Department of Neurological Sciences, Christian Medical College, Vellore, 632004, India.
Childs Nerv Syst. 2022 Mar;38(3):587-595. doi: 10.1007/s00381-021-05404-4. Epub 2021 Nov 3.
To characterize the temporal profile of pre-operative deterioration in children with lipomyelomeningocele (LMMC) including those with congenital deficits and identify risk factors for clinical worsening.
Records of 87 children who underwent surgery for LMMC were retrospectively reviewed to study the temporal profile of pre-operative deterioration, defined by the onset of new neurological dysfunction or progression of a pre-existing deficit. Preoperative magnetic resonance imaging (MRI) studies were examined to identify radiological features associated with deterioration. In children with extra-spinal placodes, the angle subtended by the terminal placode with the cord at the level of the laminar-fascial defect ("J sign") was assessed.
Pre-operative deterioration in function was seen in 37 children (43%), occurring at a median age of 36 months and was more frequent in children without congenital deficits (54% versus 27%; p = 0.016). On Cox regression analysis, extra-spinal location of the placode (p = 0.003) and presence of a congenital deficit (p = 0.009) were positively and negatively associated with deterioration respectively. On Kaplan-Meier analysis, the median deterioration-free survival time was 72 months and was positively associated with presence of congenital deficit (p = 0.026) and negatively associated with presence of an extra-spinal placode (p < 0.001) or a meningocele sac (p = 0.001).
Extra-spinal location of the neural placode was associated with higher risk of clinical deterioration in children with LMMC, whereas the presence of a congenital deficit conferred a decreased risk. Risk stratification based on clinical and radiological features can be used to guide decisions regarding early prophylactic surgery in children with LMMC.
描述伴有先天性缺陷的脂肪脊膜脊髓膨出（LMMC）患儿术前恶化的时间特征，并确定临床恶化的危险因素。
回顾性分析 87 例行 LMMC 手术患儿的病历，研究术前恶化的时间特征，定义为新发神经功能障碍或现有缺陷进展。检查术前磁共振成像（MRI）研究，以确定与恶化相关的影像学特征。在脊髓外脊膜膨出患儿中，评估终板与板层筋膜缺陷水平脊髓之间的夹角（“J 征”）。
37 例患儿（43%）出现术前功能恶化，中位年龄为 36 个月，无先天性缺陷患儿更常见（54%比 27%；p=0.016）。Cox 回归分析显示，脊膜膨出位于脊髓外（p=0.003）和存在先天性缺陷（p=0.009）分别与恶化呈正相关和负相关。Kaplan-Meier 分析显示，无恶化的中位生存时间为 72 个月，与存在先天性缺陷（p=0.026）呈正相关，与脊膜膨出位于脊髓外（p<0.001）或存在脑膜膨出囊（p=0.001）呈负相关。
LMMC 患儿中，脊膜膨出位于脊髓外与临床恶化风险增加相关，而存在先天性缺陷则降低了风险。基于临床和影像学特征的风险分层可用于指导存在 LMMC 患儿的早期预防性手术决策。
