Hayashi Toshiaki, Kimiwada Tomomi, Shirane Reizo, Tominaga Teiji
1Department of Neurosurgery, Miyagi Children's Hospital, Sendai; and.
2Tohoku University Graduate School of Medicine, Sendai, Japan.
J Neurosurg Pediatr. 2021 Nov 19;29(3):342-349. doi: 10.3171/2021.9.PEDS21413. Print 2022 Mar 1.
Lipoma of the conus medullaris (LCM) causes neurological symptoms known as tethered cord syndrome (TCS). The symptoms can be seen at diagnosis and during long-term follow-up. In this report, pediatric patients with LCMs who underwent untethering surgery, under the policy of performing surgery if diagnosed regardless of symptoms, were retrospectively reviewed to evaluate long-term surgical outcomes. Possible risk factors for retethered cord syndrome (ReTCS) were evaluated in the long-term follow-up period.
A total of 51 consecutive pediatric patients with LCMs who underwent a first untethering surgery and were followed for > 100 months were retrospectively analyzed. The surgery was performed with the partial removal technique. Pre- and postoperative clinical and radiological data were reviewed to analyze the outcomes of surgery and identify potential risk factors for ReTCS.
During follow-up, 12 patients experienced neurological deterioration due to ReTCS. The overall 10-year and 15-year progression-free survival rates were 82.3% and 75.1%, respectively. On univariate analysis, a lipoma type of lipomyelomeningocele (OR 11, 95% CI 2.50-48.4; p = 0.0014), patient age at the time of surgery (OR 0.41, 95% CI 0.14-1.18; p = 0.0070), and the mean patient growth rate after surgery (OR 2.00, 95% CI 1.12-3.41; p = 0.0040) were significant factors associated with ReTCS. Cox proportional hazard models showed that a lipoma type of lipomyelomeningocele (HR 5.16, 95% CI 1.54-20.1; p = 0.010) and the mean growth rate after surgery (HR 1.88, 95% CI 1.00-3.50; p = 0.040) were significantly associated with the occurrence of ReTCS.
More complex lesions and a high patient growth rate after surgery seemed to indicate increased risk of ReTCS. Larger prospective studies and registries are needed to define the risks of ReTCS more adequately.
圆锥脂肪瘤(LCM)可引发称为脊髓栓系综合征(TCS)的神经症状。这些症状在诊断时及长期随访期间均可出现。在本报告中,我们对接受松解手术的LCM患儿进行了回顾性研究,这些患儿遵循一旦确诊即行手术的原则,无论有无症状,以评估长期手术效果。在长期随访期间评估了再栓系综合征(ReTCS)的可能危险因素。
对51例连续接受首次松解手术且随访时间超过100个月的LCM患儿进行回顾性分析。手术采用部分切除技术。回顾术前和术后的临床及影像学数据,以分析手术效果并确定ReTCS的潜在危险因素。
在随访期间,12例患者因ReTCS出现神经功能恶化。总体10年和15年无进展生存率分别为82.3%和75.1%。单因素分析显示,脂肪瘤型脊髓脊膜膨出(OR 11,95%CI 2.50 - 48.4;p = 0.0014)、手术时患者年龄(OR 0.41,95%CI 0.14 - 1.18;p = 0.0070)以及术后患者平均生长速率(OR 2.00,95%CI 1.12 - 3.41;p = 0.0040)是与ReTCS相关的显著因素。Cox比例风险模型显示,脂肪瘤型脊髓脊膜膨出(HR 5.16,95%CI 1.54 - 20.1;p = 0.010)和术后平均生长速率(HR 1.88,95%CI 1.00 - 3.50;p = 0.040)与ReTCS的发生显著相关。
更复杂的病变以及术后患者高生长速率似乎表明ReTCS风险增加。需要更大规模的前瞻性研究和登记系统来更充分地界定ReTCS的风险。