伴有神经损害的儿童胸腰椎朗格汉斯组织细胞增生症的手术治疗

Surgical treatment of pediatric thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit

中文关键词:  朗格汉斯组织细胞增生症  儿童脊柱  胸腰椎  神经损害  手术效果

英文关键词:Langerhans cell histiocytosis  Children spine  Thoracolumbar spine  Neurologic deficit  Surgical outcomes

中文摘要:

  【摘要】 目的：总结伴有神经损害的儿童胸腰椎朗格汉斯组织细胞增生症（LCH）的手术治疗效果。方法：2005年1月～2012年12月共有14例伴有神经损害的儿童胸腰椎朗格汉斯组织细胞增生症患者在我科接受手术治疗。其中女性6例，男性8例，年龄2~16岁，平均9.1岁。均表现为不同程度的腰背痛，同时合并有进行性神经功能损害。术前神经功能评估Frankel B 级2例，C级9例，D级3例。病变节段：T3 2例，T4 1例，T8 3例，T11 2例，T12 1例，L1 3例，L2 1例，L3 1例。2006年及以前的早期病例（n=7）采取前后路联合手术，先行后路椎弓根螺钉固定，然后前路切除病变椎体及椎管内病变组织，植入同种异体骨块行椎体间融合；2007年及以后的病例（n=7）改为一期后路手术，椎弓根螺钉固定以后行椎管减压清除椎管内病变组织，并椎板重建、后外侧横突间融合，但保留病变椎体。结果：术中组织病理学检查均确诊为朗格汉斯组织细胞增生症。前后路联合手术组的手术时间为330±122min（240~410min），术中出血量为933±370ml（620~1700ml）；一期后路手术组的手术时间为157±87min（90~210min），术中出血量为523±222ml（375~710ml）。一期后路手术组患者的手术时间和出血量均低于前后路联合手术组，差异有统计学意义（P=0.000，P=0.041）。前后路联合手术组并发症发生率为42.9%（3/7），均为前路手术入路相关并发症，其中左侧肋间神经痛1例，使用非甾体类药物治疗2周后缓解；左侧胸腔积液2例，留置胸腔闭式引流1周后治愈。一期后路手术组无并发症发生。术后2~12周所有患者的神经功能均恢复至Frankel E级。所有患者随访24~64个月，平均40.8个月。随访期间无内固定松动、断裂、融合失败发生，前后路联合手术组无复发病例，后路手术组未见保留的病变椎体进展。结论：对于伴有神经功能损害的儿童LCH，前后路联合手术和一期后路手术均能获得满意的临床效果。一期后路手术能够避免前路开胸并发症，保留的病变椎体没有进展。

英文摘要:

  【Abstract】 Objectives: To review the surgical outcomes of pediatric thoracic or lumbar spinal Langerhans cell histiocytosis(LCH) complicated with neurologic deficit. Methods: Fourteen children suffering from thoracic or lumbar spinal LCH accompanied by neurologic deficit received surgical treatment from January 2005 to December 2012. There were 6 girls and 8 boys with an average age of 9.1 years old(range, from 2 to 16 years). All patients presented with local back pain and progressive neurologic deterioration. Frankel grade B was noted in 2 patients, grade C in 9 and grade D in 3. The lesion was noted at T3(2 cases), T4(1 case), T8(3 case), T11(2 cases), T12(1 case), L1(3 cases), L2(1 case) and L3(1 case). Posterior instrumentation combined with anterior corpectomy, decompression and allograft was performed in the early 7 patients(before 2006), while posterior pedicle screw fixation, laminectomy and repair of laminae with allograft was performed in 7 cases(after 2007). Results: Intraoperative histopathological examination was performed in all patients. The operation time was 330±122 minutes in combined approach group and 157±87 minutes in single approach group(P=0.000). The average blood loss was 933±370ml in combined procedure and 523±222ml in single posterior approach(P=0.041). Three of seven(42.9%) patients experiencing combined surgery were noted complications, which included left intercostal neuroglia(resolved after using NSAIDs for 2 weeks) in 1 case and left side pleural effusion(treated with drainage for 1 week) in 2 cases. However, no complications were noted in single approach group. The mean follow-up duration was 40.8 months(range, 24-64 months). No recurrence was noted in patients undergoing combined surgery. All patients had neurologic function recovered to Frankel E from 2 to 12 weeks after surgery. Conclusions: For pediatric spinal LCH, both combined and single posterior approaches can achieve good outcomes in relieving neurologic deficit. However, single posterior approach is more favorable with less complications and less risk of recurrence in residual vertebral body.