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How I manage cerebral vasculopathy in children with sickle cell disease
Valentine Brousse1,2,3,4, Manoelle Kossorotoff5, Mariane de Montalembert1,3,4
1Paediatrics and Sickle-Cell Centre, University Hospital Necker-Enfants Malades, APHP, Paris, France.
Insights
Sickle cell disease causes brain damage in children, leading to strokes and silent infarcts. Annual transcranial Doppler screening can prevent most strokes in children with sickle cell anaemia.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) significantly impacts brain health, affecting both large and small blood vessels.
- Children with SCD, particularly HbSS and HbS-β(0) genotypes (sickle cell anemia), are susceptible to overt neurovascular complications like stroke and hemorrhage.
- Silent cerebral infarction, cognitive deficits, and headaches are also prevalent in affected children.
Purpose of the Study:
- To review the neurovascular complications of sickle cell disease in children.
- To highlight the effectiveness of transcranial Doppler screening in stroke prevention.
- To discuss the implications of silent cerebral infarction and potential interventions.
Main Methods:
- Review of literature on sickle cell disease and its neurological manifestations.
- Analysis of stroke incidence and risk factors in pediatric SCD patients.
- Evaluation of screening methods like transcranial Doppler and MRI.
- Discussion of treatment strategies including blood transfusions and hydroxycarbamide.
Main Results:
- Stroke incidence in children with sickle cell anemia peaks between ages 2-5 years and correlates with anemia severity.
- Annual transcranial Doppler screening from ages 2-16 can prevent most strokes when combined with chronic blood transfusions for elevated flow velocities.
- Over one-third of children exhibit silent cerebral infarction on MRI, linked to cognitive impairments.
Conclusions:
- Transcranial Doppler screening is crucial for preventing overt strokes in children with sickle cell anemia.
- Silent cerebral infarcts are common and associated with cognitive issues, warranting screening and supportive care.
- The roles of hydroxycarbamide and chronic transfusions in managing abnormal screening results and silent infarcts require further investigation.
Abstract:
Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the HbSS or HbS-β(0) genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient-years) and increases with the severity of the anaemia. Most strokes can be prevented by annual transcranial Doppler screening from 2 to 16 years of age and providing chronic blood transfusion when this investigation shows elevated blood-flow velocities. The role for hydroxycarbamide in children with abnormal transcranial Doppler findings is under investigation. After a stroke, chronic blood transfusion is very strongly recommended, unless haematopoietic stem cell transplantation can be performed. Routine magnetic resonance imaging shows that more than one-third of children have silent cerebral infarction, which is associated with cognitive impairments. Screening for silent infarcts seems legitimate, since their presence may lead to supportive treatments. The role for more aggressive interventions such as hydroxycarbamide or chronic blood transfusion is debated.
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