This document provides an overview of pediatric stroke, including: 1. It classifies pediatric strokes as perinatal (birth to 28 days) or childhood (28 days to 18 years) and describes common types like arterial ischemic stroke and hemorrhagic stroke. 2. Risk factors and causes of childhood stroke are discussed, including cardiac lesions, hematological disorders, arteriopathies, infections, and genetic syndromes. Evaluation involves cardiac and thrombophilia testing as well as neuroimaging. 3. Acute management focuses on stabilization, with some evidence that thrombolysis may be considered in select cases. Long-term prevention emphasizes antithrombotic therapies tailored to the underlying condition. Outcomes

1. Pediatric stroke- Evaluation
&Management
PRESENTER- DR.PALLAV JAIN
DM RESIDENT(NEUROLOGY)
GMC,KOTA

2. Classification
Perinatal stroke -Stroke occurring from 28 weeks’ gestation to 28
postnatal days of life
Fetal: occurring before birth
Neonatal: Birth to 28 days of postnatal life
Presumed perinatal ischemic stroke (PPIS): events whose exact time
of onset are inferred as perinatal (birth to 28 days of life)
Childhood stroke- stroke occurring after 28 days to 18 years of age.

3. Classification
• Arterial ischemic stroke- where the infarct conforms to vascular occlusion
in an arterial territory
• Hemorrhagic stroke, including parenchymal, subarachnoid, intraventricular
hemorrhage
• Cerebral sinovenous thrombosis (CSVT)

4. Epidemiology
Perinatal stroke
• Arterial ischemic infarction- ≈80% of perinatal strokes
• Higher in newborns(6 times) than in older children.
Childhood Stroke
• Ischemic stroke- 1.0 to 2.0 in 100 000 children annually.
• Highest in infants and children <5 years, boys > girls.
Stroke recurrences
• 6.8% at 1 month and 12% at 1 year with most children.
• The strongest predictor-presence of an arteriopathy(5 fold)

5. Risk Factors and Causes of Childhood AIS
• Cardiac lesions
• Hematological-thrombophilia, SCD,
• Trauma and vascular compression
• Infections
• Vascular malformation/vasculopathy-Extracranial,intracranial
• Drug and toxins
• Metabolic
• Genetic causes

6. Cardiac
• Accounts for ≈30% of all childhood strokes
• Abnormal cardiac anatomy or associated cardiac arrhythmias lead to
abnormal flow and may predispose to thrombi.
• Surgery and cardiac catheterization can disrupt the endothelium
• Abnormal heart valve can serve as a nidus for bacterial or fungal
vegetations
• Role of PFO in childhood stroke remains uncertain

7. Hematological
Hematological disorders- SCA,PNH, b thalessemia
Prothrombotic disorders-
• Congenital- Deficiencies of protein C, protein S, and AT,Prothrombin
gene 20210A mutation
• Acquired- Nephrotic syndrome
Temporary hematological abnormalities and resultant thrombosis may
result from intercurrent illness

8. Arteriopathies
Focal cerebral arteripathy
Unifocal and unilateral stenosis/irregularity of the large intracranial
arteries of the anterior circulation
• FCA dissection type (FCA-d)- intracranial arterial dissection of the
anterior circulation, typically with trauma
• FCA inflammation type (FCA-i)- presumed inflammatory
• Can also be classified as- Intracranial ,Extracranial

9. Intracranial Arteriopatheis
Moyamoya disease
• Moyamoya is a rare, chronic, progressive steno-occlusive intracranial
vasculopathy
• Involving the distal supraclinoid ICA, proximal ACA and MCA
• Bimodal distribution, <10–15 years,adults 3RD-5TH decade
• Children usually present with recurrent transient ischemic attacks or
strokes, headaches, seizures, or movement disorders.

10. Extracranial Arteriopathy
Craniocervical arterial dissection (CCAD)
• Accounts for 7.5% of childhood stroke,High rates of recurrent
• Risk factors -male sex, head and neck trauma, connective tissue disorders.
Pseudoaneurysm and dissection in the V3 segment (C1- C2 region)
• Result of repetitive trauma of the artery with neck turning,
• High rates of recurrent stroke

11. Syndromic and Metabolic Disorders
• Marfan syndrome
• Tuberous sclerosis- Have a higher risk of embolic events.
• Nutritional deficiencies of folic acid or vitamin B1 may also caus
hyperhomocysteinemia, leading to stroke
• Genetics- familial lipoprotein disorders,Mitochondrial disorders
(MELAS)

12. Other causes
• Vasculitis-Kawasaki disease, HSP,PNA,SLE
• Drugs-oral contraceptives,Overuse of ergot alkaloids
• Oncologic- increased risk for AIS as a result of their disease,
subsequent treatment, and susceptibility to infection.

13. Evaluation
Cardiac evaluation
• ECG
• Transthoracic echocardiography with bubble study
• Look for valve leaflets
Thrombophilia
• CBC, FVL mutation, Prothrombin G20210A mutation,Protein C,Protein
S ,Antithrombin mutation
• Lupus anticoagulant,APLA

14. Neuro-Imaging in pediatric stroke
Moyamoya diease
Associated with the
formation of an abnormal
vascular network at the
base of the brain
resembling a “puff of
smoke” on angiography.

16. If moyamoya is identified on MRI, then DSA should considered
• Increased diagnostic sensitivity for moyamoya compared with MRI
• Important data germane to preoperative planning.
• Transdural collaterals visualized on DSA are critical biomarkers of
disease.

17. Grading of Moyamoya
1st-Stage – Narrowing of carotid fork only
• 2nd – Dilatation of all main cerebral arteries
• 3rd – Reduction of flow in the middle and anterior cerebral arteries
• 4th – Proximal portions of the posterior cerebral arteries become
involved
• 5th – Absence of all main cerebral arteries
• 6th – Cerebral circulation is supplied only by the external carotid
system

18. Post-varicella transient cerebral arteriopathy.

19. Vertebral artery pseudoaneurysm.
Imaging
• In cases with multiple
infarcts of the posterior
circulation
• DSA- evaluate the V3
segment
• Pseudoaneurysm or
dissection detected,
head turning during
imaging

20. MELAS
• Primarily in that they do
not conform to vascular
territories.
• Lesions are predominantly
occipital
• May have paradoxically
increased ADC on DWI.
• DWI may demonstrate
hyperintensities in
gyriform pattern, and T2
anomalies may expand on
sequential imaging.

21. Arterial Wall Imaging

22. Stroke Mimics
• Migraine with aura
• Bell palsy
• Seizure, especially with todd paresis.
• Demyelinating disease
• Brain tumours
• Metabolic disease
• Psychogenic disorders

23. Acute Management of Childhood AIS
Blood Glucose, Temperature
Management of hyperglycemia and hypoglycemia, fever as in adults
Blood Pressure
• Caution should be exercised in children with intracranial vascular
stenosis
• Often hypertensive at baseline, presumably as a compensatory
mechanism to improve cerebral perfusion

24. TIPS was an NIH-funded phase 1 clinical trial to determine the
safety and pharmacokinetics of intravenous tPA in children 2
to 18 years of age within 4.5 hours of AIS if vascular
obstruction was diagnosed on MRI.

25. Thrombolysis
• Consensus opinion- intravenous tPA is considered in children, the adult dose of
0.9 mg/ kg be used
• Differences in plasminogen levels may actually make the effective dose for
children higher
Endovascular thrombectomy
• May be reasonable for some acute AIS patients <18 years of age, using adult
parameters
• Benefits and risks are not established in this age group

26. Challenges in Mechanical thrombectomy
• Smaller size of children’s vessels may limit use of some clot retrieval devices.
• Large vessel occlusions secondary to embolism amenable to endovascular
treatment, are less common than intracranial arteriopathies.
• Radiation exposure and dose limitations for iodinated contrast during
angiography
• Delayed pediatric AIS diagnosis and interpretation of perfusion imaging in
children are additional challenges.

27. Management Of Complications
Malignant edema of the cerebral hemisphere- Decompressive surgery
Large-volume infarcts (more than half of the MCA territory)
• Performing early prophylactic hemicraniectomy within the first 24
hours
• Implementing serial imaging and frequent clinical assessments within
the first 72 hours to monitor swelling, need for surgical intervention

28. Treatment
• Both antiplatelet (eg, aspirin) and anticoagulant (LMWH or warfarin)
medications appear to be safe in initial AIS
• Relative contraindications to anticoagulant therapy include very large
acute infarcts or severe bleeding diathesis.

29. Antithrombotic Therapies Used for Stroke
Prevention
Uncharacterized childhood AIS
• Either anticoagulation or aspirin may be considered during the initial
5 to 7 days.
Cardiac embolism,prior thrombosis,prothrombotic disorder
• Maintenance therapy -continued anticoagulation for 3 to 6 months or
longer

30. Antiplatelet therapy
• In most other children, continued maintenance therapy consists of aspirin dosed
at 3 to 5 mg/kg.
• Duration of aspirin therapy-Underlying condition, Ongoing risk of recurrent
stroke.
• Most children are treated for 2 years to cover the time window when the vast
majority of recurrent strokes occur.
• Duration of antithrombotic treatment in the setting of persistent arteriopathy is
unknown

31. Management in Sickle Cell Disease
Acute Management
• Optimal hydration, correction of hypoxemia, and correction of systemic
hypotension.
Suspected acute cerebral infarction
• Prompt initial simple blood transfusion is needed to get the hemoglobin
level to 10 g/dL,
• if the hemoglobin is >10 g/ dL, an exchange transfusion is required.

32. Prevention of stroke
• Screening for cerebral infarcts with an MRI for detection can be considered
for children with hemoglobin SS or Sβ0 thalassemia.
• If a silent infarct is identified- then cognitive assessment is warranted
• Regular blood transfusions to reduce the percentage of hemoglobin S to a
maximum of <30%
• Hydroxyurea therapy after 1 year of regular blood transfusion therapy
should be offered to children.

33. Stroke Prevention in Sickle Cell Disease
• In children with SCD and an ICH- DSA for structural vascular lesion
• Reasonable to repeat a normal TCD annually
• Repeat an abnormal study in 1 month.
• Hydroxyurea may be considered in patients who will not or cannot
continue on long-term transfusion

34. Surgical Management of Moyamoya
• Indications- strokes, TIAs, or other clinical or radiographic evidence of
compromised cerebral blood flow or cerebral perfusion reserve
Surgical revascularization- Primary treatment
• Direct technique- Superficial temporal artery to MCA bypass,Middle
meningeal artery to MCA
• Indirect technique-Cortical receipnt artery not available for anastomosis

35. Rehabilitation
Constraint therapy(Level of Evidence A)
• Improves upper extremity strength by increasing the use of the affected upper
limb.
• Associated with improved function of the hemiparetic hand.
• Improvements are sustained over a prolonged period of time, and late
deterioration is rare.

36. CSVT in Childhood
• Risk Factors- fever, anemia dehydration, and infection, Behçet
syndrome, Hypercoagulable state,congenital heart disease
• Children with suspected CSVT-dedicated brain MRV or CT
venography for diagnostic confirmation.
• Children with confirmed CSVT -thorough evaluation for risk factors,
as well as acquired and genetic thrombophilia.

37. Management
• Supportive care measures
• Anticoagulation is the mainstay of treatment
• In rare circumstances, endovascular intervention with thrombolysis or
thrombectomy is an option

38. Hemorrhagic Stroke in Childhood

39. Pediatric ICH score
Intraparenchymal hemorrhage volume as percentage of TBV
<2 % = 0 , 2 to 3.99 % = 1 ,- ≥4 % = 2
 Hydrocephalus?
No = 0 ,Yes = 1
Herniation?
No = 0 ,Yes = 1
Infratentorial location?
No = 0 ,Yes = 1
• Total pediatric ICH score ranges from 0 to 5 points.

40. Management
• Includes airway, seizure control,Normoglycemia, and normothermia.
• Bleeding disorder is known, rapid correction should be instituted.
• No known bleeding disorder, MRA can be performed.
• All pediatric patients with hemorrhagic stroke should ultimately have
DSA before a hemorrhage is deemed idiopathic.

41. Aneurysms in children
Different from those in adults in the following respects
• There tends to be a male predominance in children
• Pediatric aneurysms tend to be larger in size
• There is a higher incidence of giant aneurysms in children

43. Acute ischemic stroke in Neonates
Presentation
• Seizures, characteristically focal motor seizures involving only 1 extremity
• Individuals with presumed perinatal stroke may seem normal after birth
• Later present with delayed motor milestones, epilepsy, asymmetric motor
function, or early handedness.
• Some may have had clinical or subclinical seizures that escaped detection in the
neonatal period.

44. Pathophysiology
• Emboli of cardiac, transcardiac, or aortic arch origin
• Disorders of the cerebral arteries
• Thrombosis due to disturbed hemostasis
• Thrombosis of placental vessels normally occurs as pregnancy ends
• Cerebral oxygen delivery falls- ischemic lesions develops in border
zone regions

45. Risk Factors
Neonatal factors
• Low levels of factors in the infant just before and after the time of
delivery
• Inherited thrombophilia,cardiac lesions, coagulation disorders
infection, trauma, asphyxia
Maternal factors
Primiparity,infertility, chorioamnionitis,oligohydramnios, coagulation
disorders, and preeclampsia.

46. Evaluation
• Routine thrombophilia testing is not indicated.
• Thrombophilia evaluation-limited clinical utility
• Levels of protein C, protein S, antithrombin, and factor XI are normally
decreased to 30% of adults levels
• Approach adult levels at various time points during childhood
• Obtained only in highly selected cases

47. Management
• Supportive care-control of seizures, oxygenation,correction of dehydration and
anemia.
• Aspirin and LMWH rarely indicated- low risk of recurrent stroke after neonatal AIS
• Considered in high risk of recurrent AIS
• Thrombolytics, mechanical thrombectomy- No evidence for their use.
• Endovascular procedures-small artery size of neonates precludes the use of
current endovascular devices in these individuals

48. Outcomes of stroke
• Golomb et al summarized 111 children with perinatal stroke,
including 67 who presented as neonates and 44 whose strokes were
discovered later.
• Seventy-six children (68%) exhibited cerebral palsy,55 of these
individual had at least 1 additional disability
• 45 (59%) experienced cognitive or speech impairment
• 36 (47%) had epilepsy.

49. CSVT in Neonates
• Lethargy, irritability, or seizures
• Risk Factors- Gestational or delivery complications, dehydration, sepsis,
meningitis, cardiac defects, and coagulation disorders
• Thrombophilia evaluation in the neonate has limited clinical utility
• MRI, especially MRV, should be performed to diagnose the thrombosis

50. Management
• Supportive measures -control of epileptic seizures, correction of dehydration and
anemia, treatment of underlying infections.
• Anticoagulation with LMWH or heparin may be considered in neonates with CSVT
• Those having clinical deterioration or evidence of thrombus extension on serial
imaging.
• Not on anticoagulation-Serial imaging at 5 to 7 days should be considered to
exclude propagation

51. Management
• Anticoagulation should be continued after the acute period for at least six
weeks using LMWH.
• Repeat imaging with MRI and MRV at the targeted endpoint of therapy (six
weeks)
• For neonates who have not achieved clinically significant recanalization
• Extending the duration of anticoagulation therapy for up to six months

52. Hemorrhagic Stroke in Neonates
• Presentation- seizures, encephalopathy.
• Risk Factors- postmaturity, emergency cesarean delivery, fetal distress,
male sex, coagulopathy( Aqcuired, congenital)
• Markedly low platelet counts and coagulation factor deficiencies should be
corrected.
• Large doses of vitamin K- correct factor deficiencies resulting from
maternal medications
• Surgical evacuation of a hematoma, Ventricular drainage

53. Challenges to Improving Acute Pediatric Stroke
Care
• Spectrum of Disorders Mimicking Stroke Differs From Adults
• Parental Knowledge and Care-Seeking Seeking Behavior in Pediatric
Stroke
• Pediatric Tools for Stroke Assessment
• Challenges to Accessing Rapid Diagnostic Imaging in the ED
• Safety and Efficacy of tPA in Children
• Mechanical Interventions in Pediatric Stroke

54. Opportunities for Improving Acute Stroke Care
in Children
• Increasing Stroke Awareness and Decision Support
• Tools to Improve Diagnostic Accuracy
• Developing Systems of Acute Pediatric Stroke Care and
Comprehensive Pediatric Stroke Centers
• Acute Pediatric Code Stroke and Rapid Neuroimaging Protocols
• Mechanical Thrombectomy in Pediatric Stroke
• Establishment of Pediatric Registries to Capture Safety and Efficacy
Data for Reperfusion Therapies

55. Conclusion
• Important to recognize stroke in children.
• These groups of patients have different pathophysiology for stroke
• Hyper-acute reperfusion therapies are still not advocated because of
paucity of clinical trail data.

56. Refrences
• Management of Stroke in Neonates and Children -A Scientific Statement
From the American Heart Association/American Stroke Association (Stroke.
2019;50:e51–e96)
• Manus J. Donahue. Neuroimaging Advances in Pediatric Stroke ,Stroke.
2019;50:240-248.
• Laura L. Lehman .What Will Improve Pediatric Acute Stroke Care? Stroke.
2019;50:00-00
• Nomazulu Dlamini .Arterial Wall Imaging in Pediatric Stroke,Stroke. 2018
• Bradley 7th edition
• www. Uptodate.com

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