Motosuneya T, Asazuma T, Tsuji T, Watanabe H, Nakayama Y, Nemoto K
Department of Orthopaedic Surgery, National Defense Medical College, Namiki, Tokorozawa, Saitama, Japan.
J Orthop Surg (Hong Kong). 2006 Dec;14(3):346-9. doi: 10.1177/230949900601400324.
Costello syndrome is characterised by dwarfism, unique cutaneous lesions, a distinct facial gestalt, and mental retardation. There have been no detailed reports of severe spinal deformities requiring surgical treatment as a complication of Costello syndrome. We report a case of a 10-year-old girl with progressive scoliosis associated with Costello syndrome. She underwent anterior release and posterior surgical correction and fusion from T5 to L2 using a third generation hook and rod system plus spinous process wiring. Congenital portal vein deficiency and coagulopathy were other major complications. At 15-month follow-up, the patient had good balance and no evidence of instrumentation failure.
科斯特洛综合征的特征为侏儒症、独特的皮肤损害、独特的面部形态以及智力发育迟缓。目前尚无关于作为科斯特洛综合征并发症的严重脊柱畸形需要手术治疗的详细报告。我们报告一例10岁患有与科斯特洛综合征相关的进行性脊柱侧凸的女孩。她接受了前路松解以及后路手术矫正和融合,从T5至L2使用了第三代钩棒系统加棘突钢丝固定。先天性门静脉缺如和凝血病是其他主要并发症。在15个月的随访中,患者平衡良好,且没有内固定失败的迹象。