Management of a large pseudomeningocele and cerebrospinal fluid fistula after microsurgical resection of recurrent lipomyelomeningocele in children.

OBJECTIVE

Symptomatic retethering in pediatric patients following complex spinal dysraphism surgery for lipomyelomeningocele (LMMC) repair occurs in approximately 20% of cases. Common complications after repeat surgery for retethering include infection, pseudomeningocele (PMC), and cerebrospinal fluid (CSF) fistula. The authors of this report aim to describe the treatment options for large PMCs or CSF fistulas in a series of patients who underwent repeat surgery for recurrent LMMC at their institution. Additionally, they review management strategies from the literature.

METHODS

This retrospective, descriptive case series includes patients with LMMC who required revision surgery for postoperative PMC or CSF fistula after recurrent untethering procedures at the authors' institution between 2013 and 2023. The surgical strategies for managing PMC and CSF fistula were examined. References for the narrative literature review were sourced from the PubMed and MEDLINE databases.

RESULTS

Eight patients underwent surgery for recurrent retethering due to worsening neurological deficits, including 2 (25.0%) patients who had undergone multiple previous untethering surgeries. The mean duration between symptom onset and repeat surgery was 11.30 ± 5.50 months. Of these 8 cases, 3 (37.5%) developed large postoperative PMCs and CSF fistulas. These patients required a mean of 4.7 ± 2.9 revision surgeries (range 3-8). Management often involved multiple techniques, including local wound revision, dural repair or sealing, mechanical coverage, tissue reconstruction, and external or internal fluid diversion. In all cases, PMCs and CSF fistulas were successfully treated, and at the final follow-up, all patients had stable neurological conditions compared to their preoperative status.

CONCLUSIONS

Managing symptomatic retethering after LMMC repair is challenging, often complicated by PMC and CSF fistulas due to missing normal anatomical tissue layers, large defects, and poor dorsal support. Successful treatment typically requires a combination of techniques to address CSF-related issues. In the authors' experience, a multifaceted approach and familiarity with these methods are essential for achieving optimal outcomes.