Long-term outcomes following posterior fossa decompression in pediatric patients with Chiari malformation type 1, a population-based cohort study.

OBJECTIVE

Posterior fossa decompression for Chiari malformation type I (Chiari 1) is effective and associated with a low risk of complication. However, up to 20% of patients may experience continued deficits or recurring symptoms after surgical intervention. For pediatric patients, there are no established tools to predict outcomes, and the risk factors for unfavorable postoperative outcomes are poorly understood. Hence, our aim was to investigate baseline data and early postoperative predictors of poor outcomes as determined by the Chicago Chiari outcome scale (CCOS).

METHODS

All pediatric patients (< 18 years) receiving a posterior fossa decompression for Chiari 1 between the years of 2005 and 2020 at the study center were eligible for inclusion. Patients with congenital anomalies were excluded.

RESULTS

Seventy-one pediatric patients with a median age of 9 years were included. Most patients (58%) were females. Chiari 1 was associated with syringomyelia (51%), scoliosis (37%), and hydrocephalus (7%). Perioperative complications occurred in 13 patients (18%) of which two required additional procedures under general anesthesia. On multivariable proportional odds logistic regression, motor deficits (OR: 0.09; CI95%: [0.01-0.62]; p = 0.015), and surgical complications (OR: 0.16; CI95%: [0.41-0.66]; p = 0.011) were significant predictors of worse outcomes. The presence of syringomyelia was identified as a predictor of better outcomes (OR: 4.42 CI95% [1.02-19.35]; p = 0.048). A persistent hydrocephalus during the early postoperative period after posterior fossa decompression was a strong predictor of worse long-term CCOS (OR: 0.026; CI95%: [0.002-0.328]; p = 0.005).

CONCLUSION

Results from this study indicate that the existence of motor deficits and syringomyelia prior to surgery, and surgical complications and persistent hydrocephalus despite posterior fossa decompression, were useful predictors of long-term outcome.