Jones Morgan, Breakwell Lee, Cole Ashley, Arundel Paul, Bishop Nick
Sheffield Children's Hospital, Sheffield, UK.
Eur Spine J. 2018 Sep;27(9):2079-2084. doi: 10.1007/s00586-018-5465-8. Epub 2018 Feb 19.
The objective of this article is to report a case of type V osteogenesis imperfecta (OI) undergoing posterior instrumented fusion for scoliosis. Type V OI is a moderately severe dysplasia causing primary defects in endochondral bone ossification or mineralisation. It is characterised by hyperplastic callus (HPC) formation, interosseous membrane calcifications, poor bone quality and spinal deformities including scoliosis. Data on the surgical management of spinal deformities in this patient group are lacking.
A 16-year-old patient with a confirmed diagnosis of type V OI presented with a progressive scoliosis. The patient underwent a T3-L4 posterior instrumented correction and fusion utilising pedicle screws, pedicle hooks and sub-laminar wiring. At 4 months after surgery, the pedicle hooks pulled out and required partial metalwork removal after CT scanning confirmed bony union and no evidence of HPC formation. The patient was successfully discharged with satisfactory correction, confirmed bony union, no neurologic complication and absence of any hyperplastic callus formation.
Type V OI patients requiring surgical intervention for scoliosis correction can safely undergo posterior instrumented fusion using sublaminar wiring and pedicle hook/screw constructs without apparent risk of HPC formation around neural elements. Surgery in this patient group remains challenging due to the associated poor bone quality.
V.
本文旨在报告一例Ⅴ型成骨不全症（OI）患者接受后路器械融合治疗脊柱侧弯的病例。Ⅴ型OI是一种中度严重的发育异常，可导致软骨内骨化或矿化的原发性缺陷。其特征为增生性骨痂（HPC）形成、骨间膜钙化、骨质不佳以及包括脊柱侧弯在内的脊柱畸形。目前缺乏该患者群体脊柱畸形手术治疗的数据。
一名确诊为Ⅴ型OI的16岁患者出现进行性脊柱侧弯。该患者接受了T3 - L4后路器械矫正及融合术，使用了椎弓根螺钉、椎弓根钩和椎板下钢丝。术后4个月，椎弓根钩脱出，在CT扫描确认骨愈合且无HPC形成迹象后，需要部分取出金属内固定物。患者成功出院，矫正效果满意，骨愈合良好，无神经并发症，也未出现任何增生性骨痂形成。
因脊柱侧弯矫正而需要手术干预的Ⅴ型OI患者，可以安全地接受后路器械融合术，使用椎板下钢丝和椎弓根钩/螺钉结构，且神经周围无明显的HPC形成风险。由于骨质不佳，该患者群体的手术仍然具有挑战性。
Ⅴ级。
