Cerebral infarction secondary to sickle cell disease: arteriographic findings

Area of Science:

• Neurology
• Pediatrics
• Vascular Medicine

Background:

• Sickle cell disease (SCD) is a genetic blood disorder associated with an increased risk of cerebrovascular complications, including cerebral infarction.
• Cerebral infarction in children with SCD can lead to significant neurological deficits and long-term disability.

Purpose of the Study:

• To evaluate the cerebrovascular findings in children with sickle cell disease and clinical evidence of cerebral infarction using cerebral angiography.
• To assess the utility of arteriography in monitoring the effects of hypertransfusion therapy on cerebrovascular disease in this population.

Main Methods:

• Cerebral angiograms were performed in 14 children diagnosed with sickle cell disease and presenting with clinical symptoms of cerebral infarction.
• Angiographic findings were analyzed to identify abnormalities in the cerebral arteries and their branches.

Main Results:

• Four out of 14 patients showed normal angiograms.
• Ten patients exhibited abnormalities, primarily stenosis or occlusion of the internal carotid artery or its branches, often bilateral.
• The anterior and middle cerebral arteries were involved in nine patients, with frequent branch occlusions. Posterior fossa arteries were unaffected.

Conclusions:

• Cerebral angiography is a valuable tool for diagnosing cerebrovascular abnormalities in children with sickle cell disease and stroke.
• Significant carotid artery disease, including stenosis and occlusion, is prevalent in this pediatric cohort.
• Arteriography can guide and evaluate the efficacy of therapeutic interventions like hypertransfusion for cerebrovascular disease in SCD.