Cerebral haemorrhagic risk in children with sickle‐cell disease

Abstract

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.

Intracranial Aneurysms in Sickle-Cell Disease Are Associated With the Hemoglobin SS Genotype But Not With Moyamoya Syndrome

“…7 In the general population, it is estimated that 20% of cases have multiple aneurysms. 10 We also found a higher propensity for a location in the posterior circulation as noted previously 11,12 (for comparison, in the general population, an often quoted figure is 8% from the International Cooperative Study on the Timing of Aneurysm Surgery 13 ). The reason for this remains obscure.…”

Central nervous system complications and management in sickle cell disease

“…42 In a French cohort, 2.8% of children with SCD (7 of 251) had either an intracranial hemorrhage or an unruptured aneurysm. 43,44 Risk factors for cerebral hemorrhage are poorly defined, but include hypertension associated with the use of corticosteroids 40 or phenylephrine, 45 recent transfusion, 40 splenic sequestration, 46 and hyperviscosity associated with higher hemoglobin levels. 16 Cerebral hemorrhage in adults is commonly related to aneurysm formation (see supplemental Figure 1C-D).…”

Acute spontaneous intracranial epidural haematoma and disseminated intravascular coagulation in a paediatric sickle cell patient

“…The incidence of strokes in paediatrics is about 1/100 000, with a 200-fold increase seen in SCD 15. Cerebrovascular complications of SCD develop in up to 36% of patients with SCD,16–18 with cerebral infarction accounting for 78%, intracerebral haemorrhage 20% and subarachnoid haemorrhage 2% 16.…”