Perinatal Stroke

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Perinatal strokes are a diverse but specific group of focal cerebrovascular injuries that occur early in brain development and affect an estimated 5 million people worldwide. The objective of this review is to describe the epidemiology, clinical presentations, pathophysiology, outcomes, and management for the 6 subtypes of perinatal stroke. Some perinatal strokes are symptomatic in the first days of life, typically with seizures, including neonatal arterial ischemic stroke, neonatal hemorrhagic stroke, and cerebral sinovenous thrombosis. The remaining subtypes present in the first year of life or later, usually with motor asymmetry and include arterial presumed perinatal ischemic stroke, presumed perinatal hemorrhagic stroke, and in utero periventricular venous infarction. The consequences of these injuries include cerebral palsy, epilepsy, and cognitive and behavioral challenges, in addition to the psychosocial impact on families. While there have been significant advances in understanding mechanisms of both injury and recovery, there is still a great deal to learn regarding causation and the optimization of outcomes.

Introduction

Perinatal stroke can be divided into 6 specific disease states based on clinical and imaging criteria. Precise estimates by disease type are not available but the overall combined incidence is approximately 1:1600-1:2300 live births.1, 2, 3 The timeframe for perinatal stroke includes all cerebrovascular events occurring between 20 weeks of fetal life and 28 days postnatal life.4 The neonatal period refers to the first 28 days after birth. Three of the 6 stroke subtypes present acutely in the neonatal period, usually in the first days of life, are termed acute symptomatic perinatal stroke (Fig. 1), and likely account for approximately 50% of perinatal stroke presentations.5 This makes the first week of life the most focused life-time period of risk for stroke.6 The remainder of perinatal strokes present outside the first month of life and are collectively termed presumed perinatal stroke as the precise timing cannot be determined. Thanks largely to advances in imaging techniques and increased availability of imaging, it has been shown that most cases that would have previously been labeled idiopathic hemiparetic cerebral palsy or congenital hemiplegia are due to perinatal stroke.7 Additional morbidities of perinatal stroke include epilepsy and intellectual, behavioral, and language challenges, many of which last a lifetime.8, 9 This presents a significant societal economic burden,10 as well as an economic, social, and emotional burden for families.11

Both the acute and delayed stroke presentations can be due to an arterial or venous process which is hemorrhagic or ischemic, or both. Here we discuss each specific type of perinatal stroke, including the pathophysiology, presentation, diagnosis, and management. We conclude with consideration of the outcomes shared by all types and the modern rehabilitation strategies to address them including psychosocial considerations for affected children and their families.

Section snippets

Neonatal Arterial Ischemic Stroke

Neonatal Arterial Ischemic Stroke (NAIS) is the most recognized and common type of acute stroke, likely accounting for 80%-90% of cases in the literature.12 NAIS typically presents in the first 28 days of life and is confirmed on neuroimaging with a focal area of recent ischemic infarction corresponding to 1 or more arterial territories (Fig. 2A and B). The most common presentation is seizure (either focal or generalized) occurring 12-72 hours following delivery, observed in 70%-90% of infants.

Periventricular Venous Infarction (PVI)

Periventricular venous infarction is well described in delivered preterm infants as a consequence of germinal matrix hemorrhage, typically prior to 32 weeks gestation (Fig. 4).66 It is believed the hemorrhage causes compression of the medullary veins, resulting in focal venous infarction of the periventricular white matter that is often itself hemorrhagic.78 More recently, PVI has been described in term children presenting with congenital hemiplegia, and is believed to represent an in utero

Outcomes and Rehabilitation

Most children with perinatal stroke experience lifelong neurologic disabilities. Motor deficits predominate but epilepsy and challenges with learning, behavior, language, and mental health compound the morbidity. With few acute treatment or prevention strategies, the treatment and rehabilitation of these complications is the mainstay of the care of children with perinatal stroke and their families. Although there are some parallels with adult stroke, the developing brain requires that skills be

Conclusions

Perinatal stroke is currently conceptualized as 6 specific disease states accurately classifiable based on neuroimaging and clinical presentation. Acute symptomatic types present with seizures where causation is difficult to prove and treatment strategies are limited. Presumed perinatal stroke subtypes present later with hemiparetic cerebral palsy where diagnosis is often delayed. Additional studies are needed to better understand the causative mechanisms in perinatal stroke if prevention

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    Funding: Supported by CIHR grant (FDN-143294).

    The work described has not been published previously and it is not under consideration for publication elsewhere. Its publication is approved by all authors and tacitly or explicitly by the responsible authorities where the work was carried out, and if accepted, it will not be published elsewhere including electronically in the same form, in English or in any other language, without the written consent of the copyright-holder.

    Declaration of Competing Interest: The authors have no commercial, proprietary, or financial interest in any products or companies described in this article.

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