Risk Factors for Pre-operative Functional Deterioration in Children with Lipomyelomeningocele

Purpose: 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.

Methods: 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.

Results: 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).

Conclusion: 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.