- 作者列表："Riordan CP","Zurakowski D","Meier PM","Alexopoulos G","Meara JG","Proctor MR","Goobie SM
OBJECTIVE:To evaluate patient outcomes of minimally invasive endoscopic strip craniectomy (ESC) for craniosynostosis. STUDY DESIGN:This is a retrospective cohort analysis (2004-2018) of 500 consecutive infants with craniosynostosis treated by ESC with orthotic therapy at a single center. Operative outcomes included transfusions, complications, and reoperations as well as head circumference change based on World Health Organization percentiles. Multivariable logistic regression was used to identify risk factors associated with blood transfusion. Paired t tests were used for within-patient comparisons and Fisher exact test to compare syndromic and nonsyndromic subgroups. RESULTS:ESC was associated with low rates of blood transfusion (6.6%), complications (1.4%), and reoperations (3.0%). Risk factors for transfusion included syndromic craniosynostosis (P = .01) and multiple fused sutures (P = .02). Median surgical time was 47 minutes, and hospital length of stay 1 day. Transfusion and reoperation rates were higher among syndromic patients (both P < .001). Head circumference normalized by 12 months of age relative to World Health Organization criteria in infants with sagittal, coronal, and multisuture craniosynostosis (all P < .001). CONCLUSIONS:ESC is a safe, effective, and durable correction of infantile craniosynostosis. ESC can achieve head growth normalization with low risks of blood transfusion, complications, or reoperation. Early identification of craniosynostosis in the newborn period and prompt referral by pediatricians allows families the option of ESC vs larger and riskier open reconstruction procedures.
目的: 评价微创内镜下颅骨剥脱术 (ESC) 治疗颅缝早闭的疗效。 研究设计: 这是一项回顾性队列分析 (2004-2018)，在单中心接受ESC矫形治疗的连续 500 例颅缝早闭婴儿。手术结局包括输血、并发症和再次手术以及基于世界卫生组织百分位数的头围变化。采用多变量logistic回归确定与输血相关的危险因素。采用配对t检验进行患者内比较，Fisher精确检验比较综合征和非综合征亚组。 结果: ESC与低输血率 (6.6%) 、并发症 (1.4%) 和再次手术 (3.0%) 相关。输血的危险因素包括综合征性颅缝早闭 (P = .01) 和多次融合缝线 (P = .02)。中位手术时间为 47 分钟，住院时间为 1 天。综合征患者的输血率和再次手术率较高 (均P <.001)。矢状面、冠状面和多缝线颅缝早闭婴儿头围经 12 月龄相对于世界卫生组织标准标准化 (均P <.001)。 结论: ESC是一种安全、有效、持久的治疗婴儿颅缝早闭的方法。ESC可以实现头部生长正常化，输血、并发症或再次手术的风险较低。新生儿期颅缝早闭的早期识别和儿科医生的及时转诊允许家庭选择ESC vs更大、风险更大的开放式重建手术。
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.
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.
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.