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Patients treated with perioperative Invisalign for orthognathic surgery may experience less postoperative swelling than those with fixed appliances because of a lack of mucosal irritation from bonded brackets and wires. The aims of this study were to (1) compare facial swelling after orthognathic surgery in subjects with Invisalign to those with fixed appliances using 3-dimensional (3D) subtraction imaging and (2) determine if the type of operation influences differences in swelling.This is a retrospective case-control study. To be included in the case group (Invisalign), patients had to have had: (1) LeFort I and/or bilateral sagittal split osteotomies, with or without genioplasty, (2) perioperative orthodontic treatment using Invisalign, and (3) 3D photographs at postoperative timepoints 1 week (T1), 3-4 weeks (T2), and 5-7 weeks. A sex and operation-matched control group with fixed appliances (standard) was also included. The primary outcome variable was the volume of facial swelling, measured by subtraction imposition of the T1 and T2 3D images using reference images (5-7 weeks).Twenty-two subjects (36% female; mean age 20.7 ± 3.15 years) were included: Invisalign (n = 11) and standard (n = 11). For each group, 7 subjects had 1 operation (LeFort I or bilateral sagittal split osteotomies), and 4 had bimaxillary surgery ± genioplasty. At T1, the Invisalign group had significantly less swelling than the standard group (17.52 ± 10.79 cmSubjects with Invisalign had significantly less facial swelling in the first postoperative week than those with fixed appliances.

Background: Craniofacial microsomia (CFM) is characterized by several malformations related to the first and second pharyngeal arch. Patients typically present with facial asymmetry, but extracraniofacial organ systems might be involved, including limb anomalies. The purpose of this study was to analyze the occurrence of upper and lower limb anomalies in CFM patients. Furthermore, the relation between limb anomalies and the OMENS+ (orbital distortion; mandibular hypoplasia; ear anomaly; nerve involvement; soft-tissue deficiency; and associated extracraniofacial anomalies) classification was examined. Methods: A retrospective study was conducted including patients with CFM from craniofacial units in three different countries. Patients were included when clinical and/or radiographic images were available. Demographic, radiographic, and clinical information was obtained. Results: A cohort of 688 patients was available and selected for analysis. In total, 18.2% of the patients were diagnosed with at least one upper and/or lower limb anomaly. Upper and lower limb anomalies were seen in, respectively, 13.4% and 7.8% of patients. Patients with other extracraniofacial anomalies had a significantly higher risk for limb anomalies (OR, 27.98; P = 0.005). Laterality of CFM and a higher OMENS score were not associated with limb anomalies. Conclusions: More than one in six patients with craniofacial microsomia have limb anomalies. Therefore, clinical awareness for these anomalies is warranted. Examination and, if present, follow-up on limb abnormalities in patients with CFM should be implemented in the standard assessment of CFM patients.

Purpose: Success of alveolar bone grafting has been estimated using 2-dimensional periapical radiographs that are associated with image distortion. Cone beam computed tomography (CBCT) accurately displays 3-dimensional anatomy. This study sought to develop and apply a radiographic measure of alveolar bone grafting success using CBCT. Methods: This was a retrospective case series composed of patients with cleft lip/palate who had iliac crest bone grafting by 1 surgeon from 2005 to 2020. CBCT scans were obtained ≥4 months after graft. The outcome variables included vertical bone height on cleft adjacent teeth, labiopalatal thickness, and nasal floor symmetry and were scored using a 1 to 4 ordinal scale. Vertical height was determined by the distance from the cementoenamel junction to the marginal bone level of cleft adjacent teeth (1: ≥75% root length, 2: ≥50 to

Treacher Collins syndrome (TCS) is a congenital malformation of the craniofacial structures derived from the first and second pharyngeal arches. The craniofacial deformities are well described in the literature. However, little is known about whether there are associated extracraniofacial anomalies. A retrospective study was conducted using data from four craniofacial units. Medical charts were reviewed for the presence and type of extracraniofacial anomalies, as well as age at diagnosis. A possible correlation between the severity of the phenotype and the presence of extracraniofacial anomalies was assessed using the Hayashi classification. A total of 248 patients with TCS were identified; 240 were confirmed to have TCS, of whom 61 (25.4%) were diagnosed with one or more extracraniofacial anomalies. Ninety-five different extracraniofacial anomalies were found; vertebral (n = 32) and cardiac (n = 13) anomalies were most frequently seen, followed by reproductive system (n = 11), central nervous system (n = 7), and limb (n = 7) anomalies. No correlations between tracts were found. Extracraniofacial anomalies were more prevalent in these patients with TCS compared to the general population (25.4% vs 0.001–2%, respectively). Furthermore, a positive trend was seen between the severity of the syndrome and the presence of extracraniofacial anomalies. A full clinical examination should be performed on any new TCS patient to detect any extracraniofacial anomalies on first encounter with the craniofacial team.

Objective Determine the effects of differential maxillary expansion on nasal width in patients with unilateral cleft lip and alveolus with or without cleft of the secondary palate (UCLA ± P). Design Retrospective radiographic study. Setting Institutional. Participants Forty patients with UCLA ± P who had alveolar bone grafting (ABG) between 2015 and 2020 and available preexpansion and postexpansion cone beam computed tomography (CBCT) scans. Twenty patients with UCLA ± P who underwent ABG without expansion were included as controls. Main Outcome Measure Percent change in width at the nasal pyriform, inferior turbinates, and alar base on cleft and noncleft sides. Results The study included 40 patients (24 males, mean age 9.6 years) and 20 controls (10 males, mean age 10.1 years). After maxillary expansion, there was an increase in width on the cleft and noncleft sides compared to nonexpanded controls at nasal pyriform (10.9% cleft side, P ≤ 0.001 and 4.3% noncleft side, P ≤ .001), inferior turbinate (8.7%, P ≤ .001 and 4.5%, P = .01), and alar base (6.7%, P = Conclusion Patients with UCLA ± P who undergo differential maxillary expansion before ABG exhibit greater nasal widening on the cleft side.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare inflammatory condition characterized by sterile bone lesions. There appears to be a shift in the diagnostic modalities and treatment over the past decades despite insufficient published data. The purpose of this study was to document: 1) the number of patients diagnosed with CRMO, 2) patient demographics, 3) disease characteristics at presentation, 4) diagnostic modalities employed, and 5) treatments prescribed at our institution over a 30-year period.This single-center, retrospective cohort study included children diagnosed with CRMO who presented between 1990 and 2020. The electronic medical records were queried using numerous search terms. Patients were excluded if CRMO was included in the differential diagnosis but was not confirmed at the time of chart review or if CRMO was suspected early in the disease course but the patient was ultimately diagnosed with another condition. The predictor (time in years) and outcome variables (diagnostic modalities and treatment types) were tested using bivariate analyses using IBM SPSS, Version 27 (IBM Corp., Armonk, NY).A total of 224 patients were diagnosed with CRMO during the observation period (68.3% female; 67.4% white). The number of patients diagnosed over the past decade rose by 215%, as compared to the previous 2 decades (1990 to 2010). Regional magnetic resonance imaging (83.8%) and biopsy (66.5%) were the most utilized diagnostic modalities over the past decade, with a statistically significant decline in the proportion of biopsies performed (66.5% during the past decade vs 84.9% in the previous 2 decades, P = .01). Over the past decade, nonsteroidal anti-inflammatory drugs (40.1%), disease-modifying antirheumatic drugs (27.1%), and tumor necrosis factor inhibitors (21.1%) were the most commonly used treatments, with a statistically disproportionate increase in the use of tumor necrosis factor inhibitors (21.1% during the past decade vs 3.8% in the previous 2 decades, P .001).This is one of the largest CRMO cohort studies and the only study to observe changes in diagnostic modalities and treatment over a 30-year period. Future studies should assess the impact of variations in clinical presentation, time to diagnosis, diagnostic modalities, and management as predictors of disease outcomes.

Evaluate periodontal bone support of maxillary central incisors (MCI) in patients with bilateral complete cleft lip and palate (BCCLP). Determine if syndromic diagnosis, age at time of alveolar bone graft (ABG), presence of maxillary lateral incisor (MLI), history of dentofacial orthopedics, maxillary expansion, and pre-maxillary osteotomy are associated with the periodontal bone support of MCI. Retrospective radiographic study. Tertiary care children's hospital One hundred seventy-nine patients with BCCLP (22 syndromic) who had post-operative ABG cone beam computed tomography (CBCT) scans taken between 2002-2018. Crown to root (C/R) ratio of MCI measured on CBCT scans. The C/R ratio in 65% of MCI indicated periodontally compromised teeth. Presence of a MLI improved bone support on adjacent MCI when compared to those missing a MLI (51.4% vs 28.4%, P = .010). There was no significant difference in C/R ratios for syndromic diagnosis, age at ABG, history of dentofacial orthopedics, maxillary expansion, and pre-maxillary osteotomy. The majority of MCI in patients with BCCLP are periodontally compromised but bone support is improved when cleft adjacent lateral incisors are present.

This article provides an overview of epidemiology, genetics, and common orofacial features of those with craniosynostosis. Patients with craniosynostosis require several surgical procedures along with continuum of care. The earliest surgical interventions are done during the first few years of life to relieve the fused sutures. Midface advancement, limited phase of orthodontic treatment, and combined orthodontics/orthognathic surgery treatment are usually required during later years. This article presents several examples of cases with outcomes associated with these procedures.

textabstractObjective: To provide an overview on the prevalence and types of dental anomalies in patients with craniofacial microsomia (CFM). Eligibility criteria: Inclusion criteria were CFM and dental anomalies. The following data were extracted: number of patients, methodology, mean age, sex, affected side, severity of mandibular hypoplasia, dentition stage and dental anomalies. Information sources: Cochrane, EMBASE, PubMed, MEDLINE Ovid, Web of Science, CINAHL EBSCOhost and Google Scholar, searched until the 30 August 2019. Risk of bias: The quality was examined with the OCEBM Levels of Evidence. Included studies: In total, 13 papers were included: four retrospective cohort studies, four prospective cohort studies, four case-control studies and one case series. Synthesis of results: The studies reported information on dental agenesis, delayed dental development, tooth size anomalies, tooth morphology and other dental anomalies. Description of the effect: Dental anomalies are more often diagnosed in patients with CFM than in healthy controls and occur more often on the affected than on the non-affected side. Strengths and limitations of evidence: This is the first systematic review study on dental anomalies in CFM. However, most articles were of low quality. Interpretation: Dental anomalies are common in CFM, which might be linked to the development of CFM. The pathophysiology of CFM is not entirely clear, and further research is needed.

Opioid misuse is a public health crisis. It is incumbent upon surgeons to understand analgesic requirements for operations they perform to inform responsible prescribing practices. The purpose of this study was to quantify opioid use following orthognathic surgery.This is a prospective cohort study including consecutive patients that had orthognathic surgery at the Boston Children's Hospital from May 2020 to September 2021. To be included, subjects had to have had a Le Fort I osteotomy, bilateral sagittal split osteotomies, or both. Subjects were excluded if they had a craniofacial syndrome (not including cleft lip and palate) or did not complete the study. Postoperative prescriptions and instructions were standardized. The primary outcome variable was total postoperative opioid use (inpatient + outpatient). Inpatient opioid delivery was recorded from the electronic medical record. Outpatient opioid use was ascertained via electronic questionnaire each day for 7 postoperative days. Descriptive and analytic statistics were calculated.Thirty-five subjects (54% male, age 18.7 ± 2.7 years) were included. Thirty-two subjects (91%) used postoperative opioid analgesia as inpatients, outpatient, or both, with mean total use of 18.2 ± 20.9 morphine milligram equivalents/subject (equivalent to 7.3 ± 8.4 oral oxycodone 5-mg doses). Nine (26%) subjects received inpatient opioid but did not use any oral opioid after discharge. As outpatients, a mean of 3.9 ± 5.5 oral oxycodone 5-mg dose was used per patient over 2.1 ± 2.1 postoperative days. Le Fort I osteotomy-only procedures had significantly lower (P = .032) and combined Le Fort I osteotomy and bilateral sagittal split osteotomy operations had significantly higher (P = .003) opioid requirements than the mean. Length of procedure and hospital length of stay were significant predictors of analgesic need, with an increase of 0.34 oxycodone doses/subject for each 10-minute increase in procedure time and 0.20 oxycodone doses/subject for each 1-hour increase in length of stay. Pain level on the first postoperative day was also a predictor of total opioid use (P .050).Opioid use after orthognathic surgery is less than expected. Caution is necessary to avoid overprescribing.

Objective Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. Design Cross-sectional survey-based evaluation. Setting International comprehensive cleft care workshop. Participants Total of 489 participants. Interventions Three-day simulation-based hybrid comprehensive cleft care workshop. Main Outcome Measures Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. Results The workshop included 489 participants from 5 continents. The response rate was 39.9%. Participants perceived financial factors (30.3%) the most significant barrier and improvement in training (39.8%) as the most important intervention to overcome barriers facing cleft care delivery in low to middle-income countries. All participants reported a high level of satisfaction with the workshop and a strong positive perceived short-term impact on their practice. Importantly, while this was true for both in-person and virtual attendees, in-person attendees reported a significantly higher satisfaction with the workshop (28.63 ± 3.08 vs 27.63 ± 3.93; P = .04) and perceived impact on their clinical practice (22.37 ± 3.42 vs 21.02 ± 3.45 P = .01). Conclusion Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

Objective: There is a high rate of obstructive sleep apnea (OSA) in patients with syndromic craniosynostosis (SCS). Little is known about the airway anatomy in this population. The purpose of this study is to characterize the 3 dimensional (3D) upper airway in patients with SCS with and without OSA. Design: This is a retrospective study of patients with SCS treated at Boston Children’s Hospital from 2000 to 2015. Patients were divided into OSA and no-OSA groups based on polysomnography. Predictor variables included age, sex, body mass index (BMI), and 3D upper airway measurements. The primary outcome variable was the presence or absence of OSA. Secondary outcome variables were apnea–hypopnea index and oxygen saturation nadir. Descriptive and bivariate statistics were computed, and significance was set as P < .05. Results: There were 24 patients: 16 in the OSA group and 8 in the no-OSA group. The 2 groups did not differ significantly by age, BMI, or syndromic diagnosis. The presence of OSA was associated with a smaller minimum retropalatal cross-sectional area (minRPCSA; P < .001). In a logistic regression model controlling for age, sex, and upper airway length, minRPCSA was the primary predictor of OSA ( P ≤ .002). Receiver operating characteristic analysis determined minRPCSA = 55.3 mm2 to be the optimal diagnostic threshold for OSA, with sensitivity = 100% and specificity = 87.5% ( P < .001). Conclusion: A minRPCSA ≤55.3 mm2 is predictive of the presence of OSA in patients with SCS.

Objective: Patients with cleft lip and palate (CLP) are more likely to have sinusitis. The purpose of this study is to determine whether patients with CLP have thickening of the Schneiderian membrane. Specific aims were to (1) compare Schneiderian membrane thickness in patients with CLP to noncleft controls, (2) evaluate whether membrane thickening is associated with cleft side in patients with unilateral cleft lip and palate (UCLP), and (3) evaluate if age and sex are predictors of mucosal thickening. Design: Case–control study. Setting: Tertiary care center. Patients: Patients with CLP and controls. Main Outcome Measure: The primary outcome variable was maximum Schneiderian membrane thickness measured on cone beam computed tomography. The primary predictor variable was the presence of a cleft. Additional variables were cleft phenotype, age, and sex. Results: There were 225 patients with CLP and 93 controls. Median mucosal thickness was 2.4 mm in cleft group and 0.0 mm in controls ( P = .006). In cleft group, 56.7% of sinuses had mucosal thickness >2.0 mm compared to 38.2% in controls ( P = < .004). Pathologic membrane thickening (>4.0 mm) was significantly higher in cleft group ( P = .005). No statistically significant difference in mucosal thickness between cleft and noncleft sides in patients with UCLP. Linear regression showed no association between age or sex and Schneiderian membrane thickness. Conclusion: Schneiderian membrane thickening is more common in patients with CLP and is not associated with the side of the cleft in patients with UCLP.

Distraction osteogenesis (DO) is a surgical technique that takes advantage of natural wound healing mechanisms to augment bone and soft tissues. DO is extremely versatile and can be applied to nearly any bone. In the craniofacial skeleton, the cranial vault, midface, maxilla and mandible are the most common sites for DO. This technique allows larger skeletal movements than could be achieved with conventional techniques, decreases operative time and blood loss, eliminates the need for bone grafts and associated donor site morbidity, and may improve postoperative stability. DO can be used in preparation for, in lieu of, or in combination with orthognathic surgery to correct dentofacial deformities. DO requires meticulous planning to achieve optimal results.

Current computational tools for planning and simulation in plastic and reconstructive surgery lack sufficient precision and are time-consuming, thus resulting in limited adoption. Although computer-assisted surgical planning systems help to improve clinical outcomes, shorten operation time and reduce cost, they are often too complex and require extensive manual input, which ultimately limits their use in doctor-patient communication and clinical decision making. Here, we present the first large-scale clinical 3D morphable model, a machine-learning-based framework involving supervised learning for diagnostics, risk stratification, and treatment simulation. The model, trained and validated with 4,261 faces of healthy volunteers and orthognathic (jaw) surgery patients, diagnoses patients with 95.5% sensitivity and 95.2% specificity, and simulates surgical outcomes with a mean accuracy of 1.1 ± 0.3 mm. We demonstrate how this model could fully-automatically aid diagnosis and provide patient-specific treatment plans from a 3D scan alone, to help efficient clinical decision making and improve clinical understanding of face shape as a marker for primary and secondary surgery.

Purpose There is no widely accepted protocol for management of infants with Robin sequence (RS) who present with airway obstruction and feeding impairment. The purposes of this study were to evaluate diagnostic and treatment preferences of clinicians from the United States and non-US countries and to use these data to propose an algorithm for early management of infants with RS. Materials and Methods A cross-sectional study was implemented using a survey, which was distributed to craniofacial surgeons and non-surgeon physicians involved in management of infants with RS. Predictor variables were nationality, specialty, and surgical volume. Outcome variables included survey responses regarding preferences for evaluation and early treatment of patients with RS. A diagnostic and early treatment algorithm was proposed based on the most common survey responses. Descriptive and analytic statistics were calculated and a P less than .05 was considered significant. Results A total of 275 responses were received and 82 were excluded, leaving a final sample of 193 participants: 155 (80%) surgeons and 38 (20%) non-surgeon physicians. Thirty-seven US states were represented by 143 (74%) respondents and 50 (26%) participants were from 19 non-US countries. There were 96 (50%) low-volume and 97 (50%) high-volume respondents. There was general agreement in methods for evaluation and early treatment among respondents; the only significant differences were 1) US surgeons operate earlier to address airway obstruction than those from other countries (P = .001), 2) high-volume providers tend to operate earlier (P = .017) and to choose mandibular distraction more frequently (P = .003) than low-volume respondents, and 3) mandibular distraction is the preferred operation in the United States, but several operations for airway improvement are used equally in non-US countries (P Conclusion Despite variation in the evaluation and treatment of infants with RS, this international and multispecialty survey showed trends that could represent best practices. An algorithm based on these findings is proposed.

BACKGROUND Patients with syndromic craniosynostosis exhibit exorbitism due to supraorbital and midfacial retrusion. This study documented the change in sagittal orbital-globe relationship following Le Fort III midfacial advancement. METHODS This retrospective case series comprised patients with syndromic craniosynostosis who underwent midfacial distraction from 1997 to 2016. Changes in sagittal globe position in relation to the orbital rims were measured by pre- and postoperative direct anthropometry, computed tomographic scans, or both methods. Descriptive statistics were calculated; significance was set at P

Craniofacial microsomia is characterized by an asymmetric hypoplasia of derivatives of the first and second pharyngeal arch, leading to a variety of phenotypic presentations. Studies on surgical correction of patients with craniofacial microsomia have small cohorts, leaving controversial opinions on the optimal treatment modality, the indication for surgery, and the optimal timing of surgery. The purpose of this study was to evaluate the types of, timing of, and total number of surgical corrections performed and the number of surgical procedures in correlation to the severity of the phenotype.A retrospective chart study was conducted including patients diagnosed with craniofacial microsomia from three large craniofacial units. Demographic, radiographic, and clinical information was obtained, including type and number of surgical procedures and age at the time of surgery.A total of 565 patients were included. In total, 443 (78.4 percent) of all patients underwent some form of surgery during their life, varying from skin tag removal to major craniofacial operations. The number of surgical interventions was higher with increasing severity of phenotype, bilateral presentation, and a younger age at the first intervention.Multiple surgical corrections are frequently seen in patients with a more severe or bilateral presentation. Furthermore, those who are treated earlier in life for correction of asymmetry of the mandible will undergo significantly more surgical procedures to correct the asymmetry later on, independent of the Pruzansky-Kaban type mandible. A prospective international multicenter study is designed with a uniform registration and outcome measurement tool to identify the optimal treatment strategy.

Objectives Data on normal mandibular development in the infant is lacking though essential to understand normal growth patterns and to discriminate abnormal growth. The aim of this study was to provide normal linear measurements of the mandible using computed tomography performed in infants from 0 to 2 years of age. Material and methods 3D voxel software was used to calculate mandibular body length, mandibular ramus length, bicondylar width, bigonial width and the gonial angle. Intra- and inter-rater reliability was assessed for these measurements. They were found to be sufficient for all distances; intra-class correlation coefficients were all above 0.9. Regression analysis for growth modelling was performed. Results In this multi-centre retrospective study, 109 CT scans were found eligible that were performed for various reasons (e.g. trauma, craniosynostosis, craniofacial abscesses). Craniosynostosis patients had larger mandibular measurements compared to non-craniosynostosis patients and were therefore excluded. Fifty-one CT scans were analysed. Conclusions Analysis showed that the mandible increases more in size vertically (the mandibular ramus) than horizontally (the mandibular body). Most of the mandibular growth occurs in the first 6 months. Clinical relevance These growth models provide insight into normal mandibular development in the first 2 years of life. This reference data facilitates discrimination between normal and abnormal mandibular growth.

The purpose of this study was to identify factors that influence the need for a supplemental bone graft prior to dental implant placement at previously grafted alveolar cleft sites.Retrospective case series of patients with cleft lip/palate who had both alveolar bone grafting (ABG) and placement of a dental implant(s) to replace a missing incisor(s) at the cleft site by the senior surgeon (BLP) at Boston Children's Hospital from 2005 through 2020. Primary outcome variable was need for a supplemental bone graft prior to dental implant placement. Predictor variables included gender, cleft type (unilateral vs. bilateral), implant site, number of implants placed, age at ABG and implant placement, time between ABG and implant, history of maxillary expansion and whether the patient had a Le Fort I osteotomy to correct maxillary hypoplasia before implant placement. Descriptive statistics were computed and comparative analyses were performed using Pearson XThere were 84 implants placed in 59 patients (64.2% female) with cleft lip and palate who had alveolar bone grafting of which 57.1% (n = 48) required a supplemental graft prior to placement. Median time (IQR) from alveolar bone grafting to implant placement was significantly longer in patients who required additional grafting (8.1 versus 5.4 years, P.001). Patients who required supplemental bone were significantly younger at the time of alveolar bone grafting (10.1 versus 12.3 years, P.001). Cleft sites in patients who had a Le Fort I osteotomy prior to implant placement required bony augmentation more often than cleft sites in patients who did not have a Le Fort I osteotomy (58.7% versus 33.3%, P = .03).Patients with cleft lip and/or palate who undergo alveolar bone grafting should be counseled that they are likely to require a supplemental bone graft prior to implant placement.

Purpose Chronic recurrent multifocal osteomyelitis (CRMO) is underdiagnosed and underreported because of a lack of awareness among providers. While patients with undiagnosed CRMO often present to oral and maxillofacial surgeons (OMSs) with a chief complaint of mandibular pain, to our knowledge, there is no literature regarding how well informed these providers are about this disease. Survey studies and educational efforts have been carried out among other specialists with the aim of raising awareness. The purpose of this study was to document current levels of understanding and determine knowledge gaps among OMSs regarding the diagnostic process for CRMO. Materials and Methods For this cross-sectional cohort study, the investigators sent an anonymous and electronic survey to OMSs practicing in the United States. Using a clinical vignette, the survey captured respondents' ability to evaluate, diagnosis, and take appropriate next steps for a hypothetical patient with CRMO. Results A total of 429 respondents completed the entire survey. The following proportion of respondents correctly answered questions pertaining to information gathering (10.3%), differential diagnosis (9.8%), overall diagnostic workup (76.7%), diagnostic imaging (78.8%), diagnostic laboratory tests (36.8%), biopsy and specimen (0.5%), and final diagnosis and next steps (9.6%). Conclusion Our findings demonstrate incomplete understanding of this disorder among OMSs and uncover knowledge deficiencies that can lead to misdiagnosis and/or delay in appropriate treatment. To improve patient outcomes, it is paramount to augment educational initiatives among practitioners regarding this disease.

The aim of this multicentre retrospective cohort study was to describe and categorize the types of ocular and adnexal anomalies seen in patients with craniofacial microsomia (CFM) and to determine their prevalence. In addition, the relationship between the OMENS-Plus and Pruzansky–Kaban classification for each patient and the presence of ocular anomalies was investigated. A total of 881 patients with CFM from four different craniofacial centres were included. Data on ocular anomalies were gathered from the patient charts. Ocular anomalies were present in 33.9% of patients. Four subgroups of ocular and adnexal anomalies were identified. Type I ocular anomalies were present in 22.2%, type II in 19.0%, type III in 18.4%, and type IV in 14.5%. Several potentially preventable and treatable ocular anomalies were identified. Higher OMENS-Plus classification orbit and soft tissue scores and Pruzansky–Kaban classification mandible scores were associated with an increased risk of ocular anomalies. Based on these results and the clinical implications ocular anomalies may have, we underline the importance of targeted ophthalmological screening in CFM. Healthcare professionals should be aware of the possibility of ocular anomalies in these patients, especially during the critical period for visual development.

Background: The etiology and treatment of complex mandibular defects in children differ markedly from those of adults, although treatment with free bone flaps is historical in both groups. While adult outcomes and complication rates are well known, few pediatric data exist, especially for patients with congenital deficiencies. This study reports early and late outcomes from a cohort of young, primarily syndromic patients undergoing microvascular mandibular reconstruction. Methods: This is a retrospective case series of patients who underwent microvascular mandibular reconstruction between 1995 and 2016. Results: Thirteen patients received a total of 13 fibula transfers and 1 medial femoral condyle transfer. Most patients carried a congenital diagnosis (77%), and the average age during surgery was 11.7 ± 5.7 years. The median (interquartile range) [IQR] length of follow-up was 6.3 (5.7) years. There was a 100% flap survival rate, although 86% of all patients experienced at least one complication. Half of all procedures resulted in an early complication. Nine patients (69%) developed late complications, of which temporal mandibular joint ankylosis was the most common (n = 5; 38%). Conclusions: This study is one of few detailing outcomes following mandibular reconstruction by free flap transfer in pediatric patients. These patients were primarily syndromic with appreciable complication rates higher than in other adult and pediatric studies. Some complications are manageable or self-resolving, but others lead to functional problems that may require late operative interventions to correct. Microsurgical treatment should be reserved for children with large, complex mandibular defects when other options are unavailable or have been exhausted.

Non-syndromic orofacial clefts encompass a range of morphological changes affecting the oral cavity and the craniofacial skeleton, of which the genetic and epigenetic etiologic factors remain largely unknown. The objective of this study is to explore the contribution of underlying dentofacial deformities (also known as skeletal malocclusions) in the craniofacial morphology of non-syndromic cleft lip and palate patients (nsCLP). For that purpose, geometric morphometric analysis was performed using full skull cone beam computed tomography (CBCT) images of patients with nsCLP (n = 30), normocephalic controls (n = 60), as well as to sex- and ethnicity- matched patients with an equivalent dentofacial deformity (n = 30). Our outcome measures were shape differences among the groups quantified via principal component analysis and associated principal component loadings, as well as mean shape differences quantified via a Procrustes distance among groups. According to our results, despite the shape differences among all three groups, the nsCLP group shares many morphological similarities in the maxilla and mandible with the dentofacial deformity group. Therefore, the dentoskeletal phenotype in nsCLP could be the result of the cleft and the coexisting dentofacial deformity and not simply the impact of the cleft.

Ocular anomalies may occur in craniofacial microsomia (CFM). The aim of this systematic review was to review the literature on ocular anomalies and their incidence, in order to estimate the need for ophthalmological screening in CFM patients. Online databases were searched, and data on the number of patients, type and incidence of ocular anomalies, and visual acuity were extracted. Four subgroups of ocular and adnexal anomalies were identified, to provide an overview of the different anomalies. Twenty-five papers analysing 1419 patients in total were included. Ocular anomalies were documented in 6.7-100% of patients. The most reported type I ocular anomalies were eyelid coloboma, lipodermoids, and orbital dystopia. The most reported type II ocular anomalies were epibulbar dermoid, microphthalmia, and anophthalmia. Ptosis and strabismus were the most reported type III anomalies, and irregular astigmatism was the most reported type IV ocular anomaly. Visual impairment in general was reported in 8-71.4% of patients, with severe visual impairment in 11.1-71.4% and amblyopia in 16.3%. This study provides a detailed overview of ocular anomalies in CFM and their prevalence. Furthermore, we propose a new classification to organize ocular anomalies into four clinically relevant subtypes. Finally, the high prevalence of ocular anomalies and visual impairment in this study suggests that CFM patients should undergo ophthalmological screening at least once during the sensitive period.

Background: Treacher Collins syndrome (TCS) is a rare craniofacial disorder characterized by bilateral hypoplasia of facial structures and periorbital, ocular, and adnexal anomalies. The purpose of this multicenter study was to report the prevalence of ocular and adnexal anomalies in TCS and to identify patients at risk for visual impairment. Methods: The medical records of patients seen at four craniofacial centers were reviewed retrospectively. The following data were reported: primary and secondary ocular and adnexal anomalies, orthoptic and ophthalmological findings, and severity of TCS based on the facial deformity. Results: A total of 194 patients were included, of whom 49.5% were examined by an ophthalmologist or optometrist. The mean age at the first visual acuity measurement was 6.96 ± 6.83 years (range, 1.50-47.08); at final measurement, 11.55 ± 10.64 years (range, 1.75-62.58). Primary ocular anomalies were reported in 98.5% of cases, secondary anomalies in 34.5%, strabismus in 27.3%, refractive errors in 49.5%, and visual impairment in 4.6%. We found no association between ocular anomalies and visual impairment or between the severity of TCS and ocular anomalies or visual impairment, except for an increased prevalence of secondary ocular anomalies in patients with more severe manifestations of TCS. Conclusions: Ocular anomalies were present in nearly all patients with TCS, even in mild cases.

Craniofacial microsomia (CFM) is characterized by an underdevelopment of the facial structures arising from the first and second branchial arches, but extracraniofacial anomalies such as vertebral anomalies may be present. This retrospective study was performed to determine the prevalence and types of vertebral anomalies and the association with other extracraniofacial anomalies in patients with CFM. The charts of all patients diagnosed with CFM seen in four craniofacial centres were reviewed for the presence of vertebral anomalies, symptoms, extracraniofacial anomalies, and the OMENS classification including the Pruzansky-Kaban type of mandibular deformity. A total of 991 patients were included and 28% of the patients had vertebral anomalies. The most common vertebral anomalies included scoliosis, block vertebrae, and hemivertebrae. Only 44% of the patients with vertebral anomalies had clinical symptoms; torticollis, back or neck pain, and limited neck movement were the most frequently seen. The prevalence of vertebral anomalies was greater in patients with bilateral CFM and in patients with a more severe mandibular deformity, and/or orbit, facial nerve, and/or soft tissue involvement. Patients with vertebral anomalies had significantly more extracraniofacial anomalies than patients without vertebral anomalies. Therefore, patients with vertebral anomalies should undergo cardiac, renal, and neurological evaluation.

Background Deficiency of the median tubercle is a common residual deformity after repair of cleft lip. The purpose of this study was to analyze the change in labial contour after insertion of a dermis-fat graft. Methods Patients with repaired cleft lip who underwent secondary dermis-fat grafting of the median tubercle between 2012 and 2015, along with preoperative and greater than or equal to 3-month postoperative three-dimensional photographs, were included. The photographs were registered to one another and measurements made before and after grafting using anthropometric landmarks. Outcome variables included the following: labial surface area, midline labial height (ls-sto), and lateral labial height (cphi-sto'). Results Twenty-five patients were included: 14 with unilateral and 11 with bilateral repaired cleft lip. Mean age at graft placement was 10.9 ± 3.2 years. Preoperative and postoperative three-dimensional images were taken at 13.6 ± 21.6 weeks before and 34.8 ± 25.1 weeks after the procedure, respectively. There were significant increases in labial surface area (11.5 percent; p = 0.019) and labial height (14.1 to 18.5 percent; p = 0.002) after grafting. Augmentation was greater in bilateral clefts (25.5 percent at midline and 16.8 percent laterally) compared with unilateral clefts (11.6 percent at midline and 12.5 percent on the grafted noncleft side). Intrarater and interrater reliability was excellent for all measurements. Conclusion Dermis-fat grafting significantly increases contour of the median tubercle in patients with repaired cleft lip. Clinical question/level of evidence Therapeutic, IV.

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)2D and 17β-estradiol have synergistic effects on these MSCs, and what mechanism is involved. With IRB approval, we isolated MSCs from discarded excess iliac marrow graft from children undergoing alveolar cleft repair. Plasma was available from 8 female (9.3±0.2years) and 8 male (9.6±0.1years) subjects for hormone assays [25(OH)D, total testosterone, 17β-estradiol, estrone, DHEA-S, Growth Hormone, IGF-I]. RT-PCR was used for gene expression. Alkaline phosphatase (ALP) activity was used to measure osteoblast differentiation at day 7; alizarin red was used to measure matrix mineralization at day 21. All subjects were pre-pubertal based on their hormone levels. Serum 25(OH)D levels ranged from 13.1 to 26.4ng/mL, with 75% below 20ng/mL. Constitutive gene expression of VDR and ERα, β varied from subject to subject with no association with sex or serum chemistries. In osteoblastogenic medium, 1α,25(OH)2D3 (10nM) increased ALP activity by 36% (p

Background: Infants with Robin sequence (RS) may present with airway compromise at delivery. Prenatal diagnosis would improve preparation and postnatal care. The purpose of this study was to devise a predictive algorithm for RS based on fetal magnetic resonance imaging (MRI). Methods: Retrospective case-control study including fetal MRIs from 2002 to 2017. Inclusion criteria were (1) MRI of adequate quality, (2) live-born infant, and (3) postnatal evaluation. Subjects were grouped on the basis of postnatal diagnosis: (1) RS (micrognathia, glossoptosis, airway obstruction), (2) micrognathia without airway obstruction ("micrognathia"), (3) cleft lip and palate ("CLP"), and (4) gestational age-matched controls. A series of possible predictive variables were assessed on MRI. Receiver operator curves were applied to identify cut-off values, and a multivariable algorithm was developed. Results: A total of 162 subjects with mean gestational age at MRI of 25.6 ± 4.9 weeks were included: RS, n = 27 (17%); micrognathia, n = 35 (22%); CLP, n = 46 (28%); control, n = 54 (33%). Three variables were independent predictors of RS: (1) Veau I/II cleft palate (OR = 38.8), (2) tongue shape index (>80%; OR = 8.7), and (3) inferior facial angle (80% and IFA

A retrospective cohort study was initiated to analyse the prevalence, risk factors and treatment modalities of feeding difficulties in patients with craniofacial microsomia. This study included 755 subjects with craniofacial microsomia from three craniofacial centres. Medical charts were reviewed for severity of the deformity, documented feeding difficulties, age at which feeding difficulties first presented and treatment, presence of cleft lip/palate, extracraniofacial anomalies, and obstructive sleep apnoea. In total, 199 patients (26.4%) had documented feeding difficulties. Patients with bilateral involvement, Pruzansky-Kaban III classification, cleft lip/palate, or obstructive sleep apnoea were significantly more at risk for developing feeding difficulties and significantly more often needed additional feeding via a nasogastric tube than patients without these risk factors.

Craniofacial microsomia (CFM) is characterized by underdevelopment of the structures derived from the first and second pharyngeal arches resulting in aesthetic, psychological, and functional problems including feeding and swallowing difficulties. The aim of this study is to gain more insight into swallowing difficulties in patients with CFM. A retrospective study was conducted in the population of patients diagnosed with CFM at three major craniofacial units. Patients with feeding difficulties and those who underwent video fluoroscopic swallow (VFS) studies were included for further analyses. The outcome of the VFS-studies was reviewed with regard to the four phases of swallowing. In our cohort, 13.5% of the 755 patients were diagnosed with swallowing difficulties. The outcome of the VFS-studies of 42 patients showed difficulties in the oral and pharyngeal phases with both thin and thick liquids. Patients with more severe mandibular hypoplasia showed more difficulties to form an appropriate bolus compared to patients who were less severely affected. This is the first study to document swallowing problems in patients with CFM. Difficulties were seen in both the oral and pharyngeal phases. We recommend routine screening for swallowing issues by a speech and language therapist in all patients with CFM and to obtain a VFS-study in patients with a type III mandible. Electronic supplementary material The online version of this article (doi:10.1007/s00455-017-9851-x) contains supplementary material, which is available to authorized users.

Introduction Mandibular reconstruction in craniofacial microsomia (CFM) has been described and reviewed at length although final results are not always (aesthetically) satisfactory due to maxillo-mandibular asymmetry, for which optimal correction techniques remain unclear. The aim of this systematic review is to provide an overview of the surgical options for maxillary correction in patients with unilateral CFM. Material and methods MEDLINE/Pubmed, Embase, Cochrane and Web of Science databases were searched up to April 15, 2017. Inclusion criteria were: studies reporting patients with unilateral CFM (n > 4) who had maxillary correction (with/without simultaneous mandibular correction) with a minimal follow-up of 6 months. The outcome measures included type of treatment (including preceding facial procedures), type and severity of mandibular deformity (by Pruzansky-Kaban system: Types I/IIa/IIb/III), asymmetry analysis method, outcome (i.e. occlusion, canting, stability, esthetic result, facial symmetry), complications and additional treatment needed. Results Nine studies met the inclusion criteria. Analysis showed that Le Fort I + mandibular distraction osteogenesis (LeFort + MDO) and BiMaxillary osteotomy (BiMax) were used for treatment, as single or multiple-stage procedures. All studies reported aesthetic and functional improvement. Conclusion Types I/IIa benefited from LeFort + MDO; Type IIb from LeFort + MDO or BiMax; and Type III from BiMax (with 50% of cases having preceding mandibular procedures, including patient-fitted prosthesis) at a mean age of 20.2 years. Four studies recommended additional (esthetic) procedures.

Odontogenic keratocysts (OKCs) can occur in isolation or as part of nevoid basal cell carcinoma syndrome (NBCCS). Patients with NBCCS are younger at OKC diagnosis than those with nonsyndromic OKC (NS-OKC). The purpose of this study was to determine the prevalence of undiagnosed NBCCS in children who present with an OKC and to assess differences in demographic and presenting features between children with NBCCS and those with NS-OKC.This study is a retrospective case series of children with an OKC presenting to Boston Children's Hospital (Boston, MA) from 2007 through 2018. To be included, patients had to be no older than 18 years and have a newly diagnosed OKC. Patients were excluded if they had previous or recurrent OKC or NBCCS diagnosis. Records were reviewed for age at presentation, gender, number and location of OKCs, treatment, recurrence, family history, and clinical features consistent with NBCCS. Descriptive data were summarized.The sample included 50 patients (27 boys) diagnosed with an OKC at a mean age of 11.7 years (range, 2 to 18 yr); 36% (n = 18) with NBCCS and 64% (n = 32) with NS-OKC. NBCCS diagnosis was made in 8 of 18 patients (44%) because of a family history at presentation, and in 10 patients (56%) the diagnosis was made by genetic testing or documentation of diagnostic criteria. Eight of 18 patients (44%) with undiagnosed NBCCS presented with a single OKC. Patients with NBCCS were significantly younger at presentation (NBCCS, 9.3 yr; NS-OKC, 13.0 yr), had more cysts at time of diagnosis (NBCCS, 1.7; NS-OKC, 1.0; P .05), had more maxillary cysts (NBCCS, 13; NS-OKC, 11), and had a higher recurrence rate (P .05).Given the prevalence of undiagnosed NBCCS in children with OKC, clinicians should have a low threshold for referral for complete examination or genetic testing in children with even a single OKC.

In article number 2100070, Juan M. Melero-Martin, Ruei-Zeng Lin, and co-workers present a biphasic scaffold that allows self-sustained endochondral ossification. Following seeding with human vascular and mesenchymal stem cells, this biomimetic material enables the rapid generation of osteovascular niches and ossification centers, leading to the formation of vascularized bone tissue in vivo.

Craniofacial microsomia (CFM) is characterized by a heterogeneous underdevelopment of the facial structures arising from the first and second branchial arches, but extracraniofacial malformations such as vertebral anomalies also occur. This systematic review provides an overview of the literature on the types and prevalence of vertebral anomalies found in patients with CFM. A systematic search was conducted. Data on the number of patients, patient characteristics, types and prevalence of vertebral anomalies, and other associations between CFM and vertebral anomalies were extracted from the articles identified. Thirty-one articles were included. Seventeen articles described both the prevalence and types of vertebral anomalies in CFM, five articles described solely the types of vertebral anomalies in CFM, and nine articles reported solely the prevalence of vertebral anomalies in CFM. The vertebral anomalies most often reported in CFM are hemivertebrae, block vertebrae, scoliosis/kyphoscoliosis, and spina bifida. These anomalies are mostly present in the cervical and thoracic spine and ribs. The reported prevalence of vertebral anomalies in CFM varies from 8% to 79%. To diagnose vertebral anomalies early in patients with CFM, further research should focus on determining which patients with CFM are at risk of vertebral anomalies.

Objective Patient-specific three-dimensional (3D) models are increasingly used to virtually plan rare surgical procedures, providing opportunity for preoperative preparation, better understanding of individual anatomy, and implant prefabrication. The purpose of this study was to assess the benefit of 3D models related to patient safety, operative time, and cost. Design Retrospective review. Setting Academic, tertiary care hospital. Patients, Participants Midfacial distraction was studied as a representative craniofacial operation. A consecutive series of 29 patients who underwent a single type of midfacial distraction was included. Intervention For a subset of patients, computed tomography-derived 3D models were used to study patient-specific anatomy and precontour hardware. Main Outcome Measures Complications, operative time, blood loss, and estimated cost. Results Twenty patients underwent midfacial distraction without and nine with preoperative use of a 3D model. Seven complications occurred in six patients without model use, including premature consolidation (3), cerebrospinal fluid leak (2), and hardware malfunction (2). No complications were reported in the model group. Controlling for surgeon variation, model use resulted in a 31.3-minute (7.8%) reduction in operative time. Time-based cost savings were estimated to be $1036. Conclusions Three-dimensional models are valuable for preoperative planning and hardware precontouring in craniofacial surgery, with potential positive effects on complications and operative time. Savings related to operative time and complications may offset much of the cost of the model.

BackgroundFacial infiltrating lipomatosis (FIL) is a congenital disorder that causes overgrowth of one side of the face. The purpose of this study was to determine whether PIK3CA mutations are present in tissues outside of the subcutaneous adipose.MethodsFIL tissues from three patients were dissected to enrich for cells from skin, subcutaneous tissue, orbicularis oris muscle, buccal fat, zygomatic bone, and mucosal neuroma. Endothelial cells within the affected tissue also were enriched using CD31 microbeads. Laser capture microdissection on formalin-fixed paraffin-embedded histologic sections was performed to collect specific cell types. DNA was extracted from each tissue and cell type, and measured for the abundance of mutant PIK3CA alleles using droplet digital PCR.ResultsWe detected mutant PIK3CA alleles in every tissue and cell type tested from each overgrown face; frequencies ranged from 1.5 to 53%. There were fewer mutant endothelial cells compared with nonendothelial cells, and the stromal cell compartment had the highest frequency of mutant cells in each tissue.ConclusionsPIK3CA mutations are not restricted to a single tissue or cell type in FIL. Overgrowth in this condition is likely due to the mutation arising in a cell that contributes to several different facial structures during embryogenesis.

Purpose Impacted mandibular second molars can cause caries and root resorption on adjacent molars and can lengthen orthodontic treatment. Surgical uprighting is one management option. The purposes of this study were to 1) evaluate radiographic outcomes for surgical uprighting of impacted mandibular second molars and 2) propose an etiology for impaction. Materials and Methods This was a retrospective cohort study of patients who had surgical uprighting of mandibular second molars and had preoperative and at least 12-month postoperative panoramic images and an age- and gender-matched control group of patients undergoing routine orthodontic treatment. Predictor variables included age, gender, impaction type, preoperative angle of impaction, preoperative periodontal bone level distal to an adjacent first molar, posterior eruption space, pathology, and concomitant extraction of the adjacent third molar. Outcome variables were change in impaction type, postoperative periodontal bone levels around the first and second molars, postoperative tooth angle and posterior eruption space, periapical radiolucency, pulpal obliteration, root resorption, and need for extraction. Descriptive statistics were calculated. Results The sample and control groups each had 16 patients. The mean ages at the first radiographs were 13 ± 1.1 and 13.19 ± 0.61 years for the treatment and control groups, respectively, and the mean follow-up radiographs were obtained 2.4 ± 1.4 and 2.3 ± 0.82 years later. No preoperative images showed pathologic lesions obstructing eruption. Postoperatively, all uprighted molars were Pell and Gregory type IA. The mean change in the angles of the uprighted teeth was 23.5 ± 16.1° (P

Purpose Craniofacial microsomia (CFM) is a congenital malformation of structures derived from the first and second pharyngeal arches leading to underdevelopment of the face. However, besides the craniofacial underdevelopment, extracraniofacial anomalies including cardiac, renal and skeletal malformation have been described. The aim of this study is to analyse a large population of patients with regard to demographics, typical phenotypes including craniofacial and extracraniofacial anomalies, and the correlations between the different variables of this condition. Material and methods A retrospective study was conducted in patients diagnosed with CFM with available clinical and/or radiographic images. All charts were reviewed for information on demographic, radiographic and diagnostic criteria. The presence of cleft lip/palate and extracraniofacial anomalies were noted. Pearson correlation tests and principal component analysis was performed on the phenotypic variables. Results A total of 755 patients were included. The male-to-female ratio and right-to-left ratio were both 1.2:1. A correlation was found among Pruzansky–Kaban, orbit and soft tissue. Similar correlations were found between ear and nerve. There was no strong correlation between phenotype and extracraniofacial anomalies. Nevertheless, extracraniofacial anomalies were more frequently seen than in the ‘normal’ population. Patients with bilateral involvement had a more severe phenotype and a higher incidence of extracraniofacial and cleft lip/palate. Conclusion Outcomes were similar to those of other smaller cohorts. Structures derived from the first pharyngeal arch and the second pharyngeal arch were correlated with degree of severity. Extracraniofacial anomalies were positively correlated with CFM. The findings show that bilaterally affected patients are more severely affected and should be approached more comprehensively.

A retrospective cohort study was set up to analyse the prevalence and treatment of obstructive sleep apnoea (OSA) in relation to the severity of the deformity in patients with craniofacial microsomia (CFM). This study included a population of 755 patients with CFM from three craniofacial centres. Medical charts were reviewed for severity of the deformity, types of breathing difficulty, age at which breathing difficulty first presented, treatment for OSA, and treatment outcome. In total, 133 patients (17.6%) were diagnosed with OSA. Patients with Pruzansky IIB/III classification or bilateral craniofacial microsomia were significantly more often diagnosed with OSA than unilaterally affected patients of Pruzansky I/IIA classification. The initial treatment of OSA consisted of adenotonsillectomy, tracheotomy, or non-invasive positive pressure ventilation. Thirty-seven patients received more than one treatment (range 1-3). In this study, the prevalence of OSA in patients with CFM was higher than the prevalence in the healthy population described in the literature. Although several treatment modalities are available for the treatment of OSA in patients with CFM, treatment should be individualized and based on clinical symptoms, the severity of the deformity, and comorbidities.

Purpose Patients with unilateral cleft lip and palate (UCLP) may require Le Fort I advancement to correct maxillary hypoplasia after reaching skeletal maturity. The underlying cleft anatomy, previous operations, and scarring can affect nostril changes after maxillary advancement. The purpose of the present study was to determine whether Le Fort I advancement affects the nostril configuration (ie, width, axis, shape) in patients with UCLP. The specific aims were to (1) compare cleft and noncleft nostrils in patients with UCLP after maxillary advancement and (2) compare the changes in nostril configuration in patients with UCLP with those in noncleft controls after Le Fort advancement. Patients and Methods A retrospective case-control study of nonsyndromic, skeletally mature patients with UCLP and a case-matched control group without UCLP who had undergone single-piece Le Fort I advancement with alar cinch suture from 2010 to 2014. Patients were included if they had undergone pre- and postoperative 3-dimensional photogrammetry without intervening nasal revision. Three-dimensional anthropometry was used to evaluate changes in nostril axis and width, soft triangle angle, columellar show, and nasal width after orthognathic correction. Results The present study included 19 patients with UCLP (11 males; mean age, 18.0 years) and 19 noncleft controls (11 males; mean age, 18.7 years; P = .276). The mean sagittal advancement in the patients with UCLP and noncleft controls was 7.5 mm and 6.3 mm, respectively (P = .143). On average, the nostrils widened, the soft triangles flattened, and the columellar show increased. No significant difference was found in the changes to the nostril configuration between the cleft and noncleft sides in the patients with UCLP. No significant differences were found in the nostril changes between patients with UCLP and noncleft controls. Conclusions Baseline nostril asymmetry is not altered by Le Fort osteotomy in patients with UCLP because both nostrils respond similarly to the deforming forces of maxillary advancement. Similarly, no differences were found in the nostril changes between the cleft and noncleft controls. These findings can aid proper surgical planning for cleft nasal revisions.

Objective: Measure lower lip thickness and eversion in patients with cleft lip and palate (CLP) and maxillary hypoplasia. The specific aims were to (1) compare lower lip thickness/eversion in patients with CLP to noncleft controls with maxillary hypoplasia, (2) determine differences between patients with unilateral CLP (UCLP) and bilateral CLP (BCLP), and (3) document changes in the lower lip that occur with Le Fort I advancement. Design: Retrospective case–control study. Setting: Tertiary care center. Patients/Participants: Patients with available pre- and postoperative CT scans and 2D lateral photographs who had a Le Fort I advancement between 2009 and 2017. There were 32 patients with CLP (17 females; mean age 17.7 ± 1.9 years) and 33 noncleft controls (21 females; mean age 18.8 ± 2.6 years). Main Outcome Measures: Lower lip thickness and eversion. Results: Patients with CLP and maxillary hypoplasia have a significantly thicker lower lip ( P = .019) and outward rotation of the vermilion border ( P = .003) compared to noncleft controls. The lower lip was significantly thicker in patients with BCLP than in those with UCLP ( P = .035). Lower lip thickness and rotation did not change after maxillary advancement. Conclusions: Patients with CLP and maxillary hypoplasia have a thicker and more everted lower lip than noncleft controls. Patients with BCLP have a significantly thicker lower lip than those with UCLP. Strain of the lower lip musculature appears to be an important contributor to the development of the cleft lip lower lip deformity.