Although pediatric stroke is rare, it is a leading cause of morbidity and mortality in children. The diagnosis of stroke is often delayed in children, which can contribute to death and disability. Management of pediatric stroke is challenging because there are few data to support the efficacy of interventions, and management is based on society guidelines and expert opinion, as well as extrapolation from adult stroke management. This issue reviews the most common causes of pediatric stroke, provides guidance for distinguishing stroke from stroke mimics, discusses the indications for laboratory studies and imaging modalities, and offers evidence-based recommendations for treatment.
A 7-year-old boy is brought in by ambulance after a witnessed generalized seizure lasting 2 minutes at home. He has no history of prior seizures. Upon arrival to the ED, he appears postictal and is moving all of his extremities. His blood glucose is 110 mg/dL. His vital signs are: temperature, 36.9°C (98.5°F); heart rate, 60 beats/min; blood pressure, 110/70 mm Hg; respiratory rate, 14 breaths/min; and oxygen saturation, 98% on room air. The boy vomits while the nurse is trying to obtain IV access. Per the mother, the boy has been receiving chemotherapy for lymphoma and was complaining of a headache earlier in the day. He has no history of intrathecal chemotherapy. The mother does not think he had any head trauma recently. You know that the child needs brain imaging, but you are uncertain which imaging would be most useful…
In the next room, you see a 5-year-old boy with sickle cell disease who was brought to the ED by his father. The father states that 1 hour prior to arrival, the boy started stumbling while walking. The father says he didn’t think much of it until he noticed the child's speech was slurred. The father also says he thinks his son’s face looks different on 1 side, though you cannot appreciate facial asymmetry on examination. The boy has 3 out of 5 strength of the left arm and leg, as well as dysarthria. He is alert and denies headache or visual changes. The rest of the neurological examination seems normal. The boy’s vital signs are unremarkable except for mild elevation of blood pressure. You are concerned about a stroke and begin to think about what tests to order as well as which specialists to consult before initiating treatment...
You then see a 2-year-old girl who was brought to the ED by her parents after she fell forward with a toothbrush in her mouth. The mother removed the toothbrush from the unconscious child’s mouth and noted blood on the toothbrush as well as in the child’s mouth. Although the child was unresponsive for 30 seconds after the event, no seizure-like activity was noted. On presentation, the patient is alert with normal mental status and normal vital signs. She has no obvious intraoral trauma, abnormal voice, or stridor, and she has a normal neurological examination for her age. Given the loss of consciousness and the report of blood on the toothbrush despite no oral laceration seen, you decide to obtain a CT angiogram of the neck. The CT angiogram shows a dissection of the left internal carotid artery and subcutaneous air in the area. You consider what treatments to initiate, which specialist to consult, whether further imaging is needed, and what should be the disposition of this child...
Stroke is typically thought of as a disease that occurs in older adults with risk factors for atherosclerosis. Stroke also occurs in the pediatric population, but the etiologies, risk factors, and presentation of stroke differ from those of adults. Stroke is more common in neonates than in children, and will be discussed separately, as the risk factors, treatment, and outcomes differ from those of older infants and children (see the “Neonates” section.).1,2
Annually, stroke affects 1 to 2 children per 100,000 children,1,3 and it is among the top 10 causes of pediatric mortality, with a mortality rate of up to 10% for arterial ischemic stroke (AIS) and 25% for hemorrhagic stroke.4,5 Despite potential neuroplasticity, two-thirds of children will have persistent neurological deficits after a stroke.1
There is usually an excessive delay in the diagnosis of pediatric stroke, which may contribute to morbidity and mortality. A retrospective study of children with AIS in an urban center found a median of 22.7 hours from symptom onset to diagnosis of stroke and 12.7 hours from hospital presentation to the time of diagnosis. On initial assessment, the diagnosis was suspected in only 38% of children.6 Another study found that physicians documented a suspicion of stroke in only 26% of children with AIS.7 Multiple studies have found that children with acute-onset neurological symptoms are most often brought to the emergency department (ED) by private vehicle rather than by ambulance.6,8 The delay in diagnosis is likely multifactorial, in part due to clinicians’ unfamiliarity with pediatric stroke and a bias for diagnosing more common stroke mimics. A clinician who does not know the different etiologies of stroke in children may not identify the risk factors. Children also present differently from adults with stroke. Children are more likely to have seizure and altered mental status with stroke, which generates a large list of differential diagnoses.1,6
Management of pediatric stroke is challenging because there are few data to support the efficacy of interventions, and management is based on society guidelines and expert opinion (with the exception of children with sickle cell disease). Management of stroke in children is also extrapolated from adult stroke management even though the pathophysiology and etiologies of stroke in children differ significantly from adults.
This issue of Pediatric Emergency Medicine Practice reviews common causes of pediatric stroke, offers guidance on how to distinguish stroke from its mimics, discusses the indications for imaging modalities, and provides evidence-based treatment recommendations.
A literature search was performed in MEDLINE® and the Cochrane Database of Systematic Reviews using the search terms pediatric stroke, pediatric stroke management, and hemorrhagic stroke pediatric presentation management. Results were limited to studies involving children aged 0 to 18 years, those with full text in English, and those involving human subjects. References within the articles were also reviewed to identify additional articles for inclusion. Titles and abstracts were reviewed for relevance to the topic, and 70 articles were chosen for inclusion.
Several pediatric stroke registries, prospective cohorts, and retrospective cohorts are reported in the literature.1,6,8-18 Until about 10 years ago, there were only case reports of medical interventions for pediatric stroke. Evidence for surgical interventions and mechanical thrombectomy is still based mainly on case reports.19-22 There are no completed prospective controlled trials of anticoagulation, antiplatelet medication, or thrombolysis for AIS in children, though society guidelines support the use of anticoagulation and antiplatelets, based on the observed safety of these interventions in cohort studies.3,23
2. “I obtained a CT scan instead of an MRI because it’s too difficult to obtain an MRI in the ED, and it won’t give me management-changing information.”
Though CT scan may yield important diagnostic information, MRI is the preferred modality for pediatric stroke. MRI can better detail AIS and CSVT in addition to common stroke mimics. Delaying MRI delays definitive diagnosis, treatment, and possible prevention of future stroke.
5. “This patient was last well 2 hours ago, and he has a PedNIHSS score of 10. The CT scan showed no hemorrhage, so I don’t need to involve neurology prior to giving tPA.”
While some specialists may recommend thrombolytics in pediatric AIS, emergency clinicians should never make this decision on their own. tPA may be reasonable in some situations under the guidance of a neurologist with experience treating pediatric stroke. Vascular imaging that demonstrates complete or partial occlusion of the vessel is required, in addition to other radiologic, laboratory, and clinical criteria. The safety and efficacy of thrombolytics in children have not been studied adequately.
10. “I was concerned the patient had intracerebral hemorrhage, so I rushed him to the CT scanner before wasting time with IV placement or other resuscitation.”
While it is important to rapidly diagnose and contact neurosurgery in the case of a hemorrhagic stroke, it is also important to prevent secondary brain injury from causes such as hypoxia or hypotension/hypertension. Interventions such as IV placement and supplemental oxygen or intubation should be performed to stabilize the patient prior to obtaining imaging.
Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.
To help the reader judge the strength of each reference, pertinent information about the study is included in bold type following the reference, where available. In addition, the most informative references cited in this paper, as determined by the author, are highlighted.
Why to Use
Pediatric stroke is relatively uncommon, but it remains an important cause of morbidity and mortality in children. Adult stroke scales have limited sensitivity when applied to pediatric populations (62%-67% sensitivity according to an analysis by Mackay et al ). The PedNIHSS is up to 87% sensitive (Beslow 2012). It quantifies the severity of pediatric stroke and can be trended over time to assess recovery, while the initial score may be predictive of future disability at 90 days.
When to Use
The PedNIHSS should be used in pediatric patients aged ≥ 2 years who have clinical and radiologic signs of acute ischemic stroke.
In order to improve the accuracy of the PedNIHSS, the patient’s family members should be instructed to refrain from hinting at the correct responses to the questions asked.
Abbreviation: tPA, tissue plasminogen activator.
Derek Tam, MD, MPH
Pediatric patients with sickle cell disease who present with acute ischemic stroke will likely benefit from emergent blood transfusion to reduce hyperviscosity. Early consultation with a pediatric hematologist, in addition to a pediatric neurologist, is recommended for these patients.
Pediatric stroke is rare, and the true predictive value of the PedNIHSS is subject to change as the scale continues to be studied. Retrospective application of the PedNIHSS has been shown to be valid and reliable in 1 cross-sectional study (Beslow 2012).
The PedNIHSS was developed by expert consensus of a panel of adult and pediatric stroke experts (Ichord 2011). The panel adapted the validated adult NIHSS to be appropriate for age-related variations in the comprehension of examination materials. The scoring strategy and ranges were not changed. The panel evaluated the interrater reliability of the PedNIHSS among 113 patients aged 2 to 18 years in a multicenter prospective cohort study. Overall, the interrater reliability was found to be quite high, ranging from 0.63 to 1.00 among the evaluating pediatric neurologists.
The PedNIHSS was externally validated by applying it to 75 children enrolled in a prospective study (Beslow 2012). Patients were scored both prospectively and retrospectively (based on the chart). Retrospectively applied scores correlated well with prospective scores (r2 = 0.76). Interrater reliability was good overall (intraclass correlation coefficient of 0.95; 95% confidence interval, 0.94-0.97), with similar findings for individual test items.
Rebecca N. Ichord, MD
Amy Buckowski, MD; Emily Rose, MD, FAAP, FAAEM, FACEP
Lauren A. Beslow, MD, MSCE, FAHA; Meghan Gilley, MD, FRCPC
November 2, 2019
December 1, 2022
4 AMA PRA Category 1 Credits™, 4 ACEP Category I Credits, 4 AAP Prescribed Credits, 4 AOA Category 2-A or 2-B Credits. Specialty CME Credits: Included as part of the 4 credits, this CME activity is eligible for 4 Stroke CME and 0.25 Pharmacology CME credits.
Date of Original Release: November 1, 2019. Date of most recent review: October 15, 2019. Termination date: November 1, 2022.
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