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Background Craniofacial chondromyxoid fibromas (CMFs) are a rare benign tumor of cartilaginous origin. They are commonly misdiagnosed due to the paucity of information on tumor characteristics. We performed a systematic review to characterize CMFs located in different regions of the craniofacial skeleton. Methods A search of the literature was executed using the search phrase “chondromyxoid fibroma” and included articles from 1990–2020. Sixty-eight articles met the inclusion criteria, with a total of 91 patients with analyzable data (22 with calvarial and 69 with sinonasal tumor locations). Descriptive analyses were performed to compare pre-selected characteristics between the 2 groups. Results Sinonasal CMF frequently presented with cranial nerve palsy and expectedly had a high rate of nasal symptoms. Calvarial tumors frequently presented with an external mass and headache. Gross total resection (GTR) was achieved in a higher proportion of cases in the calvarial group versus the sinonasal group (83.3% vs. 53.1%). Overall recurrence rate at 17.7% was higher in sinonasal CMF compared with the calvarial tumors at 8.3%. Recurrences after GTR were similar in the sinonasal and calvarial groups (9.7% vs. 9.1%). In patients who did not achieve GTR, recurrence was higher in the sinonasal compared with the calvarial group (27.6% vs. 0%). Conclusions Craniofacial CMF in calvarial and sinonasal locations has distinct clinical characteristics and response to treatment. Sinonasal lesions tend to have higher recurrence compared to calvarial CMF. Performance of GTR is associated with decreased recurrence in all CMFs.

Benign bone-forming tumors comprise osteomas, osteoid osteomas, and osteoblastomas. Osteomas affect a wide age range and are usually discovered incidentally. They occur predominantly in the craniofacial skeleton and are classically composed of compact bone. Osteoid osteomas and osteoblastomas are painful lesions occurring in young patients. They are morphologically similar and characterized by FOS gene rearrangement and c-FOS expression at a protein level. Osteoid osteomas are usually smaller than 2 cm in maximum dimension with limited growth potential; osteoblastomas are larger than 2 cm and may be locally aggressive. Histologically both are composed of anastomosing trabeculae of woven bone.

Funder: Natural Environment Research Council; Id: http://dx.doi.org/10.13039/501100000270, Funder: Wellcome Trust, The cichlid fishes comprise the largest extant vertebrate family and are the quintessential example of rapid "explosive" adaptive radiations and phenotypic diversification. Despite low genetic divergence, East African cichlids harbor a spectacular intra- and interspecific morphological diversity, including the hyper-variable, neural crest (NC)-derived traits such as coloration and craniofacial skeleton. Although the genetic and developmental basis of these phenotypes has been investigated, understanding of when, and specifically how early, in ontogeny species-specific differences emerge, remains limited. Since adult traits often originate during embryonic development, the processes of embryogenesis could serve as a potential source of species-specific variation. Consequently, we designed a staging system by which we compare the features of embryogenesis between three Malawi cichlid species-Astatotilapia calliptera, Tropheops sp. 'mauve' and Rhamphochromis sp. "chilingali"-representing a wide spectrum of variation in pigmentation and craniofacial morphologies. Our results showed fundamental differences in multiple aspects of embryogenesis that could underlie interspecific divergence in adult adaptive traits. First, we identified variation in the somite number and signatures of temporal variation, or heterochrony, in the rates of somite formation. The heterochrony was also evident within and between species throughout ontogeny, up to the juvenile stages. Finally, the identified interspecific differences in the development of pigmentation and craniofacial cartilages, present at the earliest stages of their overt formation, provide compelling evidence that the species-specific trajectories begin divergence during early embryogenesis, potentially during somitogenesis and NC development. Altogether, our results expand our understanding of fundamental cichlid biology and provide new insights into the developmental origins of vertebrate morphological diversity.

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.

Exposure to cigarette smoke represents the largest source of preventable death and disease in the United States. This may be in part due to the nature of the delayed harmful effects as well as the lack of awareness of the scope of harm presented by these products. The presence of “light” versions further clouds the harmful effects of tobacco products. While active smoking in expectant mothers may be reduced by educational and outreach campaigns, exposure to secondhand smoke is often involuntary yet may harm the developing embryo. In this study, we show that the main component of secondhand smoke, sidestream cigarette smoke, from several brands, including harm-reduction versions, triggered unsuccessful hatching at 3 dpf and reduced overall survival at 6 dpf in developing zebrafish. At non-lethal concentrations, craniofacial defects with different severity based on the cigarette smoke extract were noted by 6 dpf. All tested products, including harm-reduction products, significantly impacted cartilage formation and/or bone mineralization in zebrafish embryos, independent of whether the bones/cartilage formed from the mesoderm or neural crest. Together, these results in a model system often used to detect embryonic malformations imply that exposure of a woman to secondhand smoke while pregnant may lead to mineralization issues in the skeleton of her newborn, ultimately adding a direct in utero association to the increased fracture risk observed in children of mothers exposed to cigarette smoke.

Neurocristopathies form a heterogeneous group of rare diseases caused by abnormal development of neural crest cells. Heterogeneity of neurocristopathies directly relates to the nature of these migratory and multipotent cells, which generate dozens of specialized cell types throughout the body. Neurocristopathies are thus characterized by congenital malformations of tissues/organs that otherwise appear to have very little in common, such as the craniofacial skeleton and enteric nervous system. Treatment options are currently very limited, mainly consisting of corrective surgeries. Yet, as reviewed here, analyses of normal and pathological neural crest development in model organisms have opened up the possibility for better treatment options involving cellular and molecular approaches. These approaches provide hope that some neurocristopathies might soon be curable or preventable.

SUMMARY Bicoronal incisions are frequently used for exposure and access to the craniofacial skeleton. A zigzag design is often used to camouflage the resultant scar. Often, free-hand zigzag drawings require several correction attempts to ensure symmetry because of the need for replication of multiple limbs of the bicoronal incision that need to be similar lengths, distance, and angles from each other. The authors present a novel technique using a template that rapidly and consistently achieves symmetric zigzag bicoronal incisions. The device is a hairstyling device that is inherently geometric in its design. Retrospective results of pediatric craniofacial patients from 2010 to 2018 are presented. Patients undergoing endoscopic reconstructions and patients who had prior operations at other institutions were excluded from the study. Fifty-two patients met inclusion criteria, with age at surgery ranging from 3 to 207 months (mean, 17 months). Follow-up ranged from 1 to 66 months (mean, 26 months). Data collected included demographics, type of surgery, and operative outcomes, including incision-related complications. Using this dynamic hairstyling device in a novel application as a template results in a fast, effective, and easily reproducible symmetric bicoronal zigzag incision in all cases. This technique eliminates the need for adjusting the length and angles of bicoronal incisions, and it can be adapted across a variety of head sizes and shapes in both pediatric and adult populations.

Purpose. Sella turcica bridging (STB) refers to a rare anatomical variation formed by the ossification of the ligament between the anterior and posterior clinoid processes. The presence of the STB was significantly correlated with craniofacial skeleton classification and a higher prevalence rate in skeletal Class III. The current study is aimed at investigating the dimensions of sella turcica and the prevalence of STB in different sexes and on the three craniofacial skeletal patterns using cone beam computed tomography (CBCT). Materials and Methods. A total of 159 adults (66 males and 93 females), including 3 different craniofacial skeletal patterns (skeletal Classes I, II, and III), were included in the study. The sella turcica dimensions and the prevalence of STB were calculated. An independent t -test and generalized linear model were used to compare the differences in the sella turcica dimensions and the skeletal relations. The Spearman rank correlation coefficient was used to analyze the correlations between sella turcica dimensions and skeletal relation. Results. The sella length (SL) was 11.05 ± 1.80 mm for males and 10.77 ± 1.56 mm for females. The anterior clinoid distance (ACD) which was measured for the anterior width of sella turcica showed 25.83 ± 2.04 mm and 24.04 ± 2.28 mm for males and females, respectively ( p < 0.0001 ). The overall percentage of complete bridging and partial bridging was 6.6% and 56.9%, respectively. Complete bridges were more common in males (males: 9.1%, females: 4.8%), and partial bridges were more frequent in females (males: 49.2%, females: 62.4%). Both sexes differed with respect to sella turcica dimensions. Moreover, males had a significantly larger ACD, posterior clinoid distance (PCD), and diameter of sella turcica (DST), on both sides, relative to females. Conclusion. The prevalence rate of complete STB in the Taiwanese population was 6.6%. Significant differences between sexes were found in sella turcica dimensions. The prevalence rates of STB as well as the sella turcica dimensions did not significantly differ between different craniofacial skeletal patterns (skeletal Classes I, II, and III).

Distraction osteogenesis (DO) is considered one of the treatments of choice for the surgical correction of malformations of the craniofacial skeleton. This is due to its ability to generate bone at the distraction site along with the accompanying soft tissue changes, the possibility to combine DO with traditional orthognathic procedures and the successful reported long-term outcomes following its application to various craniofacial deformities. The aim of this review is to present an overview of the literature on the evolution of DO applications in craniofacial surgery through the last 35 years.

INTRODUCTION Use of patient specific Polymethyl methacrylate (PMMA) implants for the reconstruction of cranial defects has become a standard practice with excellent long-term results. However, for the reconstruction of midface and mandibular osseous defects other alloplastic materials are preferred but their use is limited due to high cost. This is a report of our experience with the use of low-cost patient specific PMMA implants fabricated using 3D printed moulds in the reconstruction of osseous defects involving different areas of the facial skeleton not limited to cranium. METHODS The 25 consecutive patients with craniofacial osseous defects who underwent reconstruction using customized PMMA implants were analyzed. All PMMA implants were fabricated intraoperatively with the use of 3D printed flexible moulds or templates. RESULTS A total of 34 implants were used in 25 consecutive patients. Out of 34 implants 25 were used for midface and mandibular osseous defects. Most common etiology was post-traumatic deformity (n = 19) followed by tumor (n = 3), craniofacial anomalies (n = 2) and post-craniotomy (n = 1). One patient out of 25 (n = 1) had postoperative implant exposure. The follow-up was ranged from 3 to 19 months with an average of 12 months. The aesthetic outcome was found to be good to excellent with mean visual analogue score of 4.08. CONCLUSIONS Polymethyl methacrylate implants fabricated intraoperatively using 3D printed moulds provide accurate and precise reconstruction at an exceptionally low cost. PMMA has an excellent moulding property with low infection rates. As shown in our study its application may be easily extended to all areas of the craniofacial skeleton.

Rare facial clefts are characterized by facial involvement that is not restricted to the lip, palate, and alveolus as in traditional cleft lip and palate. The craniofacial skeleton and the orbital structures are frequently affected in these conditions. Exposure of the eyeball, when not early treated, puts the function and the preservation of the eye at risk. We report the case of a 2-month-old boy admitted to our service with an extensive oral-ocular cleft and exposure of the eyeball with melting corneal ulcer treated with a conjunctival closure with a purse-string suture.

The purpose of this clinical report is to present the novel management of a type Tessier 3 cleft which was treated using a palatal expander in reverse fashion to reapproximate the craniofacial skeleton allowing for closure of the palate and soft tissue of the cleft. Reapproximation of the bony component of the cleft was achieved without osteotomies and allowed for easier and earlier realignment of the bony and soft tissue components of the cleft. To our knowledge, this is the first use of reverse palatal expansion in the treatment of type 3 Tessier cleft. Reverse palatal expansion made management of this cleft more straightforward and should be considered as a useful adjunct in the management of wide facial clefts.

Discrete, small, roundish lesions of localized porous bone can be occasionally seen in the craniofacial skeleton. Such lesions are rarely mentioned and only occur as an incidental discovery being assigned to plenty of diagnoses. As an example, such multiple small lesions of the facial skeleton in a well-preserved skeleton of a 40-60 year old male of the Hunno-Sarmatian Period from Kazakhstan, are discussed. Some of the lesions were examined by digital microscopy, plain radiography, and plain and polarizing microscopy. Considering possible differential diagnoses of vascular, traumatic, inflammatory/reactive, metabolic, and neoplastic entities, as well as developmental conditions, its microscopic characteristics point to a fibro-osseous process. This paper intends to open a discussion on the diagnosis of such lesions, which have been relatively neglected in previous research.

Purpose of review Leonardo Da Vinci possessed one of humanity's greatest minds, known for exploring the boundaries of art and science. The discipline of facial plastic surgery also relies on art and science for its advancement. This review focuses on key elements of Leonardo Da Vinci's work, and how they relate to concepts within facial plastic surgery. Recent findings Leonardo Da Vinci was a self-taught creative genius. Common themes that permeated his art were those of proportion, perspective, light and shadow, anatomy, and science. These principles are reflected in key aspects of facial plastic surgery, such as facial analysis, human gaze, facial subunits, surgery of the craniofacial skeleton, and evidence-based medicine. Summary Leonardo Da Vinci's approach to scientific inquiry and artistic beauty strikes a balance from which facial plastic surgeons have much to learn. In depth study of how Leonardo Da Vinci viewed the world furthers the analytical and creative sides of a facial plastic surgeon as well as informs their personal development.

ABSTRUCT Purpose By using three-dimensional (3-D) CT imaging, the maxillofacial bones and muscles can be observed from any desired angles and analyzed the bone and muscles simultaneously. However, no studies have investigated the three dimensional analysis of muscles in jaw deformity. The purpose of this study was to examine the relationship between the morphology of the masticatory muscles and the craniofacial skeleton in the patients with facial asynmetry. Methods Eight Japanese normal female adults and 10 female adult patients with laterognqthism were participated in this study. The 3-D CT images of their craniofacial skeleton and masseter, medial pterygoid and lateral pterygoid muscles were recon-structed. Then, 6 skeletal and 7 muscle variables were measured for each subject. Results Significant positive correlations were found among the volume of the masti-catory muscles in the normal adults, but not found in patients with mandibular laterognathism. The muscular variables showed significant positive correlations with skeletal variables in normal subjects, but few significant correlations were found in the patients with laterognathism. Conclusions This study suggestted that the balance between masticatory muscles and the balance between muscles and skeleton collapsed in the patients with mandibu-lar laterognathism.

BACKGROUND/PURPOSE Pediatric septorhinoplasty has sparked controversial debate amongst craniofacial surgeons, particularly due to its potential growth disturbances of the face and nose. The purpose of this study is (1) to conduct a systematic literature review that explores unique considerations and limitations associated with performing rhinoplasty in the pediatric population; (2) to discuss objective indicators for determining craniofacial skeleton maturation; (3) to perform a case series to further understand whether the age of menarche serves as a good proxy for estimating the time of facial growth maturation, and by extension, whether a post-menarchal patient is a suitable candidate for rhinoplasty. METHODS Systematic literature review was performed in order to assess unique considerations and limitations of pediatric rhinoplasty. Retrospective chart review and anthropometric (9 linear, 3 angular, 6 indices) measurements were performed for ten pediatric female patients who underwent open septo-rhinoplasty. Follow-up times ranged from 66 to 103 weeks. Patients were separated into 2 cohorts according to age at the time of menarche. Cohort 1 consisted of patients who had undergone menses for longer than 2 years at the time of rhinoplasty, whereas Cohort 2 comprised of patients who either had menses within 2 years of rhinoplasty or did not have menses at the time of rhinoplasty. Data included demographic factors, procedural variables, revision surgeries, and complications. Measurements were conducted using VECTRA software (Canfield Scientific, Parsippany, NJ). Differences in measurements between the 2 postoperative timepoints were compared. RESULTS Literature review yielded 17 articles, which had conflicting opinions regarding pediatric rhinoplasty and ultimately, highlighting the need for a more objective tool for guiding the decision to perform rhinoplasty.Patients in Cohort 1 had minimal deviations in measurements over time, within the exception of an increase in nasal tip projection and a decrease in nasolabial angle and facial width. Patients in Cohort 2 had decreases in facial height, upper facial height, nasal tip angle, and nasofrontal angle. Nasolabial angle decreased for both cohorts. Of the ten patients, 1 in Cohort 2 underwent revision rhinoplasty. CONCLUSIONS Our case series suggests that anthropometric measurements remain relatively more stable for patients who have undergone menses for more than 2 years, compared to those who have not. Although this study has limitations, further investigation is warranted in order to determine whether age of menarche may serve as a useful tool for craniofacial surgeons in guiding the decision to perform rhinoplasty.

Purpose Automated bone segmentation from MRI datasets would have a profound impact on clinical utility, particularly in the craniofacial skeleton where complex anatomy is coupled with radiosensitive organs. Techniques such as gradient echo black bone (GRE-BB) and short echo time (UTE, ZTE) have shown potential in this quest. The objectives of this study were to ascertain (1) whether the high-contrast of zero echo time (ZTE) could drive segmentation of high-resolution GRE-BB data to enhance 3D-output and (2) if these techniques could be extrapolated to ZTE driven segmentation of a routinely used non bone-specific sequence (FIESTA-C). Methods Eleven adult volunteers underwent 3T MRI examination with sequential acquisition of ZTE, GRE-BB and FIESTA-C imaging. Craniofacial bone segmentation was performed using a fully automated segmentation algorithm. Segmentation was completed individually for GRE-BB and a modified version of the algorithm was subsequently implemented, wherein the bone mask yielded by ZTE segmentation was used to initialise segmentation of GRE-BB. The techniques were subsequently applied to FIESTA-C datasets. The resulting 3D reconstructions were evaluated for areas of unexpected bony defects and discrepancies. Results The automated segmentation algorithm yielded acceptable 3D outputs for all GRE-BB datasets. These were enhanced with the modified algorithm using ZTE as a driver, with improvements in areas of air/bone interface and dense muscular attachments. Comparable results were obtained with ZTE+FIESTA-C. Conclusion Automated 3D segmentation of the craniofacial skeleton is enhanced through the incorporation of a modified segmentation algorithm utilising ZTE. These techniques are transferrable to FIESTA-C imaging which offers reduced acquisition time and therefore improved clinical utility.

In their seminal paper, Gans and Northcutt (1983) proposed that evolution of the vertebrate “New Head” was enabled by advent of the neural crest and cranial placodes(1). The neural crest is a stem cell population that arises adjacent to the forming central nervous system and contributes to important cell types including components of the peripheral nervous system, craniofacial skeleton, and elements of the cardiovascular system(2). In the past few years, the New Head hypothesis has been challenged by the discovery in invertebrate chordates of cells with some but not all characteristics of vertebrate neural crest cells(3–7). Here, we discuss recent findings regarding how neural crest cells may have evolved during the course of deuterostome evolution. The results suggest that there was progressive addition of cell types into the repertoire of neural crest derivatives throughout vertebrate evolution(8). Novel genomic tools have enabled higher resolution insight into neural crest evolution from both a cellular and gene regulatory perspective(9,10). Together, these data provide clues regarding the ancestral neural crest state and how the neural crest continues to evolve to contribute to the success of vertebrates as efficient predators.

Sutures are greatly involved in both normal craniofacial growth and developmental anomalies. Having clear parameters for defining their morphology is fundamental to properly investigate their physiological or pathological development. However, the current literature is lacking of well-defined methods for the assessment of these structures. This study performed a comprehensive microcomputed tomography (μCT) analysis of a swine model evaluating morphological variation of sutures in different skull regions. Seventy-two suture samples were removed from one swine (Sus scrofa), approximately 9-12-month-old. Each sample was analyzed with μCT in the parallel (PAR) and perpendicular (PER) plane with respect to the bone surface. Suture width (S w ), linear obliteration index (LOI), and linear interdigitation index (LII) were calculated in each of the two reference planes, and sutures were categorized in four types (S t ). Parameters were compared among the facial, craniofacial, and cranial region. Description of the main morphological parameters was provided, and differences were found between the parallel and perpendicular planes. S t varied depending on the skull region, with simple sutures more represented in the cranial region. LII in the perpendicular plane decreased from facial to craniofacial and cranial region. S w in the parallel plane decreased from facial and craniofacial to cranial region. In the swine model, the sutural width, linear interdigitation, and suture type were related to distinct skull regions. The suture type was introduced to allow a better morphological characterization of sutures as 3D structures. Clear definition of sutural parameters is important for appropriate description of these complex structures. Anat Rec, 302:2156-2163, 2019. © 2019 American Association for Anatomy.

Fibrous dysplasia is a bone disorder whereby normal medullary bone is replaced by fibrous tissue. Many sites may be affected, including the craniofacial skeleton. A 33-year-old female presented at the Bristol Dental Hospital in 2007 complaining of a prominent and expanding lower jaw as well as poor aesthetics. She was diagnosed with craniofacial polyostotic fibrous dysplasia. In order to manage her concerns and the ongoing growth of the polyostotic lesions, a combined maxillofacial and restorative approach was utilized. After ten years of ongoing management and care, including surgery and dental rehabilitation, her primary concerns have been addressed. CPD/Clinical Relevance: The clinician should be able to understand the clinical and radiographic appearance of craniofacial fibrous dysplasia, how the condition can impact a patient and the complex management that may be involved.

Perivascular epithelioid cell tumours, known as PEComas, are a family of tumours with immunoreactive melanocytic and smooth muscle markers. They are a rare heterogeneous group in adulthood and childhood with primary osseous PEComas representing an even rarer subgroup. The clinical behaviour is not well understood and the treatment options vary. We present an index case of a primary osseous PEComa of the craniofacial skeleton in a 3-year-old girl. Level of evidence: Level V, diagnostic; therapeutic; risk/prognostic study.

Bilateral cleft lip and palate (BCLP) occurs when craniofacial precursors fail to form or fuse properly during development. The aim of this retrospective, cross-sectional investigation was to quantify directional asymmetry (DA) of the facial skeleton of children born with Veau Class IV nonsyndromic BCLP. To accomplish this goal, coordinate values of anatomical landmarks were acquired from three-dimensional cone beam computed tomographic images of the craniofacial skeleton of middle- to late-aged children born with BCLP and age- and sex-matched controls and used to compare patterns of asymmetry variation. Multivariate analyses revealed different patterns of DA variation across samples and identified approximately 30% of DA measures as significantly different. Magnitudes of statistically significant linear distances differ in the craniofacial region, with most smaller DA differences located near the orbits and frontal bone, while larger differences were localized mostly to the midface, alveolar ridge, and nasal borders. Generally, areas of the craniofacial skeleton derived from the maxillary and nasal prominences demonstrated the highest magnitudes of DA. The methods and results presented will be useful to biomedical researchers when identifying the extent to which patients with BCLP diverge from typical developmental expectations. Quantifying DA and assessing local differences across the craniofacial complex can aid medical practitioners when developing treatments to improve BCLP surgical algorithms and outcomes. Anat Rec, 302:1726-1732, 2019. © 2019 American Association for Anatomy.

Treatment of pediatric skull base disorders is complex and requires individual planning. Compared to adult patients, the pathologies are more congenital and benign. Advanced surgical planning has been shown to improve outcomes and involves a variety of different software platforms to enable sophisticated review of image data sets, including virtual endoscopy and the details of surgical approach options. There are greater than 50 open and endoscopic surgical approaches described. Endoscopic approaches in patients under age 4 are extremely challenging due to the underdeveloped sinuses and overall smaller size of the craniofacial skeleton and nasal cavity. A multidisciplinary surgical team should develop and review surgical plan options with the patient and family.

Purpose Our study aimed to provide volumetric data relating to fibro-osseous lesions of the craniofacial skeleton, in order to highlight risk factors due to the different entities, and to guide clinical decisions for jeopardized patients. Methods Volumetric measurements of osteomas and ossifying fibromas were performed by applying the open-source software ITK-Snap to cone-beam computed tomography images. DICOM datasets were imported, identified, and delineated using semiautomatic segmentation; this was then verified using manual segmentation. The volumes of the lesions were computed automatically in cubic millimeters using the program. For statistical investigations, descriptive statistics and independent Student t-tests were performed. Additionally, Pearson's correlation was applied as a bivariate analysis. Values of p Results 45 patients (11 male and 34 female) were included in this study. The mean volumes were 10.02 ± 18.79 cm3 for osteomas and 4.80 ± 5.71 cm3 for ossifying fibromas (p = 0.016). Males (12.81 ± 20.38 cm3) presented significantly larger volumes than females (5.43 ± 10.32 cm3) (p = 0.042). With regard to shape, morphology, and affection of surrounding anatomical structures, irregular shape (p = 0.001; p = 0.037), multilocular morphology (p = 0.001; p = 0.037), nerve affection (p = 0.001; p = 0.002), tooth affection (p = 0.001; p = 0.594), cortical bone exceedance (p = 0.033; p = 0.001), and clinically visible symptoms (p = 0.004; p = 0.001) were statistically significantly associated with a larger volume of both entities. Conclusion Volumetric analysis revealed that osteomas significantly exceeded the mean size of ossifying fibromas, supporting the argument that special attention should be paid to this entity. In cases of difficult histopathological examination, lesions with irregular shape, multilocular morphology, nerve and tooth affection, cortical bone exceedance, and clinically visible symptoms should be considered for close clinico-radiological follow-up, irrespective of the entity.

Repair of bone deficiencies in the craniofacial skeleton remains a challenging clinical problem. The aim of this study was to evaluate and compare the effects of a plasma-derived albumin scaffold, alveolar osteoblasts and synthetic membrane implanted into experimental mandibular defects. Bilateral mandibular defects were created in twelve immunodeficient rats. The bone defect was filled with serum scaffold alone in left sides and scaffold combined with human alveolar osteoblast in right side defects. Implanted areas were closed directly in Group 1 ( n = 6) and covered by a resorbable polyglycolic-polylactic acid membrane in Group 2 ( n = 6). Bone regeneration was determined at 12 weeks as measured by and exhaustive multiplanar computed tomography analysis and histological examination. No significant differences in bone density were observed between defects transplanted with scaffold alone or scaffold seeded with osteoblasts. The use of membrane did not result in a determining factor in the grade of bone regeneration between Groups 1 and 2. Based on these results, it could be concluded that the albumin scaffold alone has osteoinductive capacity but presence of seeded ostogenic cells accelerates defect repair without being significantly influenced by covering the defect with a resorbable membrane.

Craniofacial surgery in children is a highly challenging discipline that requires extensive knowledge of craniofacial anatomy and pathology. Insults to the fronto-orbital skeleton have the potential to inflict significant morbidity and even mortality in patients due to its proximity to the central nervous system. In addition, significant aesthetic and ophthalmologic disturbances frequently accompany these insults. Craniosynostosis, facial trauma, and craniofacial tumors are all pathologies that frequently affect the fronto-orbital region of the craniofacial skeleton in children. While the mechanisms of these pathologies vary greatly, the underlying principles of reconstruction remain the same. Despite the limited data in certain areas of fronto-orbital reconstruction in children, significant innovations have greatly improved its safety and efficacy. It is imperative that further investigations of fronto-orbital reconstruction are undertaken so that craniofacial surgeons may provide optimal care for these patients.

A 19 year old man presented with a few years history of increasing headaches. Over the preceding few months, his friends and family noticed a slight protrusion of his left eye. In the last two months, he started experiencing double vision. CT and MRI scans confirmed the presence of fibrous dysplasia of the left frontal sinus with a secondary mucocele formation extending into the left orbit. The mucocele was drained and the fibrous dysplasia reduced through a combined craniotomy and endoscopic sinus surgery. The patient symptoms resolved postoperatively, and he was discharged 5 years later.

In earlier times, apart from infectious diseases, the treatment of complex wounds and fractures was a great challenge to physicians and surgeons. The craniofacial skeleton owing to its complex anatomy and proximity to vital structures is more difficult to treat when compared to other parts of the human skeletal system. Initially, surgeons used nonrigid materials to stabilize the fracture segments until the union was complete. Discovery of biocompatible metals and construction of various fixation devices took fracture management to its next level. Biocompatible metals owing to their inert nature can be fixed inside the body; thus, the term “internal fixation” came to existence. Following this, researchers continuously developed various internal fixation devices, which are being modified and developed according to the requirements, till date. Apart from the fixation devices, several instruments have also been developed, which help in fixation of these devices. Due to the unique nature of the craniomaxillofacial region, it requires armamentarium that is specially designed to be used in this region. This chapter briefly describes the various fixation devices and other armamentarium that are used in craniomaxillofacial trauma management.

The rarity of craniofacial clefts has made the amassing and complete anatomic documentation of extensive arrangement irksome. Preoperative and postoperative CT inspects with three-dimensional propagations of extensive arrangements will give the reason to quantitative examination of cautious mediations and improved understanding of the advancement components of the truly contorted craniofacial skeleton. Just with complete radiologic and employable examination of these phenomenal craniofacial changes would we have the option to esteem the giant of the reconstructive test. The assessment of this understanding and of our healing interventions will simply go with the development of a fourth estimation time.

Congenital malformations of the nose and paranasal sinuses arise from aberrations in the normal developmental pathways of the craniofacial skeleton. Understanding the embryology of the craniofacial skeleton will aid in designing appropriate surgical treatment options for congenital malformations. Radiological evaluation is an important initial step in the diagnosis and management of congenital malformations of the nose and paranasal sinuses. A multidisciplinary approach is often required for treating children with congenital malformations of the nose and paranasal sinuses.

Notch is a cell–cell signaling pathway that is involved in a host of activities including development, oncogenesis, skeletal homeostasis, and much more. More specifically, recent research has demonstrated the importance of Notch signaling in osteogenic differentiation, bone healing, and in the development of the skeleton. The craniofacial skeleton is complex and understanding its development has remained an important focus in biology. In this review we briefly summarize what recent research has revealed about Notch signaling and the current understanding of how the skeleton, skull, and face develop. We then discuss the crucial role that Notch plays in both craniofacial development and the skeletal system, and what importance it may play in the future.

Background:. Facial transplantation (FT) has become a feasible reconstructive solution for patients with devastating facial injuries. Secondary revisions to optimize functional and aesthetic outcomes are to be expected, yet the optimal timing and approach remain to be determined. The purpose of this study was to analyze all facial allograft revisions reported to date, including the senior author’s experience with 3 FTs. Methods:. A literature review was performed, with 2 reviewers independently conducting title and abstract screening, followed by a full-text review. All articles mentioning FT revision surgeries were evaluated. The medical records of the senior author’s 3 FT recipients were additionally reviewed. Results:. Initially, 721 articles were captured and 37 were included in the final analysis. Thirty-two FTs were reported to have involved posttransplant allograft revisions, with FT recipients undergoing a mean of 4.8 ± 4.6 revision procedures. The mean duration between FT and the first revision procedure was 149 ± 179 days. A wide spectrum of revisions was identified and categorized as involving the soft tissues, craniofacial skeleton, dentition, oronasal cavity, salivary glands, facial nerve, or ocular region. In the senior author’s experience, when indicated, posttransplant occlusal changes and integrity of the donor–recipient intraoral interface were successfully addressed with secondary procedures without allograft compromise or loss. Conclusions:. The worldwide experience shows that secondary procedures are nearly ubiquitous after FT and can be safely performed at various timepoints. The authors thereby establish 5 distinct categories of facial allograft revisions and define 7 critical principles to optimize posttransplant procedures.

Posttraumatic secondary reconstruction of the craniofacial skeleton is challenging. Many times the surgeon is faced with malunion and asymmetries that can be very difficult to correct using osteotomies and repositioning of bony segments. The chapter discusses the use of custom craniofacial implants as an alternative to posttraumatic secondary reconstruction.

This chapter covers the history and role of radiography, computed tomography (CT), and magnetic resonance imaging (MRI) in the evaluation of the craniofacial skeleton in the setting of blunt and penetrating trauma. The basic craniofacial anatomy is reviewed in all imaging modalities, including a video demonstrating a systematic review of a craniofacial CT. Both common and uncommon craniofacial injuries as well as their potential complications are discussed. The advantages and disadvantages of each imaging modality in both the pre- and postoperative period are also discussed, along with the effective dose of ionizing radiation.

In pediatrics, orbital fracture repair must be weighed against the potential skeletal growth disruption resulting from surgery and preventing globe malposition and motility complications. Pediatric orbital fractures are unique in presentation secondary to the evolving anatomy and physiology of the craniofacial skeleton. Therefore, the management of these fractures should be performed differently from adult patients. The understanding of these injuries is evolving and controversial in some aspects: particularly the decision to operate and the timing of the operation. For children with Type 1 orbital fractures, radiographic findings should not drive the decision to operate, independent of physical examination findings. A more conservative approach to operative management should be pursued when signs of muscle entrapment, acute enophthalmos, or hypoglobus are not present. The objective of this chapter is to highlight the pertinent differences in anatomy, presentation, and management, as well as clinical outcomes of pediatric orbital fractures.

Panfacial injury refers to fractures present in the cranio-orbital (upper third), orbitozygomaticomaxillary (middle third), as well as the mandibular (lower third) portions of the craniofacial skeleton. These injuries present challenges in surgical management to avoid long-term sequelae of inadequate correction, including increased facial width, enophthalmos, facial retrusion, and malocclusion. The purpose of this chapter is to provide an algorithm for management of these panfacial injuries in order to optimize outcomes.

The orbitozygomaticomaxillary complex (OZMC) is a structural foundation for both form and function of the craniofacial skeleton. Due to its anterolateral position and projection, it provides significant strength and stability. Fractures of the OZMC must be optimally diagnosed and classified using necessary imaging, physical examination, and additional established criteria. The interruption of its normal anatomical state results in aesthetic compromise and functional impairments. Various treatment modalities exist, so the uniform implementation of a treatment algorithm has been suggested with the goal of obtaining favorable, predictable, and reproducible results. As in any surgical intervention, even with the latest techniques, materials, precautions, and expertise, acute and long-term complications exist. The craniofacial surgeon constantly seeks the most favorable treatment modality that will lead to optimal results, both functionally and aesthetically, and diminished complications through literature, research, and experience.

The management of fractures of the midface has undergone significant evolution as practitioners have gained a more in-depth understanding of the interplay with the remainder of the craniofacial skeleton. This chapter addresses the history, classification, diagnosis, work-up, techniques for management, and complications associated with the treatment of complex Le Fort level fractures.

Juvenile ossifying fibroma (JOF) is a rare fibro-osseous neoplasm that develops among the craniofacial bones at an early age. Their aggressiveness added to their high tendency to recur, provoke real diagnostic and therapeutic challenges. We present an agressive case of a nineteen-year-old girl showing facial asymmetry with a large facial mass. The lesion was surgically excised with a satisfactory esthetic result, besides maintenance of the physiological functions. After two years there is no evidence of recurrence of the lesion. Although JOF is a benign lesion, it typically presents aggressive behavior. The high recurrence rates justifies long-term clinical and radiological surveillance.

To examine the evidence in support of light continuous forces for enhancing bone adaptation (modeling and remodeling) in orthodontics and dentofacial orthopedics. Clinical evidence suggests that light continuous orthodontic force can achieve physiologic expansion of the maxillary arch, but the long-term stability and the biological effects of the procedure are unclear. Compared to conventional orthodontic appliances that deliver heavy interrupted forces for tooth movement, the application of low-magnitude forces in animal models leads to anabolic modeling and remodeling of the alveolar bone in the path of orthodontic tooth movement. This results in dental translation and expansion of the alveolar process. Light continuous forces are preferable to heavy forces for more physiologic dentofacial orthopedics. The interaction of low-magnitude loads with soft tissue posture achieves therapeutic adaptation of the craniofacial skeleton. The increasing emphasis on genomic medicine and personalized treatment planning should focus on low-magnitude loads in orthodontics and dentofacial orthopedics.

The term fetal alcohol spectrum disorder (FASD) refers to the entire suite of deleterious outcomes resulting from embryonic exposure to alcohol. Along with other reviews in this special issue, we provide insight into how animal models, specifically the zebrafish, have informed our understanding of FASD. We first provide a brief introduction to FASD. We discuss the zebrafish as a model organism and its strengths for alcohol research. We detail how zebrafish has been used to model some of the major defects present in FASD. These include behavioral defects, such as social behavior as well as learning and memory, and structural defects, disrupting organs such as the brain, sensory organs, heart, and craniofacial skeleton. We provide insights into how zebrafish research has aided in our understanding of the mechanisms of ethanol teratogenesis. We end by providing some relatively recent advances that zebrafish has provided in characterizing gene-ethanol interactions that may underlie FASD.

Several surgical options are available for treating the different types of craniosynostosis, including fronto-orbital advancement and remodeling, total or subtotal cranial vault remodeling, barrel stave osteotomy with cranial remodeling, endoscopic suturectomy, monobloc advancement and cranioplasty, and revision cranioplasty. High-resolution, low-dose CT with 3D reconstructed images and volumetric analysis can be useful for evaluating the craniofacial skeleton following surgery. The various types of craniosynostosis surgery and corresponding imaging findings are reviewed in this article.

Vibration as a non-invasive method is currently available for clinical use with the potential to accelerate the rate of tooth movement in orthodontics. The aim of this review was to evaluate the basic science and clinical literature on the effects of vibration on the axial and appendicular skeleton including the craniofacial bone. Vibration as a dynamic load consisting of high oscillatory forces of low magnitude has shown osteogenic and anti-catabolic effects on bone. These effects have been observed in the craniofacial skeleton including the alveolar bone as increases in sutural width and alveolar bone formation. Animal studies have shown conflicting results on vibration when superposed to orthodontic tooth movement. The effects range from increasing to decreasing the rate of tooth movement. Clinical studies in accelerating the rate of tooth movement have similar findings observed in animal studies. High-frequency oscillatory forces of low magnitude are able to affect bone formation and remodelling. These effects of vibration are primarily anabolic and anti-catabolic in bone, including the craniofacial skeleton and alveolar bone. The effect of vibration on accelerating the rate of orthodontic tooth movement is contradictory. Higher levels of evidence studies have not been able to show an acceleratory effect.

Among the classification of maxillary fracture, the Le Fort classification is the best-known categorization. Le Fort (1901) completed experiments that determined the maxilla areas of structural weakness which he designated as the “lines of weakness”. According to these results, there are three basic fracture line patterns (transverse, pyramidal and craniofacial disjunction). A transverse fracture is a Le Fort I fracture that is above the level of the apices of the maxillary teeth section, including the entire alveolar process of the maxilla, vault of the palate and inferior ends of the pterygoid processes in a single block from the upper craniofacial skeleton. Le Fort fractures result in both a cosmetic and a functional deficit if treated inappropriately. In this article, authors review the management of a Le Fort I fracture with a case-based discussion.

Acoustic emission (AE) is a non-destructive test to detect energy release. This technique was applied during rapid maxillary expansion (RME) to evaluate the reaction of the craniofacial skeleton.A swine model was analysed. Soft tissues were removed from two heads, while other two were preserved intact. A palatal expander (PE) was positioned and AE sensors were placed on the intermaxillary, fronto-nasal, and maxillo-lacrimal sutures. The PE was activated and AE recorded during RME. Differences between parameters were analysed with Mann-Whitney U test and Kruskal-Wallis test, and correlations with Spearman-Rho test (significance p0.05).PE activations were accompanied by clusters of AE hits. In the presence of soft tissues, amplitudes were generally lower (p0.001) and durations were higher (p0.001). No differences were found in the respective energy values. Differences were found in the AE (p0.05) among the four channels, with AE characterised by higher values in proximity of the maxillo-lacrimal sutures. High-energy hits were represented by burst-type waves, and low-energy ones by continuous-type.Although soft tissues create possible attenuation of the signal, AE can be detected during RME with sensors on the skin. AE provided further information of energy release, on top of the mechanical parameters. Source location was one of the main limitations.

Chondromyxoid fibroma (CMF) is a rare, benign neoplasm of the chondroid, myxoid, and fibrous tissue. It characteristically affects the lower extremity long bones, although it may rarely arise within the craniofacial skeleton. We report the diagnosis and management of a 31-year-old male with a large, incidentally discovered CMF originating from the sphenoid sinus. A subsequent review of the literature reveals the need to differentiate from more aggressive neoplasms, such as chondrosarcoma and chondroma, which share radiographic features. A histopathologic examination is crucial for proper diagnosis and treatment. We discuss clinical sequelae, highlight the importance of a thorough pre-operative evaluation, and summarize previously suggested treatment paradigms.

Vertebrate cranial neural crest cells (CNCC) are multipotent. Proximal to the source CNCC form the cranial ganglia. Distally, in the pharyngeal arches, they give rise to the craniofacial skeleton and connective tissues. Fate choices are made as CNCC pattern into distinct destination compartments. In spite of this importance, the mechanism patterning CNCC is poorly defined. Here, we report that a novel β-catenin-controlled switch in the cell arrangement is critical in patterning CNCC. In mouse embryos, at the first pharyngeal arch axial level, membrane β-catenin levels correlate with the extent of cell-cell adhesion and thus, with a collective or a dispersed state of CNCC. Using in vitro human neural crest model and chemical modulators of β-catenin levels, we show a requirement for down-modulating β-catenin for the collective-to-dispersed switch. Similarly, in β-catenin gain of function mutant mouse embryos, CNCC fail to disperse, which may underlie their failure to populate first pharyngeal arch. Thus, we show that β-catenin-mediated regulation of CNCC tissue architecture, a previously underappreciated mechanism, underlies the patterning of CNCC into fate-specific compartments.Summary statementThe report shows a crucial step in cranial neural crest patterning. Neural crest cells invading the pharyngeal arches transition from a collective to a dispersed state. This transition in cell arrangement is dependent on membrane β-catenin levels.