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SCOPE

DISEASE/CONDITION(S)

Arterial and venous stroke in neonates.

GUIDELINE OBJECTIVE(S)

Review frequency, types, impact, and causes of perinatal stroke. Describe the preferred methods to investigate and acutely treat patients with stroke.

BRIEF BACKGROUND

Perinatal stroke is a focal or multifocal ischemic or hemorrhagic infarction of the neonatal brain due to cerebrovascular injury during the immediate prenatal, intranatal, or postnatal period. It is defined as “a group of heterogeneous conditions with a focal disruption of cerebral flow secondary to an arterial or a venous thrombosis or embolization between the 20 week of fetal life through the 28 post-natal day, and confirmed by neuroimaging or neuropathological studies.” Perinatal arterial ischemic stroke (PAIS), cerebral sinovenous thrombosis (CSVT), and other entities such as hemorrhagic infarct, periventricular hemorrhagic infarction (PVHI), and presumed perinatal stroke with considerable overlap are included under the umbrella term perinatal stroke.

Epidemiology and Pathogenesis

PAIS is reported in 1 in 2300–5000 live births and is caused by an embolus arising from the placenta or the heart passing through the patent foramen ovale (or rarely from a blood vessel or other causes). A large cerebral artery, most commonly the left middle cerebral artery, is occluded, leading to infarction of the region of arterial supply. Sometimes small vessels are involved, and this leads to multifocal involvement in the regions of the thalamus or the basal ganglia.

CSVT is less common, affecting 1–2 in 100,000 deliveries and results from a thrombus partially or completely occluding a cranial venous sinus, a large deep vein, or a smaller cortical or deep vein. A CSVT commonly occurs in the superior sagittal system, the internal cerebral veins, and the straight sinus leading to hemorrhagic infarction secondary to impaired venous drainage. Neonatal CSVT might be associated with mechanical factors such as occipital bone compression of the superior sagittal sinus in the supine posture. A hemorrhagic stroke can result from bleeding into the brain as a result of rupture of an arteriovenous malformation, trauma, inherited or acquired coagulopathy (including that caused by maternal ingestion of phenobarbital, phenytoin, or warfarin), or thrombocytopenia.

Risk factors associated with perinatal stroke include maternal and placental disorders (e.g., chorioamnionitis, maternal pre-eclampsia, intrauterine growth restriction, emergency cesarean section), low Apgar scores at 1 and 5 minutes, need for resuscitation at birth, perinatal asphyxia, assisted ventilation at birth, cardiac disorders, coagulation disorders, polycythemia, infection, trauma, and drugs. The risk factors and clinical presentation can overlap with those of global hypoxic ischemic encephalopathy and can occur concurrently. Prothrombotic disorders involving protein C, protein S, antithrombin III, Factor V Leiden mutation, prothrombin mutation, phospholipid antibody, or a homocysteine defect are common (40–70%) and may be contributory among neonates with PAIS.

Clinical Presentation

A neonatal ischemic stroke most commonly presents as seizures or apneas, ...

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