Oxborrow Neil, Sundarapandian Rajkumar
Royal Manchester Children's Hospital, Oxford Road, Manchester, UK.
J Surg Case Rep. 2018 Jun 8;2018(6):rjy120. doi: 10.1093/jscr/rjy120. eCollection 2018 Jun.
Mucopolysaccharidosis is a rare group of genetic disorder which results in a complex of anomalies involving various systems. In Mucopolysaccharidosis 1 progressive thoracolumbar kyphosis is a common presentation which can result in instability and neurological deficit. Posterior spinal surgery is performed to correct deformity and obtain spinal fusion. Peptide enhanced bone graft substitute (i-Factor) is relatively a new component with proven efficacy to obtain early spinal fusion. An 8-year-old child with progressive high lumbar kyphosis due to Mucopolysaccharidosis 1 was admitted for Posterior spinal fusion with i-Factor bone graft substitute. Postoperatively patient had serous discharge from the wound which settled without intervention. A month after the surgery spinal radiographs revealed heterotopic ossification at the distal end of spinal construct in the paraspinal region. Patient remained asymptomatic and clinically well.
黏多糖贮积症是一组罕见的遗传性疾病，可导致涉及多个系统的一系列异常。在黏多糖贮积症1型中，进行性胸腰椎后凸是常见表现，可导致脊柱不稳定和神经功能缺损。需进行后路脊柱手术以矫正畸形并实现脊柱融合。肽增强骨移植替代物（i-Factor）是一种相对较新的成分，已证实其在实现早期脊柱融合方面具有疗效。一名因黏多糖贮积症1型导致进行性高位腰椎后凸的8岁儿童因使用i-Factor骨移植替代物进行后路脊柱融合术入院。术后患者伤口有浆液性渗出，未经干预自行消退。术后一个月，脊柱X线片显示脊柱结构远端椎旁区域有异位骨化。患者无症状，临床情况良好。
