Overview
Definition:
Increased intracranial pressure (ICP) is a pathological condition characterized by elevated pressure within the cranial vault
This can result from various causes, including space-occupying lesions, hydrocephalus, infections, or trauma
Persistent or severe elevation of ICP can lead to secondary brain injury, herniation, and potentially irreversible neurological damage
Vomiting and headache are common, often non-specific symptoms, but in the pediatric population, their presence, particularly with associated red flags, warrants urgent evaluation for raised ICP.
Epidemiology:
The incidence of raised ICP in children varies significantly with etiology
In neonates, conditions like hydrocephalus or intraventricular hemorrhage are more common
In older children, brain tumors, trauma, and infections (meningitis, encephalitis) are significant contributors
Headache is one of the most common pediatric complaints, but only a subset presents with signs of raised ICP
Vomiting in children can be due to numerous causes, making the distinction crucial.
Clinical Significance:
Prompt recognition of increased ICP in children is paramount for preventing devastating neurological sequelae
The pediatric brain, particularly in younger children with open fontanelles, has some capacity for compensatory expansion, which can mask early signs of rising ICP
However, once these compensatory mechanisms are overwhelmed, rapid neurological deterioration can occur
Identifying red flag signs associated with vomiting and headache allows for timely diagnosis and intervention, significantly improving patient outcomes and preventing mortality.
Clinical Presentation
Symptoms:
Nausea and vomiting, often projectile and not related to feeding
Persistent or worsening headache, typically worse in the morning or with Valsalva maneuvers
Irritability or lethargy
Visual disturbances, such as blurred vision or diplopia
Seizures may occur
In infants, irritability, poor feeding, increased head circumference, and a bulging fontanelle.
Signs:
Papilledema on fundoscopy (swelling of the optic disc)
Cranial nerve palsies, particularly VI nerve palsy leading to abducens nerve dysfunction and outward deviation of the eye
Widened pulse pressure and bradycardia (Cushing's triad - a late and ominous sign)
Altered level of consciousness, from subtle irritability to deep coma
Sunsetting eyes (eyes deviating downwards)
In infants, a tense, bulging anterior fontanelle
Focal neurological deficits may emerge as brain herniation progresses.
Diagnostic Criteria:
There are no single diagnostic criteria for increased ICP
Diagnosis is primarily clinical, based on the constellation of symptoms, signs, and exclusion of other causes
Neuroimaging findings are crucial for confirming elevated ICP and identifying the underlying etiology
Lumbar puncture can be diagnostic and therapeutic but should be performed cautiously only after ruling out a mass lesion on imaging to avoid herniation.
Diagnostic Approach
History Taking:
Detailed history of onset, duration, and character of headache and vomiting
Associated symptoms: fever, photophobia, phonophobia, visual changes, focal neurological deficits, trauma
Previous medical history: epilepsy, congenital anomalies, neurodevelopmental delay, previous head trauma
Family history of neurological conditions or brain tumors
Medications
Red flags include: new onset headache in a child < 5 years, headache associated with vomiting that is projectile or persistent, visual disturbances, focal neurological signs, seizures, morning headache, rapid increase in head circumference in infants, altered mental status.
Physical Examination:
Complete neurological examination: assessment of mental status (GCS), cranial nerves (especially fundoscopy for papilledema, eye movements for CN VI palsy), motor and sensory function, reflexes, and coordination
Check vital signs: pulse, blood pressure, respiratory rate, temperature
Assess fontanelle tension and head circumference in infants
Examine for signs of trauma
Abdominal examination to rule out other causes of vomiting.
Investigations:
Neuroimaging: Computed Tomography (CT) scan of the head is often the initial imaging modality for suspected acute raised ICP, identifying mass lesions, hydrocephalus, or hemorrhage
Magnetic Resonance Imaging (MRI) provides more detailed anatomical information and is better for evaluating tumors, inflammatory processes, and subtle lesions
Fundoscopy: essential for detecting papilledema
Lumbar Puncture: to measure opening pressure and analyze cerebrospinal fluid (CSF) for infection or inflammation
CSF opening pressure > 20 cm H2O in infants and > 25 cm H2O in older children is indicative of raised ICP
Blood tests: Complete Blood Count (CBC), electrolytes, glucose, renal and liver function tests to rule out metabolic causes of vomiting and assess overall status.
Differential Diagnosis:
Migraine, tension-type headache, sinusitis, gastroenteritis, metabolic disorders (e.g., diabetic ketoacidosis), poisoning/intoxication, benign paroxysmal vertigo, post-traumatic headache, brain tumors, hydrocephalus, meningitis, encephalitis, brain abscess, intracranial hemorrhage (e.g., subdural hematoma, subarachnoid hemorrhage), venous sinus thrombosis, hypertensive encephalopathy.
Management
Initial Management:
Immediate stabilization: Airway, Breathing, Circulation (ABC)
Secure airway if GCS < 8 or vomiting is severe
Administer oxygen
Monitor vital signs closely
Elevate head of the bed to 30 degrees to facilitate venous drainage
Avoid noxious stimuli
If a mass lesion is suspected on clinical grounds and imaging is delayed, empiric treatment for raised ICP may be initiated
Sedation and analgesia may be required.
Medical Management:
Osmotic therapy: Mannitol (0.25–1 g/kg IV infused over 15–30 minutes, repeated every 4–6 hours) or hypertonic saline (3% saline) can reduce cerebral edema
Hyperventilation: transiently reduces ICP by causing cerebral vasoconstriction, but should be used cautiously and short-term to avoid ischemia
Steroids: Dexamethasone is effective in reducing edema around brain tumors or abscesses, but not for trauma or stroke
Antiemetics for symptomatic relief of vomiting
Anticonvulsants if seizures are present.
Surgical Management:
Surgical intervention is indicated for specific etiologies: Ventriculostomy or shunt placement for hydrocephalus
Excision or debulking of tumors
Evacuation of intracranial hematomas or abscesses
Decompressive craniectomy for severe refractory ICP
Surgical intervention is guided by the underlying cause and urgency.
Supportive Care:
Continuous neurological monitoring
Pain management
Nutritional support, often with nasogastric or orogastric feeding if oral intake is compromised
Fluid and electrolyte balance management
Fever control
Prevention of complications such as pressure sores and deep vein thrombosis.
Complications
Early Complications:
Brain herniation (uncal, cingulate, tonsillar), leading to coma, respiratory arrest, and death
Secondary ischemia from compression of cerebral vessels
Seizures
Hypothalamic dysfunction (temperature instability, electrolyte imbalances).
Late Complications:
Hydrocephalus
Visual impairment or blindness
Cognitive deficits
Motor deficits
Epilepsy
Neurodevelopmental delay
Behavioral problems.
Prevention Strategies:
Early recognition and prompt management of the underlying cause of increased ICP
Judicious use of osmotic therapy and hyperventilation
Careful fluid management
Prophylactic anticonvulsant therapy in specific situations
Aggressive management of fever and pain.
Prognosis
Factors Affecting Prognosis:
The underlying etiology of the raised ICP is the most critical factor
Younger age, severity of neurological deficit at presentation, degree of ICP elevation, and presence of brain herniation are associated with poorer outcomes
Timeliness of diagnosis and intervention also plays a significant role
The presence of papilledema, while indicative of raised ICP, does not always correlate with the severity of neurological impairment or prognosis independently.
Outcomes:
Outcomes vary widely
Children with reversible causes like acute meningitis or post-traumatic edema have a good prognosis with timely management
However, conditions like aggressive brain tumors or severe traumatic brain injury can lead to permanent disability or death
Early intervention significantly improves the chances of a favorable neurological outcome.
Follow Up:
Long-term follow-up is essential, particularly for children with known brain lesions or sequelae
This includes regular neurological assessments, ophthalmological evaluations for visual disturbances, neurodevelopmental assessments, and management of any chronic complications like epilepsy or hydrocephalus
Neuroimaging may be required periodically to monitor for recurrence or progression of the underlying pathology.
Key Points
Exam Focus:
Always consider raised ICP in children presenting with persistent vomiting and headache
Key red flags include projectile vomiting, morning headache, papilledema, CN VI palsy, altered mental status, and new-onset seizures
Differentiate between signs of raised ICP and other causes of vomiting/headache in children
Understand the Monro-Kellie doctrine and its implications in pediatric neurosurgery
Be familiar with management of acute ICP elevation: head elevation, osmotic therapy (mannitol, hypertonic saline), hyperventilation (temporary), and surgical decompression
Recognize Cushing's triad as a late sign.
Clinical Pearls:
In infants, check the fontanelle tension and head circumference at every visit
If a child complains of headache, ask them to describe it and when it occurs – morning headaches are particularly concerning for raised ICP
Fundoscopy is a simple yet crucial examination
Never perform a lumbar puncture in a child with suspected raised ICP without first ruling out a mass lesion on CT/MRI to prevent herniation.
Common Mistakes:
Dismissing persistent vomiting and headache in children as benign or viral gastroenteritis without a thorough neurological assessment
Delaying neuroimaging in the presence of red flag symptoms
Performing lumbar puncture without prior imaging when a mass lesion is suspected
Inappropriate use of hyperventilation without adequate monitoring
Underestimating the potential for rapid neurological deterioration in children with raised ICP.