Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment
Sarah Liane Linguet,
Suzanne Verlhac,
Florence Missud,
Laurent Holvoet-Vermaut,
Valentine Brousse,
Ghislaine Ithier,
Alexandra Ntorkou,
Emmanuelle Lesprit,
Malika Benkerrou,
Manoëlle Kossorotoff,
Berengere Koehl
Affiliations
Sarah Liane Linguet
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris
Suzanne Verlhac
INSERM UMR_S1134 BIGR, Paris
Florence Missud
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris
Laurent Holvoet-Vermaut
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris
Valentine Brousse
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris, France; INSERM UMR_S1134 BIGR, Paris
Ghislaine Ithier
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris
Alexandra Ntorkou
Radiology Unit, Robert Debre Hospital, AP-HP, Paris
Emmanuelle Lesprit
Etablissement Français du Sang
Malika Benkerrou
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris, France; INSERM UMR_S1123 ECEVE, Paris
Manoëlle Kossorotoff
French Center for Pediatric Stroke, Pediatric Neurology Department, University Hospital Necker-Enfants Malades, AP-HP, Paris, France; and INSERM U1266, Paris
Berengere Koehl
Referral Center for Sickle Cell Disease, Hematology Unit, Robert Debre Hospital, AP-HP, Paris, France; INSERM UMR_S1134 BIGR, Paris, France; Université de Paris Cité, Paris
Cerebral arteriopathy (CA) in children with sickle cell disease (SCD) is classically described as chronic stenosis of arteries in the anterior brain circulation, leading to ischemic stroke. Some studies have however reported strokes in children with SCD but without CA. In order to better understand the etiology and risk factors of these strokes, we retrospectively analyzed ischemic strokes occurring in a large cohort of children over a 13 year-period. Between 2007 and 2020, 25/1500 children with SCD had an ischemic stroke in our center. Among them, 13 (52%) had CA, described as anatomical arterial stenosis, while 12 (48%) did not. Patients with stroke without CA were older than patients with stroke attributed to SCD-CA (9.0 years old vs 3.6 years old, p=0.008), and had more frequently a SC genotype (25% vs 0% respectively). Their stroke involved posterior circulation more frequently, with cerebellar involvement in 42%. Retained stroke etiologies in patients without typical SCD-related CA were reversible cerebral vasoconstriction syndrome, cerebral fat embolism, arterial thrombosis or thromboembolism, hyperviscosity, vasculitis in a context of infectious meningoencephalitis, and severe hemodynamic failure. No recurrence was observed in the 24 months following stroke, even though 67% of the patients were no longer receiving exchange transfusions in this group. In conclusion, in a cohort of pediatric SCD patients with efficient stroke screening strategy, half of occurring ischemic strokes were related to causes other than CA. They affected a different population of SCD children and systematic long-term transfusion programs may not be necessary in these cases.
