Fatal hemorrhagic transformation after ischemic stroke in a child with sickle cell disease SS: A case report

Abstract

BACKGROUND

Sickle cell disease homozygous SS (SCD-SS) is the most prevalent hemoglobinopathy in sub-Saharan Africa, accounting for nearly 80% of global cases. Ischemic stroke affects approximately 11% of children with the SS genotype before the age of 20 years, while hemorrhagic stroke carries a significantly higher case fatality rate. The sequential occurrence of both stroke subtypes in a single pediatric patient, in the context of Plasmodium falciparum malaria and absence of secondary prevention, represents an exceptionally rare and clinically alarming event.

CASE SUMMARY

We report an 8-year-old girl with SCD-SS and a history of ischemic stroke resulting in left hemiplegia, for whom no structured secondary stroke prevention had been implemented. She was admitted to the intensive care unit for impaired consciousness, generalized tonic-clonic seizures, and high-grade fever in the setting of active Plasmodium falciparum malaria. On admission, her Glasgow Coma Scale score was 11/15, and laboratory investigations revealed severe anemia (hemoglobin level 7.6 g/dL). Initial cerebral computed tomography (CT) demonstrated a subacute ischemic infarction involving the right anterior and middle cerebral artery territories. Management included intravenous artesunate, anticonvulsants, packed red blood cell transfusions, and dexamethasone. On day 4, her Glasgow Coma Scale score dropped to 5/15, with anisocoria and meningeal signs. Emergency CT revealed diffuse subarachnoid hemorrhage, cerebral edema, and an 8-mm subfalcine herniation, consistent with hemorrhagic transformation. The patient died on day 5.

CONCLUSION

The absence of secondary stroke prevention in a child with SCD-SS proved fatal. Transcranial Doppler screening and exchange transfusion therapy are potentially life-saving interventions. Expanding access to these therapies across sub-Saharan Africa is an urgent priority.

Keywords: Anemia; sickle cell; Stroke; Malaria; Falciparum; Child; Africa South of the Sahara; Secondary prevention; Hemorrhagic transformation; Case report

Core Tip: This case highlights the fatal consequences of absent secondary stroke prevention in a child with sickle cell disease homozygous SS (SCD-SS). An 8-year-old girl who survived an initial ischemic stroke subsequently developed a fatal hemorrhagic stroke in the setting of Plasmodium falciparum malaria, without having received secondary preventive care. In sub-Saharan Africa, limited access to transcranial Doppler screening and exchange transfusion programs continues to place children with SCD-SS at high risk of recurrent cerebrovascular events. This report underscores the urgent need to strengthen early screening and secondary stroke prevention strategies in resource-limited settings.