- 作者列表："Wood RJ","Stewart CN","Liljeberg K","Sylvanus TS","Lim PK
BACKGROUND:Approximately one in 2000 babies are born with craniosynostosis, and primary open repair is typically performed before 1 year of age. Historically, the procedure has been associated with nearly 100 percent transfusion rates. To decrease the rates of transfusion, the authors' center has developed a novel multimodal blood conservation protocol. METHODS:The authors administered their standard of care to children aged 1 year or younger undergoing primary repair of craniosynostosis between 2008 and 2014. In 2014, the authors implemented the following protocol: (1) preoperative erythropoietin and ferrous sulfate, (2) local anesthetic with epinephrine infiltration of the incision, (3) PlasmaBlade incision and subgaleal dissection, (4) hypervolemic hemodilution, and (5) intravenous tranexamic acid. Procedures performed before the protocol implementation served as controls. The authors performed classic fronto-orbital advancement with anterior cranial vault remodeling for coronal and metopic craniosynostosis. For lambdoid and sagittal craniosynostosis, barrel stave osteotomies, cranial base outfracture, and interposition bone grafting were performed. RESULTS:A total of 279 children with a mean age of 6 months who had craniosynostosis repairs were included. One hundred forty-five underwent repair before the authors' protocol, and 134 had repairs during the authors' blood conservation protocol. Both groups were similar in demographics. Overall blood loss and operative times were significantly reduced by 73 percent and 11 percent, respectively. Blood transfusion rate decreased 92 percent (p < 0.001). CONCLUSIONS:These results show a strong association between the authors' blood conservation protocol and significantly reduced transfusion rates. The authors believe this is a significant step forward and can be safely applied in the great majority of children undergoing craniosynostosis repairs. CLINICAL QUESTION/LEVEL OF EVIDENCE:Therapeutic, III.
背景: 大约每 2000 个婴儿中就有一个出生时患有颅缝早闭，通常在 1 岁之前进行初次开放修复。历史上，该手术与近 100% 的输血率相关。为了降低输血率，作者中心开发了一种新型的多模式血液保护方案。 方法: 作者在 2008 年至 20 1 4 年期间对接受初次颅缝早闭修复的 1 岁或以下儿童实施了他们的标准护理。在 2 014 中，作者实施了以下方案 :( 1) 术前促红细胞生成素和硫酸亚铁，(2) 切口浸润肾上腺素的局部麻醉剂，(3) 等离子消融切口和角膜下剥离，(4) 高容量血液稀释，(5) 静脉氨甲环酸。在协议实施之前执行的程序作为控制。作者对冠状位和metopic颅缝早闭进行了经典的额眶前移伴前颅穹隆重塑。对于低颅缝和颅缝矢状早闭，行桶形壁截骨、base外骨折、间置植骨术。 结果: 共纳入 279 例平均年龄 6 个月的颅缝早闭修复术患儿。105 例在作者方案前接受修复，134 例在作者血液保护方案期间接受修复。两组人口统计学相似。总体失血量和手术时间分别显著减少 73% 和 11%。输血率下降 92% (p <0.001)。 结论: 这些结果显示作者的血液保护方案与显著降低的输血率之间有很强的相关性。作者认为这是向前迈出的重要一步，可以安全地应用于绝大多数接受颅缝早闭修复的儿童。 临床问题/证据水平: 治疗性，III。
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.