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Childhood stroke

Nature Reviews Disease Primers volume 8, Article number: 12 (2022) Cite this article

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Abstract

Stroke is an important cause of neurological morbidity in children; most survivors have permanent neurological deficits that affect the remainder of their life. Stroke in childhood, the focus of this Primer, is distinguished from perinatal stroke, defined as stroke before 29 days of age, because of its unique pathogenesis reflecting the maternal–fetal unit. Although approximately 15% of strokes in adults are haemorrhagic, half of incident strokes in children are haemorrhagic and half are ischaemic. The causes of childhood stroke are distinct from those in adults. Urgent brain imaging is essential to confirm the stroke diagnosis and guide decisions about hyperacute therapies. Secondary stroke prevention strongly depends on the underlying aetiology. While the past decade has seen substantial advances in paediatric stroke research, the quality of evidence for interventions, such as the rapid reperfusion therapies that have revolutionized arterial ischaemic stroke care in adults, remains low. Substantial time delays in diagnosis and treatment continue to challenge best possible care. Effective primary stroke prevention strategies in children with sickle cell disease represent a major success, yet barriers to implementation persist. The multidisciplinary members of the International Pediatric Stroke Organization are coordinating global efforts to tackle these challenges and improve the outcomes in children with cerebrovascular disease.

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Fig. 1: Major aetiologies of haemorraghic and ischaemic stroke in childhood.
Fig. 2: Common aetiologies of childhood arterial ischaemic stroke.
Fig. 3: Major signalling pathways and genes involved in vascular malformations that cause the majority of childhood ICH.
Fig. 4: Diagnostic clues for haemorrhagic and ischaemic childhood stroke.
Fig. 5: Treatment algorithm for acute childhood stroke.
Fig. 6: Example of a case with successful mechanical thrombectomy in a 9-year-old boy with left-sided hemiparesis.
Fig. 7: Paediatric stroke trajectories.

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Authors and Affiliations

  1. Department of Neuroradiology, Clinic of Radiology & Nuclear Medicine, University Hospital Basel, Basel, Switzerland

    Peter B. Sporns

  2. Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    Peter B. Sporns

  3. Departments of Neurology and Pediatrics, Benioff Children’s Hospital, University of California at San Francisco, San Francisco, CA, USA

    Heather J. Fullerton

  4. Division of Child Neurology, Department of Neurology & Neurological Sciences, Stanford University School of Medicine, Palo Alto, CA, USA

    Sarah Lee

  5. Departments of Anesthesia and Perioperative Care, and Epidemiology and Biostatistics, Center for Cerebrovascular Research, University of California at San Francisco, San Francisco, CA, USA

    Helen Kim

  6. Departments of Pediatrics and Neurology, Nationwide Children’s Hospital and The Ohio State University, Columbus, OH, USA

    Warren D. Lo

  7. Department of Neurology, Royal Children’s Hospital, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia

    Mark T. Mackay

  8. Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Mark T. Mackay

  9. Department of Radiology, University Hospital Munich, LMU Munich, Munich, Germany

    Moritz Wildgruber

Authors
  1. Peter B. Sporns
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  2. Heather J. Fullerton
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  3. Sarah Lee
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  4. Helen Kim
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  5. Warren D. Lo
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  6. Mark T. Mackay
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  7. Moritz Wildgruber
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Contributions

Introduction (M.W. and P.B.S.); Epidemiology (H.J.F. and H.K.); Mechanisms/pathophysiology (H.J.F., S.L., H.K., W.D.L. and M.T.M.); Diagnosis, screening and prevention (M.W., P.B.S., H.J.F., S.L., H.K., W.D.L. and M.T.M.); Management (M.W., P.B.S., H.J.F., S.L., H.K. and M.T.M.); Quality of life (H.J.F. and W.D.L.); Outlook (M.W., P.B.S., H.J.F., S.L., H.K., W.D.L. and M.T.M.); Overview of Primer (M.W.).

Corresponding author

Correspondence to Moritz Wildgruber.

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Competing interests

The authors declare no competing interests.

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Nature Reviews Disease Primers thanks A. Mallick, who co-reviewed with R. Spaull; L. Beslow; N. Dlamini; R. Westmacott; and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Glossary

Tetralogy of Fallot

Congenital disorder characterized by a ventricular septal defect, pulmonary stenosis, overriding aorta and right ventricular hypertrophy.

Transposition of the great vessels

A disorder in which the aorta arises from the right ventricle and the pulmonary trunk arises from the left ventricle.

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A disorder in which the left ventricle, and mitral and aortic valves are underdeveloped and unable to support the systemic circulation.

Sub-intimal haematoma

Vessel wall haematoma accompanied by separation of the layers of the arterial wall.

Rotational vertebral arteriopathy

Injury of the vertebral artery due to repeated mechanical impingement from adjacent bony or soft tissue structures in the neck.

Coarctation of the aorta

Narrowing of the aorta, most frequently located along the aortic arch.

String sign

A string of contrast material distal to a stenotic segment visible on angiography.

Double lumen

A sign on angiography representing the true and false lumen.

Intimal flap

A flap of the intima protruding into the perfused vessel lumen.

Arterial banding

Narrowing of the vessel following external compression.

Posterior fossa decompression

Removal of a portion of bone in the back of the skull to allow expansion of cerebellar swelling.

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Sporns, P.B., Fullerton, H.J., Lee, S. et al. Childhood stroke. Nat Rev Dis Primers 8, 12 (2022). https://doi.org/10.1038/s41572-022-00337-x

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