Departments of1Neurosurgery and.
2University of Missouri School of Medicine, Columbia, Missouri; and.
J Neurosurg Pediatr. 2024 Mar 8;33(6):554-563. doi: 10.3171/2023.11.PEDS23328. Print 2024 Jun 1.
The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery.
The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment.
Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001).
The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.
与接受后天闭合手术的患者相比，接受产前手术治疗的脊髓脊膜膨出（MMC）患者需要永久性脑脊液分流的需求较低。两组之间的大脑发育和头部生长差异尚不清楚，特别是对于那些不需要手术治疗脑积水的患者。本研究的目的是确定头部生长的差异，并为接受产前或后天手术的患者生成特定于 MMC 的头围（HC）生长曲线。
作者从圣路易斯儿童医院回顾性地确定了 2016 年至 2021 年期间接受 MMC 治疗的患者。HC 数据从出生开始到最近的随访或脑积水治疗。使用非线性最小二乘回归分析将数据拟合到四个模型中：双项幂、戈珀兹、韦布尔。随后，评估了这些曲线在预测脑积水治疗中的有效性。
本研究共纳入 61 例患者（女性 29 例[47.5%]，25 例[41%]接受产前手术，出生时平均胎龄为 36.6 周）。韦布尔模型最适合 HC 数据（产前调整后的 R2=0.95，产后调整后的 R2=0.95），而戈珀兹模型的拟合效果最差（产前调整后的 R2=0.56，产后调整后的 R2=0.39）。产前 MMC 修复患者的 HC 测量值明显大于后天修复患者。确定韦布尔曲线的第 50 百分位数作为脑积水治疗的有用阈值：HC 测量值超过并保持在该阈值以上的儿童更有可能接受脑积水治疗，无论 MMC 修复的时间如何（产前相对风险[RR]10.0[95%CI 1.424-70.220]，敏感性 85.7%[95%CI 0.499-0.984]，特异性 82.4%[95%CI 0.600-0.948]；产后 RR 4.750[95%CI 1.341-16.822]，敏感性 90.5%[95%CI 0.728-0.980]，特异性 75.0%[95%CI 0.471-0.924]）。接受产前治疗的 MMC 患者的 HC 生长曲线明显大于世界卫生组织 HC 曲线（p<0.001）。
确定韦布尔模型为 MMC 患者 HC 生长曲线的最佳拟合模型，可作为脑积水治疗的有用预测指标。对于患有脑积水的 MMC 患者，接受产前治疗的患者很好地符合该模型，但接受后天治疗的患者不符合，这可能表明脑积水发育的机制不同。与一般人群和后天治疗的患者相比，接受产前治疗的患者的 HC 测量值明显更大。需要进一步研究以了解接受产前治疗的 MMC 患者中那些具有较大 HC 测量值且无症状的患者的长期认知结果和最佳管理方法。
