The Development and Management of Cerebrospinal Fluid Leaks following Surgical Management of Spinal Dysraphism: A Systematic Review of The Literature

Introduction: Due to changes in cerebrospinal fluid (CSF) dynamics associated with neural tube defects (NTDs), CSF leaks frequently occur during primary management of NTDs such as myelomeningocele (MMC) and fatty filum terminale (FFT). This study aims to evaluate postoperative CSF leak management in pediatric patients with NTDs.

Methods: A systematic review of the literature using PubMed, Google Scholar, and Web of Science electronic databases was completed according to the PRISMA guidelines to highlight articles examining CSF leaks following management of spinal dysraphism.

Results: 54 articles including 40 retrospective studies with 3463 patients and 14 case reports totaling 24 patients were analyzed. In the retrospective studies, the average age of patients was 36.7±51.7 months, 880 (25.4%) developed CSF leaks following management of the underlying spinal dysraphism, and 328 patients (9.47%) developed secondary CSF leaks following management of the initial CSF leak. Most CSF leaks were managed surgically (1017, 43.78%). Of the 24 case report patients (36.7±51.7 months), there were 12 males (52.2%). The primary diagnoses were meningoceles (8), myelocystoceles (9), myelomeningocele (6), FFT (2), and lipomyelomeningocele (2). All 24 patients had surgical management of the CSF leak (CSF shunt: 6, 25%; bilateral V.Y. flap: 2, 8.33%). The rate of development of secondary CSF leaks (8, 50%) was not associated with the type of treatment used to correct patients’ initial CSF leak (shunt: 25.0% vs. 50%, p=0.608; bilateral V.Y. flap: 12.5% vs. 12.5%, p=1.000). Unlike with primary CSF leaks observed in this study, not all patients received surgery to correct the secondary CSF leak (5, 62.5%). Neurologic improvement at final follow-up was not significantly different (4 (80.0%) vs. 7 (100.0%), p=0.417).

Conclusion : While secondary CSF leaks did not influence overall outcome, the high prevalence rate highlights the importance of developing mechanisms to reduce CSF leak development in pediatric patients with NTDs.