Modified Technique to Protect the Superior Sagittal Sinus in Cranial Vault Reconstruction for Scaphocephaly / Técnica modificada para proteger o seio sagital superior em reconstrução da calota craniana por escafocefalia

Scaphocephaly remains the most prevalent form of isolated single-suture synostosis. In most Pediatric Neurosurgery Centers, the treatment consists of extensive calvarial reshaping operations which are performed in relatively late ages. We described a modified open technique of cranial vault reconstruction for scaphocephaly and we report the anesthetic approach and how it influences our results and enables the performance of this surgery at earlier ages. A total of 47 patients with an average age of 11 2.72 months underwent to the same surgical technique. Blood transfusion during the surgical intervention was needed in 25 patients (53%), and 22 patients (46.8%) did not need blood transfusion. None of the 14 patients who used tranexamic acid (TXA) needed blood transfusions. We reported a statistically significant association between the use of TXA and blood transfusion (p < 0.0001), with a decreased need for blood transfusion with the use of TXA. Besides sagittal suturectomy, we create new parasagittal sutures by replacing the prematurely fused one, and 1.5 cm of the anterior segment of bone of those 2 rectangular structures are cut before fixation, to enable the immediate shortening of the anteroposterior (AP) diameter of the skull. Finally, we put a mesh plate (SonicWeld Rx, KLS Martin Group, Tuttlingen, Baden-Wuerttemberg, Germany) over the superior sagittal sinus (SSS), which is fixed anteriorly and posteriorly with pins (SonicPins Rx, KLS Martin Group). This procedure enables an immediate reduction of the AP diameter of the skull, with no further therapies needed, and promotes a wide decompression and remodeling of the skull. With an experienced surgical team and an anesthesiologic protocol, we were able to perform this surgery at early ages, requiring no blood transfusions.

A escafocefalia continua sendo a forma mais prevalente de sinostose isolada de sutura única. Na maioria dos Centros de Neurocirurgia Pediátrica, o tratamento consiste em extensas operações de remodelação calvária, realizadas em idades relativamente tardias. Descrevemos uma técnica aberta modificada de reconstrução da calota craniana para escafocefalia e relatamos a abordagem anestésica e como ela influencia nossos resultados e permite a realização desta cirurgia em idades mais precoces. Um total de 47 pacientes com idademédia de 11 2,72meses foram submetidos à mesma técnica cirúrgica. A transfusão sanguínea durante a intervenção cirúrgica foi necessária em 25 pacientes (53%) e 22 pacientes (46,8%) não necessitaram de transfusão sanguínea. Nenhum dos 14 pacientes que usaram ácido tranexâmico (TXA) precisou de transfusões de sangue. Relatamos uma associação estatisticamente significativa entre o uso de TXA e transfusão de sangue (p < 0,0001), com diminuição da necessidade de transfusão de sangue com o uso de TXA. Além da suturectomia sagital, criamos novas suturas parassagitais substituindo a fundida prematuramente, e 1,5 cm do segmento anterior do osso dessas duas estruturas retangulares são cortados antes da fixação, para permitir o encurtamento imediato do diâmetro ântero-posterior (AP) do crânio. Por fim, colocamos uma placa de tela (SonicWeld Rx, KLS Martin Group, Tuttlingen, Baden-Wuerttemberg, Alemanha) sobre o seio sagital superior (SSS), a qual é fixada anterior e posteriormente com pinos (SonicPins Rx, KLS Martin Group). Este procedimento permite uma redução imediata do diâmetro AP do crânio, sem necessidade de terapias adicionais, e promove ampla descompressão e remodelação do crânio. Com uma equipe cirúrgica experiente e um protocolo anestesiológico, conseguimos realizar esta cirurgia em idades precoces, sem necessidade de transfusões sanguíneas.

Single sagittal craniosynostosis surgical treatment with the "Peau d́ours" technique. Single-center experience in Mexico.

Scaphocephaly (SC) is defined as an elongation of the anteroposterior axis of the skull resulting from the abnormal fusion of the sagittal suture. This study describes the "Peau d'ours" technique and results for correcting SC. We conducted a consecutive and retrospective analysis of patients treated from 2011 to 2016. We evaluated the gender, age, and surgical outcomes. A total of 53 patients were enrolled with a mean age of 19 months old. The advantages of this technique are healthy coronal and lambdoid suture preservation and symmetrical parietal bone flap opening. This technique is safe and simple to reproduce, allowing good surgical outcomes with a low incidence of secondary craniosynostosis. This technique is ideal for patients older than six months old.

Anthropometric changes in the skull base in children with sagittal craniosynostosis submitted to surgical correction.

PURPOSE: Studies have examined the impact of fusion of the sagittal suture in the skull base while others have evaluated the growth of the skull base before and after surgery. This study aims to perform the anthropometric measures of the skull base in children with scaphocephaly to evaluate the influence of surgical repair in the remodeling of the skull base and anthropometric measures. METHODS: Twenty-one children with diagnosis of scaphocephaly were operated between April 2007 and October 2008, and anthropometric measures at the skull base were performed before and after a year of surgery. The measures were the cranial index (CI), distance between the crista galli and tuberculum sellar (CG-TS), distance between the crista galli and the internal auditory meatus (CG-IAM), distance between the oval foramen (OF-OF), distance between the internal auditory meatus (IAM-IAM), the angle of the skull base (Â1), and the angle between the nasion, center of sella, and basion (Â2). RESULTS: There was a normalization of the CI in all children, confirming an appropriate cranial remodeling. The CG-TS measure evaluated the anterior skull base, with proportional growth of 12.5%. The mediolateral growth was observed by the increase of OF-OF measures by 8.5% and IAM-IAM by 9.5%. The CG-TS measure grew by 7.2%. There was no statistical difference in the angles analyzed. CONCLUSION: Surgical treatment of scaphocephaly led to remodeling of the skull base, confirmed by the changes of anthropometric measures taken before and after a year of surgery.

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.

Uso de almohadas de gel en recién nacidos de pretérmino se asocia a disminución de deformidad craneana / Using gel pillows in preterm infants is related to decreased cranial deformity

Introducción: la escafocefalia posicional, deformidad craneana típica del recién nacido prematuro, presumiblemente es debida a un inicio anticipado de presión sobre la calota, a un inadecuado control motor que determina un menor cambio de posición y un mayor tiempo de presión asimétrica sobre la calota. El descenso de la presión de puntos de apoyo asimétricos sobre el cráneo podría disminuir la escafocefalia. La superficie de gel puede bajar la presión localizada en el cráneo al disminuir la rigidez de la superficie de contacto. Los estudios existentes, escasos, no son concluyentes al respecto. Objetivo: Mostrar la relación entre el uso de almohadas de gel y la reducción de escafocefalia en recién nacidos de pretérmino. Método: Estudio longitudinal de pacientes prematuros hospitalizados en el servicios de neonatología de un hospital terciario, que utilizaron almohadas de gel por 5 semanas consecutivas, durante al menos el 90 por ciento del tiempo que permanecieron en decúbito. Se realizaron mediciones craneales, considerando el índice de deformidad craneana; la primera medición fue previo al uso de almohadas y luego semanalmente hasta completar 5 semanas. Resultados: 17 pacientes completaron las 5 semanas de protocolo. Edad promedio: 28,9 semanas de gestación (rango 28 a 34). Peso promedio: 1058 grs. (rango 720 a 2000). El índice de deformidad craneana varió desde 70,36 (DE 4,7) al inicio del estudio a 76,95 (DE 3,03) al término del estudio, con una diferencia significativa (p< 0,05). Conclusión: Este trabajo mostró una relación entre reducción del grado de escafocefalia, expresada en el IC, con el uso de almohadas de gel por un período de 5 semanas en RNPT.

Introduction: the positional scaphocephaly is the preterm infants’ typical cranial deformity. And probably is due to improper motor control, which determines minor change in position and longer asymmetric pressure on the skull. Decreasing the pressure of asymmetric support points on the skull, might decrease scaphocephaly. Gel’s surface may decrease localized pressure in the skull thus decreasing the stiffness of the contact surface. There are scarce reports about this issue and all are inconclusive. Objective: To show the relationship between the use of gel pillows and reduction of scaphocephaly in preterm infants. Method: Longitudinal study of preterm infants hospitalized in neonatology service of a tertiary hospital. They used gel pillows for 5 consecutive weeks, for at least 90 percent of the time in prone position. Cranial measurements were performed, considering the index of cranial deformity, the first measurement before to the use of pillows and then weekly until 5 weeks. Results: 17 patients completed the 5-week protocol. Average age 28.9 weeks of gestation (range 28-34), average weight 1058 g. (range 720-2000). The index of cranial deformity ranged from 70.36 (SD 4.7) at baseline to 76.95 (3.03) at the end of the study with a significant difference (p <0.05). Conclusions This study showed a relationship between the degree of reduction of scaphocephaly expressed in the IC, using gel pillows for a period of 5 weeks in preterm infants.

Progressive frontal morphology changes during the first year of a modified Pi procedure for scaphocephaly.

PURPOSE: The purpose of this study was to quantify the changes in frontal morphology in patients with scaphocephaly treated with a modified Pi procedure. METHODS: Consecutive scaphocephalic patients (n = 13) who underwent surgery before 12 months of age that had more than 1 year of follow-up and standard preoperative, 3-month, and 1-year photographs were included. Anthropometric measurements were used to document the craniofacial index. Computerized photogrammetric analyses of five craniofacial angles (bossing angle, nasofrontal angle, angle of facial convexity, and angle of total facial convexity) were also performed. RESULTS: Comparisons of the preoperative and postoperative direct anthropometric measurements of the cephalic index showed a significant (all p < 0.05) increase in the postoperative period, with no significant differences in early versus late postoperative period comparisons. Comparisons of the preoperative and postoperative computerized photogrammetric measurements of the craniofacial angles showed a significant (all p < 0.05) reduction (bossing angle, angle of facial convexity, and angle of total facial convexity) and increase (nasofrontal angle) in the early and late postoperative periods. CONCLUSIONS: Frontal morphology significantly changed over the first year of the modified Pi procedure.

Indicadores de severidad de la craneosinostosis no sindrómica: Cuantificación de malformaciones sagitales y metópicas / Severity indices for non-syndromic craniosynostosis: quantifying sagittal and metopic malformations

En este trabajo se presenta un nuevo conjunto de indicadores de severidad que combinan diversos rasgos craneales para cuantificar las craneosinostosis aisladas de tipo sagital y metópica. La utilidad de los indicadores se evaluó examinando las tomografías computarizadas del cráneo de un grupo de infantes afectados por craneosinostosis aislada y un grupo de infantes no afectados. La base de datos contiene estudios de 90 pacientes con craneosinostosis sagital, 40 con craneosinostosis metópica y 60 pacientes no afectados. Los indicadores de severidad se obtienen a partir de un conjunto de indices de severidad por medio de un método estadístico de regresión logística regularizada conocido como red elástica. Los índices de severidad son medidas univariadas de forma que se calculan a partir de tres planos de análisis. Los planos se estiman a partir de referencias anatómicas cerebrales radiológicamente identificables. El desempeño de los indicadores se midió estimando el grado de separación lineal (GSL), que cuantifica la capacidad de un indicador para distinguir cráneos sagitales o metópicos de cráneos no afectados. Los indicadores de severidad propuestos alcanzan un GSL del 95.83% y 98.9% en las poblaciones sagitales vs. controles y metópicos vs. controles, respectivamente. Los resultados obtenidos en este trabajo sugieren que es posible construir indicadores multivariables de severidad que son clínicamente reproducibles y cuantifican efectivamente aspectos de la morfología craneal codificada por medio de un conjunto de índices de severidad.

This work develops a new set of severity scores that combine several cranial features in order to quantify sagittal and metopic craniosynostosis. Computed tomography head scans were obtained from 90 children affected with single-suture sagittal synostosis, 40 children with single-suture metopic synostosis, and 60 age-matched nonsynostotic controls. Tridimensional reconstructions of the skull were used to trace image analysis planes defined in terms of skull-base plane and internal landmarks. For each patient, a new set of descriptive measures or severity indices of skull shape malformation were computed. A statistical classification approach (regularized logistic regression) was used for combining individual severity indices into summarizing severity scores. The linear separation index that measures the ability of a classification function to separate the affected (sagittal or metopic) and nonsynostotic populations was used to evaluate the severity scores. The proposed scores are sensitive measures of the calvarial malformation that achieve linear separation indices of 95.83% and 98.9% for sagittal vs. control and metopic vs. control populations, respectively. As opposed to individual severity indices, the summarizing severity scores encapsulate a number of distinctive calvarial features associated with sagittal and metopic synostoses crania. The proposed scores enable quantitative analysis in clinical settings of skull features observed in isolated sagittal and metopic synostoses that may not be accurately detected by separate analysis of individual severity indices.