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The management of pediatric Chiari I malformation with concomitant hydrocephalus: a multicenter retrospective
Ladina Greuter1,2, Tim Jonas Hallenberger1, Maria Licci1,2
1Department of Neurosurgery, University Hospital Basel, Basel, Switzerland.
Insights
Extradural foramen magnum decompression showed the best outcomes for children with Chiari I malformation and hydrocephalus. Intradural decompression had the least favorable success-to-complication ratio, guiding future treatment discussions.
Area of Science:
- Pediatric Neurosurgery
- Cerebrospinal Fluid Disorders
- Congenital Malformations
Background:
- Chiari I malformation (CIM) co-occurs with hydrocephalus in 7-10% of pediatric cases.
- The causal relationship between CIM and hydrocephalus is debated, complicating treatment decisions.
- Optimal surgical strategies for pediatric CIM with hydrocephalus remain unclear.
Purpose of the Study:
- To analyze management strategies and outcomes in children with CIM and hydrocephalus.
- To evaluate the effectiveness and complication rates of different surgical interventions.
- To identify risk factors for treatment failure in this patient population.
Main Methods:
- Retrospective multicenter study of pediatric patients (<18 years) treated for CIM and hydrocephalus (2005-2021).
- Analysis of revision surgery rates as the primary outcome for treatment failure.
- Descriptive survival and multivariable analyses to identify risk factors.
Main Results:
- Extradural foramen magnum decompression (FMD) demonstrated a 100% success rate with a 20% complication rate.
- Intradural FMD had a 33% success rate and an 8.3% complication rate.
- Ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV) showed varying success and complication rates, with extradural FMD being most favorable.
Conclusions:
- Significant heterogeneity exists in surgical approaches for pediatric CIM with hydrocephalus.
- Extradural FMD offers the most favorable success-to-complication ratio compared to other methods.
- Further prospective studies are necessary to optimize treatment strategies for these complex cases.
Background:
Chiari I malformation (CIM) rarely occurs alongside hydrocephalus (7-10%), and the relationship between the two-whether one is the cause or the effect of the other-remains contentious. Consequently, the optimal treatment approach for these patients is unclear. This multicenter, retrospective study analyzed management strategies and outcomes for children presenting with CIM and hydrocephalus, with or without syringomyelia.
Methods:
We reviewed cases from 2005 to 2021 involving children (< 18 years) who underwent surgical treatment for CIM with hydrocephalus, with or without syringomyelia, at ten international pediatric neurosurgical centers. The primary outcome was the rate of revision surgery due to treatment failure, defined as the need for additional surgery due to unsatisfactory treatment of CIM and/or hydrocephalus by the initial intervention. Descriptive survival analyses and a step-down multivariable analysis to identify possible risk factors influencing the outcome were performed.
Results:
The study included 49 patients (mean age 68.5 months, 65.3% male). Increased head circumference was noted in 40.8% of patients, and 24.4% reported preoperative headaches. Primary surgical treatments included CSF diversion in 61.2% of cases (42.9% VPS, 18.4% endoscopic third ventriculostomy [ETV]), intradural foramen magnum decompression (FMD) in 24.5%, and extradural FMD in 10.2%. The success-to-complication ratio was most favorable for extradural FMD (100% success, 20% complication), followed by ETV (44% success, 0% complication), VPS (76.2% success, 47.6% complication), and intradural FMD (33% success, 8.3% complication). At discharge and at the latest follow-up, 76% and 69% of patients showed improved Chicago Chiari Outcome Scale scores, respectively. The primary treatment modality and the bulging of the lamina terminalis were significant potential risk factors for the failure rate after multivariate analysis.
Conclusion:
Our data reveals considerable heterogeneity in treatment approaches. Extradural FMD exhibited the most favorable success-to-complication ratio, while intradural FMD had the least favorable ratio. These findings provide some basis for informed discussion with families. Larger prospective studies are still needed to refine treatment strategies.
