Pediatric stroke evaluation ;management- Dr Pallav Jain

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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