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BACKGROUND Bilateral cleft lip and palate (BCLP) remains a difficult surgical problem due to the severely protruding premaxillary segment, with no consensus of optimal treatment sequence in older patients. A systematic review of the literature was performed to assess the current status of BCLP repair based on age. METHODS A PRISMA systematic review of the PubMed, Web of Science, and Embase databases was performed using a series of search terms related to BCLP. Studies were categorized based on the age of presentation, repair sequence, and technique. RESULTS The database search identified 381 articles. Of these, 72 manuscripts were ultimately included. The lip was repaired first in 1077 patients (86.0%), palate first in 161 patients (12.9%), and simultaneous lip and palate in 14 patients (1.1%). Patients less than 6 months old received lip repair first (n = 959, 98.6%), with complications of unaesthetic appearance (n = 86, 62.3%) and midface retrusion (n = 41, 34.1%) in younger patients and wound dehiscence (n = 8, 40%) in older patients. Primary lip repair was preceded by presurgical orthopedics (n = 760) or lip adhesion (n = 272) to reduce lip tension with nasoalveolar molding (n = 452, 62.9%) or the Latham device (n = 282, 37.1%). In older patients, the palate was repaired first or premaxillary setback (n = 222) was indicated in protruded premaxillae greater than 10 mm, but carried the risk of premaxilla mobility (n = 20, 37.7%) and midface retrusion (n = 10, 18.9%). CONCLUSION In younger patients, lip repair is performed first with preoperative orthopedics or lip adhesion. In older patients, the palate is more commonly repaired first compared with the lip; however, there is no difference in complication rate.

The Le Fort III advancement was first described in 1950 and has since become a key technique in the armamentarium of craniofacial surgeons. The application of distraction osteogenesis to the craniofacial skeleton has allowed for large movements to be performed safely in young patients. This technique is valuable for correcting exorbitism, airway obstruction owing to midface retrusion, and class III malocclusion. It can be performed with either an external distractor or internal distractors. Although serious complications have been reported, these occur rarely when performed by experienced providers.

BACKGROUND Midface retrusion is a common problem in patients with cleft lip and palate owing to the lack of adequate growth of the maxilla. Midface advancement surgery is a definite treatment for midface retrusion; however, the subsequent oropharyngeal airway changes might result in velopharyngeal insufficiency (VPI) in affected patients. Determining the predictors of VPI beforehand and modifying the surgical procedure would be beneficial. MATERIALS AND METHODS A retrospective review of 42 patients with cleft lip and palate who underwent orthognathic surgery from 2013 to 2014 was performed. A total of 25 patients met the inclusion criteria and had undergone complete pre- and postsurgery videofluoroscopy, nasopharyngoscopy (NPS), and speech assessment. We compared 2 groups of patients, those who showed NPS finding changes of more than 0.1 (8 patients) and those without changes (17 patients), by measuring the distances of the contact point to the tip of the velum, gap size at maximum closure (MC) and rest, contact area length, lateral wall closure rate, closure velocity, and 2 angles (from the velum to the hard palate and genu) at MC and rest. The amount of maxillary advancement was also recorded. RESULTS Among the 8 patients with NPS finding changes, 3 underwent Furlow palatoplasty for VPI. No significant difference was found in the amount of maxillary advancement between the groups (5.8 vs 5.7 mm). Significant differences were found in the preoperative gap size at MC and angle at the genu at MC (P = 0.035 and .012). These could be considered as the predictors before surgery. After surgery, a significant difference was found in the contact area, lateral wall closure rate, and gap size at MC (P = 0.005, 0.018, and 0.01). CONCLUSION Videofluoroscopy is a relatively applicable method of determining dynamic changes in the velum function. By performing videofluoroscopy before midface advancement surgery in patients with cleft lip and palate, we may predict the risk of VPI and consider alternative surgical strategies.

BACKGROUND Midface hypoplasia dramatically affects the normative facial cascade. Simultaneous Le Fort III and Le Fort I procedures (Le Fort III/I) provide a powerful tool for achieving significant midface advancement. This study presents the authors' approach for addressing midface hypoplasia in the setting of class III malocclusion using Le Fort III/I advancement. METHODS This was an institutional review board-approved retrospective review of patients who underwent Le Fort III/I advancement at the authors' institution from 2009 to 2019. Demographic, surgical, and postoperative data were recorded. The authors' operative technique and surgical pearls are described. RESULTS Twenty-five patients met inclusion criteria, 15 male patients (60 percent) and 10 female patients (40 percent). Patient age ranged from 14.9 to 21.6 years. Diagnoses included Crouzon syndrome, nonsyndromic developmental skeletal dysplasia, cleft lip/palate, Klippel-Feil syndrome, Apert syndrome, Van den Ende-Gupta syndrome, and Pfeiffer syndrome. Le Fort III advancements averaged 6.18 ± 1.38 mm and Le Fort I advancements averaged 6.70 ± 2.48 mm. Thirteen patients underwent simultaneous bilateral sagittal split osteotomy with average movement of 5.85 ± 1.21 mm. Average follow-up was 1.3 ± 1.0 years. One patient experienced intraoperative cerebrospinal fluid leak that resolved with expectant management. Three patients experienced major complications (12 percent) postoperatively necessitating repeated orthognathic operations. Ten patients experienced minor complications (40 percent). Average length of stay was 10 days, with all patients achieving improvement of their facial profile. CONCLUSIONS The authors' experience reaffirms the relative safety of simultaneous Le Fort III/I advancement. This technique should be considered in select patients with global midface retrusion and class III malocclusion. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

We report the clinical findings of 26 individuals from 16 unrelated families carrying variants in the COL2A1 or COL11A1 genes. Using Sanger and next-generation sequencing, 11 different COL2A1 variants (seven novel), were identified in 13 families (19 affected individuals), all diagnosed with Stickler syndrome (STL) type 1. In nine families, the COL2A1 disease-causing variant arose de novo. Phenotypically, we observed myopia (95%) and retinal detachment (47%), joint hyperflexibility (92%), midface retrusion (84%), cleft palate (53%), and various degrees of hearing impairment (50%). One patient had a splenic artery aneurysm. One affected individual carrying pathogenic variant in COL2A1 showed no ocular signs including no evidence of membranous vitreous anomaly. In three families (seven affected individuals), three novel COL11A1 variants were found. The propositus with a de novo variant showed an ultrarare Marshall/STL overlap. In the second family, the only common clinical sign was postlingual progressive sensorineural hearing impairment (DFNA37). Affected individuals from the third family had typical STL2 signs. The spectrum of disease phenotypes associated with COL2A1 or COL11A1 variants continues to expand and includes typical STL and various bone dysplasias, but also nonsyndromic hearing impairment, isolated myopia with or without retinal detachment, and STL phenotype without clinically detectable ocular pathology.

Goals/purpose Rhinoplasty is the most common procedure seen in the teenage population. Many of these patients have facial imbalance both recognized and unrecognized by the individual and family. Most often it involves chin or midface deficiency. When the discussion involves the possibility of additional surgery, such as an alloplastic chin implant or a surgical osteotomy, the conversation halts because the patient and family seek a simpler solution.Autologous fat transfer is a useful adjunct to achieve facial balance in chin and cheek in the teen population. More recently, we have been using this technique to correct facial disharmony in rhinoplasty patients. Methods/technique Fat grafting was performed at the time of rhinoplasty in 22 patients (age, 15-19 years). Presurgical planning involved cephalometric and computer-enhanced photographic analysis of the face. Midface retrusion and underprojected mentum were treated. Deficient sites were treated with small aliquots of fat, which were injected into the supraperiosteal plane. The average amounts of fat grafting injected per region were 2 to 3 mL for the malar region and 8 to 10 mL for the chin and geniomandibular borders. Results/complications Twenty-two patients underwent augmentation of soft tissue and skeletal deficiencies. Analysis of postoperative results showed excellent outcomes with enhancement of facial profile and proportions. The mean follow-up was 3 years. The majority of patients reported high degree of satisfaction. No complications were observed. Conclusions The combination of rhinoplasty and autologous fat grafting offers very satisfactory esthetic outcomes and improvement of facial balance with minimal added time, cost, and risk.

Background Le Fort III advancement and/or distraction involve osteotomies and dysjunction in the region of the maxillary tuberosity in proximity to the maxillary posterior tooth buds. The purpose of this study was to determine the effect of early conventional Le Fort III advancement and/or distraction on development and eruption of the maxillary posterior permanent molars. Methods A retrospective review of patients diagnosed with syndromic craniosynostosis, who underwent early Le Fort III or early midface distraction and late surgical intervention, was analyzed. Results In the early conventional Le Fort III surgery group, 93 percent of maxillary second molars and 28 percent of maxillary first molars experienced a disturbance in eruption. In the early distraction group, 82 percent of maxillary second molars and 20 percent of maxillary first molars experienced a disturbance in eruption. In the control group, the late conventional Le Fort III and the late distraction groups, only 26 percent of maxillary second molars and none of maxillary first molars experienced a disturbance in eruption. Conclusions A common disruption seen postoperatively in the early Le Fort III and distraction groups was displacement of the second molars. The majority of the displaced tooth buds were located in the maxillary sinus. Overall, the early Le Fort III surgery groups experienced more frequent disturbances for both first and second molars, with the common sequela of displacement in the maxillary sinus, leading to the question of whether presurgical planning in cases of early intervention to address midface retrusion should include extraction/enucleation of the second molar tooth buds. Clinical question/level of evidence Therapeutic, III.

Pfeiffer syndrome is one of the autosomal dominant craniofacial syndromes. Classical clinical manifestations are coronal suture synostosis causing brachycephaly, midface retrusion, airway compromise, broad thumbs, and toes. Pfeiffer syndrome type I (classic type) is associated with FGFR1 mutation. However, wide range of clinical manifestations, with and without craniosynostosis, have been reported. Here, we present a family of Pfeiffer syndrome across 3 generations with identical FGFR1: c.755C>G (p.Pro252Arg) mutation. Where the members of the youngest generation have no cranial involvement. Lastly, we propose a guideline management for familial Pfeiffer syndrome management.

Correction of the skeletal framework is increasingly viewed as the new frontier in facial rejuvenation. The major forces contributing to facial aging include gravity, skeletal remodeling secondary to bone resorption, subcutaneous fat redistribution and loss, hormonal imbalance, chronic solar exposure, and smoking. The regions of the face affected are the following: (a) In the periorbital region, there is a curvilinear distortion of the orbital aperture in the superomedial upper orbit and inferolateral lower orbit curve progressively with age. These bony periorbital age-related changes, particularly of the upper half, will support the clinical appearance of a descending brow, lateral orbital hooding, and in the lower half the descent of the lower lid and its junction with the cheek and deepening of the nasojugal groove. (b) Midface retrusion does occur with aging in dentulous patients. The nose lengthens and the tip droops, with the columella and the lateral crura displacing posteriorly. Bone loss is not uniform, with the greatest resorption occurring in the ascending process of the maxilla. The posterior displacement of the bone rim is greatest at the lower pyriform aperture, which is the critical area for support of the lateral crura and the external nasal valves. Bone loss here contributes also to deepening of the nasolabial fold with age, which previously had been attributed solely to soft tissue laxity and descent. The anterior nasal spine also recedes with aging, and this reduced skeletal support contributes to retraction of the columella, with downward tip rotation and apparent lengthening of the nose with aging .(c) In the lower face, the main change is a reduction in alveolar height secondary to resorption increasing labiomental fold and the appearance of a “witch’s chin.” The depository effect will lead to a change in contour from the typical geometric form of the parabola. A recognized aging feature of the lower mandibular border is the “prejowl sulcus,” the geniomandibular groove, resorption of the bone just below the mental foramen will cause this triangular defect. The decrease in BMD (Bone Mineral Density) of the facial bones with age may also have an impact on facial rejuvenation. The changes in facial aging have become more evident that the most effective approach toward facial rejuvenation should include restoring volume and contour, in addition to reducing the skin envelope. Soft tissue fillers and fat can be used successfully to restore volume loss and, when injected more deeply, result in the restoration of supportive structures and the skeletal foundation. Skeletal augmentation, using implants made of porous polyethylene or silicone, is also an effective method to reverse age-related changes of the facial skeleton in patients with intact occlusion. The type of volume augmentation should be tailored to each patient to rejuvenate the face and minimize potential complications. A better understanding of facial bone strength and support will further our understanding of facial aging and the future possibilities for facial rejuvenation.

Background Facial advancement represents the essence of the surgical treatment of syndromic craniosynostosis. Frontofacial monobloc distraction is an effective surgical approach to correct midface retrusion although someone consider it very hazardous procedure. The authors evaluated a group of patients who underwent frontofacial monobloc distraction with the aim to identify the advancement results performed in immature skeletal regarding the midface morphologic characteristics and its effects on growth. Methods Sixteen patients who underwent frontofacial monobloc distraction with pre- and postsurgical computed tomography (CT) scans were evaluated and compared to a control group of 9 nonsyndromic children with CT scans at 1-year intervals during craniofacial growth. Three-dimensional measurements and superimposition of the CT scans were used to evaluate midface morphologic features and longitudinal changes during the craniofacial growth and following the advancement. Presurgical growth was evaluated in 4 patients and postsurgical growth was evaluated in 9 patients. Results Syndromic maxillary width and length were reduced and the most obtuse facial angles showed a lack in forward projection of the central portion in these patients. Three-dimensional distances and images superimposition demonstrated the age did not influence the course of abnormal midface growth. Conclusion The syndromic midface is hypoplastic and the sagittal deficiency is associated to axial facial concavity. The advancement performed in mixed dentition stages allowed the normalization of facial position comparable to nonsyndromic group. However, the procedure was not able to change the abnormal midface architecture and craniofacial growth.

Purpose Subcranial midface distraction is used to treat central midface deficiency in syndromic synostosis. Our aim was to determine which maxillary movements were associated with improvement in measures of obstructive sleep apnea. Methods This was a retrospective cohort study that reviewed patients with syndromic midface retrusion and documented sleep apnea who underwent subcranial midface distraction via either Le Fort 3 osteotomy or Le Fort 2 osteotomy with zygomatic repositioning. The predictor variables measured on cephalograms were the magnitude and direction of midface and mandibular movements. The primary outcome was the change in the apnea hypopnea index (AHI) from polysomnography before and after surgery. The secondary outcomes were volumes of upper airway containing bone spaces calculated from computed tomography scans. Data analysis included linear regression to estimate the effect of distraction vectors on bone space volumes and AHI changes. Results We included 18 patients primarily with Crouzon or Apert syndrome. The magnitude of distraction in a horizontal direction was the most significant factor for AHI improvement and primarily expanded the nasopharyngeal space, but with a smaller impact on the oral cavity space. Clockwise palate rotation was most influenced by a downward direction of distraction, with 24° below horizontal creating a neutral advancement. The greater the magnitude of advancement, the more likely a counterclockwise rotation was observed. Conclusions Horizontal magnitude of advancement had the greatest impact on AHI improvement. Vertical lengthening and closure of anterior open bite deformities can be done without compromising airway results as long as total advancement is not compromised. Palate rotation is best controlled by a downward distraction vector, but counterclockwise rotation increases with greater advancement.

Keutel syndrome (KS) is an extremely rare autosomal recessive cartilage tissue disorder. We report a patient with morphological signs of KS. Case. A 14-month-old Moroccan male was referred to our center, he suffered from persistent stridor from birth and dysmorphic features. Family background: Consanguineous, healthy parents. Personal background: Term newborn (weight 3,500gr). Respiratory distress that required resuscitation and oxygen therapy for 48h. The parents noticed persistent progressive stridor since he was one-month old, increasing during respiratory infections and crying. Weight: 11.36 kg (P87), Height: 73 cm (P3). Vital signs and oxygen saturation: normal. Biphasic stridor (inspiratory predominance). Face: broad and depressed nasal bridge, low-hanging columella, short philtrum, underdeveloped alae nasi, midface retrusion. Normal cardiorespiratory auscultation. Limbs: brachytelephalangism of both hands. Normal cardiologic examination. Skeletal x-ray: 2-5o brachythephalanges of both hands and distal phalanx of the first finger with two ossification nuclei. 2 calcifications visualized in the external ears. Chest x-ray: Calcifications of tracheal, bronchial and thyroid cartilages. Bronchoscopy: Generalized tracheal stenosis mainly in the middle third (decrease in the transverse diameter). No calcifications were observed. Thorax TC: Decreased tracheal size. Diffuse calcifications in trachea and both main bronchi. Normal mediastinal vessels. With a suspected diagnosis of KS, we are waiting for the results of genetic test. Conclusion: KS is rare, however the presence of tracheobronchial calcifications in combination with short, broad distal phalages and midface hypoplasia, should suggest this disorder. Data on long term outcomes are limited

A cleft lip, palate, and alveolus is one of the most common birth defects. Depending on the type of cleft, multiple surgeries may be required throughout the growth stage. Traditionally, an orthodontic-surgical approach has been adopted to treat maxillary retrusion. Osteodistraction it is the surgical choice in patients with severe midface retrusion that require maxillary advancement. Our objective is to present our experience using this orthodontic and surgical approach.

Crouzon syndrome is a rare form of syndromic craniosynostosis (SC) characterized by premature fusion of the cranial and facial sutures, elevated intracranial pressure, varying degrees of ocular exposure due to exorbitism, and airway compromise caused by midface retrusion. Craniolacunae and upper and lower extremity anomalies are not frequently found in Crouzon syndrome. We present a girl with Crouzon syndrome caused by c.1040 C > G, p.Ser347Cys, a pathogenic mutation in the FGFR2 gene with atypical characteristics, including craniolacunae resembling severe Swiss cheese type of bone formation, and upper and lower extremity anomalies which are more commonly associated with Pfeiffer syndrome patients. Distinguishing between severe Crouzon syndrome patients and patients who have mild and/or moderate Pfeiffer syndrome can be challenging even for an experienced craniofacial surgeon. An accurate genotype diagnosis is essential to distinguishing between these syndromes, as it provides predictors for neurosurgical complications and facilitates appropriate family counseling related to long-term outcomes.

Cleft lip and palate is the most common congenital craniofacial anomaly. Up to 60% of these patients will benefit from cleft orthognathic surgery, which consists primarily of maxillary advancement and mandibular setback to address midface retrusion and relative mandibular protrusion, respectively. It is believed that maxillary advancement can enlarge the airway whilst mandibular setback can reduce the airway, but this has not previously been quantified for cleft patients undergoing orthognathic surgery. This unique longitudinal prospective study of 18 patients was conducted between April 2013 and July 2016. No significant changes occurred by six months postoperatively in body mass index, apnoea-hypopnoea index or lowest oxygen saturation (LSAT). There was a mean increase of 0.73 cm3 in velopharyngeal volume, a mean decrease of 0.79 cm3 in oropharyngeal volume, an improvement in snoring index, and no statistically significant change in hypopharyngeal volume. In conclusion, cleft orthognathic surgery that produced anterior advancement of the maxilla, setback of the mandible and clockwise rotation of the maxillo-mandibular complex resulted in increased velopharyngeal, decreased oropharyngeal and unchanged hypopharyngeal airways, and improved snoring, but did not significantly alter objective sleep-related breathing function.

Purpose Meeting patient desires for enhanced facial esthetics requires that providers have standardized and objective methods to measure esthetics. The authors evaluated the effects of objective 3-dimensional (3D) facial shape and asymmetry measurements derived from 3D facial images on perceptions of facial attractiveness. Materials and Methods The 3D facial images of 313 adults in Iowa were digitized with 32 landmarks, and objective 3D facial measurements capturing symmetric and asymmetric components of shape variation, centroid size, and fluctuating asymmetry were obtained from the 3D coordinate data using geo-morphometric analyses. Frontal and profile images of study participants were rated for facial attractiveness by 10 volunteers (5 women and 5 men) on a 5-point Likert scale and a visual analog scale. Multivariate regression was used to identify the effects of the objective 3D facial measurements on attractiveness ratings. Results Several objective 3D facial measurements had marked effects on attractiveness ratings. Shorter facial heights with protrusive chins, midface retrusion, faces with protrusive noses and thin lips, flat mandibular planes with deep labiomental folds, any cants of the lip commissures and floor of the nose, larger faces overall, and increased fluctuating asymmetry were rated as significantly (P Conclusion Perceptions of facial attractiveness can be explained by specific 3D measurements of facial shapes and fluctuating asymmetry, which have important implications for clinical practice and research.

Crouzon syndrome is a rare genetic disorder with autosomal-dominant inheritance that shows a triad of hallmark characteristics: craniosynostosis, exophthalmos, and midface retrusion. General treatment protocol for patients with Crouzon syndrome has already been established, but there is no standard treatment strategy for adult patients with Crouzonoid appearance. The authors present clinical patients of 1-stage orthognathic and face contouring surgery to achieve functional and aesthetic improvement.One-stage surgery was performed in adult patients with Crouzonoid appearance without a history of facial surgery. Orthognathic 2-jaw surgery was first performed to treat class III malocclusion. Face contouring surgery was conducted next to improve the aesthetic appearance using midface augmentation with onlay rib bone graft, advancement genioplasty, augmentation rhinoplasty, and ancillary procedures such as neck lift, fat graft, or fillers.Five patients (2 men, 3 women) were enrolled in this study. The mean age at the time of surgery was 19.0 years. No specific surgery-related complications such as infection, nerve injury, or recurred malocclusion were observed during the 3.5 years of follow-up. Normal class I occlusion was reached in all patients. The frontonasal angle, nasolabial angle, and labiomental angle improved from 125.1° to 135.1° (P 0.05), 60.9° to 86.3° (P = 0.018), and 146.3° to 125.0° (P = 0.018), respectively. The Global Aesthetic Improvement Scale score was 4.75 and 5.00 in frontal and profile views, respectively.One-stage orthognathic and face contouring surgery may be an effective surgical option with good postoperative functional and aesthetic improvement in adult patients with Crouzonoid appearance.

Correction of secondary deformities of the midface can happen even for the most experienced surgeons due to inadequate reduction. This chapter categorizes the correction of secondary defects based on the initial fracture patterns and deformity of midface caused by medial canthal deformity, orbitozygomaticomaxillary complex (OZMC) fractures and Le Fort fractures. The secondary correction of the central midface will be covered briefly in the context of Le Fort II and III fractures. Medial canthal correction can be done by osteotomy or epicanthoplasty techniques. Complications of OZMC fractures include poor 3-dimensional reduction resulting in residual cosmetic deformities and an increase in orbital volume with subsequent enophthalmos or dystopia. This can be corrected by onlay of bone or other materials if there are no associated ocular findings and reconstruction of the internal orbit. OZMC osteotomy is the optimal technique to address orbital volume, dystopia and projection, and width of the face. The key fit of the bony fractures at the zygomaticofrontal sutures, orbital rim, and lateral buttress of the maxilla are lost in delayed repair due to bone remodeling. Hence techniques such as intraoperative navigation, imaging, preoperative surgical cutting, and repositioning guides are of value. The most common maxillary defects encountered after Le Fort fractures are midface retrusion, decreased midfacial height, and anterior open-bite. Patients with Le Fort II or III fractures would benefit from reestablishing the old fractures and mobilization to help correct the orbital rim position and the malocclusion at the same time. Often recreation of the Le Fort II or III fractures may not help obtain the desired occlusion due to remodeling and associated soft tissue changes. In such situations, the Le Fort I osteotomy should be completed at the same time. The technique is similar to that of the Le Fort I osteotomy performed in orthognathic surgery, utilizing dental models and surgical splints.

OBJECTIVE Midface retrusion is a concern in patients with complete unilateral cleft lip and palate (CUCLP). Maxillary segment alignment may influence the prognosis after corrective surgery. This study assessed the association between maxillary segment alignment and interarch relationship observed in the early mixed dentition. The null hypothesis was that there is no relation between the initial alignment of cleft segments and the width of the cleft in the infant, and the early mixed dentition interarch relationship. SETTING AND SAMPLE POPULATION The sample consists of 352 children with CUCLP treated at the Hospital for Rehabilitation of Craniofacial Anomalies/USP. MATERIAL AND METHODS Dental models were obtained before lip repair at three months and at the age of six. Based on the first, the sample was then divided into two groups: anteroposterior aligned and anteroposterior misaligned maxillary segments. They were also divided into 4 groups according to the cleft width (Narrow, Medium, Wide and Very Wide). Interarch relationship was graded using the 5-year-old index. The statistical association was evaluated using the chi-squared test (P

INTRODUCTION Maxillary hypoplasia after cleft lip and palate (CLP) repair can result in significant functional and aesthetic impairments. Le Fort I osteotomy & advancement and Le Fort I distraction osteogenesis are standard treatment options for individuals with CLP-associated midface retrusion. However, both of these modalities continue to be associated with a high relapse rate. This study describes surgical outcomes of a 2-stage technique utilizing distraction osteogenesis combined with bone grafting and rigid fixation, which may optimize skeletal stability by reducing relapse. METHODS A retrospective review of CLP patients with severe maxillary hypoplasia evaluated by a single surgeon from 2003 to 2014 was performed. Twenty-one subjects were identified that underwent maxillary advancement via a 2-stage technique: (1) Le Fort I external rigid distraction using a HALO device, followed by (2) autologous iliac crest bone graft application and plate-fixation. Post-operative cephalograms were taken on average 1-year following surgery. RESULTS Twelve subjects met the inclusion/exclusion criteria. A distraction rate of 1 mm/day was achieved with an average of 14 mm of maxillary advancement. Average increase in SNA was +9.03°, with an increase from 71.84° to 80.88° (normal = 82.0°, P value

Patients with cleft lip and palate could develop dentofacial deformity characterized by malocclusion, midface retrusion, midline discrepancy and asymmetry. Cleft orthognathic surgery has evolved from the simple maxillary LeFort I advancement with correction of dental malocclusion to the current model of patient-centered approach focusing on skeletofacial reconstruction using computer-assisted diagnosis and planning. Three-dimensional imaging and surgical simulation have provided valuable information for facial aesthetics and surgical feasibility. Surgery-first approach and two-jaw orthognathic surgery have gradually become prevalent replacing the conventional method. A better dentofacial outcome is achieved with reduction of the burden of care.

Introduction: The management of syndromic and non-syndromic craniosynostosis is controversial with different units performing various procedures at different points in time and for different reasons. This study was performed to compare protocols of assessment and surgery in craniofacial units in Australasia. Methods: All paediatric craniofacial surgery centres in Australia and New Zealand were surveyed with respect to their current protocols for the preoperative assessment, intraoperative management and postoperative follow-up of syndromic and non-syndromic craniosynostosis patients. Results: All eight centres completed the survey with 100 per cent response rate. There was considerable homogeneity in the management of the various synostoses. Differences in management were notable in the preoperative assessments that were offered and the management of midface retrusion in syndromic craniosynostoses. Conclusion: Single-suture management among surveyed centres was relatively homogenous. Developmental assessment is not routinely performed for single-suture disease but is desired by the majority of surveyed centres. Postoperative CT scans are not commonly used but a number of centres desired routine use of 3D photometry for outcome assessment. In multi-suture cases, expansion of the posterior vault was performed in all cases but management of midface retrusion was variable.

Introduction Midface retrusion creates a size deficiency problem in the upper airway that has been improved in children using surgical midface advancement and orthopedic protraction of the maxilla. The results of these treatments have been mostly promising at enlarging the pharyngeal airway. Recently introduced bone anchored maxillary protraction (BAMP) uses implant inserted devices in the jaws to pull the maxilla forward against a backward pressure to the lower jaw. This is a pilot study that examines the use of BAMP as a strategy to treat maxillary retrusion, malocclusion and children with obstructive sleep apnea. Methods 15 children, ages 9–16 years with maxillary retrusion creating a skeletal malocclusion were treated with bone anchored maxillary protraction (BAMP) and the results were compared against an untreated control group. 8 children in the treatment group also had sleep disordered breathing/obstructive sleep apnea. All subjects had lateral cephalograms before and after BAMP therapy. The OSA cohort completed the pediatric sleep questionnaire (PSQ) and polysomnography prior to and at the end of BAMP. Results The majority of the OSA children (n = 5) showed improvement in their apnea-hypopnea index (AHI) and OSA symptoms after BAMP. Preliminary results of BAMP therapy show improvement in respiratory and airway parameters in OSA children with a highly significant change in the forward position of the upper jaw and enlargement in the nasopharyngeal to oropharyngeal junction as compared to an age and sex matched untreated control group. The outcomes were dependent on the age of treatment initiation and patient compliance. Conclusions This preliminary work suggests that bone anchored maxillary protraction may be considered as an adjunctive treatment option in adolescents for improving midface retrusion and sleep apnea, but further work is needed to explore this therapy.

Introduction LeFort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion. This study investigates long-term changes in patients undergoing distraction as children, and compares outcomes to an unaffected, untreated control. Methods Fifteen patients (9 males, 6 females) with syndromic craniosynostosis treated by LeFort III distraction at an average age of 4.9 ± 1.5 years were identified. Lateral cephalograms at predistraction, immediate, 1-, 5-, and 10-year postdistraction were superimposed using the best-fit of cranial base details. An untreated, unaffected matched control was obtained from the American Association of Orthodontists Foundation Legacy Collection. Differences in landmark location and cephalometric relationships were assessed between time points and between treatment and control groups. Results LeFort III distraction produced an average advancement of 14.86 ± 5.14 mm at A-point and 10.54 ± 3.78 mm at orbitale. This advancement produced overcorrection of anteroposterior occlusal relationships and phenotypic correction of midface position. Surgical stability over a 10-year follow-up was excellent. Posttreatment growth was characterized by absent anteroposterior maxillary growth, preservation of dentoalveolar development and maxillary remodeling, and delayed mandibular growth. Subsequent growth resulted in a long-term phenotypic relapse of pretreatment Class III maxillomandibular relationship and negative overjet. Conclusions LeFort III distraction osteogenesis produces stable advancement of the midface. Overcorrection is required for long-term phenotypic stability because of deficient postdistraction sagittal midface growth. Late mandibular growth contributes to underestimation of the amount of distraction required to produce long-term phenotypic correction.

Le Fort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion and proptosis. This study assesses the stability of proptosis correction over 10-years.A retrospective review identified 15 patients with syndromic craniosynostosis treated by Le Fort III distraction prior to age 10 (9 males, 6 females; age 4.9 ± 1.5 years). Untreated, non-craniosynostotic age- and gender-matched controls were obtained from historical growth records. Lateral cephalometric tracings at pre-surgery (T1), immediate (T2), 1 year (T3), 5 years (T4), and 10 years (T5) (n = 11) post-distraction were superimposed using the best-fit of cranial base. Proptosis severity was defined as the horizontal distance between the Ant. Globe cephalometric point and orbital rim landmarks Orbitale and Lat. Orbit.The orbital rim advanced 10.54 ± 3.78 mm (P 0.001) at Orbitale and 9.73 ± 4.54 mm (P 0.001) at Lat. Orbit from T1 to T2; Ant. Globe advanced 3.13 ± 3.02 mm (p 0.001). Proptosis decreased 7.41 ± 5.29 mm (P .001) from Orbitale and 6.60 ± 6.50 mm (p 0.002) from Lat. Orbit. Comparison to controls demonstrated phenotypic correction. In craniosynostotic patients from T2 to T5, the bony orbital rim demonstrated non-significant remodeling posteriorly and inferiorly. Anterior Globe moved 3.79 ± 1.47 mm anteriorly (P .001), which did not differ significantly from controls. Proptosis increased by 4.18 ± 2.94 mm in craniosynostotic patients from T2 to T5.Le Fort III distraction was stable, with no significant anteroposterior relapse of the maxilla or bony orbit. Phenotypic relapse of proptosis to pre-treatment levels occurred through deficient growth of the midface, surface resorption at the orbital rim, and preservation of normal forward movement of Ant. Globe.

Less than one hundred cases of the acrofacial dysostosis, Nager syndrome, have been described. The cardinal features of Nager syndrome are micrognathia, midface retrusion and limb malformations, predominately of the radial ray of upper extremities. Within the past three years haploinsufficiency of SF3B4 has been confirmed as the major cause of Nager syndrome. Different loss-of-function point-mutations in SF3B4 have been found in approximately 2/3 of patients diagnosed with Nager syndrome. Whole gene deletions of SF3B4 have also been suggested to be the cause of Nager syndrome in SF3B4 point mutation negative patients. Only four prenatal cases displaying Nager-like features in the 2nd or 3rd trimester which have been genetically confirmed with SF3B4 point-mutation after birth have been described. We report a case of a 12-week-old fetus with micrognathia, malformed wrists, bilateral club foot and short long bones diagnosed prenatally by chromosomal microarray with a de novo 0.4 Mb deletion at chromosome 1q21.2 involving SF3B4. To our knowledge, this is the first report of Nager syndrome caused by a SF3B4 whole gene deletion. The case presented also shows that high-resolution chromosomal microarray in early pregnancy can confirm Nager syndrome caused by SF3B4-deletion prenatally.

Congenital Hypopituitarism (CH) has traditionally been associated with specific facial phenotypes subsumed under the term midface retrusion, based on cephalometric studies. In this study, we used a systematic anthropometric approach to facial morphology in 37 individuals with CH and their parents, primarily of French Canadian ancestry, and compared them to a control group of 78 French Canadian patients with well-controlled type 1 diabetes and their parents. We were able to demonstrate clear morphological differences, which were more prevalent in the affected group than in the control group. More specifically, we showed the presence of a shorter skull base width (P

Achondroplasia is the most common form of inherited disproportionate short stature. Affected individuals have short arms and legs, a large head, and characteristic facial features with frontal bossing and midface retrusion, formerly known as midface hypoplasia. Mode of inheritance of achondroplasia is autosomal dominant. Two mutations in FGFR3, G380R and G375C are known to cause achondroplasia. The G380R mutation accounts for 98% of the achondroplasia cases. Here we review the ultrasound, radiographic and clinical features, genetic aspects and molecular pathogenesis of the most common form of dwarfism in humans, providing a model of genetic counseling given to a family with an antenatal diagnosis of a fetal genetic disorder, due to a novel mutation, as a part of the feto-maternal diagnostic strategy.

INTRODUCTION LeFort III midface advancement using the distraction technique became the standard method for the correction of various craniofacial syndromes accompanied by the midface retrusion. Midface hypoplasia is known to be related to the imbalance in facial aesthetics, malocclusion, and the airway problem. This study aimed to evaluate the change in airway volume after performing a LeFort III midface advancement with the distraction techniques. PATIENTS AND METHODS Between April 2008 and February 2013, 7 patients aged 5 to 7 years underwent standard LeFort III osteotomy, followed by distraction with a rigid external distractor with or without internal distractor. The degree of advancement of the midface and the airway volume were evaluated with 3-dimensional computed tomography scans and the 3-dimensional software. RESULTS The average latent period was 3.57 days. The average manual distraction distance was 17.55 mm for 3.82 weeks. The consolidation period ranged from 3 to 7 months. Changes between the preoperative and postoperative airway volumes were remarkable. The average preoperative postpharyngeal airway volume was 5649.33 mm compared with an average postoperative airway volume of 7403.44 mm. Therefore, the average postpharyngeal airway space increased by a remarkable 32.78%. CONCLUSIONS This investigation revealed that the LeFort III midface advancement using distraction could increase postpharyngeal airway space by approximately 33% when the midface is advanced by approximately 18 mm. This method could be used as a future reference for LeFort III midface advancement with distraction.

Evidence exists for the presence of a specific facial phenotype in nonaffected first-degree relatives of persons with CL/P. An increased risk for olfactory dysfunction has also been reported in CL/P-relatives. These phenotypic features can probably be explained via the presence of CL/P-related susceptibility genes. We aimed at confirming the occurrence of these endophenotypic traits in first-degree CL/P-relatives, and we investigated the link between the facial phenotype and the smell capacity in this group. We studied the facial morphology of 88 nonaffected first-degree relatives of patients with CL/P and 33 control subjects without family history of facial clefting by 3D surface imaging and a spatially dense analysis of the images. Smell testing was performed in 30 relatives and compared with 23 control subjects. Nonaffected relatives showed midface retrusion, hypertelorism, and olfactory dysfunction, compared to controls. In addition, we show for the first time that olfactory dysfunction in relatives is correlated to a smaller upper nasal region. This might be explained by a smaller central olfactory system. The different facial morphology in the relatives with olfactory impairment as compared to the total group may be an illustration of the contribution of different genetic backgrounds to the occurrence of CL/P via different biological pathways. Roosenboom J., Saey I., Peeters H., Devriendt K., Claes P., Hens G., ''Facial characteristics and olfactory dysfunction: two endophenotypes related to nonsyndromic cleft lip and/or palate'', Biomed research international, vol. 2015, Article ID 863429, 8 pp., 2015. ispartof: BioMed Research International vol:2015 pages:1-8 ispartof: location:United States status: published

In this review, we aimed to depict the clinical, radiological, and genetic features of cleidocranial dysplasia (CCD) and to suggest management guidelines, based on our experience of 8 cases, with an emphasis given to dental complications.The most common craniofacial features of CCD that stand out are a patency of the anterior fontanelle, an inverted pear-shaped calvaria, a hypertelorism, a general midface retrusion, and a mandible prognathism, associated with an excessive mobility of the shoulders, a short stature, and teeth abnormalities such as supernumerary teeth and failure of eruption, in particular. RUNX2 is the only gene in which mutation is known to cause CCD, but mutations are detected in only 65% of all patients with a clinical diagnosis of CCD. Panoramic radiography is a valuable adjunct in confirming the diagnosis of CCD.Our experience allowed us to conclude that orthodontically aided eruption should always be attempted. However, to stabilize the occlusion and to improve facial esthetics, we recommend associated orthognathic surgery. When orthodontic treatment is partially efficient, prosthetic treatment options bring satisfactory results, in terms of occlusion. Nevertheless, when orthodontic treatment fails, we recommend to preserve as many native teeth as possible, and to combine orthognathic preprosthetic surgery and implant-supported prosthesis.In any case, an individualized treatment protocol, depending on the needs and demand of the patient, the age at diagnosis and social and economic circumstances, should be put forward.

Background Gomez-Lopez-Hernandez syndrome is a rare genetic disease characterized by scalp alopecia with trigeminal anesthesia, brachycephaly or turribrachycephaly, midface retrusion, and rhombencephalosynapsis. We report the second case with this condition who presented with consanguineous parents. Patient This boy was evaluated shortly after birth because of suspected craniosynostosis. He was the only son of healthy, consanguineous parents (his maternal grandmother and his paternal great-grandfather were siblings). His examination was notable for turribrachycephaly, prominent forehead, bilateral parietotemporal alopecia, midfacial retrusion, anteverted nostrils, micrognathia, low-set and posteriorly rotated ears, and short neck with redundant skin. Radiographs and tridimensional computed tomography scan of skull revealed lambdoid craniosynostosis. Brain magnetic resonance imaging revealed complete rhombencephalosynapsis, aqueductal stenosis, fused colliculi, abnormal superior cerebellar penducle, mild ventriculomegaly, and dysgenesis of the corpus callosum. Conclusions Since its first description, 34 patients with this condition have been reported. The etiology of Gomez-Lopez-Hernandez syndrome is unknown. However, it is noteworthy that the patient in this report presented with a family history of consanguinity because this finding reinforces the possibility of an autosomal-recessive inheritance for this condition.

Gomez–Lopez-Hernandez syndrome (GLHS) is a rare neurocutaneous syndrome characterized by the triad of rhombencephalosynapsis, trigeminal anesthesia, and bilateral parieto-occipital alopecia. We herein describe the first Japanese patient with GLHS characterized by the standard triad with typical craniofacial anomaly including hypertelorism, brachyturricephaly and midface retrusion, and a short stature. This female patient had also exhibited fever-induced convulsive seizures and psychomotor developmental delay since infancy. Brain magnetic resonance imaging showed severe rhombencephalosynapsis, supratentorial abnormalities (aplasia of the septum pellucidum, severe ventricular enlargement, and hypoplasia of the corpus callosum), and hippocampus atrophy. Bilateral ectopic cerebellums were also observed. This report describes the long-term clinical outcome of GLHS and a new neuroradiological finding regarding rhombencephalosynapsis.

Rhombencephalosynapsis (RES) is a rare hindbrain malformation that could occur in isolation or as a part of a syndrome for example, Gomez-Lopez-Hernandez syndrome (GLH) or VACTERL-H. We identified male patient with severe RES. Ventriculomegaly, agenesis of septum pellucidum, very thin corpus callosum with interhemispheric cyst were additional neuroimaging findings. He had brachyturricephaly, midface retrusion, low-set posteriorly rotated ears and bilateral parietal well circumscribed areas of alopecia. No corneal anesthesia was observed; thus, demonstrating many of the diagnostic criteria of GLH. Interestingly, he additionally had bilateral bipartite parietal bone (BPB) that is an extremely rare anomaly of the parietal sutures. This is the first co-occurrence of this rare anomaly with GLH. We believe the presence of this unique finding could represent an important clue for understanding the pathogenesis of this malformation. © 2013 Wiley Periodicals, Inc.

Pycnodysostosis is a rare autosomal recessive skeletal disorder involving a constellation of craniofacial manifestations including midface retrusion. We report the case of a 13-year-old girl with pycnodysostosis who presented with exorbitism, midface retrusion, malocclusion, and obstructive sleep apnea. Here, we de- scribe the successful use of subcranial Le Fort III advancement using distraction osteogenesis with internal Kawamoto distracters. After a latency of 5 days, distraction for 10 days, and consolidation for 12 weeks, her midface was advanced by 10 mm with slight over- correction at the occlusion level. At 2 years postoperatively, the patient had complete remission of her sleep apnea, resolution of her exorbitism, and amelioration of her class III malocclusion to class I. To the best of our knowledge, this is the first report of a successful subcranial Le Fort III midface advancement with distraction osteo- genesis for craniofacial reconstruction of a pycnodysostosis. Our report highlights the surgical options that have been described for this craniofacial deformity and presents a novel and expedient ap- proach for patients with pycnodysostosis presenting with exorbitism, midface retrusion, and/or sleep apnea.

Porous polyethylene (PPE) implants are biocompatible alloplastic materials commonly used for facial augmentation. However, the effect of sub-periosteal PPE application on the surrounding tissues has not been analyzed clearly. This report documents the case of a 22-year-old woman who underwent peri-alar augmentation with PPE to improve midface retrusion. Although no infection or inflammation occurred at the surgical site, the patient requested removal of the PPE implant for aesthetic reasons alone at 1 year after the surgery. The removed implant was subjected to histological and morphological evaluation using conventional histological staining and scanning electron microscopy (SEM). Histopathological staining revealed bone ingrowth into the pores of the implant near the boundary with the host bone. Little evidence of a foreign body reaction was observed. SEM revealed densely arranged collagen fibres and osteoblastic cells in the pores. Moreover, the outer surface of the PPE implant in contact with the periosteum showed fibrous tissue ingrowth, leading to tissue adhesion. These findings confirm bone ingrowth into the PPE pore structure in humans.

This report describes a case of successful orthodontic treatment using maxillary anterior segmental distraction osteogenesis with an internal maxillary distractor and bilateral sagittal split ramus osteotomy in a girl with cleft lip and palate. A 16-year-old girl with unilateral cleft lip and palate exhibited midface retrusion because of growth inhibition of the maxillary complex and mandibular excess. After the presurgical orthodontic treatment, 6.0-mm advancement of the maxillary anterior segment and 4.0-mm set back of the mandible were performed. After a retention period, the patient's midface convexity was greatly improved and the velopharyngeal competence was preserved without relapse.

BACKGROUND In traditional Le Fort III distraction, the transport segment is advanced en bloc without addressing differences in the relative retrusion of the facial structure. The authors describe three methods for correcting these asymmetries with differential facial advancement. METHODS Eight patients (age range, 4 to 20 years) with asymmetric facial hypoplasia were treated by Le Fort III midface distraction using an external device. Two patients with nasal bone hypoplasia exceeding midface retrusion were treated with nasal passenger grafts at the time of osteotomy. Two patients with asymmetric rotational midface deformities underwent wire cerclage swing advancement of the affected side to achieve differential advancement. Four patients with central nasomaxillary retrusion exceeding zygomatic retrusion underwent segmental Le Fort III osteotomy with simultaneous zygoma repositioning and Le Fort II distraction. RESULTS Differential midface advancement was achieved in all patients. Midface distraction and nasal passenger grafts resolved obstructive sleep apnea, improved globe protection, and improved fit of prescription glasses. After Le Fort III swing advancement, the centric relation and malar asymmetry were corrected with differential advances of 10 and 15 mm compared with the unaffected side. In the segmental osteotomy Le Fort III group, the central face was distracted independently of the zygoma repositioning, thus correcting the shortened retruded central midface without distorting the orbitomalar relationship and improving airway obstruction, anterior open bite, short nose, and proptosis. CONCLUSIONS Midface distraction techniques have evolved to include the principles of segmentation, graft augmentation, and controlled rotation. The benefits of gradual distraction can be realized without compromising the aesthetic and functional result.

The functional matrix theory was first introduced in the orthodontic literature by Melvin Moss half century ago. Since its original introduction, several attempts have been made to test the validity of this theory with mixed results. In this paper, we present evidence generated using transgenic mice to test the role of neuronal function in craniofacial development. Our data confirmed previous observations that midface retrusion accompanied neuronal dysfunction in the HexB −/− mouse model of Sandhoff disease. Importantly, restoration of neuronal function after targeted expression of a therapeutic gene selectively in the neurons of HexB −/− mice resulted in normalization of midface development in this mouse model. Histological analysis of the cranial base revealed that abnormal development of the synchondroses underlies the attendant midface retrusion in this model: Neuronal dysfunction led to the absence of hypertrophic chondrocytes in the synchondroses, whereas restoration of neuronal function resulted in normalization of the cranial base development. Taken together, our studies suggest that neuronal function is critical for normal midface development, underscoring the importance of the functional matrix theory as originally proposed by Melvin Moss.

This study compares internal and external distraction devices in the treatment of midface retrusion. 20 patients were treated with midface distraction (12 Crouzon, 4 Apert, 4 others); 12 with internal distraction (MID device), 8 with external distraction (Red or Blue device). The two groups were compared regarding operation time, peroperative blood loss and complications. The groups were comparable regarding patient age, sex, weight and diagnosis. In the MID-group, 7 of 12 patients (58%) underwent Le Fort III, 5 underwent 12 monobloc (32%). In the Blue device group, three of eight patients underwent Le Fort II (38%), three of eight underwent Le Fort III (38%), and two of eight underwent monobloc (25%). Operation time was shorter in the Blue device (mean 298 min) than in the MID group (mean 354 min). Peroperative blood loss and complication rates were similar. The internal distraction device is the 'gold standard' for treating midface retrusion. The use of an external distraction device in midface distraction osteogenesis is associated with a shorter operation time; peroperative blood loss and complications were similar. An external device affords better 3-dimensional control during the distraction process, so external distraction is preferable in patients who will tolerate this treatment.

Nasomaxillary depression, which may be seen with major saddle nose deformities, especially as a sequel to childhood nasal trauma, generally is overlooked during management of the saddle nose. To handle both the saddle nose deformity and nasomaxillary depression, the authors used a one-stage intraoral and external open rhinoplasty approach for 12 patients. Lateral nasal osteotomies and correction of midface retrusion with crescent-shaped autogenous rib cartilage grafts were performed through two bilateral intraoral gingivobuccal incisions. The external open rhinoplasty approach was used for the correction of the saddle nose deformities. The mean follow-up period after the surgery was 2 years (range, 1-3.5 years). The correction of nasal tip depression resulting from midface retrusion was evaluated by lateral cephalometric radiographs. The mean movement of the nasal tip anteriorly in the sagittal plane 1 year after the operation was 5.5 mm (range, 4-7 mm). The functional and aesthetic results were satisfactory for all the patients. As the findings show, the intraoral part of the approach provides a route for the placement of cartilage grafts to correct the depressed nasal tip, augment the paranasal area, and provide support to the lateral alar bases and the controlled low-to-low lateral osteotomies. The external part of the approach provides correction of the saddle nose deformity.

Structured Abstract Authors – Weinberg SM, Naidoo SD, Bardi KM, Brandon CA, Neiswanger K, Resick JM, Martin RA, Marazita ML Objective – Various lines of evidence suggest that face shape may be a predisposing factor for non-syndromic cleft lip with or without cleft palate (CL/P). In the present study, 3D surface imaging and statistical shape analysis were used to evaluate face shape differences between the unaffected (non-cleft) parents of individuals with CL / P and unrelated controls. Methods – Sixteen facial landmarks were collected from 3D captures of 80 unaffected parents and 80 matched controls. Prior to analysis, each unaffected parent was assigned to a subgroup on the basis of prior family history (positive or negative). A geometric morphometric approach was utilized to scale and superimpose the landmark coordinate data (Procrustes analysis), test for omnibus group differences in face shape, and uncover specific modes of shape variation capable of discriminating unaffected parents from controls. Results – Significant disparity in face shape was observed between unaffected parents and controls (p

Le Fort III osteotomy with midface distraction osteogenesis (Le Fort III DO) can improve the midface form and change the upper airway space. Some surgeons believe that midface advancement can improve respiratory outcome dramatically, but others think it does not predictably result in the cure of obstructive sleep apnea (OSA). In this study, we evaluated the structural and functional changes of the upper airway before and after Le Fort III DO; we hope these studies can improve future protocols for midface advancement. A retrospective study of 11 patients with severe midface retrusion who underwent Le Fort III osteotomy with midface external distractor system was undertaken. These patients had an average of 5.4 months of follow-up. Three-dimensional volumetric assessment of the upper airway was used before and after surgery. We also evaluated the two-dimensional cross-sectional area of the upper airway to show the changes in different airway levels. Two patients with preoperative evidence of OSA were evaluated both preoperatively and postoperatively by overnight polysomnography. The midface was distracted for an average of 20.27 +/- 8.04 mm. Comparison between preoperative and postoperative three-dimensional computed tomographic data showed an average 64.30% increase in upper airway volume, an improvement of 9.13 +/- 6.94 mL (P0.05). The two-dimensional measurement also showed that the cross-sectional area at the posterior nasal spine and uvula airway level increased (P0.05), but the cross-sectional area at the epiglottis level and the separation of airway and esophagus level did not increase (P0.05). Two patients with preoperative evidence of OSA had both preoperative and postoperative sleeping studies that showed improvement. Le Fort III DO can significantly improve the upper airway space in the cases of syndromic craniosynostosis. The upper airway space above the uvula level was significantly enlarged after Le Fort III DO according to two-dimensional and three-dimensional image measurements, and according to the polysomnography, the OSA was alleviated. Le Fort III DO is a promising procedure in the treatment of severe midface retrusion with OSA in young patients.

Maxillonasal dysplasia (Binder's syndrome) is a congenital malformation characterized by an extremely flat and retruded nose.This report describes an 18-year-old woman with maxillonasal dysplasia (Binder's syndrome). The septal deficiencies and maxillary retrusion of the patient were corrected by using an L-shaped implant and a crescent-shaped high-density porous polyethylene implant through the oral vestibular sulcus via an external rhinoplasty approach.After the operation, the tip of the nose had moved 5.5 mm anteriorly in the sagittal plane and 11.9 mm coronally in the vertical plane. Also, the nasolabial angle had increased after the surgery. Follow-up evaluation at 24 months showed good correction of the nasal and midface projection.High-density porous polyethylene implants are a good alternative for patients with Binder's syndrome, especially those who will not accept costal cartilage grafts or orthognathic surgery and who have tendency for hypertrophic scarring.

To compare craniofacial structural characteristic of individuals with different types of cleft palate and to lay a foundation for better treatment protocol for patients with cleft palate, we chose a sample consisting of 12 patients with Treacher Collin syndrome, 15 patients with Pierre Robin sequence, 40 patients with unilateral complete cleft lip and palate, and 40 patients with isolated cleft palate who met certain criteria. Lateral cephalometric radiographs were obtained from each subject. A total of 22 variables, comprising 11 angular, 9 linear, and 2 ratio measurements, were studied. The z-scores were analyzed during paired Student t test. The data showed us that there seems to be no difference in craniofacial structures between patients with isolated cleft palate and normal persons. Patients with unilateral complete cleft lip and palate who had only cleft lip repaired exhibit such characteristics as midface retrusion, relatively excessive lower facial height, and more obtuse gonial angle. The cranial base areas of individuals with Treacher Collin syndrome and Pierre Robin sequence are similar to those of normal persons. Mandibular hypoplasia in both vertical and horizontal dimensions and maxillary retrusion can be found in patients with Treacher Collin syndrome, while only mandibular hypoplasia in the horizontal dimension can be found in patients with Pierre Robin sequence. The developmental deficiency of craniofacial structures seems to be a separate deformity, not the direct outcome of cleft palate defect.

BACKGROUND Orbital dysmorphology and midface retrusion are the hallmarks of Crouzon and Apert syndromes. The precise nature of this deficiency is not known. METHODS Untreated Crouzon and Apert syndrome patients and age- and sex-matched controls were included. Computed tomographic scans were digitized and reconstructed. Craniometric and volumetric data relating to the orbit were collected. RESULTS Thirty-one scans were included (control, n = 12; Crouzon; n = 9; Apert, n = 10). The mean age of the Apert group was 5.31 ± 5 years; Crouzon, 5.77 ± 2.7 years; and control, 6.4 ± 3.6 years. The bony orbit length was 12 percent shorter in Apert (p = 0.004) and 17 percent shorter in the Crouzon group when compared to controls (p < 0.0001). The bony orbital volume was 21 percent smaller in the Apert children (p = 0.0006) and 23 percent smaller in Crouzon when compared to controls (p = 0.003). Globe volume was 15 percent larger in Apert (p = 0.008) and 36 percent larger in the Crouzon group when compared to controls (p < 0.0001). Orbital soft-tissue volume was 19 percent less in the Apert group (p = 0.004) and 29 percent less in the Crouzon group (p = 0.001) when compared to controls. CONCLUSIONS A shortened bony orbit, decreased orbital and orbital soft-tissue volumes, and an increased volume of the globe were found in both conditions. Despite normal volume of the overall orbital contents, the contents are altered, and the bony orbit is shorter and holds less volume.

There has been no ideal option for patients who are unhappy with their facial shape but do not want to undergo complex surgeries or pay the high price of temporary injectable treatments. In this article, we describe the treatment of facial complaints such as the gaunt face, long face, bottom-heavy face, chubby face, midface retrusion, and asymmetric face, as well as the modification of racial and ethnic characteristics, through the use of less invasive methods. Fat grafting was used to accentuate the cheekbones in most patients complaining about their facial shape. For the long face, this technique increased the horizontal facial diameter. Lipoplasty combined with buccal lipectomy effectively decreased the fullness of the lower face and improved facial contour in the bottom-heavy face and chubby face. A skin-tightening face lift may also be necessary in patients with neck laxity. Fat grafting was used to fill in depressions or defects in the gaunt face and to treat midface retrusion and facial asymmetry. Added facial projection was obtained with nasal and chin implants. Photographs of representative patients treated for facial reshaping using the described techniques are presented. By using the less invasive techniques described, plastic surgeons can reshape a patient's face without the prolonged downtime or morbidities associated with more invasive procedures.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences. Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.