Pediatric Sickle Cell Disease and Stroke: A Literature Review

Parikh, Taral; Goti, Ashish; Yashi, Kanica; Ravikumar, Naveen Prasad Gopalakrishnan; Parmar, Narendrasinh; Dankhara, Nilesh; Satodiya, Vimal

doi:10.7759/cureus.34003

Cited by 7 publications

(8 citation statements)

References 48 publications

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“…Diverse mechanisms underlie stroke in patients with SCD, including large artery occlusion, Moyamoya syndrome, vasculopathy, and small vessel occlusion [28]. Transcranial doppler screening and preventive monthly transfusion can lower the risk of stroke up to 10-fold and is standard of care in the developed countries [29,30]. Lower-cost strategies of reducing risk of stroke in children with SCD, such as hydroxyurea, are an area of ongoing www.co-pediatrics.com research.…”

Section: Childhood Ischemic Strokementioning

confidence: 99%

Epidemiology, causes, and morbidities of stroke in the young

Janas,

Barry,

Lee

2023

Current Opinion in Pediatrics

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Purpose of review The purpose is to describe the latest research on epidemiology, causes, and morbidities of stroke in neonates and children. Recent findings The global incidence of childhood stroke is approximately 2 per 100 000 person-years, which is significantly lower compared to neonates (20–40 per 100 000 live births) and adults (80–90 per 100 000 person-years). Placental abnormalities are a risk factor for perinatal stroke, although cause is usually multifactorial. In children, nonatherosclerotic arteriopathies and arteriovenous malformations are major causes of ischemic and hemorrhagic strokes, respectively. The perinatal period confers a high risk of stroke and can lead to long-term disability, including motor delay, cognitive or speech impairment, and epilepsy. Recent studies suggest that at least 50% of survivors of perinatal stroke have abnormal neurodevelopmental scores in long-term follow up. Childhood stroke is associated with significant morbidity, including epilepsy, motor impairments, and behavioral disability. Recent studies have also identified an association between pediatric stroke and behavioral disorders, such as attention deficit hyperactivity disorder and autism. Summary Perinatal and childhood strokes are important causes of neurological morbidity. Given the low incidence of childhood stroke, prospective research studies on epidemiology, causes, and outcomes remain limited, highlighting the need for continued multisite collaborations.

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Section: Childhood Ischemic Strokementioning

confidence: 99%

Epidemiology, causes, and morbidities of stroke in the young

Janas,

Barry,

Lee

2023

Current Opinion in Pediatrics

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“…Chronic anemia leads to high cardiac output, increasing shear forces on blood vessel walls [48,49]. Children with SCD have a very high risk of stroke, estimated at 10% to 15% [4,50]. Without intervention, approximately 11% of patients suffer from clinical IS by age 20.…”

Section: Pathophysiological Association Between Scd and Strokementioning

confidence: 99%

“…Without intervention, approximately 11% of patients suffer from clinical IS by age 20. Moreover, strokes tend to recur in these patients [4,50].…”

Section: Pathophysiological Association Between Scd and Strokementioning

confidence: 99%

“…Both significant artery stenosis and Moyamoya syndrome are observed; the latter causes fragile collateral vessels prone to rupture and hemorrhage [56]. Overall, both IS and HS are increased in SCD due to this multitude of vascular effects [4,50]. Blood transfusions can significantly reduce stroke risk by diluting sickle cell concentration [11,52].…”

Section: Pathophysiological Association Between Scd and Strokementioning

confidence: 99%

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Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Hakami,

Alhazmi,

Busayli

et al. 2023

Cureus

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Sickle cell disease (SCD) is a genetic blood disorder that affects hemoglobin and increases stroke risk, particularly in childhood. This review examines the pathophysiological association between SCD and stroke, the classification of stroke types, risk factors, diagnosis, management, prevention, and prognosis. A comprehensive literature search was conducted via PubMed, Scopus, and Cochrane databases. Relevant studies on SCD and stroke pathophysiology, classification, epidemiology, diagnosis, treatment, and prevention were identified.Sickle cell disease causes red blood cells to become rigid and sickle-shaped, obstructing blood vessels. Recurrent sickling alters cerebral blood flow and damages vessel walls, often leading to ischemic or hemorrhagic strokes (HS). These occur most frequently in childhood, with ischemic strokes (IS) being more common. Key risk factors include a prior transient ischemic attack (TIA), low hemoglobin, and a high leukocyte count. Neuroimaging is essential for diagnosis and determining stroke type. Primary prevention centers on blood transfusions and hydroxyurea for those at high risk. Acute treatment involves promptly restoring blood flow and managing complications. However, significant knowledge gaps remain regarding stroke mechanisms, optimizing screening protocols, and improving long-term outcomes. This review synthesizes current evidence on SCD and stroke to highlight opportunities for further research and standardizing care protocols across institutions. Ultimately, a holistic perspective is critical for mitigating the high risk of debilitating strokes in this vulnerable patient population.

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“…However, four other children with SCD had their first stroke associated with MMS before the age of four years. Three out of 29 infants aged seven to 48 months were found to have MRA abnormalities in one study [22]. Since MRI/MRA was performed following a clinically overt stroke, it may be beneficial to screen this group of children for MM changes by performing MRI/MRA before age four in areas where TCD is unavailable.…”

Section: Mms Age At Diagnosismentioning

confidence: 99%

Moyamoya Syndrome in Children With Sickle Cell Disease in Saudi Arabia: A Single-Center Experience

Zayed,

Al-Muhaimeed,

Al-Otaibi

et al. 2023

Cureus

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BackgroundSickle cell disease (SCD) is relatively common in Saudi Arabia. Its neurologic complications such as stroke and Moyamoya syndrome (MMS) can be severe and devastating. Such complications can be minimized by modern investigative tools such as transcranial Doppler (TCD) ultrasound, which is unavailable in many high-risk places. Our aim is to investigate the prevalence and characteristics of these complications in children with SCD in our center where TCD is not available. MethodsWe conducted a retrospective record review of children with SCD admitted to the pediatric ward and visited the pediatric hematology outpatient clinic of King Fahad Military Medical Complex, Dhahran, Saudi Arabia, from January 2010 to December 2021. The target population was children aged six months to 14 years with SCD and a history of stroke or transient ischemic attacks (TIAs). Their magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) of the brain radiographic features were reviewed. A descriptive analysis was used to summarize the demographic characteristics and clinical features of patients with and without MMS. ResultsTwenty-six children (out of 385 with sickle cell anemia, originating mainly from the southwestern and eastern provinces of Saudi Arabia) experienced an overt stroke with an overall prevalence of 6.7%. All patients with stroke were originally from the Southwestern province. Their genotype was SS, and the median age at the onset of the first stroke was six years (IQR: 5.5). The main presenting symptoms were seizures (57.7%), motor weakness (42.3%), headache (15.3%), cranial nerve palsies (11.5%), cognitive deficit (7.6%), and dysphasia (3.8%). The majority of strokes were ischemic (92.3%). MMS was detected in 61.5% and was seen at the onset of the first stroke in all patients with this MRA abnormality. Seven children with moyamoya (43.8%) had recurrent strokes. ConclusionIn this study, the prevalence of overt stroke is 9% in children with SCD originating from the southwestern region of Saudi Arabia (26/286), and 61.5% of them (16/26) had MMS. It is absent in the children of Eastern origin (99 children). In places lacking TCD facilities, further studies are required to determine if MRA brain screenings of children with SCD may detect MMS before the onset of stroke and help start protective therapy.

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Pediatric Sickle Cell Disease and Stroke: A Literature Review

Cited by 7 publications

References 48 publications

Epidemiology, causes, and morbidities of stroke in the young

Epidemiology, causes, and morbidities of stroke in the young

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke

Moyamoya Syndrome in Children With Sickle Cell Disease in Saudi Arabia: A Single-Center Experience

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