Pediatric Stroke
1. Introduction and Definition
- Definition: A stroke is defined as the sudden onset of a focal neurological deficit lasting > 24 hours (or leading to death) caused by a disturbance in blood supply to the brain.
- Pediatric Stroke Classification:
- Perinatal Stroke: 20 weeks gestation to 28 days of life.
- Childhood Stroke: > 28 days to 18 years of age.
- Types:
- Arterial Ischemic Stroke (AIS): Obstruction of an artery (Commonest).
- Cerebral Sinovenous Thrombosis (CSVT): Thrombosis of venous sinuses.
- Hemorrhagic Stroke: Rupture of a vessel.
2. Epidemiology
- Incidence: 2β13 per 100,000 children per year.
- Mortality: A top 10 cause of death in children.
- Morbidity: > 60% of survivors have permanent neurological deficits (Cerebral Palsy, Epilepsy).
3. Etiology and Risk Factors (The "Black Box")
Unlike adults (where atherosclerosis/HTN dominates), pediatric stroke is multifactorial.
| Category | Specific Causes / Risk Factors |
|---|---|
| Arteriopathies (50-80% of AIS) | β’ Focal Cerebral Arteriopathy (FCA): Transient inflammatory stenosis (often post-Varicella). β’ Moyamoya Disease: Chronic progressive stenosis of distal ICA. β’ Arterial Dissection: Post-trauma (even minor neck trauma) or spontaneous. β’ Vasculitis: PAN, Takayasu, Primary CNS vasculitis. |
| Cardiac Disorders (25%) | β’ Congenital Heart Disease: Cyanotic > Acyanotic (R-to-L shunt). β’ Procedures: Cardiac catheterization, ECMO. β’ Infection: Infective Endocarditis. β’ Cardiomyopathy/Arrhythmias. |
| Hematologic | β’ Sickle Cell Disease: Highest risk (10% risk without screening). β’ Prothrombotic States: Protein C/S deficiency, Factor V Leiden, Antithrombin III deficiency. β’ Iron Deficiency Anemia: Thrombocytosis/hyperviscosity. |
| Infectious | β’ Post-Varicella Angiopathy: Stroke 3β12 months after Chickenpox. β’ Meningitis: Bacterial (Pneumococcal/TB) causing vasculitis. β’ Head/Neck Infections: Mastoiditis (Risk of CSVT). |
| Metabolic / Genetic | β’ Homocystinuria: Thromboembolism. β’ MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke-like episodes. β’ Fabry Disease. |
4. Clinical Presentation
Symptoms vary significantly by age.
A. Neonates / Infants
- Seizures: Focal clonic seizures are the most common presentation.
- Encephalopathy: Lethargy, apnea, poor feeding.
- "Handedness": Pathological early handedness (< 1 year) indicates contralateral hemiparesis.
B. Older Children
- Hemiparesis: Sudden onset weakness of face/arm/leg.
- Speech: Aphasia or dysarthria.
- Visual: Hemianopia.
- Headache/Vomiting: More common in Hemorrhagic stroke or CSVT.
- Ataxia/Vertigo: Posterior circulation stroke.
5. Differential Diagnosis (Stroke Mimics)
- Seizures (Toddβs Paresis): Post-ictal weakness.
- Hemiplegic Migraine: Strong family history; headache follows aura.
- Intracranial Infection: Abscess, ADEM (Acute Disseminated Encephalomyelitis).
- Metabolic: Hypoglycemia (focal signs possible).
- Tumor: Acute hemorrhage into a tumor.
6. Diagnostic Evaluation
A. Neuroimaging (Stat)
- MRI Brain with DWI (Diffusion Weighted Imaging): Gold Standard.
- DWI: Shows restriction (bright) within minutes of ischemia.
- ADC Map: Confirms true restriction (dark).
- MRA / MRV (Angiography/Venography): Essential to visualize arteriopathy (Moyamoya, dissection) or sinus thrombosis.
- CT Head: Less sensitive for acute ischemia but excellent for Hemorrhage. Used if MRI is unavailable or for rapid exclusion of bleed.
B. Etiological Workup (The "Stroke Protocol")
- Cardiac: Echocardiography (Transthoracic/Transesophageal) with bubble study (for PFO). ECG.
- Vascular: Carotid Doppler (if dissection suspected).
- Blood Work:
- CBC (Sickle cell, Polycythemia, Platelets).
- Coagulation profile (PT/APTT).
- Thrombophilia screen (Protein C, S, AT3, Factor V Leiden, Lipoprotein-a, Homocysteine).
- Inflammatory markers (ESR, CRP, ANA).
- Lipid profile.
7. Management
A. Acute Management (The "Golden Hour")
-
Stabilization (ABC):
- Maintain Oxygen saturation > 92%.
- Blood Pressure: Permissive hypertension allowed (to maintain cerebral perfusion). Treat only if >95th percentile + 15-20%.
- Glucose: Treat hypoglycemia immediately. Avoid hyperglycemia (worsens infarct).
- Fever: Aggressive antipyretics (hyperthermia worsens outcome).
- Seizures: Treat aggressively (Benzodiazepines
Levetiracetam).
-
Specific Reperfusion Therapy:
- tPA (Tissue Plasminogen Activator): Controversial in children. Not routinely FDA approved. Considered only if:
- Adolescent / Large child.
- Within 4.5 hours window.
- Confirmed large vessel occlusion.
- Mechanical Thrombectomy: Increasing evidence for benefit in older children with large vessel occlusion (LVO) within 24 hours.
- tPA (Tissue Plasminogen Activator): Controversial in children. Not routinely FDA approved. Considered only if:
-
Antithrombotic Therapy (Standard of Care):
- Aspirin: (3β5 mg/kg/day). First-line for most Arterial Ischemic Strokes (non-cardioembolic, non-dissection).
- Anticoagulation (LMWH/Unfractionated Heparin):
- Indicated for Arterial Dissection (extracranial).
- Indicated for Cardioembolic Stroke.
- Indicated for Cerebral Sinovenous Thrombosis (CSVT).
- Contraindication: Hemorrhagic stroke / large infarct with hemorrhagic transformation.
B. Disease-Specific Management
- Sickle Cell Disease: Exchange Transfusion to reduce HbS < 30%. Chronic hypertransfusion program thereafter.
- Moyamoya Disease: Surgical Revascularization (Pial synangiosis / EDAMS).
- Autoimmune/Vasculitis: High-dose Steroids + Cyclophosphamide.
C. Chronic Management and Rehabilitation
- Secondary Prevention: Long-term Aspirin (usually 2 years or lifelong depending on cause).
- Rehabilitation: Physical therapy (constraint-induced movement therapy), Occupational therapy, Speech therapy.
- Education: Individualized Education Plan (IEP) for cognitive deficits.
8. Prognosis
- Recurrence: 20β30% overall. Highest risk in cases with Arteriopathy (Moyamoya/Vasculitis).
- Neurological Outcome:
- Hemiplegic Cerebral Palsy: Most common motor sequela.
- Epilepsy: Develops in 25% of survivors.
- Cognitive: Learning disabilities, ADHD, behavioral issues.
- Plasticity: Young brains have higher plasticity, so motor recovery is often better than in adults, but cognitive deficits may emerge years later ("growing into the deficit").
9. Summary Algorithm
- Suspicion: Sudden focal deficit.
- Confirm: MRI Brain (DWI) + MRA.
- Acute Tx: Neuroprotection + Aspirin (or LMWH if dissection/cardiac).
- Investigate: Echo + Blood work.
- Prevent: Treat underlying cause + Rehab.