Application and development of orthognathic surgery in treatment of syndromic craniosynostosis / 中国修复重建外科杂志

OBJECTIVE@#To summarize the application and recent development of orthognathic surgery in treating syndromic craniosynostosis.@*METHODS@#The related literature at home and abroad in recent years was extensively reviewed, and the indications, routine procedures, and protocols of orthognathic surgery in the treatment of syndromic craniosynostosis were summarized and analyzed.@*RESULTS@#Craniosynostosis is a common congenital craniofacial malformation. Syndromic craniosynostosis usually involves premature fusion of multiple cranial sutures and is associated with other deformities. Orthognathic surgery is the necessary and effective means to improve the midfacial hypoplasia and malocclusion. Le Fort I osteotomy combined with sagittal split ramus osteotomy are the common surgical options. Orthognathic surgery should combine with craniofacial surgery and neurosurgery, and a comprehensive long-term evaluation should be conducted to determine the best treatment plan.@*CONCLUSION@#Orthognathic surgery plays an important role in the comprehensive diagnosis and treatment of syndromic craniosynostosis. The development of digital technology will further promote the application and development of orthognathic surgery in the treatment of syndromic craniosynostosis.

Anterior fontanelle closure and diagnosis of non-syndromic craniosynostosis: a comparative study using computed tomography

Abstract Objective: Suspicion of early anterior fontanel (AF) closure is a common reason for referral to a pediatric neurosurgeon because of the suspected increased risk of developing craniosynostosis (CS) in spite of the absence of evidence in the literature. The aim of this study was to analyze the association between AF closure and the diagnosis of non-syndromic CS in Brazilian children. Methods: An observational and case-cohort study was conducted to compare the incidence of closed AF between healthy children (group 1) and children diagnosed with non-syndromic CS (group 2) at a pediatric neurosurgery referral center. The accuracies of completely closed AF and diagnosis of CS were assessed. Results: High-resolution three-dimensional reconstruction computed tomography scans were obtained for 140 children aged < 13 months, of whom 62.9% were boys and 37.1% were girls (p < 0.001). The most common types of non-syndromic CS were trigonocephaly (34, 48%) and scaphocephaly (25, 35.7%). Closed fontanel (27, 38.6%) was observed in both groups, and a sensitivity of 36.1%, specificity of 72%, the positive predictive value of 59%, and negative predictive value of 51% were observed in the patients diagnosed with CS when AF closure occurred before the age of 6 months. Conclusion: The results of this comparative study of AF closure and CS diagnosis suggest that early AF closure does not imply a diagnosis of CS. Pediatricians should be aware of the risk of misdiagnosis of CS in cases with a widely open AF in spite of the presence of CS.

Cloverleaf Skull Syndrome: A Rare Case Report

Cloverleaf deformity is an extremely rare skull deformity that happens when mutiple joints (sutures) between a baby's skull bones begin to fuse too premature. Also known as Kleeblattschädel syndrome, the cloverleaf deformity causes bossing at the front and sides of the skull, resembling a cloverleaf shape. Cloverleaf skull syndrome is an abnormal configuration of the calvaria classified as Craniosynostosis, consisting of premature ossification of cranial bones. It is a deformity characterized by a remarkable enlargement of the head, with a trilobed configuration of the frontal view, resembling a three leaved clover .The first report about such a syndrome in the literature occurred in 1973 and, over the years only some tens of cases have been documented worldwide. The present report describes a case of a severe craniofacial condition that is known as "cloverleaf skull syndrome". On Physical examination multiple malformations were observed, with altered craniofacial configuration, low set ear, hypertelorism, exopthalmos, syndactyly, closed anterior fontanelle, high arched palate, bilateral CTEV. The vast majority of patients with kleeblattschädel have mentalretardation and hydrocephalus. It is usually seen in thanatophoric dysplasia: classically type II only, severe Apert syndrome, severe Crouzon syndrome, Boston-type craniosynostosis & Carpenter syndrome. Craniosynostosis has an overall incidence of 1 in 2100 to 1 in 2500 live births with multi-suture craniosynostosis constituting about 5% of all the cases

Shprintzen-goldberg craniosynostosis: craniofacial and oral characteristics, diagnosis, and clinical management of a very rare syndrome / Craniossinostose de shprintzen-goldberg: características craniofaciais e orais, diagnóstico e manejo clínico de uma síndrome muito rara

ABSTRACT Shprintzen-Goldberg craniosynostosis syndrome, characterized by craniosynostosis and marfanoid habitus, is a very rare entity described in 75 individuals worldwide. This study aimed to present a case report of a 6-year-old female Brazilian child with Shprintzen-Goldberg's craniosynostosis syndrome. We described the craniofacial and oral characteristics and its clinical management, comparing to the existing literature. The patient presented with intellectual disability, craniosynostosis, ocular proptosis, low-set anomalous ears, and other skeletal and connective tissue defects. Oral features included malocclusion, micrognathia, pseudo-cleft palate, dental caries, and inefficient biofilm control. The treatment started with guidance on cariogenic foods, oral hygiene, and an indication to discontinue bottle feeding. Subsequently, the carious lesions were restored with composite resins, and the patient was referred for orthopedic surgery, orthodontic treatment, speech therapy, and nutritional counseling. The patient was followed for 5 years. This case report emphasizes the importance of knowing the craniofacial and oral characteristics for the diagnosis and clinical management of a female child with a rare Shprintzen-Goldberg's craniosynostosis syndrome. The case also highlights the need for oral health care in individuals with intellectual disabilities.

RESUMO A síndrome Shprintzen-Goldberg é uma entidade muito rara descrita em 75 pessoas, caracterizada por craniossinostose e habitus marfanóide. O objetivo deste estudo é apresentar o relato de um caso clínico de uma criança brasileira de 6 anos com síndrome da craniossinostose de Shprintzen-Goldberg, apresentando as características craniofaciais e orais e seu manejo clínico, comparando com a literatura. O paciente apresentava deficiência intelectual, craniossinostose, proptose ocular, orelhas anômalas de implantação baixa e outros defeitos esqueléticos e do tecido conjuntivo. As características orais incluíram má oclusão, micrognatia, pseudo fenda palatina, atividade de cárie e controle ineficiente do biofilme. O tratamento foi iniciado com orientações sobre alimentos cariogênicos, higiene oral e indicação de suspensão do uso de mamadeira. Em seguida, as lesões cariosas foram restauradas com resina composta e o paciente encaminhado para cirurgia ortopédica, tratamento ortodôntico, fonoaudiologia e nutricionista. O paciente foi acompanhado periodicamente por 5 anos. Este relato de caso enfatiza a importância do conhecimento das características craniofaciais e orais para o diagnóstico e manejo clínico de uma criança do sexo feminino com síndrome de craniossinostose de Shprintzen-Goldberg rara, destacando a necessidade de cuidados com a saúde bucal em indivíduos com deficiência intelectual.

Simulation method of skull remodellingsurgeryfor infant with craniosynostosis / 生物医学工程学杂志

Craniofacial malformation caused by premature fusion of cranial suture of infants has a serious impact on their growth. The purpose of skull remodeling surgery for infants with craniosynostosis is to expand the skull and allow the brain to grow properly. There are no standardized treatments for skull remodeling surgery at the present, and the postoperative effect can be hardly assessed reasonably. Children with sagittal craniosynostosis were selected as the research objects. By analyzing the morphological characteristics of the patients, the point cloud registration of the skull distortion region with the ideal skull model was performed, and a plan of skull cutting and remodeling surgery was generated. A finite element model of the infant skull was used to predict the growth trend after remodeling surgery. Finally, an experimental study of surgery simulation was carried out with a child with a typical sagittal craniosynostosis. The evaluation results showed that the repositioning and stitching of bone plates effectively improved the morphology of the abnormal parts of the skull and had a normal growth trend. The child's preoperative cephalic index was 65.31%, and became 71.50% after 9 months' growth simulation. The simulation of the skull remodeling provides a reference for surgical plan design. The skull remodeling approach significantly improves postoperative effect, and it could be extended to the generation of cutting and remodeling plans and postoperative evaluations for treatment on other types of craniosynostosis.

Craneosinostosis, una perspectiva pediátrica / Craniosynostosis, a pediatric perspective

La craneosinostosis se define como el cierre prematuro de una o más suturas del cráneo, que se manifiesta por una forma anormal de la cabeza. Es una condición infrecuente, pero requiere ser reconocida y derivada oportunamente a Neurocirugía para prevenir complicaciones. El objetivo de esta revisión es describir las características clínicas y genéticas más frecuentes de esta patología, su clasificación de acuerdo a la forma del cráneo y los signos más característicos para lograr reconocerla oportunamente. Se realizó una búsqueda de artículos científicos en bases de datos Pubmed, Scielo y EMBASE con las palabras craneosinostosis, plagio, escafo y braquicefalia. Se seleccionaron artículos en español e inglés que describieran las características de la patología y su manejo, optando por revisiones sistemáticas o recomendaciones de sociedades científicas cuando estuvieran disponibles. La craneosinostosis puede presentarse en forma aislada o asociada a otras deformidades. Su clasifi cación depende de la(s) sutura(s) afectada(s), lo que lleva a la forma característica del cráneo y de la presencia de otras malformaciones. Suele diagnosticarse y derivarse de forma tardía, lo que se asocia a complicaciones como hipertensión endocraneana y alteración del desarrollo encefálico. La cirugía precoz tiene menor comorbilidad y mejores resultados estético. En conclusión, la forma anormal del cráneo debe hacer sospechar la presencia de craneosinostosis, aunque se presente en forma aislada. El manejo quirúrgico antes del año de vida se asocia a mejor pronóstico.

Craniosynostosis is defined as the premature fusion of one or more skull sutures, characterized by an abnormal shape of the head. It is a rare condition but should be recognized and timely referred to Neurosurgery in order to prevent complications. The objective of this review is to describe the most frequent clinical and genetic characteristics of this pathology, its classification according to the shape of the skull, and the most characteristic signs to achieve timely recognition. A search for scientific articles in Pubmed, Scielo, and EMBASE databases was performed using the terms craniosynostosis, plagiocephaly, scaphocephaly, and brachycephaly. We selected articles in Spanish and English that described the characteristics of the pathology and about its management, choosing systematic reviews or recommendations from scientific societies when available. Craniosynostosis may occur in isola tion or associated with other deformities. Its classification depends on the affected suture(s), leading to the characteristic shape of the skull and the presence of other malformations. This condition is usually diagnosed and referred late, which is associated with complications such as intracranial hy pertension and impaired brain development. Early surgery has less comorbidity and better esthetic results. In conclusion, the abnormal shape of the skull must raise the suspicion of craniosynostosis, even if it occurs in isolation. Surgical management before one year of life is associated with a better prognosis.

Craniossinostoses não sindrômicas: uma análise retrospectiva / Non-syndromic craniosynostosis: a retrospective analysis

Introdução: Craniossinostoses são definidas como fusões prematuras de uma ou mais suturas cranianas. Podem ser classificadas de acordo com as suturas envolvidas, malformações correlacionadas e alterações genéticas, podendo ser sindrômicas ou não. Sua incidência estimada em 1:2.000-2.500 nascidos vivos, sendo apenas 8% sindrômicas. Existem múltiplas opções cirúrgicas para a correção destas malformações, e o encaminhamento precoce para centros especializados no tratamento destes doentes é necessário para uma melhor indicação terapêutica. O diagnóstico é pautado no exame físico e tomografia computadorizada. O tratamento cirúrgico traz melhoras significativas estéticas e no convívio social dos pacientes. O objetivo é analisar os dados, retrospectivamente, dos pacientes submetidos à cirurgia para correção de craniossinostoses, no HC da UNESP-Botucatu. Métodos: Análise retrospectiva dos prontuários de pacientes submetidos à correção cirúrgica de craniossinostoses entre 2012 e 2017. Resultados: A prevalência de escafocefalia e trigonocefalia foi igual (38,5%), idade média de abordagem de 24 meses, o tempo cirúrgico médio foi de 3h48min, sendo menor nas escafocefalias, 2h50min. Todos os pacientes receberam transfusão de sangue no perioperatório com média percentual em relação ao volume sanguíneo pré-operatório de 24,9% e fizeram pós-operatório em UTI pediátrica. Não houve óbitos ou complicações, e os resultados tidos como bom pela equipe e familiares. Conclusão: O tratamento cirúrgico aberto das craniossinostoses não sindrômicas é um procedimento seguro. As equipes de cirurgia plástica conseguem alcançar resultados equiparáveis aos descritos na literatura, com baixas taxas de complicações, bom resultado estético, quando executado de maneira adequada, e com abordagem multidisciplinar.

Introduction: Craniosynostoses are defined as premature fusions of one or more cranial sutures. They can be classified according to the sutures involved, correlated malformations and genetic alterations, and maybe syndromic or not. Its incidence is estimated at 1: 2,000-2,500 live births, with only 8% being syndromic. There are multiple surgical options for the correction of these malformations, and early referral to specialized centers for the treatment of these patients is necessary for a better therapeutic indication. The diagnosis is based on physical examination and computed tomography. The surgical treatment brings significant aesthetic and social improvements to patients. The objective is to analyze retrospectively patient data who underwent surgery to correct craniosynostosis at HC of UNESP-Botucatu. Methods: Retrospective analysis of the medical records of patients who underwent surgical correction of craniosynostosis between 2012 and 2017. Results: The prevalence of scaphocephaly and trigonocephaly was the same (38.5%). The mean age of the approach was 24 months; the mean surgical time was 3h48min, lower in scaphocephaly, 2h50min. All patients received blood transfusions in the perioperative period, with a mean percentage of 24.9% in relation to the preoperative blood volume. They underwent postoperative in a pediatric ICU. There were no deaths or complications, and the results were considered good by the team and family. Conclusion: Open surgical treatment of non-syndromic craniosynostosis is a safe procedure. Plastic surgery teams can achieve results comparable to those described in the literature, with low complication rates, good aesthetic results when performed correctly and with a multidisciplinary approach.

Deviation of paradigmatic mutations found in Shprintzen-Goldberg syndrome

Shprintzen-Goldberg (S-G) Syndrome known as rare congenital connective tissue disorder where craniosynostosis and marfanoid habitus found to be the usual presentation. Craniofacial dysmorphism with multi-organ involvement documented to be amongst prominent features of this syndrome. Case characteristics is five-month-old male infant with craniosynostosis, and motor developmental delay was evaluated for congenital connective tissue disorder. Dysmorphic craniofacial features like dolichocephaly, triangular forehead, ocular hypertelorism, micrognathia and retrognathia were noticed besides congenital umbilical hernia, empty scrotal sac, clinodactyly with long slender fingers, hyper-mobile joints, hypotonia. Subsequent investigations revealed normal male karyotype (46, XY) while genetic analysis depicted missense mutations in six different genes. Conventionally, mutation in SKI gene reported for its' associated with S-G syndrome where dysregulation of TGF-? signaling was discussed as the primary reason. In the present case discussed here, it was found to have polygenic mutational association where few novel genetic mutations were seen.

Apert Syndrome Presenting with Omphalocele

Apert syndrome is a congenital acrocephalosyndactylysyndrome. It is mainly presented by craniosynostosis,syndactyly of the hands and feet and dysmorphic facialfeatures. The condition has an autosomal dominantinheritance assigned to mutations in the FibroblastGrowth Factor Receptors (FGFR-2) gene. The reportedcase is a 9 months old boy with clinical suspicion ofApert syndrome as he had turricephaly, stubby handswith short fingers, omphalocele measuring 10 × 10 cm,bilateral undescended testes, Congenital TalipesEquinovarus (CTEV) and polydactyl and syndactyl ofgreat and second toes. He was developmentallynormal. The omphalocele was surgically repaired.Early diagnosis and intervention helps rehabilitation ofpatients with Apert syndrome and help them to lead abetter life.

Review of spring-assisted surgery in craniosynostosis / 中华整形外科杂志

Traditional total cranial vault reconstruction for craniosynostosisis traumatic with long operative time and many serious complication. A brand new surgery technique, spring-assisted surgery(SAS), has been invented and performed for 20 years. It showed that SAS has the advantages of less invasive and good result. It has gradually become one of the important methods to treat early craniosynostosis. This article reviewed the SAS related literatures in the past 20 years, focusing on various surgical methods of SAS and possible surgical complications. The osteogenesis principle, prognosis and development of SAS were also summerized.

Management of nasal deformity with unicoronal craniosynostosis using the nasal bones were wedge removed / 中华整形外科杂志

Objective@#To investigate the treatment of nasal deformity in patients with unicoronal craniosynostosis.@*Methods@#In patients over 6 months old, the nasal bones were wedge-removed without fixation. The management of all patients with unicoronal craniosynostosis was distraction osteogenesis of pedicled unilateral frontal bone flap.@*Results@#Postoperative extended distance of the frontal bone was 28—41 mm (mean, 35.4 mm). After extension, three-dimensional reconstruction of cranial CT was carried out, which showed that CVAI was 0.8—1.2 (mean, 0.98), tending to normal. After discharge, dynamic cranial braces were put on for 1 year. Postoperatively, the children were followed up for 8—36 months (mean, 28 months). The shape and nasal deformity of all children were improved compared with those before surgery.@*Conclusions@#Nasal wedge resection should be used to correct nasal deformity in children over 6 months with unicoronal craniosynostosis.

Treatment of syndromic craniosynostosis with distraction osteogenesis / 中华整形外科杂志

Objective@#To explore the effect of distraction osteogenesis in the treatment of syndromic craniosynostosis.@*Methods@#The clinical data of 6 children with syndromic craniosynostosis from January 2014 to September 2018 were retrospectively analyzed. There were 5 males and 1 female, aged from 1 month and 21 days to 6 years and 1 month, with an average age of 30 months. There were 3 Crouzon syndrome, 1 Pfeiffer syndrome, 1 Vogt syndrome (ACS Ⅱ) and 1 Clove leaf skull syndrome. The distraction osteogenesis apparatus was used in this procedure. The distraction osteogenesis was prolonged twice a day, 0.4 mm each time, and the prolongation was stopped when the skull shape was significantly improved. Three-dimensional CT scans of the skull were reviewed after 6 months, suggesting that distraction osteogenesis was good, then the lengthener was removed. Complications were recorded, and extended distances were measured. Cranial indices before and after operation were compared to evaluate the efficacy, safety and feasibility of the operation.@*Results@#The procedure of operation and traction was successful in all 6 children. There were no complications such as cranial spinal fluid (CSF) leakage or intracranial infection occurred. The increased distance was (19.1±3.3) mm, ranging from 15.2 to 25.6 mm. The preoperative cranial index was 89.6 ±7.3, while the postoperative cranial index was 74.2 ±3.6. All patients were followed up from 3 to 20 months, with the average of 14 months. The posterior cranial flatness was improved, and the patients were satisfied with the surgical results.@*Conclusions@#Distraction osteogenesis is effective and reliable in treating the premature fusion of cranial suture, and it produces excellent result with low rate of CSF leak and infection.

A case of Crouzon syndrome with plagiocephaly and scaphocephaly / 中华整形外科杂志

In October 2017, a female patient, 3 years and 5 months of age, with Crouzon syndrome, associated with multiple craniosynostoses was admitted to Plastic Surgery Hospital. Combined intracranial and extracranial approaches of fronto-orbital advancement and cranial suture release were performed to treat plagiocephaly and scaphocephaly. The patient′s families were investigated. Corresponding mutations were detected by DNA sequencing. Therapeutic effect was satisfactory. The mutation was inherited for 5 generations. Genomic sequencing results showed that the exons of fibroblast growth factor receptor 2 gene in the child was mutated, which excessively activated downstream signals and caused craniosynostosis.

Three dimensional finite element biomechanical analysis of unilateral coronal synostosis and reconstruction / 中华整形外科杂志

Objective@#To establish finite element models of skull, fronto-orbital advancement and fronto-orbital distraction osteogenesis of craniosynostosis, to analyze the mechanical characteristics of skull base and fronto-orbital operation area, so as to guide the later application of tractors.@*Methods@#One 6-year-old male patient with unilateral coronal synostosis was enrolled in October 2015. Three-dimensional (3D) computed tomography (CT) scan of skull was performed. DICOM data was imported into Mimics 17.0 for contour extraction and cranial 3D reconstruction. The skull model was processed by Mimics, Geomagic Studio 12.0, Hypermesh 12.0 and other software to establish a three-dimensional finite element model. The unilateral and bilateral fronto-orbital anterior osteotomy models were simulated respectively. The mechanical analysis was performed at point A in forehead area and point B in temporal area. Three different groups of traction forces were loaded: (1) 50 Newton for point A, 50 Newton for point B; (2) 80 Newton for point A and 50 Newton for point B; (3) 100 Newton for point A and 50 Newton for point B, to obtain the optimized traction force.@*Results@#Stress analysis was performed on established cranial finite element model, as well as unilateral and bilateral fronto-orbital advancement procedures. The stress distribution of the anterior and middle cranial fossae was found to be concentrated. After unilateral fronto-orbital advancement, the stress of anterior cranial fossa, especially the affected side, was decreased. The stress on both side in anterior cranial fossa was decreased after bilateral fronto-orbital advancement. After force was applied to point A and point B, the optimum deviation result at supraorbital notch point, midpoint of supraorbital margin, frontal temporal point and frontal zygomatic suture point in 3D (Deviation result of X value: -29.4%, -20.5%, -8.6%, -9.3%; Deviation result of Y value: 20.9%, 31.5%, 73.0%, 539.4%; Deviation result of Z value: 4.4%, 1.9%, 0.1%, 11.8%) demonstrated the application of traction force can inwardly, downwardly and forwardly move the bone flap. The optimized traction was 80 Newton at point A and 50 Newton at point B by preliminary assessment.@*Conclusions@#The finite element analysis of the fronto-orbital advancement can be used for more accurate preoperative simulation, to clarify the influence of fronto-orbital advancement on craniofacial morphology and development, as well as skull base. It also facilitates surgical decision and predicts the postoperative distraction vectors.

Multiple osteotomies with combined distraction osteogenesis for non-syndromic scaphocephaly / 中华整形外科杂志

Objective@#To evaluate the clinical effect of multiple osteotomies combined with distraction osteogenesis in the treatment of non-syndromic scaphocephaly infants and young children.@*Methods@#Clinical records of 8 non-syndromic scaphocephaly patients, during January 2017 to October 2018, from the Department of Burn and Plastic Surgery, Children′s Hospital of Nanjing Medical University, were retrospectively analyzed. There were 3 males and 5 females, aged from 3 to 28 months, with an average of 13 months. Among them, 4 cases were typical scaphocephaly, 2 cases were anterior scaphocephaly and 2 cases were posterior scaphocephaly. According to the subtypes of scaphocephaly, different osteotomy methods and distraction directions were designed. The cranial vault was osteotomized by piezosurgery osteotomy in a multiple way. The dura did not separate from cranial flap. The internal distractors were installed subsequently. After a mean latency period of 5 days, the devices were activated at a rate of 0.4-0.6 mm/d. Distractor was extended twice a day. After 10-15 days activation period and 6 months consolidation, all the distractors were removed. Three-dimensional CT scans were performed before and after operation to measure the anteroposterior and bitemporal diameters, so as to calculate the cranial index.@*Results@#Follow-up ranged from 6 to 14 months (average, 10 months). The average anteroposterior length of 8 children was(158.2±12.9) mm before operation, and (145.5±14.2) mm after operation, which was shortened(12.6±3.4) mm. Bitemporal diameters was(99.6±8.6) mm preoperatively and(113.9±7.5) mm postoperatively, which was widened (14.2±2.8) mm. Preoperative and postoperative cranial index was 63.2 and 78.3, respectively. The scaphocephalic head shape was significantly improved in all patients. There was no serious complications such as skull necrosis or intracranial infection.@*Conclusions@#Different types of multiple osteotomy combined with distraction osteogenesis are useful to correct various scaphocephaly of infants and children, by improving skull shape and cranial index.

Progress and considerations in the treatment and diagnosis of pediatric craniomaxillofacial surgery / 中华整形外科杂志

Research progress and clinical achievements of pediatric craniomaxillofacial surgery in recent 30 years were reviewed. It highlighted significant discoveries and innovations in the field of craniomaxillofacial abnormalities, such as craniosynostosis, facial cleft, maxillary and mandibular malformation, and craniomaxillofacial trauma. Additionally, the author elaborated his opinions on the further developments of pediatric craniomaxillofacial surgery.

Deformidades craneales sinostósicas y no sinostósicas / Synostosic and non-synostosic cranial deformities

Las alteraciones de la forma del cráneo han aumentado en las últimas décadas a expensas de las plagiocefalias, como consecuencia de las recomendaciones de la Academia Americana de Pediatría de dormir al lactante en decúbito supino. El clínico debe diferenciar entre plagiocefalia posicional y craneosinostosis, ya que la conducta terapéutica será potencialmente neuroquirúrgica de comprobar una sinostosis craneal. Si bien la tomografía de cráneo tridimensional con ventana ósea es el estudio de mayor sensibilidad y especificidad diagnóstica, la mayoría de los casos pueden confirmarse por la clínica, reservando la radiografía simple o de preferencia la ecografía con enfoque de suturas para casos dudosos. Las craneosinostosis deben derivarse tempranamente a un equipo craneofacial para definir la indicación, oportunidad y técnica quirúrgica más adecuada para evitar futuras complicaciones neurocognitivos y psicosociales. Los niños con plagiocefalias posicionales, independientemente del tratamiento de la deformidad craneal, pueden tener más riesgo de retraso del desarrollo motor. Para su corrección se sugiere reposicionamiento y fisioterapia en formas leves a moderadas, reservando el uso de ortésis craneal en formas graves.

In the last decades alterations in the skull shape have increased at the expense of plagiocephaly, as consequence of the American Academy of Pediatrics recommendations to sleep the infant in the supine position. The clinician must differentiate between positional plagiocephaly and craniosynostosis, since if a cranial synostosis is proven, the therapeutic behavior will be potentially neurosurgical. Although three-dimensional skull tomography with bone window is the study of greater sensitivity and diagnostic specificity, the majority of cases can be confirmed by the clinic, reserving the radiography or ultrasound with a suture approach for doubtful cases. Craniosynostosis must be early referral to a craniofacial team to define the indication, opportunity and most appropriate surgical technique, in order to avoid future neurocognitive and psychosocial complications. Children with positional plagiocephaly regardless of the treatment of cranial deformity may have a higher risk of motor development delay. For correction, repositioning and physiotherapy are suggested in mild to moderate forms, reserving the use of cranial orthesis in severe forms.

Craneosinostosis sindrómica: Características craneodentofaciales, tratamiento ortodón-tico-quirúrgico y factores asociados a tipos de síndrome / Craniossinostose sindrômica: Características craniodentofaciais, tratamento ortodônti-co-cirúrgico e fatores associados aos tipos de síndrome / Syndromic Craniosynostosis: Craniodentofacial features, orthodontic-surgical treatment and factors associated to types of syndrome

Objetivos: Describir las características craneodentofaciales, tratamientos ortodónticos-quirúrgicos y establecer una aso-ciación entre los tipos de síndrome presentes en pacientes con craneosinostosis sindrómica (CS). Material y métodos: Estudio retrospectivo de registros médicos y de ortodoncia de pacientes con CS. Los datos se recogieron en una forma específica y se sometieron a un análisis estadístico descriptivo para observar la distribución de frecuencias y se utilizó la prueba de Chi cuadrado con un nivel de significación del 5% para asociar el síndrome y los tipos de variables. Resultados: El síndrome de Crouzon fue el tipo predominante (59.2%) y la braquicefalia fue el tipo más frecuente de craneosinostosis (63.6%). Hubo una asociación significativa entre las variables braquicefalia (p = 0,014), presencia de paladar hendido (p = 0,043), mordida cruzada posterior (p = 0,013), distracción osteogénica realizada por elásticos intermaxilares (p = 0,030), barra de Erich (p = 0.007) y la extracción (p = 0.041) y los síndromes estudiados. Conclusión: Los pacientes con CS a menudo tienen cambios craneodentofaciais y algunas variables tienen asociaciones significativas en relación con los tipos de síndromes.

Objectives: Describe the craniodentofacial characteristics, orthodontic-surgical treatments and establish an association between the syndrome types present in patients with syndromic craniosynostosis (SC). Material and methods: Retros-pective study of medical and orthodontic records of patients with SC. Data was collected on a specific form and subjected to descriptive statistical analysis to observe the distribution of frequencies and chi-square test with level of significance of 5% was used to associate syndrome and the types of variables. Results: Crouzon syndrome was the predominant type (59.2%) and brachycephalia was the most prevalent type of craniosynostosis (63.6%). There was a significant association between the variables brachycephaly (p=0.014), presence of cleft palate (p=0.043), posterior cross bite (p=0.013), distrac-tion osteogenesis performed by intermaxillary elastics (p= 0.030), Erich bar (p= 0.007) and extraction (p=0.041) and the syndromes studied. Conclusion: Patients with SC often have craniodentofaciais changes and some variables had signifi-cant associations in relation to the types of syndromes.

Descrever as características craniodentofaciais, tratamentos ortodôntico-cirúrgicos e estabelecer uma associação entre os tipos de síndrome presentes em pacientes com craniossinostose sindrômica (CS). Material e métodos: Estudo retrospectivo de prontuários médicos e ortodônticos de pacientes com CS. Os dados foram coletados em formulário específico e submetidos à análise estatística descritiva para observar a distribuição das frequências e o teste qui-quadrado com nível de significância de 5% foi utilizado para associar a síndrome e os tipos de variáveis. Resultados: A síndrome de Crouzon foi o tipo predominante (59,2%) e a braquicefalia foi o tipo mais prevalente de craniossinostose (63,6%). Houve associação significativa entre as variáveis braquicefalia (p = 0,014), presença de fissura de palato (p = 0,043), mordida cruzada posterior (p = 0,013), distração osteogênica realizada pelos elásticos intermaxilares (p = 0,030), barra de Erich (p= 0,007) e extração (p = 0,041) e as síndromes estudadas. Conclusão: Pacientes com CS frequentemente apresentam alterações craniodentofaciais e algumas variáveis apresentaram associações significativas em relação aos tipos de síndromes.

O papel da simulação na prática cirúrgica e a criação de uma nova ferramenta para treinamento neurocirúrgico / The role of simulation in surgical practice and the creation of a new tool for neurosurgical training

OBJETIVOS: Testar uma nova ferramenta para educação neurocirúrgica, um "quebra-cabeça" para simular a correção cirúrgica de craniossinostose (especificamente escafocefalia), usando a técnica "H" de Renier. MÉTODOS: O modelo do crânio foi criado por meio da obtenção de imagens de tomografia computadorizada multi slice (camadas de 1mm) no formato Digital Imaging and Communications in Medicine (DICOM). Essas imagens foram então processadas usando um algoritmo computadorizado para gerar um modelo tridimensional em resina. O quebra-cabeça e suas possibilidades de treinamento foram avaliados qualitativamente por uma equipe de neurocirurgiões especialistas. Após, os especialistas avaliaram a aplicação da ferramenta para residentes em neurocirurgia e os residentes também avaliaram a experiência. RESULTADOS: Participaram da avaliação cinco especialistas neurocirurgiões e 10 residentes em neurocirurgia. Todos consideraram a ferramenta positiva para o treinamento proposto. Os especialistas fizeram observações sobre o quanto o modelo pode ser interessante por instigar a compreensão dos porquês das etapas cirúrgicas e como atuar em cada uma delas. Segundo a percepção dos especialistas, após o uso do modelo os residentes apresentaram melhor clareza na visualização tridimensional do passo a passo, indiretamente auxiliando na compreensão da técnica cirúrgica. Além disso, ressaltaram uma notável redução de erros a cada tentativa de montagem do quebra-cabeça. Os residentes consideraram ser um método de ensino que torna a avaliação objetiva e clara. Entre os entrevistados, a nota fornecida ao simulador teve média de 9,9. CONCLUSÕES: O quebra-cabeça em formato de crânio mostrou-se uma ferramenta complementar para o ensino, que permite diferentes graus de imersão e realismo. Fornece uma noção de realidade física, oferecendo informações simbólicas, geométricas e dinâmicas, com rica visualização tridimensional. O uso do simulador pode, potencialmente, abreviar e melhorar a curva de aprendizado dos neurocirurgiões, de uma forma segura.

AIMS: To test a new tool for neurosurgical education, a "puzzle" to simulate the craniosynostosis surgical correction (specifically scaphocephaly) using Renier's "H" technique. METHODS: The cranial model was created by obtaining images through a multi slice (1 mm) CT scan in the Digital Imaging and Communications in Medicine (DICOM) format. This information was then processed using a computing algorithm to generate a three-dimensional biomodel in resine (performed on a computer or via computer simulation). The puzzle and its training possibilities were evaluated qualitatively by a team of expert neurosurgeons. Subsequently the experts evaluated the application of the tool for residents in neurosurgery, and the residents also evaluated the experience. RESULTS: Five experts neurosurgeons and 10 neurosurgery residents participated in the evaluation. All considered the tool positive for the proposed training. The experts have commented on how interesting the model may be by instigating the understanding of the reasons for each surgical step and how to act in them. According to the experts perceptions, the residents presented better clarity in the three-dimensional visualization of the step by step, indirectly aiding in the understanding of the surgical technique. In addition, they noted a notable reduction of errors with each attempt to assemble the puzzle. Residents considered it to be a teaching method that makes assessment objective and clear. Among the interviewers, 9,9 was the averaged note given to the simulator. CONCLUSIONS: The puzzle in cranial shape can be a complementary tool, allowing varying degrees of immersion and realism. It provides a notion of physical reality, offering symbolic, geometric and dynamic information, with rich tridimensional visualization. The simulator use may potentially improve and abbreviate the surgeons learning curve, in a safe manner.

The volumetric change of cranial cavity in the patients with craniosynostosis after fronto-orbital advancement / 中华整形外科杂志

Objective@#To assess the surgical outcome of fronto-orbital advancement (FOA)in the patients with craniosynostosis by quantitative analysis of the cranial volume using the three-dimensional digital technology.@*Methods@#From January 2010 to February 2016, 10 cases of craniosynostosis were included in the study. The average age of patients was 3.2 years (1 year old to 6.5 years old). All patients underwent modified FOA.Based on the uncompressed digital imaging and communications in medicine (DICOM) data of 3D cranial CT scan, the intracranial volume was measured by Proplan CMF 1.4.The average follow-up time was 23.6 months. We quantitatively analysed brain volume in ten patients with craniosynostosis, comparing with the normal data of age- and sex-matched healthy controls. Paired t tests were performed to compare mean value of the variables.@*Results@#The volume of intracranial cavity was(1 117.39 ± 105.83) ml before surgery and the preoperative volume of intracranial cavity in the control group was (1 251.37 ± 114.35) ml. The difference between the preoperative group and the preoperative control group was statistically significant(P=0.00030). The volume of intracranial cavity was (1 260.84 ± 122.08) ml after surgery. The volume was increased significantly after surgery(P=0.00002). There was no significant difference between the postoperative group and the preoperative control group(P=0.60000). The volume of intracranial cavity was (1 368.65 ± 94) ml at least one year follow-up and the cranial volume of the control group was (1 321.96 ± 80.21) ml. There was no statistically significant difference between the two groups(P=0.07000). There were statistically significant differences between the cranial volume at two weeks after operation and that of one year follow-up (P=0.00300). This result showed that the cranial vault and brain of patients grew and developed normally after FOA surgery.@*Conclusions@#Preoperative cerebral volume in the patients with craniosynostosis was lower than normal. The cranial volume has been effectively expanded and basically reached the normal level after fronto-orbital advancement. Especially, the intracranial volume maintained normal during follow-up.