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.

Perioperative management and complication control of Le Fort Ⅲ osteotomy in children with syndromic craniosynostosis / 中华口腔医学杂志

Objective: To summarize the preliminary efficacy, perioperative management and complications of Le Fort Ⅲ osteotomy and midface distraction in patients with syndromic craniosynostosis by retrospective analysis, and to provide clinical experience for reference. Methods: From October 2017 to January 2020, 20 patients with syndromic craniosynostosis underwent Le Fort Ⅲ osteotomy and distraction in The Department of Oral and Maxillofacial Surgery of Peking University International Hospital, including 11 males and 9 females, were involved. The median age was 7 years (1.5 to 15 years). Preoperative risk prevention plan was put forward by multidisciplinary evaluation, and preoperative intervention was carried out. The diagnostic data of SNA, airway volume, polysomnography (PSG), ophthalmology and occlusal relationship were obtained through specialized examination, and osteotomy and distraction surgical plan was formulated through virtual surgical planning. CT was taken 1 week and 3, 6, 12 months after operation, PSG and eye protrudence examination were conducted to evaluate the therapeutic effect, syndrome type, multiple disciplinary treatment (MDT) intervention, occurrence and outcome of complications were summarized. Results: There were 15 cases of Crouzon syndrome and 5 cases of Pfeiffer syndrome. Sleep apnea was the first complaint in 18 cases and exophthalmia in 2 cases. Preoperative interventional therapy included 4 cases of adenoid surgery, 2 cases of continuous positive airway pressure and 2 cases of maxillary expansion. The most common surgical complications were accidental fracture (14/20 cases, 70%), cerebrospinal fluid fistula (2 cases), internal carotid cavernous sinus fistula (1 case), postoperative hyponatraemia (5 cases), crying syndrome (2 cases), wound infection (2 cases), trichiasis of lower eyelid (4 cases), and nasal malformation (1 case). Three cases underwent unplanned secondary surgery. SNA, airway volume and mean percutaneous arterial oxygen saturation (SpO2) six months after operation were significantly higher than those before operation (F=10.09, P=0.001; F=5.13, P<0.001; F=10.78, P=0.001), and the protrusion and apnea hypopnea index were significantly lower than those before surgery (F=6.73, P=0.010; F=18.47, P<0.001). There were no significant differences in SNA, airway volume, mean SpO2, ophthalmology between 6 months after surgery and 1 year after surgery (P>0.05). Conclusions: Perioperative safety assessment and early intervention of MDT is an effective diagnosis and treatment model of Le Fort Ⅲ osteotomy and distraction for syndromic craniosynosis. The operative complications are mainly local, and systemic complications are controllable.

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.

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.

Cráneo en trébol y fisura facial bilateral / Cloverleaf skull and bilateral facial clefts

Introducción: El síndrome del cráneo en trébol, o síndrome de Kleeblattschädel, es una malformación rara en la cual el cráneo presenta un aspecto de trébol. Es causado por el cierre prematuro de varias suturas, evidenciándose desde antes del nacimiento. Objetivo: presentar nuestra experiencia en un caso de síndrome del cráneo en trébol, y actualizar la información de la literatura. Caso clínico: lactante de sexo femenino, 5 meses de edad, diagnósticos al nacimiento de Fisura labio-Palatina e Hidrocefalia. A los 30 días de vida se instaló válvula ventrículo peritoneal, y se procedió a enucleación ocular bilateral por proceso infeccioso. Se controla en Genética donde se confirma macrocefalia y craneosinostosis tipo cráneo en trébol. El estudio citogenético 46XX, Ecocardiografía normal, TAC de cerebro mostró anomalias múltiples asociadas a hidrocefalia y malformaciones inespecíficas. Conclusión: El cráneo en trébol puede presentarse aisladamente o asociado a otras anomalías congénitas, conformando varios síndromes de craneosinostosis, como Crouzon, Pfeiffer o Carpenter. También puede ser componente de la secuencia de rotura amniótica o de diversas displasias, como la campomélica, tanatofórica tipo ii, o la distrofia torácica asfixiante de Jeune. El caso descrito no cumple con todas las características necesarias para incluirlo dentro de un síndrome específico, y no habiendo antecedentes familiares que sugieran patrón de herencia ni anomalías cromosómicas se concluye que se trata de un caso de anomalías congénitas de presentación esporádica.

Introduction: Cloverleaf skull syndrome, or Kleeblattschädel syndrome, is a rare malformation in which the skull has a cloverleaf appearance. It is caused by the premature closure of several sutures, being evident before birth. Objective: To present our experience in a case of cloverleaf skull syndrome, and update the information from the literature. Clinical case: A female infant of 5 months of age, diagnosed at birth with cleft lip and palate and hydrocephaly. A peritoneal ventricle valve was implanted at 30 days of life, and an ocular enucleation was performed due to an infectious process. The patient was followed-up in Genetics, where it confirmed a macrocephaly and craniosynostosis type cloverleaf skull. The 46XX cytogenetic study and echocardiography were normal. The brain CT scan showed multiple anomalies associated with hydrocephaly and non-specific malformations. Conclusion: Cloverleaf skull may be present in isolated form or associated with other congenital abnormalities, leading to various craniosynostosis syndromes, such as Crouzon, Pfeiffer or Carpenter. It may also be a component of the amniotic rupture sequence or to different dysplasias, such as campomelic dysplasia, thanatophoric dysplasia type 2, or the asphyxiating thoracic dystrophy of Jeune. The case presented does not fulfil all the characteristics needed to be included within a specific syndrome, and on not having a family history that suggests a hereditary pattern or chromosome abnormalities, it is concluded that it is a case of a congenital anomaly of sporadic presentation.

Nautilus: craniotomia dinâmica - nova técnica cirúrgica e resultados preliminares / Nautilus-shaped dynamic craniotomy: a new surgical technique and preliminary results

INTRODUÇÃO: Considerando-se que as craniossinostoses são afecções basicamente suturais, o fato de o cérebro estar aprisionado em um compartimento fechado, que não possui a complacência necessária para acompanhar seu crescimento, se constitui no desafio principal de seu tratamento. O objetivo do tratamento é restabelecer a complacência da sutura estenótica e corrigir a deformidade craniana compensatória. Este trabalho propõe a associação de osteotomia helicoide à distração osteogênica proporcionada pelo uso das molas distratoras para remodelar defeitos craniofaciais causados por craniossinostoses. MÉTODO: Entre julho de 2010 e julho de 2012, foram tratados 10 pacientes portadores de craniossinostoses, sendo 5 oxicefalias, 3 escafocefalias, 1 turricefalia e 1 trigonocefalia. O tratamento consistiu na aplicação de molas de Lauritzen, para corrigir a deformidade primária da craniossinostose, com a associação de craniotomia helicoide em forma de Nautilus nos sítios de deformação secundária do crânio, sem descolamento dural. RESULTADOS: Foi observada resolução da deformidade craniana e remissão dos sinais clínicos de hipertensão intracraniana. Nenhum paciente apresentou complicações, como fístula liquórica, infecção local, seroma ou hematoma. CONCLUSÕES: A associação da osteotomia helicoide com a distração ou contração promovida pelas molas permitiu remodelar ativamente o crânio, facilitando a acomodação do conteúdo cerebral no continente craniano.

INTRODUCTION: Considering that craniosynostosis is a suture-related condition, the main challenge for its treatment is the fact that the brain is located in a closed compartment that does not have the required adaptability to accommodate its growth. The goal of treatment is to restore stenotic suture adaptability and correct the compensatory cranial deformity. This paper proposes the combined use of spiral osteotomy with distraction osteogenesis by the use of distracting springs to remodel craniofacial defects caused by craniosynostosis. METHODS: Between July 2010 and July 2012, 10 patients with craniosynostosis were treated: 5 with oxycephaly, 3 with scaphocephaly, 1 with turricephaly, and 1 with trigonocephaly. The treatment consisted of the application of Lauritzen springs to correct the primary craniosynostosis defect in combination with a nautilus-shaped spiral craniotomy at the secondary deformation sites without dural detachment. RESULTS: Resolution of cranial deformity and remission of the clinical signs of intracranial hypertension were observed. None of the patients had complications such as cerebrospinal fluid fistula, local infection, seroma, or hematoma. CONCLUSIONS: The combined use of spiral osteotomy with spring-mediated distraction or contraction enables active reshaping of the skull and facilitates accommodation of the brain by the cranial cavity.

Idade e indicações de osteotomias para avanço frontofacial em pacientes com craniossinostoses sindrômicas / Age and indications for osteotomy for frontofacial advancement in patienets with syndromic craniosynostosis", "_e": "n

INTRODUÇÃO: Desde o início da Cirurgia Craniofacial, muitos desafios foram ultrapassados. Problemas operatórios técnicos e de infraestrutura básica de atendimento especializado foram solucionados. Agora, 25 anos após as publicações iniciais dos avanços frontofaciais, há ainda algumas dúvidas quanto às indicações precisas da idade e do tipo de cirurgia a ser realizada. O objetivo deste estudo foi avaliar a evolução de pacientes submetidos a tratamento de craniossinostoses sindrômicas operados nos últimos 10 anos em nossa instituição. MÉTODO: Todos os pacientes sindrômicos submetidos a avanço frontofacial em monobloco ou somente facial isolado foram selecionados no período de 2001 a 2011. Foram selecionados 70 pacientes, 56 submetidos a avanço frontofacial em monobloco e 14, a avanço facial após remodelagem frontorbitária prévia. Todos os dados referentes a esses pacientes foram correlacionados, avaliando a idade e o resultado final. Os pacientes foram selecionados de acordo com idade à época da cirurgia, complicações existentes e resultados finais correlacionados com os principais problemas existentes previamente. RESULTADOS: Os pacientes sindrômicos apresentaram graus variados de resultados finais, dependendo da síndrome e da idade de realização do procedimento. Os avanços frontofaciais em monobloco apresentaram baixo índice de complicações pós-operatórias imediatas, porém ficou demonstrada a necessidade de procedimentos futuros ao final do crescimento facial. Nos pacientes submetidos a cirurgias mais tardiamente, o índice de resultados positivos foi maior. CONCLUSÕES: Nos casos de craniossinostoses graves, com problemas funcionais, a indicação de avanço frontofacial em monobloco continua sendo a melhor opção terapêutica.

BACKGROUND: Craniofacial surgery has overcome many challenges since its initiation into clinical practice. Several technical issues have been addressed and the basic infrastructure of the specialty has now been developed. At present, 25 years after the first publications on frontofacial advancement, questions still remain as to the appropriate age for surgery and the appropriate type of surgery that should be performed. The aim of this study was to evaluate patients surgically treated for syndromic craniosynostosis over the last 10 years at our institution. METHODS: All syndromic patients who underwent monobloc frontofacial advancement or only isolated facial advancement from 2001 to 2011were selected. Out of 70 patients in total, 56 underwent monobloc frontofacial advancement and 14 underwent facial advancement after fronto-orbital remodeling. All data concerning these patients were correlated with patient age and final result. Moreover, age at surgery, complications, and final results were correlated with the main preexisting problems. RESULTS: Final results for syndromic patients varied, depending on the syndrome and the age at which the procedure was performed. Monobloc frontofacial advancements had a low index of immediate postoperative complications, but there was a clear need for further procedures at the time of final facial growth. The index of positive outcome was higher in patients who underwent surgery at an older age. CONCLUSIONS: In cases of severe craniosynostosis with functional problems, monobloc frontofacial advancement is still the best therapeutic option.

Deformidades craneoestenoticasfaciales su estudio en nuestro medio (1992- 2009) / Craniofacialsynostosis malformations a study in our department (1992- 2009)

Las deformidades craneoestenóticas-faciales o alteraciones morfo-volumétricas del cráneo y la cara, de etiopatogenia discutida y tratamiento siempre quirúrgico y precoz, motivan esta investigación longitudinal y retrospectiva de 108 sujetos operados de la craneoestenosis que forma parte de todas ellas, teniendo en cuenta las siguientes variables: edad, sexo, tipos de craneoestenosis, estudios imagenológicos y neurofisiológicos pre y postoperatorios, complicaciones acaecidas en el trans o postoperatorio, se llegó a los siguientes resultados: Edad predominante, 1 año (51.9 por ciento), sexo masculino (83.3 por ciento), tipo de craneoestenosis mayor número de veces presente, la escafocefalia (68 niños/63 por ciento). Estudios Preoperatorios. Imagenología: La radiografía simple de cráneo en tres vistas mostró mayor número de veces el diagnóstico de imagen, con aumento de presencia de impresiones digitiformes (18 pacientes/16.6 por ciento); mientras que TAC de Cráneo permitió diagnosticar tempranamente (7 niños/6.5 por ciento) signos de hidrocefalia y presencia de atrofia cerebral ( 27 sujetos/25 por ciento); imágenes diagnósticas fundamentales para confirmar diagnóstico precoz de craneoestenosis. Estudios Neurofisiológicos (EEG), patológicos (73 pacientes/67.5 por ciento), resultados no encontrados en estudios evolutivos posteriores a los 6 meses de la cirugía. Siendo muy escasas la presencia de complicaciones trans y postoperatorias, no obstante ocurrir una muerte súbita (0.9 por ciento), en el postoperatorio inmediato (72 horas).

Craniofacialsynostotic malformations and morpho-volume alterations of the skull and the face of discussed etiopathogenesis and early surgical treatment have motivated a longitudinal and retrospective research of a 108 subjects who underwent surgery of craniosynostosis taking into consideration the following variables: age, sex, type of craniosynostosis, imaging and neurophysiologic studies either pre or post operatory, as well as during and post operatory complications; resulting in the following : predominant age 1 year (51.9 percent), male (83.3 percent), type of craniosynostosis of greater number of presence, scafocephaly (68 children/63 percent). The pre operatory imaging studies.: were simple radiography of the skull of three views showing greater number of times an image diagnosis, with increased presence of digit forms impressions (18 patients/16.6 percent); whereas skull CT scan allowed to early diagnose (7 children/6.5 percent) signs of hydrocephaly and the presence of cerebral atrophy, (27 subjects/25 percent); essential diagnostic images to confirm early diagnosis of craniosynostosis. Neurophysiologic studies (EEG), pathologic (73 patients /67.5 percent), with no result in follow up studies after six months of surgery. Having little presence of complications during or post operatory; however, one sudden death occurred (0.9 percent), in the immediate post operatory (72 hours).

Spring-mediated skull expansion: overall effects in sutural and parasutural areas. An experimental study in rabbits / Expansão craniana com molas: efeitos globais nas áreas suturais e parassuturais. Estudo experimental em coelhos

Purpose: The use of springs in cranial expansion has proven to be effective in the treatment of craniosynostosis. Spring-mediated expansion has been studied both in the sagittal and in parasagittal regions, especially in scaphocephaly. A rabbit model was used in the present study to analyze the effects of springs on the cranial vault and sutures. Methods: Thirteen 4-week-old New Zealand rabbits were divided into 4 groups: in group I, only amalgam markers were used as control; in group II, amalgam markers were used and sagittal suturectomy was performed; in group III, amalgam markers were used, a sagittal suturectomy was performed and an expansible spring was fitted in the interparietal region and in group IV, markers were used and linear parasagittal craniectomy was carried out with springs. Animals were sacrificed after 2, 4, 8 and 12 weeks. Radiological control and histological analysis were performed in the area of spring implantation. RESULTS: In the groups using springs distraction of the craniectomy borders was greater than in those that did not use springs. New bone formation was observed in all groups, and was faster in group II. Bone growth started from the borders and depth. Bone regeneration presented a similar histological pattern in the groups with spring in the sagittal and parasagittal region. Conclusion: The rabbit model proved to be adequate for the analysis proposed by the study. The use of springs in the groups with sagittal and parasagittal osteotomy led to a similar distraction of amalgam markers and both groups had similar ossification histological pattern.

Objetivo: O uso de molas na expansão craniana tem provado ser efetivo no tratamento da craniossinostoses. A expansão com molas tem sido estudada nas regiões sagital e parassagital, especialmente nas escafocefalias. Um modelo com coelho foi usado no presente estudo para analizar os efeitos das molas sobre a calota craniana e suturas. Métodos: Treze coelhos Nova Zelândia, com quarto semanas de vida, foram divididos em quatro grupos: no grupo I, somente marcadores de amálgama foram implantados como controle; no grupo II, marcadores de amálgama foram implantados e foi retirada a sutura sagital; no grupo III, marcadores de amálgama foram implantados, foi retirada a sutura sagital e foi colocada uma mola expansora na região interparietal e no grupo IV, marcadores de amálgamas foram implantados, uma craniectomia linear parassagital foi realizada e foi colocada uma mola expansora. Os animais foram sacrificados com 2, 4, 8 e 12 semanas. Foi realizado controle radiológico e histológico nas áreas de implantação das molas. RESULTADOS: Nos grupos que utilizaram molas a distração das margens ósseas de craniectomia foi maior do que nos grupos que não utilizaram molas. Neoformação óssea foi observada em todos os grupos, tendo sido mais rápida no grupo II. O crescimento ósseo teve início a partir das margens e da profundidade. A regeneração óssea apresentou padrões histológicos similares nos grupos com o uso de molas na região sagital e parassagital. Conclusões: O modelo experimental com coelho provou ser adequado para a análise proposta pelo estudo. O uso das molas nos grupos com osteotomia sagital e parassagital promoveu uma distração similar dos marcadores de amálgamas e ambos os grupos tiveram padrão histológico de ossificação similar.

Evaluation of cephalometric points in midface bone lengthening with the use of a rigid external device in syndromic craniosynostosis patients / Avaliação de pontos cefalométricos no alongamento ósseo do terço médio da face em portadores de craniossinostose sindrômica com a utilização de dispositivo externo rígido

Distraction osteogenesis has been extensively used to correct severe midface hypoplasia in syndromic craniosynostosis patients. However few studies have reported midface distraction outcomes through cephalometric evaluation. AIM: The purpose of the present study was to evaluate outcomes with midface distraction rigid external device (RED) in patients with syndromic craniosynostosis, in terms of quantity of bone lengthening, skeletal stability and facial growth. MATERIALS AND METHODS: Eleven patients were retrospectively evaluated in this study. Cephalometrics was carried out through three teleradiographies from each patient (T1 -before surgery; T2- immediate postop, rigth after distractor removal; T3 - late postop, obtained with a minimal interval of 12 months after surgery). RESULTS: Significant midface advancement was achieved with the procedure. The rate of horizontal relapse was minimal. We noticed a clear vertical facial growth, contrary to what was seen in the horizontal aspect, when there was a mild posterior relapse and no growth evidence. CONCLUSION: Cephalometric evaluation showed adequate results in midface bone lengthening with rigid external distractor.

A distração osteogênica tem sido extensamente empregada na correção da grave hipoplasia do terço médio da face de portadores de craniossinostose sindrômica. Poucos estudos têm apresentado os resultados da distração do terço médio da face através de avaliação cefalométrica. OBJETIVO: O objetivo do estudo foi o de avaliar os resultados obtidos com o avanço ósseo do terço médio da face seguida da utilização de dispositivo rígido externo de distração (RED), em portadores de craniossinostose sindrômica, através de análise cefalométrica. MATERIAL E MÉTODOS: O estudo teve desenho retrospectivo, onde onze pacientes foram avaliados retrospectivamente. A análise cefalométrica foi realizada a partir de três telerradiografias de cada paciente (T1- préoperatório; T2 - pós-operatório recente, logo após a remoção do distrator; T3 - pós-operatório tardio, obtida com um intervalo mínimo de 12 meses após cirurgia) RESULTADOS: Avanço significativo do terço médio da face foi obtido. A taxa de perda de resultado foi mínima. Foi observado evidente crescimento vertical da face, ao contrário do que corre na direção horizontal, onde existiu um pequeno reposicionamento posterior e nenhuma evidência de crescimento. CONCLUSÃO: Observou-se resultados adequados no alongamento do terço médio da face com o uso de distrator externo rígido, através da análise cefalométrica.

Fraciocraniosynostosis treatment strategy: one or two stages?

O tratamento cirúrgico da craniossinostose envolve técnicas complexas para prevenir danos cerebrais secundários e corrigir a retrusão facial, incluindo suas conseqüências (exorbitismo e danos às vias aéreas superiores). O avanço fronto-orbital convencional (antes de um ano de idade) pode corrigir problemas associados em um único procedimento na grande maioria dos casos apesar do crescimento cerebral estar completo aos três anos, a retrusão facial pode ser corrigida posteriormente, sabendo-se que o crescimento facial está completo oa redor de 16-18 anos. Uma osteotomia Le Fort III definitiva realizada tardiamente poderia não necessitar de distração. Pacientes com indicação de correção precoce, como crianças que apresentam malformações mais graves como risco de sequelas psicológicas importantes, podem ser operados antes, assim, considerando-se que o distúrbio genético de crescimento irá permanecer, cirurgia futura seá necessária. Os autores apresentam uma discussão sobre a realização do procedimento em um ou dois estágios para tratar esta desafiadora malformação, apresentando sua experência com milhares de casos já operados.

Plagiocefalia frontal: experiência de 17 casos operados

A plagiocefalia frontal pode ter como causas o fechamento prematuro unilateral da sutura coronal (plagiocefalia frontal sinostótica) ou pode ser causada por compressão externa intra-útero ou pós-natal (plagiocefalia frontal posicional). A forma posicional pode ter tratamento inicial conservador, com o uso de capacetes ou mudanças de decúbito, porém a forma sinostótica exige tratamento cirúrgico precoce. O tratamento cirúrgico, preferencialmente ao redor de 3 a 8 meses, ou quando o paciente se apresenta, o que pode ocorrer mais tardiamente, visa à correção da deformidade fronto-orbital, prevenindo o aparecimento de deformidades de terço médio da face, nariz e órbitas (em arlequim), além de permitir um crescimento cerebral adequado e uma expansão da base do crânio (estreitada pela deformidade). Apresentamos dados referentes a características dos pacientes operados e detalhes da técnica cirúrgica. Nove pacientes do sexo masculino e oito pacientes do sexo feminino, todos com a forma de plagiocefalia frontal sinostótica não sindrômica, com mediana de idade ao procedimento cirúrgico de 12 meses. O princípio cirúgico seguido é o de craniotomia com o avanço e rotação fronto-orbital assimétricos. O tratamento cirúrgico precoce é mandatório, evitando as seqëlas estético-funcionais e o retardo do desenvolvimento neuropsicomotor decorrentes da diminuição do fluxo sangüíneo cerebral do lado afetado pela compressão.

Materiais absorvíveis na cirurgia da cranioestenose: revisão de 72 casos operados com 2 anos de acompanhamento / Resorbable internal fixation in 72 craniosynostosis surgery in a 2 year follow-up

A cranioestenose ou craniosinostose é o fechamentoprecoce das suturas cranianas, que pode restringir o crescimentoencefálico, levando a seqüelas como hipertensão intracraniana,distúrbios de desenvolvimento e alterações morfológicas doesqueleto craniofacial. Nos últimos dez anos, o tratamento cirúrgicoda cranioestenose evoluiu muito em decorrência da introdução denovos materiais para osteossíntese e fixação da barra supra-orbitária.Objetivo: Avaliar a incidência de complicações e intercorrências nosítio operatório associadas ao uso de materiais absorvíveis empacientes submetidos a tratamento cirúrgico de cranioestenoses(co-polímero polilacticoglicólico 82-18%). Método: Estudo retrospectivoque avaliou 72 pacientes, no período de março de 2002 adezembro de 2005, comparando dois grupos: grupo controle, quenão utilizou o material; e grupo de estudo, que utilizou. As variáveis dedesfecho foram clínicas: presença de deiscência de ferida operatória,seroma e reintervenção cirúrgica no sítio operatório. Resultados: Nãohouve diferença estatisticamente significativa na freqüência dasvariáveis estudadas à comparação dos dois grupos. Conclusão: Aosteossíntese com sistemas absorvíveis em cirurgia craniofacialpediátrica é segura e proporciona maior estabilidade de resultados,sem correlação com intercorrências pós-operatórias relacionadas àincidência de reações em sítio operatório.

The craniostenose or craniosynostose is the earlyclosure of cranial sutures that can limit encephalic growth withsequelas like cranial hypertension, growth disturbs andmorphologic alterations of craniofacial skeleton. In the last tenyears, the surgical treatment of the craniostenose has developeda lot due to the introduction of new materials for osteosyntheseand supraorbital fixation. Objective: Evaluate the incidence ofcomplications in the operative site associated with thereabsorbable materials in patients submitted to surgicaltreatment of craniosynostose (polilacticoglicolico co-polymers82-18%). Methods: Retrospective study that evaluated 72patients from March 2002 to December 2005 comparing 2groups – control group that didn’t use the material and groupthat used. The results were evaluated clinically: presence ofdehiscence on operative wound, seroma and surgicalreintervention. Results: No statistic difference in the frequence ofthe 2 groups compared. Conclusion: The osteosynthese withreabsorbable systems used in pediatric craniofacial surgery aresafe and afford more stability of results without correlated postoperativecomplications due to local reactions.

Two Cases of Cerebral Salt Wasting Syndrome Developing after Cranial Vault Remodeling in Craniosynostosis Children

Hyponatremia has been recognized as an important postoperative metabolic complication after central nervous system (CNS) operations in children. If not appropriately treated, the postoperative hyponatremia can cause several types of CNS and circulatory disorders such as cerebral edema, increased intracranial pressure. The postoperative hyponatremia after CNS surgery has been considered as one of the underlying causes of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). In some cases, however, the cerebral salt wasting (CSW) syndrome has been detected. CSW syndrome is far less well-known than SIADH and also different from SIADH in diagnosis and treatment. It causes an increase in urine output and urine sodium after a trauma of CNS and dehydration symptoms. The appropriate treatment of CSW syndrome is opposite the usual treatment of hyponatremia caused by SIADH. The latter is treated with fluid restriction because of the increased level of free water and its dilutional effect causing hyponatremia, whereas the former is treated with fluid and sodium resuscitation because of the unusual loss of high urinary sodium. Early diagnosis and treatment of CSW syndrome after CNS surgery are, therefore, essential. We made a diagnosis of CSW syndrome in two craniosynostosis children manifesting postoperative hyponatremia and supplied them an appropriate amount of water and sodium via intravenous route. The hyponatremia or natricuresis of the children improved and neurologic and circulatory sequelae could be prevented.

Dificuldade de intubaçäo traqueal em paciente com craniossinostose: relato de caso / Tracheal intubation difficulty in patient with craniosynostosis: case report

Justificativa e objetivos - Existem formas complexas de craniossinostose acompanhadas de malformações da face e das vias aéreas, que podem levar a dificuldade de intubação traqueal (IOT). O objetivo deste relato é apresentar um caso de intubação traqueal difícil, em criança submetida à cirurgia para correção da craniossinostose. Relato de caso - Criança com 57 dias, 3700 gramas, perímetro cefálico 39 cm, ASA II, com craniossinostose, retrognatia, macroglosia e exoftalmo bilateral, dificuldade inspiratória e retração intercostal leve. Após decúbito dorsal, coxim sob os ombros, pré-oxigenação e infusão de propofol (10 mg) e succinilcolina (5 mg), realizou-se laringoscopia (lâmina nº1 reta), sem visualização da epiglote. Foi feita ventilação assistida seguida de novas tentativas (4) de IOT, sem sucesso. Optou-se pela ventilação espontânea assistida e IOT, novamente sem sucesso. Pela capnografia não havia CO2 exalado. Com auxílio de fibroscópio intubou-se a traquéia, porém a sonda era estreita e foi trocada por outra com balonete. A SpO2 era de 98 por cento, porém com instabilidades, ás vezes com 60 por cento. A PetCO2 apresentava hipocapnia com morfologia irregular. Á auscultura mostrava ventilação pulmonar bilateral, por com entrada de ar no estômago. Suspeitou-se de fístula tráquo-esofágica traumática. Com endoscopia esofágica constatou-se que a sonda de intubação estava no esôfago; como o balonete foi insuflado na cavidade oral posterior, que estava edemaciada, impedia o vazamento de gás. O volume corrente ventilava o estômago e os pulmões simultaneamente. A sonda foi introduzida corretamente na traquéia com fibroscópio e realizou-se a cirurgia. Conclusões - É necessário distinguir craniossinostose simples daquelas acompanhadas de malformações faciais e de vias aéreas. E fundamental que se tenha equipamentos adequados para IOT e além da monitorização básica, a capnografia é de grande valor na confirmação da intubação traqueal

Dois casos de craniossinostoses e a importância do diagnóstico precoce / The two cases of craniosynostosis and the importance of early diagnosis

As craniossinostoses (CSO) ou, como säo classicamente denominadas, "cranioestenoses" säo entidades já bem conhecidas há mais de vinte séculos e que, se näo adequadamente manejadas, trazem inúmeras complicaçöes principalmente no que diz respeito à limitaçäo do espaço intracranial e deformidades craniais, hipertensäo intracraniana e retardo no desenvolvimento neuropsicomotor. Neste trabalho, os autores descrevem aspectos importantes sobre essa entidade no que se refere a: histórico, etiopatogenia, classificaçäo, tratamento cirúrgico e, ainda, através do relato de dois casos distintos de CSO, um de sutura sagital e outro de sutura bicoronal, demonstram a importância do diagnóstico do tratamento precoce de modo a otimizar os resultados cirúrgicos e prevenir as temidas complicaçöes.

Surgical treatment of premature sagittal synostosis

A series of 50 consecutive children with premature sagittal synostosis is reported. All were treated surgically; 43 were male, 47 were leukodermic and two are siblings. In the pre-operative examination, the head shape, skull measurements and radiologic findings were evaluated; 38 children were operated on before six months of age and 12 of them, between six and 12 months of age. The surgical technique used was a wide biparietal craniectomomy. Blood transfusions were occasional, being necessary for only six (12 percent) children. The children were admitted at the day of surgery and discharged between the second and the third post-operative day. No local or general complications were observed and no one died. The aesthetic result was considered good. The altered skull measurements before surgery reached normalization as far as the end of the first year after the treatment. It may be concluded that wide biparietal craniectomy is a procedure of great effectiveness in the treatment of the premature fusion of the sagital suture.

Plagiocefalia Frontal Sinostosica. Resultados del Tratamiento Quirurgico / Frontal synostotic plagiocephaly. Results of the surgical treatment

Se estudiaron de forma retrospectiva 10 ninos con craneosinostosis tipo plagiocefalia frontal sinostosica operados en el Servicio de Neurocirugia Infantil del Instituto de Neurologia y Neurocirugia. Como tecnica quirurgica se empleo la craniectomia lineal en el sitio de la hemisura coronal sinostosada, ampliada hasta la region del pterion. La totalidad de los infantes evolucionaron con hipoplasia orbitaria y abombamiento frontal y a mas de la mitad se le realizo operacion en los primeros 6 meses de la vida. Hubo importante variacion del indice cefalico, asi como mejoria estetica comparativamente, antes de la operacion y despues de esta