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Cerebral haemorrhagic risk in children with sickle-cell disease
Manoelle Kossorotoff1, Valentine Brousse, David Grevent
1Paediatric Neurology, French Centre for Paediatric Stroke, Necker-Enfants Malades University Hospital, APHP and Inserm UMR-S1140, Paris, France.
Insights
Modern stroke prevention strategies did not alter the risk ratio of hemorrhagic to ischemic events in children with sickle cell disease (SCD). Intracranial aneurysms in pediatric SCD share traits with adult SCD, suggesting a common pathophysiology.
Area of Science:
- Pediatric Neurology
- Hematology
- Vascular Medicine
Background:
- Sickle cell disease (SCD) poses a significant risk for cerebrovascular events in children.
- Modern stroke prevention strategies aim to mitigate these risks, but their impact on hemorrhagic complications requires further investigation.
Purpose of the Study:
- To evaluate the risk of cerebral hemorrhage in children with SCD managed with contemporary stroke prevention methods.
- To characterize intracranial aneurysms in pediatric SCD and compare them to adult SCD findings.
Main Methods:
- A retrospective analysis of a pediatric SCD cohort over a 9-year period.
- Defining hemorrhagic risk by intracranial hemorrhage or aneurysm diagnosis.
- Defining ischemic risk by ischemic events or neuroimaging findings.
Main Results:
- Among 251 patients, 36 had ischemic risk and 7 had hemorrhagic risk; 5 patients had both.
- The average age at first hemorrhagic symptom was older (10.4 years) compared to ischemic risk (6.2 years).
- Nine intracranial saccular aneurysms were identified, predominantly in the posterior circulation.
Conclusions:
- The ratio of ischemic to hemorrhagic risk in pediatric SCD remains unchanged despite modern management.
- Pediatric SCD-associated intracranial aneurysms exhibit characteristics similar to those in adults with SCD.
- Findings suggest a shared pathophysiology between anterior stenosis and posterior dilation in SCD, potentially guiding unified management strategies.
Aim:
To address risk of first or recurrent cerebral haemorrhage in children with sickle-cell disease (SCD) who are being managed with modern stroke prevention strategies.
Method:
A systematically followed SCD paediatric cohort was retrospectively studied over a 9-year period. Haemorrhagic risk was defined as intracranial haemorrhage occurrence or intracranial aneurysm diagnosis during the study period. Ischaemic risk was defined as cerebrovascular ischaemic event occurrence or transcranial Doppler/magnetic resonance imaging hallmarks of ischaemic risk finding during the study period.
Results:
Among the 251 patients in the cohort, 36 patients were included in the ischaemic group. Seven patients were included in the haemorrhagic group, of which five also met the criteria for the ischaemic group. Age at first haemorrhagic symptom/hallmark of risk was older (10.4 vs 6.2 years old, p=0.036). Nine intracranial saccular aneurysms were found, mostly on the posterior circulation. Two patients had endovascular embolization.
Interpretation:
The ratio of ischaemic to haemorrhagic risk was not modified with modern management compared with historical series. Intracranial aneurysm in children with SCD had specific characteristics, close to intracranial aneurysms described in adults with SCD. Data favoured concurrent development of intracranial SCD-associated anterior stenosis and posterior dilation, suggesting common pathophysiology and management strategies.
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