A systematic quantitative morpho-volumetric analysis in infants with sagittal craniosynostosis and relationship with the severity of scaphocephalic deformity.


  • 影响因子:1.82
  • DOI:10.1007/s11547-020-01150-w
  • 作者列表:"Calandrelli R","Pilato F","Massimi L","Panfili M","Colosimo C
  • 发表时间:2020-06-01

PURPOSE:Among patients with isolated sagittal synostosis (ISS), the head shape varies considerably in relation to the severity of the abnormality. This study aimed to quantify skull base morphometry and intracranial volume to investigate their relationships with the severity of scaphocephaly. METHODS:We studied 66 infants with ISS identifying three groups according to the morphological severity of cranial deformity (group I: mild deformity; group II: moderate deformity; group III: severe deformity), by combining two scaphocephaly severity indices as descriptors of the relation of three morphological measurements (length, width and height) We perform a quantitative analysis using high-resolution CT images calculating following parameters: cranial fossae dimensions, supratentorial (ICV) and infratentorial (PCFV) cranial volume, supratentorial (WBV) and infratentorial (PCFBV) brain volume, ICV/WBV, PCFV/PCFBV, supratentorial and infratentorial cerebrospinal fluid (CSF). RESULTS:In all subgroups, anterior and middle skull base lengths were increased, while posterior hemifossae lengths were unchanged. In mild subgroup, ICV/WBV was significantly different and ICV, WBV and CSF supratentorial volume increased (p < 0.05). In moderate and severe subgroups, FCPV/FCPBV was significantly different and CSF infratentorial volume was reduced (p < 0.05); FCPBV was increased only in the severe subgroup (p < 0.05). CONCLUSION:This morpho-volumetric study provides new insights in understanding the compensatory changes occurring in infants at different stages of scaphocephaly severity. In particular, our study suggests that patients with severe deformity might have an earlier depletion of reserve mechanisms with a reduced compliance of the overall skull during encephalic growth and these patients might require early surgical cranial expansion.


目的: 在孤立性矢状面滑膜病 (ISS) 患者中,头部形状与异常严重程度有很大关系。本研究旨在量化base形态测量学和颅内体积,以研究其与scaphophealy严重程度的关系。 方法: 我们研究了 66 例ISS婴儿,根据颅骨畸形的形态严重程度确定三组 (ⅰ 组: 轻度畸形; Ⅱ 组: 中度畸形; Ⅲ 组: 重度畸形),通过结合两个scaphophaly严重程度指数作为三个形态测量 (长度,宽度和高度) 的关系的描述符我们使用高分辨率CT图像进行定量分析,计算以下参数: 颅窝尺寸、幕上 (ICV) 和幕下 (PCFV) 颅体积、幕上 (WBV) 和幕下 (PCFBV) 脑体积、ICV/WBV、PCFV/PCFBV、幕上及幕下脑脊液 (CSF)。 结果: 在所有子组,前中柱头骨base长度的增加,而后路hemifossae长度不变.在轻度亚组中,ICV/WBV有显著性差异,ICV、WBV和CSF幕上体积增加 (p <0.05)。中、重度亚组FCPV/FCPBV差异有显著性,CSF幕下体积缩小 (p <0.05); FCPBV仅在重度亚组增加 (p <0.05)。 结论: 这项形态容积研究为理解婴儿在鼻疽严重程度不同阶段发生的代偿性变化提供了新的见解。特别是,我们的研究表明,严重畸形的患者可能会更早地耗尽储备机制,在颅内生长过程中整体颅骨的顺应性降低,这些患者可能需要早期手术颅骨扩张。



作者列表:["Peyroux E","Babinet MN","Cannarsa C","Madelaine C","Favre E","Demily C","Michael GA"]

METHODS::Impairments in social cognition have been frequently described in 22q11.2 deletion syndrome (22q11.2DS) and are thought to be a hallmark of difficulties in social interactions. The present study addresses aspects that are critical for everyday social cognitive functioning but have received little attention so far. Sixteen children with 22q11.2DS and 22 controls completed 1 task of facial expression recognition, 1 task of attribution of facial expressions to faceless characters involved in visually presented social interactions, and 1 task of attribution of facial expressions to characters involved in aurally presented dialogues. All three tasks have in common to involve processing of emotions. All participants also completed two tasks of attention and two tasks of visual spatial perception, and their parents completed some scales regarding behavioural problems of their children. Patients performed worse than controls in all three tasks of emotion processing, and even worse in the second and third tasks. However, they performed above chance level in all three tasks, and the results were independent of IQ, age and gender. The analysis of error patterns suggests that patients tend to coarsely categorize situations as either attractive or repulsive and also that they have difficulties in differentiating emotions that are associated with threats. An isolated association between the tasks of emotion and behaviour was found, showing that the more frequently patients with 22q11.2DS perceive happiness where there is not, the less they exhibit aggressive behaviour.

作者列表:["Sheridan GA","Nagle M","Howells C","Gallagher O","Kiely PJ","O'Toole P","Kelly PM","Moore DP"]

METHODS:BACKGROUND:We describe the first radiographic clinic in the literature for DDH and how this novel clinic can significantly improve the efficiency and cost-effectiveness of service in a tertiary referral centre. AIMS:A radiographic clinic for the management of developmental dysplasia of the hip was introduced in 2017 in our institution. We performed a detailed cost analysis to assess the economic savings made with the introduction of this new clinic. We assessed the efficiency of the service by identifying how many unnecessary outpatient visits were prevented. We also assessed the difference in times from referral to review between the two clinics. METHODS:Analysis of the clinic activity in 2017 was possible as all data was collected prospectively by the DDH CNS and stored in our database. Cost analysis was performed, and the savings made per patient along with the financial benefit to our institution was recorded. RESULTS:The new radiographic clinic reduced the cost of reviewing one patient by €162.51 per patient. There was a 73% discharge rate from the clinic which prevented 251 unnecessary patient visits to the outpatient department over the course of the year. There was a significant 11-day reduction in waiting times between referral and review when comparing the radiographic to the conventional clinic (p < 0.05). CONCLUSIONS:A radiographic clinic for the management of developmental dysplasia of the hip has a significant effect on the efficiency and overall cost-effectiveness of service provision in a tertiary referral centre.

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作者列表:["Grigoryan G","Korcek L","Eidelman M","Paley D","Nelson S"]

METHODS:INTRODUCTION:Triple pelvic osteotomy (TPO) involves periacetabular osteotomies of the ilium, ischium, and pubis to reorient the acetabulum. This operation is indicated in certain situations for the treatment of developmental dysplasia of the hip, dysplastic neuromuscular hips, and for containment of the femoral head in cases of Legg-Calvé-Perthes disease. METHODS:This retrospective cohort study compares radiographic outcomes of patients who underwent TPO using two different techniques and describes a novel single-incision direct lateral approach. TPO was performed on 22 patients by the senior author. The first 10 patients underwent TPO through a single-incision anterolateral approach. The last 12 patients underwent TPO using the direct lateral approach. Preoperative and postoperative pelvic radiographs were reviewed for each patient, and the migration index and center-edge angle were recorded. RESULTS:The migration index and center-edge angle were evaluated and were not found to be significantly different between the anterolateral and direct lateral groups. The direct lateral approach is described. CONCLUSION:The direct lateral approach for TPO is equivalent to the anterolateral approach on radiographic evaluation. Advantages of the direct lateral approach include direct visualization of the ischial osteotomy, effective mobilization of the acetabulum, and safety of the sciatic nerve.

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