Presentation of Ischemic Cerebrovascular Disease in Children

Symptoms and signs

Perinatal stroke (62)

• Seizures: Neonates will often present with seizures. One study reported seizures in 94% of neonates with arterial ischemic stroke (193).

MRI of a male neonate presenting with seizures: (A) Axial and (B) coronal T2-weighted MRI demonstrate relatively hyperintense regions of the left hemisphere with evidence of encephalomalacia (yellow arrows). (C) Corresponding hypointensities on ADC and (D) hyperintensities on DWI (yellow arrows). (Images courtesy of Dr. Fabricio Goncalves and Caroline Davies of the University of Alabama at Birmingham Heersink School of Medicine.)

• Nonspecific symptoms: Lethargy and irritability may be observed.
• Symptoms of perinatal stroke discovered late: Children who suffered perinatal stroke that was not detected early may present with delayed motor milestones, epilepsy, asymmetric motor function, and hand preference.

Childhood stroke (62)

• Weakness and motor symptoms: Hemiparesis and hemifacial weakness are common. Ataxia may be observed.
• Speech and language symptoms: Aphasia and dysarthria may be observed.
• Vision disturbance: A variety of vision disturbances may be observed, depending on the region of the visual pathway affected.
• Nonlocalizing symptoms: Headache and altered mental status may be observed.
• Seizures: Seizures may be observed and are more common in children with ischemic stroke, as compared to adults.

Patterns of evolution

• Variable patterns of evolution: Symptoms and signs of pediatric ischemic stroke vary by age and brain regions affected. Thus, there is no stereotypic pattern of acquisition. Symptoms and signs may be transient and may not be immediately apparent (194).

Time for evolution

• Perinatal stroke: Neonates may present acutely with focal seizures and/or lethargy (62,194). However, delayed motor milestones, epilepsy, asymmetric motor function, hand preference, and focal neurological deficits may not present until weeks or months later.
• Childhood stroke: Children with ischemic stroke present similarly to adults and often present with acute symptoms and signs (62). Older children may report prior, brief episodes that resemble TIAs (194).

Evaluation at Presentation

• Urgent evaluation: The differential diagnosis of pediatric ischemic stroke is broad and includes hemorrhagic stroke, TIA, migraine, seizures not caused by stroke, Bell’s palsy, conversion disorders, and intracranial infection (195,196). Misdiagnosis and delayed diagnosis of pediatric ischemic stroke is very common (197,198). Urgent evaluation and imaging are required to confirm the diagnosis. The evaluation is notoriously challenging because the proportion of actual arterial ischemic strokes in children presenting with symptoms suggesting one is low — the opposite is true of adults, who have few cases of stroke mimics and many cases of stroke.
• History: Determine the last known normal time and whether the patient has a history of any of the following: CHD, sickle cell hemoglobinopathy, coagulopathy, vasculitis, metabolic disorder, recent or ongoing infection or illness, recent head or neck trauma, and drug or medication use (199).
• Neuroimaging: Obtain a brain MRI with DWI and ADC mapping, in addition to TOF MRA, if available, as soon as possible (200). Non-contrast CT can be obtained early to exclude hemorrhagic stroke (194). Head CT and CTA can be obtained if urgent MRI is not possible or is contraindicated (201). Radiation exposure in young children should be minimized where possible.
• Laboratory studies: Obtain CBC, urea, and electrolytes; prothrombin time/international normalized ratio and partial thromboplastin time ; serum glucose; venous or capillary blood gas; and type and screen (202,203).
• Vital signs: Initiate continuous monitoring of blood pressure, temperature, oxygen saturation (SpO2), heart rate, and respiratory rate (202).
• Neurological exam: Use the pediatric adaptation of the National Institutes of Health Stroke Scale (PedNIHSS) and the GCS for initial assessment of neurologic deficits (202-204).
• Cardiac evaluation: Use transthoracic echocardiography to evaluate for cardiac thrombus, vegetations, and right-to-left cardiac shunt. A bubble (saline contrast) study can evaluate for a PFO (203).
• Additional studies: In some patients, additional studies may be indicated early, including lumbar puncture for suspected infectious etiology, toxicology screen, and thrombin time (202,203).

Intervention at Presentation

Stabilization

• Goal of stabilization: The goal of stabilization in pediatric ischemic stroke is supportive therapy to achieve and maintain normoxemia, normocapnia, normotension, and normoglycemia. The ABCDE (Airway, Breathing, Circulation, Disability/Seizure, and Exposure/Examination) approach is often used (205).
• Airway: Ensure airway patency. Intubation with mechanical ventilation is indicated if GCS ≤8, there is a loss of airway reflexes, or if ICP is elevated (202,205).
• Breathing: High flow oxygen with a target SpO2 ≥92%, pulse oximetry, and mechanical ventilation (if indicated) are important interventions used to achieve normoxemia and normocapnia (202,205).
• Circulation: Continuous blood pressure monitoring should inform whether fluid resuscitation (10 mL/kg isotonic fluid bolus), vasopressors, or antihypertensives are required; normotension for age is the goal (202,205).
• Seizure: Anticonvulsants for seizures may be required (205).
• Exposure/Examination: Regular clinical examination, temperature management, and achievement of normoglycemia (6-10 mmol/L; insulin or 2 mL/kg of 10% dextrose may be required) are recommended (202,205).

Preparation for definitive intervention, nonemergent

• Continuous monitoring: Pursue supportive therapy if there is any deviation from normoxemia, normocapnia, normotension, and normoglycemia.
• Additional evaluation: Stable patients may not require immediate intervention and can undergo additional evaluation for etiology. This includes evaluation for hypercoagulability, inflammatory etiology, infection, connective tissue disorders, and metabolic disorders (194). Coagulation tests, tests for inflammatory markers, and genetic testing can be considered.
• Electroencephalography: Continuous EEG can be used to monitor seizure status, and anticonvulsants can be administered if needed (203).
• Patients with HbSS: Supportive therapy, IV hydration, and exchange transfusion with a target hemoglobin S level of <30% are important steps in the treatment of children with HbSS who present with stroke. If exchange transfusion is not immediately available, simple transfusion can be an initial intervention (203).

Preparation for definitive intervention, emergent

• Consideration of medical, surgical, or interventional therapy: In the absence of contraindication, medical and interventional therapies have varying time windows, so appropriate emergent consultation with stroke neurology, endovascular neurosurgery, and interventional neuroradiology is appropriate to consider time-sensitive treatments.
• Close observation and supportive therapy: Continuous monitoring of vital signs and supportive therapy to achieve and maintain normoxemia, normocapnia, normotension, and normoglycemia should be continued prior to definitive intervention.
• Repeat imaging as necessary: Repeat the head and neck MRI/MRA (or head CT/CTA if MRI/MRA are not available) when a change in the neurologic exam results is observed (203).

Admission orders

• Head of bed, positioning, and activity: The HOB should be flat to maximize cerebral perfusion pressure. The patient should not eat or drink until oral intake is determined safe via a swallowing study (203). Strict bed rest is required, keeping the neck midline.
• Vital signs: Continuous monitoring of blood pressure, temperature, oxygen saturation, heart rate, and respiratory rate is critically important (202). Adjust oxygen to maintain SpO2 >94%.
• Blood pressure parameters: Normotension is the goal; a blood pressure goal of 50th to 95th percentile for age/height has been recommended, with hypertension up to 20% above the 95th percentile permitted (203). Treat significant hypertension with labetalol or an angiotensin-converting enzyme inhibitor, but avoid a rapid and dramatic drop in blood pressure.
• IV fluids: Maintain euvolemia and normoglycemia; infuse normal saline with or without D5W (dextrose 5% in water) at 1 to 1.5 times the maintenance fluid requirements with normoglycemia (maintain serum glucose levels of 60-200 mg/dL) (203).
• Temperature: Normothermia is the goal. Prevent hyperthermia (greater than 37.5°C) with acetaminophen or cooling blanket application (203).
• Neurological exam: Instruct nursing staff to conduct hourly neurological exams (203).
• Other testing: As necessary or if not already performed, order electrocardiogram, echocardiogram, EEG, and repeat neuroimaging (203).
• Consults: Consult neurology for all patients. Consult hematology for a child with HbSS.