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Long-term outcomes following posterior fossa decompression in pediatric patients with Chiari malformation type 1, a
Victor Gabriel El-Hajj1, Erik Öhlén1, Ulrika Sandvik1
1Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Insights
Posterior fossa decompression for Chiari malformation type I is effective, but predictors of poor outcomes in pediatric patients are unclear. Motor deficits, surgical complications, and persistent hydrocephalus predict worse long-term results, while syringomyelia predicts better outcomes.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Surgical Outcomes Research
Background:
- Posterior fossa decompression (PFD) is a standard treatment for Chiari malformation type I (CMI).
- Up to 20% of pediatric patients experience persistent or recurring symptoms post-surgery.
- Predictive tools for long-term outcomes in pediatric CMI patients are lacking.
Purpose of the Study:
- To identify baseline and early postoperative predictors of unfavorable outcomes in pediatric CMI patients.
- To utilize the Chicago Chiari Outcome Scale (CCOS) for outcome assessment.
- To improve understanding of risk factors for poor surgical results in children.
Main Methods:
- Retrospective study of pediatric patients (<18 years) undergoing PFD for CMI (2005-2020).
- Exclusion of patients with congenital anomalies.
- Multivariable proportional odds logistic regression analysis to identify predictors of CCOS.
Main Results:
- Motor deficits and surgical complications were significant predictors of worse outcomes (p<0.05).
- Syringomyelia was associated with better outcomes (OR: 4.42, p=0.048).
- Persistent hydrocephalus post-PFD strongly predicted worse long-term CCOS (OR: 0.026, p=0.005).
Conclusions:
- Preoperative motor deficits and syringomyelia are key indicators of surgical outcome.
- Postoperative surgical complications and persistent hydrocephalus are critical predictors of long-term CCOS.
- These findings can aid in patient counseling and management strategies for pediatric CMI.
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.
