What is the approach for a pediatric patient with multiple lacunar cerebral and cerebellar infarcts?

Approach to Pediatric Multiple Lacunar Cerebral and Cerebellar Infarcts

In a pediatric patient with multiple lacunar infarcts affecting both cerebral and cerebellar territories, immediate transfer to an intensive care or stroke unit with neurosurgical consultation is essential, followed by comprehensive investigation for underlying vasculopathy (particularly arterial dissection, vasculitis, or moyamoya), with treatment directed at the identified etiology rather than the lacunar pattern itself. 1, 2

Immediate Triage and Monitoring

• Transfer immediately to a stroke unit or intensive care unit with neuromonitoring capabilities, as multiple infarcts in a pediatric patient represent a potentially progressive condition requiring close observation 1, 2
• Obtain urgent neurosurgical consultation if cerebellar infarcts are present, as these can deteriorate rapidly with brainstem compression or hydrocephalus 1, 2
• Monitor closely for at least 5 days if territorial cerebellar infarctions are present, even if the patient appears stable initially 1, 3
• Assess level of consciousness frequently, as decreased alertness is the most reliable sign of tissue swelling and impending deterioration 1, 3
• Watch for brainstem compression signs: pupillary changes (anisocoria or pinpoint pupils), loss of oculocephalic responses, breathing irregularities, cardiac dysrhythmias, and bradycardia 4, 3

Critical Diagnostic Workup

The multiple lacunar pattern in a pediatric patient is atypical and demands investigation for underlying arteriopathy rather than typical small vessel disease seen in adults:

• Obtain high-resolution MRI with fat-saturated T1 imaging of the neck and contrast-enhanced MRA to evaluate for cervical or intracranial arterial dissection, which can present with multiple embolic infarcts 1
• Perform conventional angiography or CTA if dissection is suspected, looking for string sign, double-lumen sign, or smooth tapered stenosis 1
• Evaluate for vasculitis in any pediatric patient with multiple infarcts, particularly if accompanied by encephalopathic changes or recurrent strokes 1
• Consider moyamoya syndrome, especially if there is progressive narrowing of arteries at the base of the brain, as this predisposes to both ischemic and hemorrhagic stroke 1
• Screen for sickle cell disease in patients of African or Mediterranean descent, as the pathophysiology involves large artery arteriopathy with progressive narrowing 1
• Assess for fibromuscular dysplasia, though rare in children, particularly affecting the extracranial ICA 1
• Investigate infectious causes: mycoplasma, aspergillosis, Borrelia burgdorferi, cat-scratch disease, and neurocysticercosis can all cause arteritis and multiple infarcts 1

Acute Medical Management

General Supportive Care

• Ensure adequate cerebral oxygenation and maintain cerebral perfusion pressure >60 mmHg 1, 4
• Correct hypovolemia with isotonic fluids only—avoid hypotonic or dextrose-containing solutions 1, 2, 4
• Elevate head of bed 0-30 degrees during periods of increased intracranial pressure 1, 4
• Treat hyperthermia aggressively and maintain normoglycemia (glucose <8 mmol/L) 1, 4
• Avoid oral intake initially until swallowing is assessed 1, 4

Antithrombotic Therapy Decisions

The approach depends critically on the identified underlying etiology:

If Cervical Arterial Dissection is Identified:

• Begin UFH or LMWH immediately as a bridge to oral anticoagulation for extracranial dissection 1
• Continue anticoagulation with LMWH or warfarin for 3-6 months (target INR 2.0-3.0) 1
• Consider antiplatelet agents as an alternative to anticoagulation, particularly for intracranial dissections where subarachnoid hemorrhage risk is higher 1
• Extend therapy beyond 6 months if recurrent symptoms develop or residual arterial abnormality persists 1

If Sickle Cell Disease is Confirmed:

• Initiate chronic blood transfusion therapy to reduce hemoglobin S to <30% of total hemoglobin for secondary stroke prevention 1
• Use hydroxyurea as an alternative only if transfusion therapy is not available or practical, though it is less effective (10% recurrent stroke rate vs. 0% with transfusion) 1
• Avoid routine antithrombotics unless there is evidence of another stroke mechanism (e.g., atherosclerosis), as SCD patients have higher hemorrhagic stroke risk 1

If Vasculitis is Suspected:

• Antiplatelet therapy with aspirin (1-5 mg/kg/day) is reasonable as initial therapy until embolic causes are excluded 5
• Consider UFH or LMWH initially (dosing to achieve aPTT corresponding to anti-FXa 0.35-0.7 U/mL for UFH, or anti-FXa 0.5-1.0 U/mL for LMWH) 5

If No Specific Etiology Identified:

• Initiate antiplatelet therapy with aspirin (1-5 mg/kg/day) until dissection and embolic causes are excluded 5
• Provide thromboembolism prophylaxis with subcutaneous low-dose heparin or LMWH 1, 4

Surgical Considerations for Cerebellar Involvement

If cerebellar infarcts are present with any signs of deterioration:

• Perform suboccipital decompressive craniectomy with dural expansion if the patient develops decreased consciousness, GCS <12, or decline of ≥2 points 1, 2, 4
• Do NOT perform ventriculostomy alone for hydrocephalus—this can cause fatal upward herniation; always combine with suboccipital craniectomy 2, 4
• Radiographic indications for surgery include fourth ventricular compression and hydrocephalus 4, 3
• Outcomes after cerebellar surgery are often good if performed before irreversible brainstem injury occurs 1

Critical Pitfalls to Avoid

• Do not assume typical small vessel disease as in adults—multiple lacunar infarcts in children nearly always indicate an underlying arteriopathy requiring specific investigation 1
• Do not delay neurosurgical consultation if cerebellar infarcts are present, as deterioration can be rapid and fatal 2, 4
• Do not use corticosteroids, barbiturates, or hypothermia for cerebral edema—these lack evidence and are not recommended 1, 4
• Do not miss arterial dissection by relying only on standard imaging—use high-resolution MRI with fat-saturated sequences 1
• Do not overlook sickle cell disease in appropriate populations, as it requires specific transfusion-based therapy 1
• Do not use full-dose therapeutic anticoagulation acutely without identifying the underlying mechanism, as this may increase hemorrhagic risk 1, 4

Prognosis and Long-term Management

• Recanalization occurs in 60% of children with arterial dissection, with 12% risk of recurrent stroke or TIA 1
• Continue antiplatelet agents long-term if residual arterial abnormality persists 1
• Monitor for cognitive decline and dementia risk, as even lacunar infarcts carry increased long-term risk 6, 7, 8
• Aggressive risk factor modification is essential: blood pressure control, lifestyle modifications, and addressing any identified prothrombotic conditions 6, 7