摘要
Background: Turner syndrome (TS) affects approximately one in 2500 live births in females. Scoliosis is one of the skeletal manifestations of TS, but most cases only require observation or conservative treatment. We experienced two adolescent TS cases in which progression of scoliosis required surgical intervention, which is very rare in TS. Case Presentation: Case 1: An 11-year-old female with TS had a single thoracic curve that rapidly progressed to a triple major curve with a 76˚ main thoracic curve at age 13.5 years. Case 2: A 14-year-old female with TS had a 59˚ single thoracic curve. In both cases, growth hormone and estrogen replacement therapy were administered preoperatively and planned postoperatively. Posterior correction and instrumented fusion using simultaneous translation on two rods technique and direct vertebral rotation with the use of multiple rod introducers were successfully performed in both cases. No crankshaft phenomenon or distal adding on were observed during those postoperative courses. Conclusions: Although curve pattern of the deformity is similar to adolescent idiopathic scoliosis (AIS), bone quality in patient with TS is lower. In the context of surgical interventions for scoliosis associated with TS, it is imperative to employ surgical techniques that take into account the suboptimal bone quality. If continuation of hormone replacement therapy is planned after corrective surgery for scoliosis in TS patients, it is essential to follow the patient closely postoperatively until bone maturation is complete.
Background: Turner syndrome (TS) affects approximately one in 2500 live births in females. Scoliosis is one of the skeletal manifestations of TS, but most cases only require observation or conservative treatment. We experienced two adolescent TS cases in which progression of scoliosis required surgical intervention, which is very rare in TS. Case Presentation: Case 1: An 11-year-old female with TS had a single thoracic curve that rapidly progressed to a triple major curve with a 76˚ main thoracic curve at age 13.5 years. Case 2: A 14-year-old female with TS had a 59˚ single thoracic curve. In both cases, growth hormone and estrogen replacement therapy were administered preoperatively and planned postoperatively. Posterior correction and instrumented fusion using simultaneous translation on two rods technique and direct vertebral rotation with the use of multiple rod introducers were successfully performed in both cases. No crankshaft phenomenon or distal adding on were observed during those postoperative courses. Conclusions: Although curve pattern of the deformity is similar to adolescent idiopathic scoliosis (AIS), bone quality in patient with TS is lower. In the context of surgical interventions for scoliosis associated with TS, it is imperative to employ surgical techniques that take into account the suboptimal bone quality. If continuation of hormone replacement therapy is planned after corrective surgery for scoliosis in TS patients, it is essential to follow the patient closely postoperatively until bone maturation is complete.
