先天性单侧桡尺近端滑膜病 1 例。
- 作者列表："Jia Y","Geng C","Song Z","Lv S","Dai B
RATIONALE:Congenital proximal radioulnar synostosis is a rare genetic malformation of the upper limb. This deformity, which is found mainly in preschool-aged children, has no recognized diagnosis and treatment. Current diagnostic methods cannot effectively assess both bone structure and soft tissue abnormalities, and most surgical treatments introduce complications and do not prevent recurrence. More work is needed; therefore, to address the diagnosis and treatment of this disease. PATIENT CONCERNS:An 8-year-old male patient was hospitalized in our department. He reported deformity and limited motion in his right elbow for the past 2 years. He denied a traumatic or family history of bony malformation. The chief complaint at the time of the hospitalization was the limitation in forearm rotation. DIAGNOSIS:Digital radiography of the right elbow joint showed proximal radioulnar synostosis and a valgus deformity. A 3-dimensional computed tomography scan further showed proximal ulna and radius dysplasia as well as anterior dislocation of the radius head. The patient was diagnosed with congenital right proximal radioulnar synostosis. INTERVENTIONS:Surgical procedures included arthrolysis of the right proximal radioulnar joint, osteotomy of the proximal radius, internal fixation with Kirschner wires, and reconstruction of the annular ligament. The right elbow was immobilized in plaster in a flexion and supination position for 2 weeks. OUTCOMES:Recurrence of the right proximal radioulnar synostosis was observed during the 6-month follow-up, but the rotation function of the patient's forearm was significantly improved. LESSONS:The findings from this case suggest that we should carefully monitor all patients younger than 6 years old who report long-term issues with forearm rotation. This case also highlights the need to assess soft tissue and epiphysis abnormalities in addition to bone assessments via digital radiography and 3-dimensional computed tomography. We suggest that surgery should not be performed until the proximal radius epiphysis has closed. Not all cases require surgical treatment, but when surgery is needed, a suitable method should be selected according to the individual needs of the patient. Any surgery performed should treat both the bony malformations and soft tissue abnormalities to maximize the therapeutic effect and reduce complications during and after surgery.
基本原理: 先天性近端桡尺滑膜病是一种罕见的上肢遗传畸形。该畸形主要见于学龄前儿童，目前尚无公认的诊断和治疗方法。目前的诊断方法不能有效评估骨结构和软组织异常，大多数手术治疗引入并发症，不能防止复发。需要做更多的工作; 因此，解决这种疾病的诊断和治疗。 患者顾虑: 患者男性，8 岁，在我科住院。他报告了近 2 年来右肘畸形和活动受限。他否认有骨性畸形的外伤或家族史。住院时的主诉是前臂旋转受限。 诊断: 右侧肘关节数字化摄片显示桡尺近端滑膜和外翻畸形。三维计算机断层扫描进一步显示尺骨和桡骨近端发育不良以及桡骨头前脱位。患者被诊断为先天性右桡尺近端滑膜。 干预措施: 外科手术包括右桡尺近端关节关节松解术、桡骨近端截骨术、克氏针内固定术和环状韧带重建术。右肘关节采用石膏固定，屈曲旋后位固定 2 周。 结果: 在 6 个月的随访中观察到右桡尺近端滑膜复发，但患者前臂的旋转功能明显改善。 教训: 本病例的发现建议我们应该仔细监测所有报告前臂旋转长期问题的小于 6 岁的患者。本病例还强调了除了通过数字x线摄影和三维计算机断层扫描进行骨评估外，还需要评估软组织和骨骺异常。我们建议在桡骨近端骨骺闭合之前不要手术。并非所有病例都需要手术治疗，但当需要手术时，应根据患者的个体需要选择合适的方法。任何手术都应该同时治疗骨畸形和软组织异常，以最大限度地提高治疗效果，减少术中及术后并发症。
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.