Association of cranial base suture/synchondrosis fusion with severity of increased intracranial pressure in Crouzon syndrome.

This study investigated how the fusion states of the cranial base is related to the degree of increased intracranial pressure (ICP) in patients with Crouzon syndrome. This retrospective cohort study enrolled patients who were diagnosed with Crouzon syndrome between May 2007 and April 2022. We categorized the patients into three groups: A, B, and C, according to the severity of increased ICP and the number of cranial vault remodeling procedures for corrective operation. The preoperative fusion states of the cranial base sutures/synchondroses were examined using facial bone computed tomography and compared between groups. Overall, 22 patients were included in Groups A, B, and C, including 8, 7, and 7 patients, respectively. The preoperative average grades of the total cranial base suture/synchondrosis fusion appeared to significantly increase with severity, except for the frontoethmoidal suture, which showed the opposite tendency. In the subgroup analysis, frontosphenoidal, sphenoparietal, sphenosquamosal, parietomastoid, and occipitomastoid suture and petro-occipital synchondrosis were associated with earlier fusion in the more severe group. Premature closure of the cranial base sutures/synchodroses seems to be associated with increased ICP severity in patients with Crouzon syndrome. Precise evaluation of minor sutures/synchondroses at the first visit might help build subsequent operative plans and predict disease prognosis.

Correlation analysis of airway-facial phenotype in Crouzon syndrome by geometric morphometrics: A promising method for non-radiation airway evaluation.

AIM: This study aimed to verify the correlation of the airway-facial phenotype and visualize the morphological variation in Crouzon syndrome patients. Additionally, to develop a non-radiation methodology for airway assessments. METHOD: In this study, 22 patients diagnosed with Crouzon syndrome (Age: 7.80 ± 5.63 years; Gender distribution: 11 females and 11 males) were analysed. The soft tissue surface and airway were three-dimensionally reconstructed, and the entire facial phenotype was topologized and converted into spatial coordinates. Geometric morphometrics was employed to verify the correlation and visualize dynamic phenotypic variation associated with airway volume. A total of 276 linear variables were automatically derived from 24 anatomical landmarks, and principal component analysis (PCA) identified the 20 most significant parameters for airway evaluation. Correlation analyses between parameters and airway volume were performed. Then, patients were classified into three groups based on airway volume, and the differences among the groups were compared for evaluating the differentiating effectiveness of parameters. RESULTS: The facial phenotype was strongly correlated with the airway (coefficient: 0.758). Morphological variation was characterized by (i) mandibular protrusion and anticlockwise rotation; (ii) midface retrusion; (iii) supraorbital frontward and (iv) lengthening of the facial height. All the anthropometric parameters were strongly associated with the airway, and the differences among the groups were statistically significant. CONCLUSION: This study confirmed the strong correlation between facial phenotype and airway parameters in Crouzon syndrome patients. Despite the development of the airway, pathological midface retrusion was still aggravated, suggesting that surgical intervention was inevitable. Three-dimensional facial anthropometry has potential as a non-radiation examination for airway evaluation.

LeFort III Versus Monobloc Frontofacial Advancement: A Comparative Analysis of Soft Tissue Changes.

INTRODUCTION: The LeFort III and monobloc are commonly used midface advancement procedures for patients with syndromic craniosynostosis with well characterized postoperative skeletal changes. However, the differential effects of these procedures on facial soft tissues are less understood. The purpose of this study was to critically analyze and compare the effects of these 2 procedures on the overlying soft tissues of the face. METHODS: Frontal and lateral preoperative and postoperative photographs of patients undergoing monobloc or LeFort III were retrospectively analyzed using ImageJ to measure soft tissue landmarks. Measurements included height of facial thirds, nasal length and width, intercanthal distance, and palpebral fissure height and width. Facial convexity was quantified by calculating the angle between sellion (radix), subnasale, and pogonion on lateral photographs. RESULTS: Twenty-five patients with an average age of 6.7 years (range 4.8-14.5) undergoing monobloc (n=12) and LeFort III (n=13) were identified retrospectively and analyzed preoperatively and 6.4±3.6 months postoperatively. Patients undergoing LeFort III had a greater average postoperative increase in facial convexity angle acuity (28.2°) than patients undergoing monobloc (17.8°, P =0.021). Patients in both groups experience postoperative increases in nasal width ( P <0.001) and decreases in palpebral fissure height ( P <0.001). CONCLUSIONS: Both subcranial LeFort III advancements and monobloc frontofacial advancements resulted in significant changes in the soft tissues. Patients undergoing LeFort III procedures achieved greater acuity of the facial convexity angle, likely because the nasion is not advanced with the LeFort III segment.

A relevância da odontologia na Síndrome Treacher Collins: relato de caso / The relevance of dentistry in Treacher Collins Syndrome: case report

Treacher Collins também chamada de disostose mandibulofacial, é uma alteração genética dominante rara caracterizada pela má-formação dos ossos e tecidos da face. É uma síndrome crânio-facial que apresenta alterações bilaterais e simétricas de estruturas originadas do primeiro e segundo arcos branquiais. A maioria dos casos possui transmissão autossômica dominante e expressividade variável. O objetivo do presente estudo é realizar um relato de caso sobre o impacto do tratamento odontológico na qualidade de vida do paciente portador de Treacher Collins. Paciente, 39 anos, sexo feminino compareceu a uma clínica odontológica em Belo Horizonte, com a queixa principal de falhas dentárias e sensibilidade. Durante a anamnese a paciente relatou ter a STC, durante o exame clínico extraoral verificou a presença de hipoplasia malar e mandibular, malformação dos pavilhões auriculares com perda auditiva, obliquidade e coloboma palpebral inferior. Ao exame intraoral observou ser classe II de Angle, ausência dos dentes 11, 12, 13, 21 e 22 e extrusão do dente 41 e recessão gengival e periodontite estágio I grau A. Após exames de periodontograma e complementares foi realizado uma raspagem nas áreas com profundidade de sondagem maior que 3mm, frenectomia labial inferior, aplicação de laser para sensibilidade, enxerto gengival e colocação de prótese parcial removível. A paciente ao final do tratamento relatou ter se sentido realizada e contente com a sua conclusão, ela foi encaminhada ao Sistema único de Saúde para realizar as cirurgias para corrigir as alterações crânio-faciais. O tratamento odontológico deve ser adaptado a cada indivíduo de acordo com sua necessidade, tendo uma abordagem multidisciplinar, possibilitando uma melhora na qualidade de vida e estética do paciente(AU)

Treacher Collins syndrome is a rare dominant genetic disorder characterized by malformation of the bones and tissues of the face. It is a craniofacial syndrome that presents bilateral and symmetrical alterations of structures originating from the first and second branchial arches. The aim of the present study is to perform a case report on the impact of dental treatment on the quality of life of a patient with CTS. Patient, 39 years old, female, attended a dental clinic in Belo Horizonte, with the main complaint of dental flaws and sensitivity. During the anamnesis the patient reported having CTS, during the extraoral clinical examination she verified the presence of malar and mandibular hypoplasia, malformation of the pinnae with hearing loss, obliquity and lower eyelid coloboma. Intraoral examination revealed Angle class II, missing teeth 11, 12, 13, 21 and 22, extrusion of tooth 41, gingival recession and stage I periodontitis grade A. After periodontogram and complementary exams it was performed a scaling in areas with a probing depth greater than 3mm, lower lip frenectomy, laser application for sensitivity, gingival graft and placement of partial removable prosthesis. The patient at the end of treatment reported feeling fulfilled and happy with its completion, she was referred to the Unique Health System to undergo surgery to correct the craniofacial changes. The current treatment aims at functional and aesthetic correction and the need for psychosocial support, having the joint participation of a multidisciplinary team to achieve this goal(AU)

Plea for systematic prenatal genes panel testing when facing isolated craniosynostosis on fetal imaging.

BACKGROUND: Prenatal diagnosis of craniosynostosis remains rare and challenging, easier in syndromes with craniosynostosis due to the association with other sonographic anomalies. Crouzon syndrome is the most frequent syndrome with craniosynostosis but is difficult to detect antenatally because of mild skull deformation without specific associated anomaly during gestation. CASE: This report presents the case of a fetus with Crouzon syndrome related to the variant c.1646A>C in exon 14 of the FGFR2 gene and presenting with apparently isolated scaphocephaly on fetal US. CONCLUSION: This observation supports the interest of systematic prenatal panel genes testing when facing an apparently isolated craniosynostosis diagnosed on fetal imaging, even if non-syndromic craniosynostosis are much more frequent in such situation. TEACHING POINTS: Syndromic craniosynostosis can appear as apparently isolated form on fetal imaging. Systematic prenatal panel genes testing can be contributive even when facing an apparently isolated craniosynostosis on fetal imaging.

An unusual presentation of bilateral optic pathway glioma in Crouzon Syndrome.

Crouzon Syndrome is a genetic craniosynostosis disorder associated with a high risk of ophthalmologic sequelae secondary to structural causes. However, ophthalmologic disorders due to intrinsic nerve aberrations in Crouzon Syndrome have not been described. Optic pathway gliomas (OPGs) are low grade gliomas that are intrinsic to the visual pathway, frequently associated with Neurofibromatosis type 1 (NF-1). OPGs involving both optic nerves without affecting the optic chiasm are rarely seen outside of NF-1. We report an unusual case of bilateral optic nerve glioma without chiasmatic involvement in a 17-month-old male patient with Crouzon Syndrome without any clinical or genetic findings of NF-1. This case suggests that close ophthalmologic follow up and orbital MRIs may benefit patients with Crouzon Syndrome.

Long-term follow-up of a patient diagnosed with Crouzon syndrome who underwent Le Fort I and III distraction osteogenesis using a rigid external distractor system.

OBJECTIVE: This case report describes the successful treatment of a patient with Crouzon syndrome with severe midfacial deficiency and malocclusion, including reverse overjet. MATERIALS AND METHODS: In Phase I treatment, maxillary lateral expansion and protraction were performed. In Phase II treatment, after lateral expansion of the maxilla and leveling of the maxillary and mandibular dentition, an orthognathic approach including simultaneous Le Fort I and III osteotomies with distraction osteogenesis (DO) was used to improve the midfacial deficiency. RESULTS: After DO, 12.0 mm of the medial maxillary buttress and 9.0 mm of maxillary (point A) advancement were achieved, which resulted in a favorable facial profile and stable occlusion. CONCLUSION: Even after 8 years of retention, the patient's profile and occlusion were preserved without any significant relapse.

Displasia frontonasal / Frontonasal dysplasia

Los trastornos del desarrollo son aquellos padecimientos que se manifiestan por defectos en la embriogénesis de la región afectada. La cara del ser humano comienza su formación alrededor de la cuarta semana de vida intrauterina y se manifiesta gracias a la fusión de cinco prominencias: dos pares conocidas como maxilar y mandibular, y una impar conocida como frontonasal. Cuando esta fusión no se lleva a cabo de una forma óptima, aparecen numerosas alteraciones del desarrollo como el labio y paladar hendido, y la displasia frontonasal. La displasia frontonasal produce frecuentemente afecciones oculares, nasales y orales. Dentro de las manifestaciones orales destacan una forma atípica de hendidura labial o palatina, afecciones dentales y alteraciones en el crecimiento de la cara. Dada la gran relación que este padecimiento tiene con la cavidad oral resulta importante que el odontólogo conozca la etiología y las características clínicas de este trastorno (AU)

Developmental disorders are those conditions that are manifested by defects in the embryogenesis of the affected region. The human face begins its formation around the fourth week of intrauterine life and is manifested thanks to the fusion of five prominences: two pairs known as maxillary and mandibular and odd one known as frontonasal. When this fusion is not carried out in an optimal way, numerous developmental alterations appear, such as cleft lip and palate and frontonasal dysplasia. Frontonasal dysplasia frequently produces ocular, nasal and oral affections. Among the oral manifestations, and atypical form of clef lip and/or palate, dental affections and alterations in the growth of the face stand out. Given the great relationship that this condition has with the oral cavity, it is important for the dentist to know the etiology and clinical characteristics of this disorder (AU)

Métodos de avaliação cognitiva de pacientes com Síndrome de Apert e de Crouzon / Cognitive assessment methods of patients with Apert and Crouzon syndrome

A Craniossinostose Coronal bilateral implica em diminuição do Perímetro Craniano (PC) no eixo ântero-posterior (Braquicefalia) e frequentemente se associa ao aumento do eixo céfalo-caudal (vertical-altura) do crânio (Turricefalia), sendo um dos achados mais comuns nas Síndromes de Crouzon e Apert. Objetivo: Identificar, analisar e sintetizar os métodos de avaliação cognitiva apropriados para o acompanhamento da evolução de pacientes com cranioestenoses sindrômicas, em particular as síndromes de Apert e de Crouzon. Método: Trata-se de uma revisão de escopo. Para a formulação da pergunta norteadora da pesquisa e da estratégia de busca, foi utilizada a estratégia Population [((Apert OR Crouzon) AND (Disease OR Syndrom*))], Concept [((cognit* OR neurobehavioral OR neurocognit* OR neuropsyc*) AND (evaluation OR evaluations OR assessment OR "test" OR tests OR status OR development OR disorder OR disorders OR impairment OR impairments OR impaired OR function OR functions))] e Context (em qualquer contexto). Foram inclusos os artigos escritos em inglês, português e espanhol em qualquer período. A busca foi realizada nas bases de dados: Embase, Scopus, PubMed/MEDLINE e rede BVS Salud. Resultados:Inúmeros testes de avaliação cognitiva validados internacionalmente foram aplicados aos pacientes com Apert e Crouzon, mas não se observou uma padronização (protocolo) seguida pelas várias unidades de assistência. Dos 75 tipos de Testes Cognitivos aplicados houve o predomínio da Escala de Inteligência de Wechsler (e seus subtestes), 50%. Na população avaliada predominou duas faixas etárias: escolares e adolescentes. As crianças com Apert e Crouzon obtiveram escores piores nos transtornos de socialização, atenção e internalização quando comparadas com o grupo normativo, sendo os piores resultados encontrados em Apert. Fatores que interferem no desenvolvimento neuropsicomotor: pressão intracraniana, malformações encefálicas, genética, idade na correção cirúrgica (postergação da primeira cirurgia após um ano de idade associou-se a um quociente de inteligência mais baixo), institucionalização, ambiente familiar, escolaridade dos cuidadores e nível socioeconômico. Considerações finais: os resultados obtidos contribuíram para maior conhecimento do perfil cognitivo dos pacientes com estas síndromes. Somente conhecendo as habilidades e dificuldades neuropsicomotoras, cognitivas e psicossociais dos pacientes com Apert e Crouzon é que as equipes de saúde, da escola e de cuidadores poderão entender melhor a capacidade perceptiva destes no processo de aprendizado e estarão mais aptas em atender as necessidades especiais destes pacientes e poderão ofertar os estímulos mais adequados no momento mais oportuno (AU).

Bilateral Coronal Craniosynostosis implies a decrease in the Cranial Perimeter (CP) in the anteroposterior axis (Brachycephaly) and is frequently associated with an increase in the cephalocaudal (vertical height) axis of the skull (Turrycephaly); being one of the most common findings in Crouzon and Apert Syndromes (Syndromic Craniosynostosis). In this Scope Review study, among the Syndromic Craniosynostosis, Apert and Crouzon Syndromes will be of special interest. Objective: This study aimed to identify, analyze, and synthesize the appropriate cognitive assessment methods for monitoring the evolution of patients with syndromic craniosynostosis, in particular Apert's and Crouzon's syndromes. Method: This is a scope review. In order to formulate the research guiding question and the searching strategy, the Population [((Apert OR Crouzon) AND (Disease OR Syndrom*))], Concept [((cognit* OR neurobehavioral OR neurocognit* OR neuropsyc*) AND (evaluation OR evaluations OR assessment OR "test" OR tests OR status OR development OR disorder OR disorders OR impairment OR impairments OR impaired OR function OR functions))] and Context (in any context) strategy was used. The articles written in English, Portuguese, and Spanish in any period were included. The search was performed in the following databases: Embase, Scopus, National Library of Medicine (PubMed/MEDLINE), and in the BVS Salud network (PAHO, WHO, BIREME, LILACS). Results: many internationally validated cognitive assessment tests were applied to patients with Apert and Crouzon, but no standardization (protocol) was followed. Of the 75 types of Cognitive Tests applied, the Wechsler Intelligence Scale predominated, 50%. In the evaluated population, two age groups predominated: school children and adolescents. Children with Apert and Crouzon had worse scores on disorders of socialization, attention, and internalization when compared to the normative group, with the worst results found in Apert. Factors that interfere with cognitive development: intracranial pressure, brain malformations, genetics, age at surgical correction, institutionalization, family environment, caregiver education, and socioeconomic status. Conclusion: the results contributed to a better understanding of the cognitive profile of patients with these syndromes and only by knowing about the neuropsychomotor, cognitive, and psychosocial skills and difficulties of these patients with Apert and Crouzon that health, school, and caregiver teams will be able to understand the perceptive capacity in the learning process of these patients deeply and will be able to offer the most appropriate stimuli at the most opportune time. Keywords: Apert, Crouzon, Neuropsyc, Tests, Development (AU).

Molecular Scalpels: The Future of Pediatric Craniofacial Surgery?

SUMMARY: CRISPR-Cas genome editing tools are among the most substantial advances in the life sciences in modern history. Single-dose gene therapies to correct pathogenic mutations have moved quickly from bench to bedside, with several therapeutics designed by CRISPR pioneers entering various stages of clinical investigation. Applications of these genetic technologies are poised to reshape the practice of both medicine and surgery. Many of the most morbid conditions treated by craniofacial surgeons are syndromic craniosynostoses caused by mutations in fibroblast growth factor receptor genes, including Apert, Pfeiffer, Crouzon, and Muenke syndromes. The fact that pathogenic mutations in these genes are recurrent in the majority of affected families presents a unique opportunity to develop "off-the-shelf" gene editing therapies to correct these mutations in affected children. The therapeutic potential of these interventions could reshape pediatric craniofacial surgery, potentially first eliminating the need for midface advancement procedures in affected children.

Comparison of Internal and External Distraction in Frontofacial Monobloc Advancement: A Three-Dimensional Quantification.

BACKGROUND: Crouzon syndrome is characterized by complex craniosynostosis and midfacial hypoplasia. Where frontofacial monobloc advancement (FFMBA) is indicated, the method of distraction used to achieve advancement holds an element of equipoise. This two-center retrospective cohort study quantifies the movements produced by internal or external distraction methods used for FFMBA. Using shape analysis, this study evaluates whether the different distraction forces cause plastic deformity of the frontofacial segment, producing distinct morphologic outcomes. METHODS: Patients with Crouzon syndrome who underwent FFMBA with internal distraction [Hôpital Necker-Enfants Malades (Paris, France)] or external distraction [Great Ormond Street Hospital for Children (London, United Kingdom)] were compared. Digital Imaging and Communications in Medicine files of preoperative and postoperative computed tomographic scans were converted to three-dimensional bone meshes and skeletal movements were assessed using nonrigid iterative closest point registration. Displacements were visualized using color maps and statistical analysis of the vectors was undertaken. RESULTS: Fifty-one patients met the strict inclusion criteria. Twenty-five underwent FFMBA with external distraction and 26 with internal distraction. External distraction provides a preferential midfacial advancement, whereas internal distractors produce a more positive movement at the lateral orbital rim. This confers good orbital protection but does not advance the central midface to the same extent. Vector analysis confirmed this to be statistically significant ( P < 0.01). CONCLUSIONS: Morphologic changes resulting from monobloc surgery differ depending on the distraction technique used. Although the relative merits of internal and external distraction still stand, it may be that external distraction is more suited to addressing the midfacial biconcavity seen in syndromic craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Craniofacial characteristics in Crouzon's syndrome: A systematic review and meta-analysis.

The purpose of this meta-analysis was to strengthen the credibility of primary research results by combining open-source scientific material, namely a comparison of craniofacial features (Cfc) between Crouzon's syndrome (CS) patients and non-CS populations. All articles published up to October 7, 2021, were included in the search of PubMed, Google Scholar, Scopus, Medline, and Web of Science. The PRISMA guidelines were followed to conduct this study. PECO framework was applied in the following ways: Those who have CS are denoted by the letter P, those who have been diagnosed with CS via clinical or genetic means by the letter E, those who do not have CS by the letter C, and those who have a Cfc of CS by the letter O. Independent reviewers collected the data and ranked the publications based on their adherence to the Newcastle-Ottawa Quality Assessment Scale. A total of six case-control studies were reviewed for this meta-analysis. Due to the large variation in cephalometric measures, only those published in at least two previous studies were included. This analysis found that CS patients had a smaller skull and mandible volumes than those without CS.in terms of SNA° (MD = -2.33, p = <0.001, I2 = 83.6%) and ANB°(MD = -1.89, p = <0.005, I2 = 93.1%)), as well as ANS (MD = -1.87, p = 0.001, I2 = 96.5%)) and SN/PP (MD = -1.99, p = 0.036, I2 = 77.3%)). In comparison to the general population, people with CS tend to have shorter and flatter cranial bases, smaller orbital volumes, and cleft palates. They differ from the general population in having a shorter skull base and more V-shaped maxillary arches.

Two-Center Review of Posterior Vault Expansion following a Staged or Expectant Treatment of Crouzon and Apert Craniosynostosis.

BACKGROUND: The timing of posterior cranial expansion for the management of intracranial pressure can be "staged" by age and dysmorphology or "expectant" by pressure monitoring. The authors report shared outcome measures from one center performing posterior vault remodeling (PCVR) or distraction (PVDO) following a staged approach and another performing spring-assisted expansion (SAPVE) following an expectant protocol. METHODS: Apert or Crouzon syndrome patients who underwent posterior expansion younger than 2 years were included. Perioperative outcomes and subsequent cranial operations were recorded up to last follow-up and intracranial volume changes measured and adjusted using growth curves. RESULTS: Thirty-eight patients were included. Following the expectant protocol, Apert patients underwent SAPVE at a younger age (8 months) than Crouzon patients (16 months). The initial surgery time was shorter but total operative time, including device removal, was longer for PVDO (3 hours 52 minutes) and SAPVE (4 hours 34 minutes) than for PCVR (3 hours 24 minutes). Growth-adjusted volume increase was significant and comparable. Fourteen percent of PCVR, 33% of PVDO, and 11% of SAPVE cases had complications, but without long-term deficits. Following the staged approach, 5% underwent only PVDO, 85% had a staged posterior followed by anterior surgery, and 10% required a third expansion. Following the expectant approach, 42% of patients had only posterior expansion at last follow-up, 32% had a secondary cranial surgery, and 26% had a third cranial expansion. CONCLUSION: Two approaches involving posterior vault expansion in young syndromic patients using three techniques resulted in comparable early volume expansion and complication profiles. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Crouzon syndrome complicated with binocular strabismus and extraocular muscle fibrosis: a case report.

BACKGROUND: Crouzon syndrome, a rare genetic disorder characterized by premature closure of coronal sutures, results in skull and facial deformities along with abnormal brain and ocular development. CASE PRESENTATION: Here, we report a case of a 27-year-old ethnic han male patient who presented with complex binocular strabismus secondary to Crouzon syndrome. At the time of surgery, extraocular muscles were found to be fibrotic and results of the pathological examination revealed degeneration of muscle fibers, which were replaced by adipose tissue. The entire exome sequencing DNA testing indicated that the patient and his father possessed the fibroblast growth factor receptor 2 (FGFR2) gene c.G812T:p.G271V heterozygous mutation. Binocular strabismus corrective surgery was performed in this patient with a satisfactory outcome. CONCLUSIONS: This case demonstrates that Crouzon syndrome patients can show an FGFR2 gene c.G812T:p.G271V mutation and display clinical symptoms such as extraocular muscle fibrosis, exotropia, exophthalmos, and a pointed head deformity.

A new class of transformable kirigami metamaterials for reconfigurable electromagnetic systems.

The rapid development of radio frequency (RF) components requires smart multifunctional materials that can adapt their physical shapes and properties according to the environment. While most current reconfigurable systems provide limited flexibility with high manufacturing cost, this research proposes to harness the transformable properties of kirigami-inspired multistable mechanical metasurfaces that can repeatedly deform and lock into different configurations to realize a novel class of low-cost reconfigurable electromagnetic structures with a broad design space. The metasurfaces are formed by designing kinematic-based unit cells with metallised coating that can provide adjustable resonant electromagnetic (EM) properties while rotating with respect to each other. Tailoring the cut length and geometry parameters of the patterns, we demonstrate programming of the topologies and shapes of different configurations. The influence of critical parameters on the structural multistability is illustrated by means of both a simplified energy model and finite element simulations. As examples of the reconfigurable electromagnetic devices that can be realized, we report the development of a tuneable half-wave dipole and two frequency selective surface (FSS) designs featuring isotropic and anisotropic responses. While the kirigami dipole can be tuned by mechanically stretching its arms, the FSSs exhibit distinct transmittance and reflectance spectra in each of the kirigami patterns stable states. The functionality of these kirigami devices is validated both by full-wave EM simulations and experiments. The proposed transformable structures can be mechanically actuated to tune the EM response in frequency or induce anisotropies for wave propagation.

Synchondrosis fusion contributes to the progression of postnatal craniofacial dysmorphology in syndromic craniosynostosis.

Syndromic craniosynostosis (CS) patients exhibit early, bony fusion of calvarial sutures and cranial synchondroses, resulting in craniofacial dysmorphology. In this study, we chronologically evaluated skull morphology change after abnormal fusion of the sutures and synchondroses in mouse models of syndromic CS for further understanding of the disease. We found fusion of the inter-sphenoid synchondrosis (ISS) in Apert syndrome model mice (Fgfr2S252W/+ ) around 3 weeks old as seen in Crouzon syndrome model mice (Fgfr2cC342Y/+ ). We then examined ontogenic trajectories of CS mouse models after 3 weeks of age using geometric morphometrics analyses. Antero-ventral growth of the face was affected in Fgfr2S252W/+ and Fgfr2cC342Y/+ mice, while Saethre-Chotzen syndrome model mice (Twist1+/- ) did not show the ISS fusion and exhibited a similar growth pattern to that of control littermates. Further analysis revealed that the coronal suture synostosis in the CS mouse models induces only the brachycephalic phenotype as a shared morphological feature. Although previous studies suggest that the fusion of the facial sutures during neonatal period is associated with midface hypoplasia, the present study suggests that the progressive postnatal fusion of the cranial synchondrosis also contributes to craniofacial dysmorphology in mouse models of syndromic CS. These morphological trajectories increase our understanding of the progression of syndromic CS skull growth.