Sickle Cell Trait and Stroke in Children

Abstract

Cerebrovascular complications of sickle cell anemia are well known to be one of the more common causes of stroke in the pediatric age group. Although it is far less common, the association of stroke with sickle cell trait has been described in young adults and only twice in children in the English literature. In this report, we describe a case of a posterior circulation infarct occurring in a 12-year-old male who also had a normal variant vertebral artery, which may have been a contributing factor in his presentation. The literature is reviewed and other possible risk factors for stroke in sickle cell trait are discussed.

Prospective Brain Imaging Evaluation of Children with Sickle Cell Trait: Initial Observations

“…Sickle cell trait has been associated with sudden death from unexplained causes (13,46 -50), but children with sickle cell trait are apparently not at greatly increased risk of stroke (25). However, the long-term risk of stroke in adults with sickle cell trait is not known.…”

“…Investigators in most prior studies of sickle cell trait included case histories of one or a few patients and did not assess the population prevalence of cerebrovascular abnormalities (24). Researchers in several case studies described findings in a pediatric patient with stroke who had hemoglobin AS (15,17,25,26), but researchers in none of these studies addressed the prevalence of risk Pediatric Imaging 208 factors associated with stroke. With several exceptions (14,16,17,22,25,26), investigators in prior studies of stroke in patients with sickle cell trait used autopsy material, with the inherent problem that such studies are neither prospective nor cross sectional.…”

“…Researchers in several case studies described findings in a pediatric patient with stroke who had hemoglobin AS (15,17,25,26), but researchers in none of these studies addressed the prevalence of risk Pediatric Imaging 208 factors associated with stroke. With several exceptions (14,16,17,22,25,26), investigators in prior studies of stroke in patients with sickle cell trait used autopsy material, with the inherent problem that such studies are neither prospective nor cross sectional. Furthermore, in many of the early reports, sickle cell trait was not diagnosed by using electrophoresis (9 -11), and other tests used to diagnose sickle cell trait were often inaccurate (13).…”

Acute Ischaemic Stroke Agnieszka Anna Ardelt

“…122 The gene is thought to confer a bene fi cial effect on heterozygotes consisting of a relative resistance to malaria. Sporadic reports exist of stroke in young adults 123 and children 124 with no known risk factors other than sickle cell trait. Children with heterozygous sickle cell trait were found to have parenchymal abnormalities on MRI in 10% and vasculopathy manifesting as tortuosity in 19%.…”

Defining stroke risk in children with sickle cell anaemia

“…SCD is the most common genetic disorder predisposing to stroke in childhood, accounting for 39% of childhood strokes in one population study (Earley et al , 1998). Although the increased risk of sudden death in military recruits with sickle cell trait is well described, stroke in individuals with sickle cell trait (HbAS) has only been sporadically reported, usually in association with dehydration, anaemia, or the perioperative period (Partington et al , 1994; Kerle & Nishimura, 1996; Drehner et al , 1999). In a recent study, arterial tortuosity was more frequently demonstrated on MRA in children with sickle cell trait compared with normal controls (Steen et al , 2003).…”