Stroke in paediatric patients with sickle-cell anaemia

Introduction. Sickle-cell anaemia is the severe homozygotic form of drepanocytosis, a genetic disorder that often occurs among black people and which is characterised by the production of haemoglobin S, chronic hemolytic anaemia and tissue ischaemia due to alterations in blood flow. A quarter of the patients presented neurological manifestations; 8-10% of children will have a stroke. Aim. To analyse the cases of stroke in children with sickle-cell anaemia in our centre. Patients and methods. We conducted a retrospective descriptive study of children with sickle-cell anaemia and stroke. Results. Five patients (two Dominicans and three Guineans) with sickle-cell anaemia and stroke; one patient suffered two episodes of stroke. The mean age was 27 months. Five of the episodes were ischaemic infarctions. Stroke was the initial form of presentation of drepanocytosis on three occasions. Two of the strokes occurred within a context of pneumococcal meningitis. Four of the patients had previously reported fever. The initial clinical picture was hemiparesis in four cases. Mean haemoglobin on diagnosing the stroke was 6.5 g/dL. Transcranial ultrasound imaging revealed alterations in three patients and, in all the patients, magnetic resonance imaging revealed lesions, which were bilateral in half the cases. Following the stroke, a hypertransfusion regimen protocol was established and only one patient presented a new stroke. This same patient went on to develop moya-moya disease and was submitted to an indirect revascularisation; the patient progressed well, without presenting any new ischaemic events. Conclusions. Drepanocytosis is a disease that is emerging in our setting as a result of immigration. It should be suspected in cases of paediatric strokes associated to anaemia, above all in black children under the age of five who were not submitted to neonatal screening.