluded. The primary predictor variable was the subject side (cleft vs noncleft side), and the primary outcome variable was the turbinate cross-sectional area. The secondary predictor variables included the site of measurement along the sagittal axis of the turbinate (anterior, middle, posterior) and tissue type (turbinate whole, bone, mucosa). The Wilcoxon signed rank test for paired samples compared the turbinate dimensions on the cleft and noncleft sides, with statistical significance set at P ≤ .05. Results The sample included 53 patients (32 females and 21 males). The inferior turbinates were measured bilaterally on CT images obtained at a mean age of 12.2 ± 0.8 years. The inferior turbinate on the noncleft side was significantly larger in both bone and mucosa ( P = .003). This relationship did not change when controlling for age and gender. Conclusions The results of the present study have confirmed bony and mucosal enlargement of the inferior turbinate on the noncleft side in patients with UCLP. This might contribute to bilateral nasal obstruction and should be considered during treatment planning for nasopharyngeal and orthognathic surgery.

Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

Purpose Opioid abuse is a public health concern. Oral opioids are prescribed after removal of third molars, but the amount needed for adequate postoperative analgesia is unknown. The purpose of this study was to quantify opioid need after third molar extractions. Materials and Methods This is a prospective cohort study of consecutive patients who had asymptomatic third molars extracted with intravenous sedation at the Boston Children's Hospital (Boston, MA) from June through October 2018 by 3 attending surgeons. To be included, patients had to have had 4 third molars removed. Patients were excluded if they had a concomitant procedure, preoperative infection, postoperative inflammatory complication, chronic pain condition, or did not complete the study. Postoperative prescriptions and instructions included 1) oxycodone 5-mg tablets with 1 tablet taken every 6 hours as needed (dispensed, 6); 2) ibuprofen 600-mg tablets with 1 tablet taken every 6 hours as needed (dispensed, 20); and 3) acetaminophen 325-mg tablets with 2 tablets taken every 6 hours as needed (dispensed, 40). Participants reported medication use by electronic questionnaire each day for 7 postoperative days (PODs). Descriptive statistics were calculated. Results Eighty-one patients (56% female; mean age, 19.4 ± 7.7 yr) were included. The average number of oxycodone tablets used was 0.04 ± 0.24, and the highest daily use of oxycodone was on POD 2 (1.0 ± 0.0 tablet). Oxycodone was taken by 3 patients (4%) on POD 1, 4 (5%) on POD 2, 2 (3%) on PODs 3 and 4, and 0 on PODs 5 to 7. Seventy-five patients (93%) used no postoperative oxycodone; 466 prescribed oxycodone tablets remained unfilled or unused. Ibuprofen 600 mg was used for 4.6 ± 2.2 PODs and acetaminophen 650 mg was used for 3.4 ± 1.9 PODs. Conclusions Oral opioid use after third molar extractions is minimal. Caution is necessary to avoid over-prescribing.

Craniofacial microsomia (CFM) is characterized by unilateral or bilateral underdevelopment of the facial structures arising from the first and second pharyngeal arches, but extracraniofacial anomalies may also be present. This retrospective study provides an overview of the prevalence, types, and characteristics of extracraniofacial anomalies in patients with CFM. All patients diagnosed with CFM seen at four craniofacial centres were included. The patient charts were reviewed and data on patient characteristics and extracraniofacial anomalies were extracted. Of the 991 patients included, 462 (47%) had extracraniofacial anomalies. The prevalence of extracraniofacial anomalies in the various tracts was as follows: vertebral 28%, central nervous system 11%, circulatory system 21%, respiratory tract 3%, gastrointestinal tract 9%, and urogenital tract 11%. Compared to patients without extracraniofacial anomalies, those with an extracraniofacial anomaly were at higher risk of having additional extracraniofacial anomalies in other tracts. The prevalence of extracraniofacial anomalies was greater in patients with bilateral CFM, a more severe mandibular deformity, or facial nerve or soft tissue deformity. Patients with CFM should be screened for extracraniofacial anomalies by physical examination with specific attention to the circulatory, renal, and neurological tracts. Diagnostically, electrocardiography, echocardiography, spine radiography, and renal ultrasound should be performed for patients at risk of extracraniofacial anomalies.

Tongue-lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) are the most common operations for obstructive apnea (OA) in infants with Robin sequence (RS). The purpose of this study was to compare early outcomes of TLA and MDO on resolution of OA.This is a retrospective cohort study of infants with RS treated with TLA or MDO from 2005 through 2018. To be included, patients had to have polysomnograms pre- and postoperatively unless intubated or tracheostomy dependent. Patients were excluded if the operation occurred after 1 year of age. The primary predictor variable was type of operation (TLA or MDO). The primary outcome variable was "successful resolution of OA," defined as 1) postoperative apnea-hypopnea index (AHI) score lower than 5, 2) postoperative OA severity score of 0 to 1 (no to mild OA), and 3) no need for additional airway intervention during the first year of life. Descriptive, bivariate, and regression analyses were performed. Statistical significance was set at a P value less than .05.Forty-three patients were included (TLA, n = 19 [44%]; MDO, n = 24 [56%]). Eighteen patients (41%) were syndromic. Patients in the TLA group were significantly younger at operation (28.2 ± 23.1 days) compared with those in the MDO group (87.1 ± 81.7 days; P = .002). The MDO group had more severe preoperative OA (AHI score, 20.5 ± 14.9; OA severity score, 4.7 ± 0.8) than the TLA group (AHI score, 17.6 ± 31.0; OA severity score, 3.6 ± 1.4; P .041). Postoperative AHI score and OA severity score for the TLA group were 11.7 ± 19.5 (33.5% decrease; P = .496) and 2.3 ± 1.8 (improvement by 1.3 ± 2.4 levels; P = .051), respectively. Postoperative AHI score and OA severity score for the MDO group were 1.1 ± 1.8 (94.6% decrease; P .001) and 0.2 ± 0.4 (improvement by 4.6 ± 0.8 levels; P .0001), respectively. Successful resolution of OA occurred in 9 patients (47%) in the TLA group and 22 patients (92%) in the MDO group. Postoperative complications were similar.MDO is more effective than TLA in relieving OA in infants with RS.

Three-dimensional surgical planning is used widely in orthognathic surgery. Although numerous computer programs exist, the accuracy of soft tissue prediction remains uncertain. The purpose of this study was to compare the prediction accuracy of Dolphin, ProPlan CMF, and a probabilistic finite element method (PFEM). Seven patients (mean age 18years; five female) who had undergone Le Fort I osteotomy with preoperative and 1-year postoperative cone beam computed tomography (CBCT) were included. The three programs were used for soft tissue prediction using planned and postoperative maxillary position, and these were compared to postoperative CBCT. Accurate predictions were obtained with each program, indicated by root mean square distances: RMSDolphin=1.8±0.8mm, RMSProPlan=1.2±0.4mm, and RMSPFEM=1.3±0.4mm. Dolphin utilizes a landmark-based algorithm allowing for patient-specific bone-to-soft tissue ratios, which works well for cephalometric radiographs but has limited three-dimensional accuracy, whilst ProPlan and PFEM provide better three-dimensional predictions with continuous displacements. Patient or population-specific material properties can be defined in PFEM, while no soft tissue parameters are adjustable in ProPlan. Important clinical considerations are the topological differences between predictions due to the three algorithms, the non-negligible influence of the mismatch between planned and postoperative maxillary position, and the learning curve associated with sophisticated programs like PFEM.

Purpose Enlarged mental tubercles are rare benign exostoses that occur in children. The purpose of this study was to report a case series of patients with hypertrophic mental tubercles and propose a theory for their etiopathogenesis. Patients and Methods In this retrospective case series of patients with hypertrophic mental tubercles, we reviewed patients' medical records for age, gender, clinical presentation, findings on physical examination and radiographic images, and management details. Descriptive data were summarized. Results The sample included 14 patients (71% of whom were male patients) who presented at a mean age of 12.9 ± 4.8 years (range, 6 to 21 years). Most patients had bilaterally enlarged mental tubercles (n = 8); unilateral cases were primarily on the left side (5 of 6 patients). On clinical examination, a bony prominence in the parasymphyseal region was noted on palpation and occasionally by visual inspection. No tenderness to palpation or overlying skin changes were noted. Typical radiographic features included asymmetry along the inferior border of the mandible in the parasymphyseal region on panoramic radiographs and prominence and/or enlargement of the mental tubercles on computed tomography scans. No evidence of cortical expansion, perforation, or periosteal reaction was found. Three patients requested contouring of the hypertrophied tubercles. Conclusions Hypertrophic mental tubercles are rare bony exostoses that occur in children in a region where a confluence of facial muscles attach to the mandible. The etiology likely involves bone deposition occurring in an area of muscle pull.

Objective: Glossoptosis causes airway obstruction in patients with Robin sequence (RS), but little is known about the in-utero tongue. The purpose of this study was to assess shape and position of the fetal tongue on prenatal magnetic resonance imaging (MRI) to determine if this is predictive of postnatal RS. Design: Retrospective case-control study including fetuses with prenatal MRIs performed from 2002 to 2017. Inclusion criteria were (1) prenatal MRI of adequate quality, (2) live born and evaluated postnatally for craniofacial findings. Subjects were divided into groups based on postnatal findings: (1) RS, (2) micrognathia without RS, and (3) a gestational-age-matched control group with normal craniofacial morphology. Outcome variables were based on the prenatal MRI and included fetal tongue height, length, and width, tongue shape index (TSI, ratio of height to length), and observation of tongue touching the posterior pharyngeal wall. Results: A total of 116 subjects with mean gestational age at MRI of 25.6 ± 5.1 weeks were included: RS, n = 27 (23%); micrognathia, n = 35 (30%); control, n = 54 (47%). Tongue length was significantly shorter ( P = .009) and TSI was significantly larger in the RS group ( P < .0001). The tongue touched the posterior pharyngeal wall in 5 (19%) of the RS group and in no subjects in the other groups ( P < .0001). Conclusion: In utero tongue shape and position were significantly different in fetuses with postnatal RS compared to those with isolated micrognathia and controls. Prenatal MRI tongue characteristics may be predictors for postnatal RS.

Purpose Craniofacial microsomia (CFM) is characterized by malformations of structures derived from the first and second pharyngeal arches. The orbit is variably affected. The purpose of this study was to determine whether there is a difference in orbital volume between affected and unaffected sides in patients with unilateral CFM. The specific aims were to 1) measure orbital volume, 2) compare affected and unaffected sides, 3) evaluate the correlation between clinical evaluation of orbital size and volumetric measurement, and 4) determine whether there is a correlation between orbital volume and severity of mandibular deformity. Materials and Methods This study is a retrospective case series of patients with unilateral CFM from Boston Children's Hospital (Boston, MA) who had a computed tomographic (CT) scan. Manual segmentation of the orbit using Mimics software (Materialise, Leuven, Belgium) was performed on CT images of the 2 orbits. The predictor variable was laterality (affected vs unaffected side) and the primary outcome variable was orbital volume. Wilcoxon signed rank test was used to compare these measurements and determine whether the affected side differed from the unaffected side. The correlation between orbital volume and Pruzansky-Kaban type of mandibular deformity, as documented in the medical record, was determined using the Spearman rank correlation coefficient. Results Thirty-nine patients were included. Orbital volume was 10% smaller on the affected side (P = .001) in 80% of patients. There was no correlation between orbital size and severity of mandibular involvement. Conclusion The results of this study showed a marked difference in orbital volume between affected and unaffected sides in patients with unilateral CFM. These differences were small and might not be clinically relevant. Orbital volume did not correlate with severity of mandibular deformity.

Purpose The etiology of the palatal cleft in Robin sequence (RS) is unknown. The purpose of this study was to assess the position of the fetal tongue at prenatal magnetic resonance imaging (MRI) and to suggest a potential relation between tongue position and development of the cleft palate seen in most patients with RS. Materials and Methods This is a retrospective case-and-control study including fetuses with prenatal MRIs performed in the authors' center from 2002 to 2017. Inclusion criteria were 1) prenatal MRI of adequate quality, 2) liveborn infant, and 3) postnatal diagnosis of RS (Robin group) or cleft lip and palate (CLP group). Patients with postnatal RS without a palatal cleft were excluded. A control group with normal facial morphology was matched by gestational age. The outcome variable was tongue position at fetal MRI, described as within the cleft, along the floor of the mouth (normal), other, or indeterminate. Results One hundred twenty-two patients with mean gestational age at MRI of 25.8 ± 4.9 weeks were included (Robin, n = 21 [17%]; CLP, n = 47 [39%]; control, n = 54 [44%]). The tongue was visualized within the palatal cleft in 76.2% of the Robin group and 4.3% of the CLP group. The tongue was found along the floor of the mouth (normal) in the remainder of the Robin and CLP groups and in 100% of the control group. Conclusion These findings suggest a relation between in utero tongue position and the development of cleft palate in RS.

Objective To summarize the clinical characteristics and surgical and speech outcomes for patients with Van der Woude/popliteal pterygium syndromes (VWS/PPS) and to compare them with a historic cohort of patients with nonsyndromic cleft lip/cleft palate (CL/P) Design Retrospective chart review. Setting Tertiary care center. Patients All patients with VWS/PPS seen at Boston Children's Hospital from 1979 to 2012: 28 patients with VWS (n = 21)/PPS (n = 7) whose mean age was 17.3 ± 10.4 years, including 18 females (64%) and 10 males (36%); 18 patients (64%) had a family history of VWS/PPS. Main Outcome Measures Cleft type, operative procedures, speech, and midfacial growth. Data were compared with historic cohorts of patients with nonsyndromic CL/P treated at one tertiary care center. Results There were 24 patients (86%) with CP±L, Veau types I (n = 4,17%), II (n = 4, 17%), III (n = 5, 21%), and IV (n = 11, 46%). Nine patients (38%) had palatal fistula after palatoplasty. Fourteen of 23 (61%) patients with CL/P age 5 years or older had midfacial retrusion, and 10 (43%) required a pharyngeal flap for velopharyngeal insufficiency. Fisher's exact test demonstrated higher frequencies of Veau type IV CP±L ( P = .0016), bilateral CL±P ( P = .0001), and complete CL±P ( P < .0001) in VWS/PPS compared with nonsyndromic patients. Incidences of midfacial retrusion ( P = .0001), palatal fistula ( P < .0001), and need for pharyngeal flap ( P = .0014) were significantly greater in patients with VWS/PPS. Conclusions Patients with VWS/PPS have more severe forms of labiopalatal clefting and higher incidences of midfacial retrusion, palatal fistula, and velopharyngeal insufficiency following primary repair as compared with nonsyndromic CL/P.

Objective Characterize mandibular morphology in patients with syndromic craniosynostosis and document changes in mandibular position following midfacial advancement using distraction osteogenesis (DO). Design Retrospective chart review and analysis of cephalometric radiographs. Setting Tertiary care center. Patients Patients with syndromic craniosynostosis who had midfacial advancement with DO at Boston Children's Hospital between 2000 and 2012. Mandibular morphology was characterized in 26 patients (15 boys and 11 girls) with a mean age of 11 years, 9 months. Pre- and postoperative analyses were performed for 17 (10 boys and 7 girls) of the 26 patients with a mean age of 11 years, 9 months. Main Outcome Measures Mandibular morphology and mandibular position. Data were compared to standard data from the Michigan Growth Study. Results Comparison of preoperative mandibular measurements to standard data showed that patients with syndromic craniosynostosis have a shorter mandibular body and length and an obtuse gonial angle. Comparison of pre- and postoperative cephalograms showed that, following midfacial advancement with DO, the maxilla moved forward and the mandible moved backward and downward. Conclusions Patients with syndromic craniosynostosis have a smaller mandible length and obtuse gonial angle. Correction of midfacial hypoplasia with DO results in inferior and posterior mandibular movement. Clinicians can use this information to counsel patients regarding anticipated changes in facial profile and the need for adjunct procedures.

The reported prevalence of obstructive sleep apnea (OSA) in patients with syndromic craniosynostosis (SCS) varies due to inconsistent definitions of OSA, lack of uniform diagnostic testing, and different mixes of syndromic diagnoses. The purpose of this study was to determine the prevalence of OSA in symptomatic patients with SCS, and to determine whether this differs by phenotypic diagnosis. A retrospective cohort study of children with SCS was conducted. The primary outcome was presence of OSA diagnosed by polysomnography. The prevalence of OSA was calculated and stratified by diagnosis to compare differences in prevalence and severity (mild, moderate, or severe). The prevalence of OSA in symptomatic patients was 74.2%. Patients with Apert syndrome had the highest prevalence (80.6%), followed by Pfeiffer, Crouzon with acanthosis nigricans, and Crouzon syndromes (72.7%, 66.7%, and 64.7%, respectively). Severe OSA was most common in patients with Pfeiffer syndrome (45.5%), while patients with Apert and Crouzon syndromes were more likely to have moderate OSA (29.0% and 23.5%, respectively). Given that 56.4% of patients with SCS are symptomatic and that 74.2% of these symptomatic patients have OSA, it is recommended that a screening level I polysomnography be part of the clinical care for all patients with SCS.

Introduction Patients with Craniofacial Microsomia (CFM) mandibles Types I/IIa benefit from combined LeFort 1 osteotomy and Mandibular Distraction Osteogenesis (LeFort + MDO); Type IIb from LeFort + MDO or Bimaxillary osteotomy (BiMax); and Type III from BiMax (with 50% of cases having preceding mandibular procedures, including patient-fitted prosthesis); as seen in Part 1. This leads to the question how maxillary and mandibular hypoplasia are correlated and influence the types of maxillary correction. Material and methods A retrospective chart study was conducted including patients diagnosed with CFM from 2 large craniofacial units. Radiographic and clinical information were obtained. Unilateral affected patients with available (ConeBeam) CT-scan of the maxillary-mandibular complex, without treatment of the upper jaw prior to the CT-scan were included. A maxillary cant grading system was set up and evaluated. Pearson correlation coefficients were used to correlate the maxillary cant and the severity of the mandibular hypoplasia. Results Eighty-one patients were included, of whom 39.5% had a Pruzansky-Kaban type III mandible and 42% a mild maxillary cant. There was a significant positive correlation between severity of the mandibular hypoplasia and the categorized canting (r = 0.370; p Conclusion There is a positive correlation between the severity of mandibular hypoplasia and maxillary cant. The severity of mandibular hypoplasia seems to dictate an intervention for both maxillary and mandibular surgery.

Purpose Micrognathia is the initiating feature of Robin sequence (RS) and leads to airway obstruction. Prenatal identification of micrognathia is currently qualitative and has not correlated with postnatal findings in previous studies. Oropharyngeal airway space has not been evaluated prenatally. The purposes of this study were to 1) quantitate mandibular characteristics and oropharyngeal size at prenatal magnetic resonance imaging (MRI) and 2) identify differences in fetuses with postnatal RS compared with those with micrognathia (without RS) and normal controls. Materials and Methods This is a retrospective case-control study of fetuses with prenatal MRIs performed from 2002 through 2017 who were live born and evaluated postnatally for craniofacial findings. Postnatal findings were used to divide patients into 3 groups: 1) RS (micrognathia, glossoptosis, and airway obstruction), 2) micrognathia without RS (“micrognathia”), and 3) a gestational-age matched control group with normal craniofacial morphology (“control”). Inferior facial angle (IFA), jaw index, and oropharyngeal space (OPS) were calculated and compared among groups. Results Of 116 patients in this study, 27 had RS (23%), 35 had micrognathia (30%), and 54 were control subjects (47%). IFA, jaw index, and OPS were statistically significantly smaller in the RS group compared with the comparison groups ( P Conclusions Prenatal MRI measurements of micrognathia and OPS are considerably different in patients with RS compared with other groups, including those with micrognathia alone. These measurements might serve as reliable prenatal predictors of RS.

The authors present a 16-year-old patient with no known history of cleft palate who developed velopharyngeal insufficiency after a Le Fort I osteotomy performed for the correction of maxillary hypoplasia and a Class III malocclusion. Postoperative evaluation revealed the presence of velopharyngeal insufficiency and subtle findings of an occult submucous cleft palate. She had a pharyngeal flap 6 months later with successful correction of the velopharyngeal insufficiency. This case illustrates the need to screen for submucous cleft palate prior to orthognathic surgery.

Vitamin D is essential for mineral homeostasis and contributes to bone metabolism by stimulating osteoblast differentiation of marrow stromal cells (MSCs). In this study, we used MSCs from pre-pubertal girls and boys to test the hypothesis that 1α,25(OH)

Purpose Three-dimensional (3D) stereophotogrammetry is a reliable, accurate, and noninvasive tool for measuring changes in soft tissue volume over time. The purpose of this prospective study was to quantify changes in postoperative swelling after orthognathic surgery using serial 3D photographs. Patients and Methods Three-dimensional photographs of 49 orthognathic surgery patients (Le Fort I and/or bilateral sagittal split osteotomy) were captured using the 3D Vectra imaging system pre-operatively (T0) and at one week (T1), two weeks (T2), three weeks (T3), four weeks (T4), six weeks (T5), three months (T6), six months (T7) and one year (T8) postoperatively. Canfield Mirror imaging software was used to quantify volume differences between serial 3D images. Descriptive statistics and repeated measures analysis of variance were calculated. Data were stratified by gender, preoperative body mass index (BMI), and procedure performed. Results On average, 50% of the initial swelling resolved after the third postoperative week (T3), and, after 3 months (T6), only 20% of the initial edema remained. Patients with a higher BMI had the greatest amount of swelling and fastest rate of resolution in the initial weeks after the operation. Patients with a lower BMI had less postoperative edema and a slower rate of reduction between all time points. Initial swelling and resolution did not vary significantly by gender or type of malocclusion. Conclusions Facial edema resolves rapidly during the first three post-operative weeks; significant decrease in soft tissue swelling still occurs between 6-12 months post-operatively.

Patients with Beckwith-Wiedemann syndrome (BWS), a genetic disorder characterized by macroglossia, abdominal wall malformations, and gigantism, are at risk for sleep-disordered breathing (SDB). SDB is defined by abnormal breathing that is exacerbated during sleep and is a spectrum ranging from apnea of prematurity to obstructive sleep apnea (OSA). The aim of this study is to determine the prevalence of SDB in children with BWS, and to document clinical characteristics and method(s) of treatment.This is a retrospective cohort study of children with BWS seen at Boston Children's Hospital between 1979 and 2010. Clinical features, presence, type, treatment, and outcomes of SDB were documented.The prevalence of SDB in the 118 patients with BWS was 48% (n = 57). This included 16 subjects who had airway obstruction limited to the neonatal period (26%), 26 subjects with snoring or other obstructive symptoms not diagnosed by polysomnography (46%), and 15 patients with OSA confirmed by polysomnography (28%). Macroglossia was not a significant predictor of SDB, although adenotonsillar hypertrophy was (P0.001). Laryngomalacia, feeding problems, and gastroesophageal reflux also predicted a higher risk for SDB (P0.018). OSA was most commonly treated with partial glossectomy and/or adenotonsillectomy.The prevalence of SDB in this cohort of patients with BWS was 48%. The etiology of SDB in these patients is multifactorial and may not be solely the result of a large tongue. Further information as to the site(s) of airway obstruction in patients with BWS will help guide treatment strategies.

BACKGROUND Facial infiltrating lipomatosis is a nonheritable disorder characterized by hemifacial soft-tissue and skeletal overgrowth, precocious dental development, macrodontia, hemimacroglossia, and mucosal neuromas. The authors tested the hypothesis that this condition is caused by a somatic mutation in the phosphatidylinositide-3 kinase (PI3K) signaling pathway, which has been indicted in other anomalies with overgrowth. METHODS The authors extracted DNA from abnormal tissue in six individuals, generated sequencing libraries, enriched the libraries for 26 genes involved in the PI3K pathway, and designed and applied a sequential filtering strategy to analyze the sequence data for mosaic mutations. RESULTS Unfiltered sequence data contained variant reads affecting ~12 percent of basepairs in the targeted genes. Filtering reduced the fraction of targeted basepairs containing variant reads to ~0.008 percent, allowing the authors to identify causal missense mutations in PIK3CA (p.E453K, p.E542K, p.H1047R, or p.H1047L) in each affected tissue sample. CONCLUSIONS Affected tissue from individuals with facial infiltrating lipomatosis contains PIK3CA mutations that have previously been reported in cancers and in affected tissue from other nonheritable, overgrowth disorders, including congenital lipomatous overgrowth, vascular, epidermal, and skeletal anomalies syndrome, Klippel-Trenaunay syndrome, hemimegalencephaly, fibroadipose overgrowth, and macrodactyly. Because PIK3CA encodes a catalytic subunit of PI3K, and in vitro studies have shown that the overgrowth-associated mutations increase this enzyme's activity, PI3K inhibitors currently in clinical trials for patients with cancer may have a therapeutic role in patients with facial infiltrating lipomatosis. The strategy used to identify somatic mutations in patients with facial infiltrating lipomatosis is applicable to other somatic mosaic disorders that have allelic heterogeneity.

The role of adenotonsillectomy in the treatment of obstructive sleep apnea in patients with craniosynostosis is not well established.A retrospective review was conducted of all children with syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer syndromes) seen at Boston Children's Hospital from January 2001 through April 2011. The primary outcome measure was the apnea/hypopnea index before and after adenotonsillectomy.There were 47 patients (66 percent Apert syndrome, 19 percent Pfeiffer syndrome, and 15 percent Crouzon syndrome) less than 21 years of age (mean, 1.2 ± 3.3 years at first visit) who were followed for a mean of 14.6 ± 8.6 years. Of children with at least one polysomnogram, 83 percent had obstructive sleep apnea (42 percent severe, 19 percent moderate, 22 percent mild). Adenotonsillectomy was performed in 62 percent of patients. Preoperative and postoperative polysomnography was performed in 45 percent of children undergoing adenotonsillectomy. Following adenotonsillectomy, the mean apnea/hypopnea index was not significantly different, and obstructive sleep apnea persisted in 11 of 13 children.Obstructive sleep apnea is commonly present in children with syndromic craniosynostosis and is a complex disease with airway obstruction at several different levels. Adenotonsillectomy often does not improve the severity of obstructive sleep apnea in this population.

Three-dimensional (3D) soft tissue prediction is replacing two-dimensional analysis in planning for orthognathic surgery. The accuracy of different computational models to predict soft tissue changes in 3D, however, is unclear. A retrospective pilot study was implemented to assess the accuracy of Dolphin 3D software in making these predictions. Seven patients who had a single-segment Le Fort I osteotomy and had preoperative (T0) and >6-month postoperative (T1) cone beam computed tomography (CBCT) scans and 3D photographs were included. The actual skeletal change was determined by subtracting the T0 from the T1 CBCT. 3D photographs were overlaid onto the T0 CBCT and virtual skeletal movements equivalent to the achieved repositioning were applied using Dolphin 3D planner. A 3D soft tissue prediction (TP) was generated and differences between the TP and T1 images (error) were measured at 14 points and at the nasolabial angle. A mean linear prediction error of 2.91±2.16mm was found. The mean error at the nasolabial angle was 8.1±5.6°. In conclusion, the ability to accurately predict 3D soft tissue changes after Le Fort I osteotomy using Dolphin 3D software is limited.

Purpose Virtual surgical planning (VSP) and 3-dimensional printing of surgical splints are becoming the standard of care for orthognathic surgery, but costs have not been thoroughly evaluated. The purpose of this study was to compare the cost of VSP and 3-dimensional printing of splints (“VSP”) versus that of 2-dimensional cephalometric evaluation, model surgery, and manual splint fabrication (“standard planning”). Materials and Methods This is a retrospective cohort study including patients planned for bimaxillary surgery from January 2014 to January 2015 at Massachusetts General Hospital. Patients were divided into 3 groups by case type: symmetric, nonsegmental (group 1); asymmetric (group 2); and segmental (group 3). All cases underwent both VSP and standard planning with times for all activities recorded. The primary and secondary predictor variables were method of treatment planning and case type, respectively. Time-driven activity-based micro-costing analysis was used to quantify the differences in cost. Results were analyzed using a paired t test and analysis of variance. Results The sample included 43 patients (19 in group 1, 17 in group 2, and 7 in group 3). The average times and costs were 194 ± 14.1 minutes and $2,765.94, respectively, for VSP and 540.9 ± 99.5 minutes and $3,519.18, respectively, for standard planning. For the symmetric, nonsegmental group, the average times and costs were 188 ± 17.8 minutes and $2,700.52, respectively, for VSP and 524.4 ± 86.1 minutes and $3,380.17, respectively, for standard planning. For the asymmetric group, the average times and costs were 187.4 ± 10.9 minutes and $2,713.69, respectively, for VSP and 556.1 ± 94.1 minutes and $3,640.00, respectively, for standard planning. For the segmental group, the average times and costs were 208.8 ± 13.5 minutes and $2,883.62, respectively, for VSP and 542.3 ± 118.4 minutes and $3,537.37, respectively, for standard planning. All time and cost differences were statistically significant (P Conclusions The results of this study indicate that VSP for bimaxillary orthognathic surgery takes significantly less time and is less expensive than standard planning for the 3 types of cases analyzed.

Purpose Controversy exists as to the most appropriate treatment strategy for myofibromas of the jaws: en bloc resection versus enucleation. The purpose of the present study was to evaluate the treatment outcomes in children with these uncommon benign tumors. Materials and Methods We performed a retrospective chart review of pediatric patients with jaw myofibromas. The predictor variables included patient demographics, clinical presentation, imaging characteristics, pathologic features, treatment, and follow-up. The outcome variable was cure or recurrence. The descriptive statistics were computed. Results A total of 12 patients (mean age 6.7 years) met the inclusion criteria. There were 2 presentations: exophytic soft tissue mass in dentoalveolar segment (n = 5); and intraosseous mass (n = 7). No distinct histopathologic differences were found between the 2 groups. Exophytic myofibromas displayed rapid growth, tooth displacement and/or mobility, bony expansion, and/or cortical thinning/perforation. Most were treated by resection. The intraosseous lesions were asymptomatic and/or incidentally discovered. They were treated by enucleation and curettage. The mean follow-up for the 2 groups was 6.5 and 3.9 years, respectively. There were no recurrences. Conclusions The results of the present study indicate that there are 2 clinical presentations of myofibromas of the jaws in children: an aggressive exophytic type and a nonaggressive intraosseous type. They are histopathologically indistinguishable.

Purpose To identify the nasolabial soft tissue changes that occur after Le Fort I advancement. Materials and Methods A prospective study of patients who had Le Fort I advancement at the Children's Hospital Boston from 2005 to 2007. The pre- and postoperative anthropometeric nasolabial measurements were recorded by a single examiner. Results A total of 37 patients with a mean age of 18.6 years at the time of operation and a mean follow-up of 12.6 months were recruited. Nearly one half of the study sample (16 of 37) had cleft lip/palate. Direct anthropometry showed a reduction of the nasal length by 1.3 mm while the nasal tip protrusion increased by 1.1 mm. The nasofrontal angle decreased by 9.8° and the upper lip moved forward by 4.15 mm, reflecting the advancement in the maxilla. The height of the cutaneous upper lip increased by 0.4 mm. No significant differences were found in the soft tissue response observed between the cleft and noncleft subjects. Conclusions Le Fort I advancement produces elevation of the nasal tip, as seen by a reduction in the nasal length, an increase in the nasal tip protrusion, and a concomitant reduction in the nasofrontal angle. Additionally, the cutaneous lip height increased, most likely due to an unfurling of the upper lip.

Purpose Children with unoperated cleft lip/palate have nearly normal facial growth, whereas patients who have had labiopalatal repair often exhibit midfacial retrusion. The aim of this study was to compare cephalometric data in patients with repaired unilateral or bilateral complete cleft lip/alveolus (UCCLA or BCCLA) with patients with repaired unilateral or bilateral complete cleft lip/palate (UCCLP or BCCLP). This study might provide insight into the etiology of impaired facial growth in patients with repaired cleft lip/palate. Materials and Methods This was a retrospective, cross-sectional analysis of nonsyndromic patients with UCCLA, BCCLA, UCCLP, and BCCLP. Angular and linear measurements of the midfacial region were made on traced lateral cephalograms. Paired t tests were used to compare each group with normative controls from the Michigan Growth Study. Multivariate analysis of variance was used to determine possible differences among the groups. Results There were 77 patients (38 male and 39 female) with a mean age of 11.2 years (range, 6 to 16 years; UCCLA, n = 25; BCCLA, n = 7; UCCLP, n = 18; and BCCLP, n = 27). There was no significant difference in midfacial position between the UCCLA and BCCLA groups and noncleft age-matched controls. In contrast, the maxilla in patients with UCCLP and BCCLP was significantly smaller and more retruded ( P Conclusions Children with UCCLA and BCCLA appear to have normal midfacial growth, whereas the maxilla in children with UCCLP and BCCLP is small and retrusive. This study suggests that the presence and/or repair of the secondary palate is responsible for midfacial hypoplasia in these patients.

Facial infiltrating lipomatosis causes diffuse overgrowth of subcutaneous fat, muscle, and bone. Because adipose tissue mass is angiogenesis dependent, the purpose of this study was to determine whether neovascularization is upregulated in this disease.Infiltrating lipomatosis tissue was collected prospectively from the preauricular cheek of 5 patients; neovascularization was compared to normal postauricular adipose. Specimens were analyzed using immunofluorescence for CD31 (microvascular density), α-smooth muscle actin (pericyte marker), CD31/Ki67 (proliferating endothelial cells), and CD34/CD133 (endothelial progenitor cells). Quantitative reverse transcription-polymerase chain reaction was used to determine messenger RNA expression of progenitor cells (CD133) and factors that recruit them: vascular endothelial growth factor (VEGF-A), hypoxia-inducible factor 1α, matrix metalloproteinase 9 (MMP-9), and stromal cell-derived factor 1α. Angiopoietin 1 and 2, MMP-2, VEGF receptors, and neuropilin receptors were quantified using quantitative reverse transcription polymerase chain reaction. There was no difference in microvascular density, pericytic density, or endothelial proliferation between infiltrating lipomatosis and normal adipose tissue (P = 0.2). Expressions of VEGF-A, hypoxia-inducible factor 1α, stromal cell-derived factor 1α, angiopoietin 1 and 2, MMP-2 and -9, VEGF receptors 1 and 2, neuropilin receptors 1 and 2, and CD133 messenger RNA were not elevated compared to control fat (P = 0.1). Endothelial progenitor cells were not present in specimens of infiltrating lipomatosis. Infiltrating lipomatosis does not exhibit elevated angiogenic or vasculogenic factors compared to normal fat; the vasculature is stable. Neovascularization does not seem to play a role in the pathogenesis of this condition.

Purpose Approximately 25% to 40% of patients with cleft lip/palate develop maxillary retrusion that requires Le Fort I osteotomy. Maxillary advancement brings the soft palate forward, and this may cause velopharyngeal insufficiency (VPI). The goal of this study was to identify predictors that place patients with repaired cleft palate at risk of developing VPI after Le Fort I advancement. Materials and Methods This was a retrospective study of nonsyndromic patients with cleft lip/palate who had a Le Fort I osteotomy between 2000 and 2008. Charts were reviewed and data were collected on patient characteristics, preoperative speech assessments, and nasopharyngoscopic reports. Pre- and postoperative cephalometric radiographs were used to measure maxillary advancement and to assess the structure of the velopharynx. Simple logistic regression analysis examined the association between each predictive variable and postoperative VPI, as indicated by need for pharyngeal flap. Predictors with P ≤ .10 were included in the multivariate regression model. In both the univariate and the multivariate analyses, P ≤ .05 was considered statistically significant. Results Univariate analysis showed a significant association between preoperative soft palatal length and need for a pharyngeal flap (P = .005). By multivariate analysis, both preoperative soft palatal length and postoperative pharyngeal depth were associated with need for pharyngeal flap (P = .003 and P = .030). Conclusion This study shows that a short soft palate is associated with VPI after Le Fort I osteotomy. Assessment of palatal length and pharyngeal depth on cephalometric radiographs is helpful in predicting postoperative VPI and need for a pharyngeal flap in patients with cleft palate after maxillary advancement.

Purpose The purpose of this study was to evaluate the stability of Le Fort I osteotomy using self-reinforced biodegradable poly-70L/30DL-lactide miniplates and screws. Patients and Methods Nineteen patients who had Le Fort I osteotomy and internal fixation using self-reinforced biodegradable poly-70L/30DL-lactide miniplates and screws were evaluated both radiographically and clinically. Changes in maxillary position after operation were documented 1 week, 1, 3, 6 mo, and/or 1-yr postoperatively with lateral cephalometric tracings. Complications of the self-reinforced biodegradable poly-70L/30DL-lactide miniplates and screws were evaluated by follow-up roentgenograms and clinical observation. A mixed model analysis for repeated measures was used for statistical analysis. Results Maxillary position was stable after operation with no change between time points ( P > .05). There were no complications with the self-reinforced biodegradable poly-70L/30DL-lactide miniplates and screws. Conclusions Internal fixation of the maxilla after Le Fort I osteotomy with self-reinforced biodegradable poly-70L/30DL-lactide miniplates and screws is a reliable method for maintaining the postoperative maxillary position after Le Fort I osteotomy.

Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele. In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach. Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosis places them at greater risk for fracture of the cribriform plate and dural tears during this procedure. Unrecognized dural injury is the etiology of this complication in this young patient; however, elevated intracranial pressure may have been a confounding factor. Attention to the anatomy of the anterior cranial base, as seen on sagittal CT images, will aid in preventing this complication.

An 11-year-old Egyptian boy presented with mild pain in his eft lower jaw accompanied by swelling. Clinical examination howed a hard, tender mandibular enlargement extending rom the chin to the left molar area. The orthopantomogram OPG) showed poorly defined multilocular radiolucency inolving the mandibular body from the right lateral incisor to he left first molar (Fig 1A). There were discrete signs of esorption of root apices of the adjacent teeth. Computed omography examination disclosed a large expansive osteoytic lesion with a soap bubble appearance and erosion of both estibular and lingual mandibular cortices (Fig 1B). Incisional biopsy was performed under local anesthesia. ections contained spindle cells within fibrous stroma and rregularly distributed multinucleated giant cells clustering round areas of hemorrhage. Periphery of the lesion howed reactive osteoid tissue formation (Fig 1C). The overall clinical, radiologic, and histopathologic picture as consistent with central giant cell granuloma. Laboratory nvestigations including complete blood count, alkaline phoshatase, calcium, and parathormone levels were within noral limits, while phosphorus level was slightly elevated.

Purpose Patients with hemifacial microsomia (HFM) and Kaban-Pruzansky type III mandibular deformities require ramus construction with autologous tissue. The free fibula flap, an alternative to the costochondral graft, has favorable characteristics for this construction but may be associated with temporomandibular joint ankylosis. The purposes of this study were to present a series of patients with HFM who underwent free fibula flap ramus construction, to determine the incidence of ankylosis, and to identify perioperative factors associated with ankylosis. Materials and Methods We performed a retrospective cohort study of patients with HFM who underwent ramus construction with a free fibula flap at Boston Children's Hospital from 2003 to 2015. Patients who had at least 1 year of follow-up and complete medical records were included. The predictor variables included demographic information, HFM severity, surgical history, and operative details. The primary outcome variable was the occurrence of ankylosis. Descriptive statistics were calculated, and significance was set at P Results We included 8 patients (75% of whom were female patients) in the study sample. Patients underwent construction at a mean age of 11.4 ± 5.9 years (range, 5 to 21 years). In 5 patients (63%), ankylosis developed during the follow-up period of 7.3 ± 4.8 years. The average time from construction to ankylosis was 4.2 ± 3.7 years. The only predictor variable statistically significantly associated with ankylosis was the use of a contralateral releasing osteotomy, which reduced the rate of ankylosis (P = .035). There was a trend toward a younger age in patients in whom ankylosis developed (8.8 ± 2.6 years) compared with those without ankylosis (15.5 ± 8.1 years, P = .392). Conclusions The free fibula flap can be associated with a high rate of ankylosis when used for ramus construction in patients with HFM. Passive flap insertion and/or use of a contralateral releasing osteotomy may reduce this risk.

Children with repaired cleft lip/palate require secondary closure of the alveolar cleft and, often, nasolabial revision. We describe a technique performed in 61 patients for harvesting bone for the alveolar defect and dermis for augmentation of the median tubercle, taking both from the posterior iliac region. The advantages of the posterior approach are as follows: (1) the same donor site is used for cancellous bone and dermal graft and (2) the child's appearance is improved along with alveolar cleft grafting.

Purpose To report the use of a semiburied curvilinear distraction device, with a 3-dimensional (3D) computed tomography treatment planning system, for correction of mandibular deformities. Materials and Methods This was a retrospective evaluation of 13 consecutive patients, with syndromic and nonsyndromic micrognathia, who underwent correction by curvilinear distraction osteogenesis. A 3D computed tomography scan was obtained for each patient and imported into a 3D treatment planning system (Slicer/Osteoplan). Surgical guides were constructed to localize the osteotomy and to drill holes to secure the distractor's proximal and distal footplates to the mandible. Postoperatively, patients were followed by clinical examination and plain radiographs to ensure the desired vector of movement. At end distraction, when possible, a 3D computed tomography scan was obtained to document the final mandibular position. Results Of the 13 patients, 8 were females and 5 were males, with a mean age of 11.9 years (range 15 months to 39 years). All 13 underwent bilateral mandibular curvilinear distraction. Of the 13 patients, 8 were 16 years old or younger and 5 were younger than 6 years of age. The diagnoses included Treacher Collins syndrome (n = 3), Nager syndrome (n = 3), craniofacial microsomia (n = 2), post-traumatic ankylosis (n = 1), and micrognathia (syndromic, n = 3; nonsyndromic, n = 1). The correct distractor placement, vector of movement, and final mandibular position were achieved in 10 of 13 patients. In the other 3 patients, the desired jaw position was achieved by “molding” the regenerate. Conclusions The use of a semiburied curvilinear distraction device, with 3D treatment planning, is a potentially powerful tool to correct complex mandibular deformities.