Initial Management of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients
The initial management of PRES in pediatric patients requires immediate and stringent blood pressure control, antiepileptic therapy if seizures occur, and removal of any precipitating agents. 1
Immediate Priorities
1. Blood Pressure Management
Very stringent control of blood pressure is crucial 1. The goal is gradual BP reduction to avoid sudden hypoperfusion of vital organs 2. While there is no specific BP threshold that defines hypertensive emergencies, the rate of BP increase is more important than the absolute value 3. In pediatric PRES cases, hypertension is frequently associated with underlying renal disease, particularly post-streptococcal glomerulonephritis 4, 5.
- Monitor BP continuously in an intensive care setting
- Reduce BP gradually with IV antihypertensives
- Avoid precipitous drops that could cause cerebral hypoperfusion
2. Seizure Management
Seizures occur in 60-90% of PRES cases, particularly in younger populations 6. Antiepileptic treatment is required when seizures are present 1. Benzodiazepines are specifically mentioned as symptomatic treatment 1. Some pediatric patients may require intubation for refractory seizures 5.
3. Remove Precipitating Agents
Treatment requires cessation of anticancer therapy (if applicable) and withdrawal of causative agents 1. Common triggers in pediatrics include:
- Immunosuppressive medications (cyclosporin)
- Chemotherapy agents
- Steroids, NSAIDs
- Underlying conditions: renal disease, autoimmune disease, malignancy, transplantation 7
Diagnostic Confirmation
While initiating treatment, confirm diagnosis with neuroimaging:
- MRI with T2-weighted or FLAIR sequences is the gold standard 3, 5, showing hyperintensities in bilateral parietal-occipital lobes
- CT scan is useful to exclude intracranial hemorrhage 3
- Typical vasogenic edema involves posterior regions but atypical patterns with anterior involvement occur frequently 8, 6
Supportive Management
Address underlying conditions:
- Correct electrolyte imbalances (particularly sodium disturbances) 1
- Manage acid-base disorders 5
- Treat intracranial hypertension if present 5
- Address underlying renal disease, sepsis, or other precipitants 7, 4
Critical Care Setting
PRES is best managed by monitoring and treatment in a neurointensive care unit 8. Pediatric patients may require PICU transfer for severe cases with reduced consciousness, refractory seizures, or need for intubation 4, 5.
Prognosis and Follow-up
PRES is usually reversible with appropriate supportive management within 2 weeks 1. Most children recover completely 7, with clinical and radiologic improvement within days when adequate treatment is immediately initiated 8. However, delay in diagnosis and treatment may lead to permanent neurological sequelae 8, 6, with reported mortality of 10-19% and persistent deficits in up to 40-44% of cases 6.
Common Pitfalls to Avoid:
- Delaying treatment while awaiting imaging - initiate BP control and seizure management immediately based on clinical presentation
- Overly aggressive BP reduction - gradual lowering prevents cerebral hypoperfusion
- Missing underlying renal disease - nephropathies are the main cause of PRES in pediatrics 5
- Assuming complete reversibility - lack of resolution of imaging findings portends worse prognosis 7
Recurrence is possible 7, so reintroduction of prior causative therapy must be decided individually 1.