先天性桡尺骨近端融合的诊断及治疗研究进展

Research advances in the diagnosis and treatment of congenital proximal radioulnar synostosis

先天性桡尺骨近端融合(congenital proximal radioulnar synostosis,PRUS)是一种较为常见的儿童上肢先天性畸形,常见于男性患儿。目前诊断主要依靠患儿临床表现与影像学资料,Cleary&Omer分型系统是较为常用的分型方式,一些影像学指标如桡骨旋前角度等可能对选择治疗方案、评估预后提供依据。PRUS的治疗比较困难,手术是重度畸形患儿主要的治疗方式,传统的手术方式如单纯融合区分离术并发症发生率较高,术后易发生再融合。目前使用的手术方式主要包括旋转截骨术与融合区分离并游离筋膜脂肪瓣植入术,但疗效及患儿的长期预后尚存在争议。以多中心研究为基础的大样本、随机对照临床研究仍待进一步开展,以探究PRUS患儿的最佳治疗方案。

Congenital proximal radioulnar synostosis(PRUS),a common pediatric malformation,predominates in boys.A definite diagnosis of PRUS is largely based upon clinical manifestations and imaging data.And Cleary&Omer system is widely applied for PRUS classification.Several imaging parameters such as radial pronation angle aid in treatment options and prognostic predication.Proper treatment of PRUS remains challenging and surgery is recommended for children with severe malformation.Traditional surgical mobilization of synostosis is usually accompanied by a relatively high incidence of complications,including recurrence of synostotic bridge.Currently rotational osteotomy and mobilization of synostosis with free vascularized adipofascial graft are adopted.However the efficacy and long-term outcome are controversial.Large-scale multi-center randomized controlled trials on the treatment of PRUS are required.