先天性脊柱侧凸伴高肩胛症的临床评估与手术

The clinical assessment and surgical treatment of congenital scoliosis combined with the Sprengel's deformity

前言 先天性脊柱侧凸（congenital scoliosis,CS）伴高肩胛症（Congenital elelvation of the scapula,Sprengel＇sdeformity）是一种少见而复杂的先天性发育畸形.Tsirikos等[1]分析了537例先天性脊柱畸形患者的肋骨、胸廓及肩胛畸形,在497例CS患者中43例（8.6%）合并高肩胛畸形.Cavendish等[2]报道的100例先天性高肩胛症患者中,有39例（39%）伴CS;与单纯的先天性高肩胛症或CS相比,CS伴高肩胛症的临床评估及手术策略更加复杂,现对这种复杂畸形的临床评估与手术策略作一综述.

Congenital scoliosis （CS） combined with the Sprengel＇s deformity was a rare and complicated congenital deformity. Although the pathogenesis of this complicated deformity was still unclear, some existing studies revealed that Sprengel＇s deformity occurred most frequently in congenital scoliosis with unilateral failure of vertebral segmentation in cervicothoracic or thoracic segments. According to the relationship between the congenital scoliosis and the Sprengel＇s deformity, the complicated congenital deformity could be divided into 3 types： the elevated shoulder occurred on the convex side of the scoliosis, on the concave side of the scoliosis and CS combined with bilateral Sprengel＇s deformity. A thorough physical examination and comprehensive using of three-dimensional computed tomography （CT） and magnetic resonance imaging （MRI） were very important for the assessment of this complicated deformity. The Sprengel＇s deformity occurred on the convex side of upper thoracic congenital scoliosis usually required one-stage surgery to elevate scapula and correct the spinal deformity. The one-stage reduction of the elevated scapula was not necessarily needed when the Sprengel＇s deformity occurred on the concave side of the scoliosis, because the elevated scapula could partially compensate the cosmetic deformity and minimize shoulder asymmetry.