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Functional outcome after initial and multiple intracerebral hemorrhages in children with cerebral cavernous
Alejandro N Santos1,2, Laurèl Rauschenbach1,2, Hannah Hadice Gull1,2
1Department of Neurosurgery and Spine Surgery, University Hospital Essen, Essen, Germany.
Insights
Pediatric patients with brainstem cavernous malformations face worse outcomes after a second hemorrhage. Functional status declines with each subsequent bleed, highlighting the need for careful management of pediatric cerebral cavernous malformations.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroscience
Background:
- Cerebral cavernous malformations (CCMs) can cause intracerebral hemorrhage (ICH) in children.
- Multiple ICH events can lead to significant neurological deficits.
Purpose of the Study:
- To evaluate functional outcomes after single and multiple ICH in pediatric CCM patients.
- To identify predictors of poor outcomes following recurrent ICH.
- To assess the risk of a third ICH within the first year after a second ICH.
Main Methods:
- Retrospective analysis of 55 pediatric patients (≤18 years) with CCM-related ICH treated between 2003-2021.
- Assessment of neurological functional status using modified Rankin Scale scores.
- Kaplan-Meier analysis to determine the 1-year risk of third ICH after a second ICH.
Main Results:
- Brainstem cavernous malformation (BSCM) was a significant predictor of unfavorable outcome after a second ICH (p=0.019).
- Outcomes worsened significantly after the second ICH compared to the first, and further after a third ICH.
- The cumulative 12-month risk of rebleeding after a second ICH was 10.7%.
Conclusions:
- Pediatric patients with BSCM have an elevated risk of worse functional outcomes after a second ICH.
- While functional status may improve over time, each subsequent ICH event leads to a decline compared to baseline or prior states.
- Recurrent ICH in pediatric CCM patients is associated with progressive neurological deterioration.
Background And Purpose:
We aimed to assess the course and predictors of functional outcome after single and multiple intracerebral hemorrhage (ICH) in pediatric patients with cerebral cavernous malformations (CCMs) and to conduct a risk assessment of a third bleed during the first follow-up year after second ICH.
Methods:
We included patients aged ≤18 years with complete baseline characteristics, a magnetic resonance imaging dataset, ≥1 CCM-related ICH and ≥1 follow-up examination, who were treated between 2003 and 2021. Neurological functional status was obtained using modified Rankin Scale scores at diagnosis, before and after each ICH, and at last follow-up. Kaplan-Meier analysis was performed to determine the cumulative 1-year risk of third ICH.
Results:
A total of 55 pediatric patients (median [interquartile range] age 12 [11] years) were analyzed. Univariate analysis identified brainstem cavernous malformation (BSCM; p = 0.019) as a statistically significant predictor for unfavorable outcome after second ICH. Outcome after second ICH was significantly worse in 12 patients (42.9%; p = 0.030) than after first ICH and in five patients (55.6%; p = 0.038) after a third ICH compared to a second ICH. Cumulative 12-month risk of rebleeding during the first year after a second ICH was 10.7% (95% confidence interval 2.8%-29.37%).
Conclusions:
Pediatric patients with a BSCM have a higher risk of worse outcome after second ICH. Functional outcome improves over time after an ICH but worsens following each ICH compared to baseline or previous ICH. Second bleed was associated with neurological deterioration compared to initial ICH, and this deteriorated further after a third ICH.
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