Sickle cell disease (SCD) is the most common type of hereditary anemia with a particularly high prevalence in sub-Saharan Africa. More than 230,000 children are affected in this geographical region every year, making up 80% of the global total.[1] SCD is characterized by the abnormal polymerization of the β-globin protein, resulting in obstruction to the microcirculation and tissue hypoxia. Cerebral hemorrhage is quite uncommon, with an incidence ranging from 0.5 to 2%. Cerebrovascular complications contribute significantly to the morbidity and mortality of the disease.[2] [3] [4] Variable risk factors include acute hypertension, blood transfusion, use of steroids, and nonsteroidal anti-inflammatory drugs (NSAIDs).[5]

Here, we present a 10-year-old Ghanaian girl who was admitted to our emergency department because of diffuse erythematous lesions and an acute pain crisis, requiring NSAIDs. One month before admission, the patient started a blood transfusion program due to anemia following SCD diagnosis. Admission blood tests revealed a white blood cell count of 14 × 109 g/L and a hemoglobin level of 6.9 g/dL, which increased to 9.4 g/dL after two blood transfusions. At day (D) 7 of hospitalization, she experienced acute chest syndrome. At D9, 5 days after the last blood transfusion, the patient became lethargic and dysarthric with increasing blood pressure values. A cranial computed tomography (CT) scan revealed synchronous right frontoparietal and left temporoparietal hemorrhages ([Fig. 1Aa]). Magnetic resonance (MR) angiography revealed the absence of aneurysms or any vascular malformation, and the dural venous sinuses appeared patent ([Fig. 1Ae, Af]). Because of an acute convulsive status epilepticus, the patient was moved to the pediatric intensive care unit the same day. A repeated CT scan performed at D10 showed increased volumes of both intracranial hemorrhages ([Fig. 1Ab]). Due to uncal herniation ([Fig. 1Ac]), the patient subsequently underwent an emergency left-sided craniotomy for evacuation of the left temporoparietal collection. To monitor the nonevacuated hemorrhage in the right frontoparietal region ([Fig. 1Ad]), a right intraparenchymal intracranial pressure (ICP) transducer was placed ([Fig. 1Ba]). At postoperative D1, the ICP course showed progressive increasing values. The patient underwent a right hemispheric decompressive craniectomy (DC) without evacuation of the hemorrhage in order to protect the underlying eloquent brain regions ([Fig. 1Bb]). Subsequent head CT scans demonstrated a progressive, complete resolution of the right hemorrhage and limited brain swelling through the craniectomy area. The ICP course showed decreased values with no further need for neurointensive care. During the postoperative course, the patient had two additional surgeries for superficial surgical site infections. At D40 following DC, she underwent autologous bone cranioplasty. At D60 from admission, she was discharged to an in-hospital neurorehabilitation program. At discharge, the patient was in good condition, with mild left monoparesis of the upper limb. At 1 year postoperative, the neurological status was unchanged, and the cerebral MR images showed a regular neurodiagnostic course ([Fig. 1C]). To our knowledge, this is the first report of a massive synchronous spontaneous bilateral hemorrhagic stroke complicating SCD among all published intracerebral hemorrhages in pediatric patients.[6]

In summary, SCD-related intracranial hemorrhage is an intricate and relatively rare complication, particularly in the absence of underlying causes, such as trauma, and among children. Guidelines for hemorrhagic stroke prevention are difficult to apply. Blood transfusions are considered a protective factor against ischemic stroke but appear to be a frequent and time-related factor associated with intracerebral hemorrhage in the pediatric SCD population, as highlighted by this case and previously published reports of hemorrhagic events in SCD patients.[6] Hence, the risk for cerebral hemorrhage ought to be considered in pediatric patients treated for SCD. It is important to balance the advantages and disadvantages of aggressive blood evacuation while considering the neuroplasticity of children and evaluating surgical methods. Multidisciplinary management by various specialists, including pediatrician, hematologist, and neurosurgeon, is essential to improve the outcomes of these patients.