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Intracranial Hemorrhage, Pediatric

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Intracranial Hemorrhage, Pediatric

Basics

Description

Epidemiology

The pathologic accumulation of blood into the epidural, subdural, subarachnoid, intraparenchymal, or intraventricular space within the cranium due to loss of blood vessel integrity or coagulopathy пїЅ

Incidence of hemorrhagic (nontraumatic) stroke is 1.1 per 100,000 person years. пїЅ

Increased frequency with hereditary disorders of coagulation, congenital heart disease, and polycystic kidney disease associated with intracranial aneurysms пїЅ

Multiple cerebral cavernomas may be associated with autosomal dominant trait with CCM1, CCM2, and CCM3. пїЅ

ICH, especially in young infants and children without an obvious etiology, should raise the suspicion of nonaccidental trauma. пїЅ

Increasing intracranial pressure from hemorrhage or hydrocephalus is life threatening. пїЅ

Early subarachnoid hemorrhage may not be apparent on initial CT and may require lumbar puncture (if safe to perform) or serial CT evaluation while the patient is under clinical observation. пїЅ

Physiotherapy, occupational therapy, speech therapy as required пїЅ

Avoid D5 and excessive fluid administration; maintain euvolemia. пїЅ

  • Intraventricular hemorrhage is rare beyond the newborn period.
  • Trauma: common cause of ICH in children
  • Arteriovenous malformations (AVMs): most common cause of nontraumatic ICH in children

Risk Factors

General Prevention

  • Automobile seat belts
  • Bicycle, skating, and skateboarding helmets
  • Child abuse prevention
  • Diving safety practices
  • Preventing falls
  • Maintaining safe driving speeds
  • Keeping children away from firearms
  • Hematologic monitoring for those at risk for hemorrhage due to bleeding disorders

Pathophysiology

  • Epidural hematoma (blood between the dura mater and the skull) is frequently arterial, related to skull fracture; typically middle meningeal artery bleeding following temporal bone fracture; may also arise from dural venous sinus laceration.
  • Subdural hematoma (blood between the dura mater and the arachnoid membrane) is frequently venous from trauma causing stretching and tearing of bridging cortical veins or coagulopathy.
  • Subarachnoid hemorrhage (blood between the arachnoid membrane and brain): ruptured intracranial aneurysm, AVM, or trauma
  • Intraparenchymal hemorrhage: trauma, infections (herpes simplex encephalitis, bacterial endocarditis), coagulopathy, brain tumor, Moyamoya arteriopathy, venous sinus thrombosis, or cerebral infarction (occurs mostly with rupture of medium or smaller branches of major cerebral arteries)
  • Intraventricular hemorrhage: may occur in isolation (more frequent in preterm infants <36 weeks gestation) or in a mixed pattern with intraparenchymal or subarachnoid hemorrhage. In term infants, rule out venous sinus thrombosis (especially in patients with accompanying thalamic hemorrhage).
  • 4 grades of intraventricular hemorrhage:Grade I: isolated to 1 or both germinal matricesGrade II: intraventricular hemorrhage without ventricular dilatationGrade III: intraventricular hemorrhage with ventricular dilatation (hydrocephalus)Grade IV: intraventricular hemorrhage with ventricular dilatation and extension into the periventricular white matter
  • Grade I: isolated to 1 or both germinal matrices
  • Grade II: intraventricular hemorrhage without ventricular dilatation
  • Grade III: intraventricular hemorrhage with ventricular dilatation (hydrocephalus)
  • Grade IV: intraventricular hemorrhage with ventricular dilatation and extension into the periventricular white matter

Etiology

  • VascularCongenital vascular anomalies: aneurysm, AVM, cavernous hemangioma, arteriovenous fistula, vein of Galen malformationDevelopmental/acquired vasculopathy: Ehlers-Danlos syndrome type IV, Moyamoya arteriopathy, sickle cell disease, hypertension (posterior reversible encephalopathy syndrome [PRES]), infective aneurysm, vasculitis (cocaine, inflammatory diseases), cerebral venous sinus thrombosis, hemorrhagic conversion of ischemic stroke, brain tumor
  • Hematologic abnormalities: thrombocytopenia, hemophilia, sickle cell disease, liver failure, disseminated intravascular coagulation, iatrogenic (ECMO or anticoagulation therapy)
  • TraumaticAccidental injuryNonaccidental injury
  • Congenital vascular anomalies: aneurysm, AVM, cavernous hemangioma, arteriovenous fistula, vein of Galen malformation
  • Developmental/acquired vasculopathy: Ehlers-Danlos syndrome type IV, Moyamoya arteriopathy, sickle cell disease, hypertension (posterior reversible encephalopathy syndrome [PRES]), infective aneurysm, vasculitis (cocaine, inflammatory diseases), cerebral venous sinus thrombosis, hemorrhagic conversion of ischemic stroke, brain tumor
  • Accidental injury
  • Nonaccidental injury

Commonly Associated Conditions

  • Prematurity
  • Hemophilia (prevalence of ICH 3-12%)
  • Sickle cell disease (250-fold increased risk of ICH)
  • Bacterial endocarditis
  • Venous infarction
  • Arterial infarction
  • Alcohol, cocaine, and other sympathomimetics

Diagnosis

History

  • Delivery complications
  • Head trauma
  • Infection
  • Cardiac disease
  • High-output cardiac failure
  • Patient or family history of coagulopathy
  • Drug use
  • Cerebral venous sinus thrombosis: dehydration, coagulopathy, polycythemia, sepsis, asphyxia (especially in newborns)
  • Arterial aneurysms: polycystic kidney disease, coarctation of the aorta, fibromuscular dysplasia, connective tissue disease
  • Vein of Galen malformation: presents as failure to thrive, hydrocephalus, seizures, high-output cardiac failure
  • Clinical presentation of ICH: headache (severity, quality, onset, location), neck pain or stiffness, vomiting, irritability, altered level of consciousness ("lucid interval"пїЅ with epidural hematoma), seizures, visual problems (diplopia, blurred vision), focal neurologic deficits, epistaxis (may occur if skull fracture is present)Posterior fossa bleed: disconjugate gaze, ataxia, and rapid deterioration to coma
  • Posterior fossa bleed: disconjugate gaze, ataxia, and rapid deterioration to coma

Physical Exam

  • Signs of increased intracranial pressure or herniation, such as Cushing triad (hypertension, bradycardia, abnormal respiratory pattern); papilledema; fixed, dilated pupil; ophthalmoparesis
  • In infants, increased intracranial pressure may result in a bulging fontanelle, splayed sutures, and increasing head circumference.
  • Low-grade fever
  • Meningeal signs
  • In setting of trauma:Leakage of CSF from the ear or noseBattle sign: bruising over the mastoid process suggestive of basilar skull fractureRaccoon eyes: periorbital ecchymosis suggestive of basilar skull fractureRetinal hemorrhages
  • Leakage of CSF from the ear or nose
  • Battle sign: bruising over the mastoid process suggestive of basilar skull fracture
  • Raccoon eyes: periorbital ecchymosis suggestive of basilar skull fracture
  • Retinal hemorrhages

Diagnostic Tests & Interpretation

  • CBC
  • Metabolic profile
  • INR, prothrombin time, activated partial thromboplastin time (PT/aPTT)
  • Fibrinogen
  • Factors VIII, IX, XI
  • Von Willebrand factor antigen
  • Urine toxicology screen
  • Electrocardiogram (for evaluation of cardiac disease or endocarditis)
  • Lumbar puncture: Lumbar puncture will show RBCs, reduced glucose, and xanthochromia. Consider spinal fluid evaluation if CT negative.
  • Rapid CT of the headThe most important study to obtain when considering ICH in the differential diagnosis because of its relative convenience, speed, and low false-negative rateAcute intracerebral blood demonstrates increased density; between 1 and 6 weeks becomes isodense with adjacent brain parenchyma. Acute ICH may appear isodense if hemoglobin <8-10 g/dL.Epidural hemorrhage: biconvex, lens-shaped hemorrhage, displacing gray-white matterSubdural hemorrhage: crescent-shaped hemorrhage; bilateral subdural hemorrhage frequent in nonaccidental injury
  • Contrast-enhanced CT: Contrast extravasation within the hematoma may identify patients at high risk of ICH expansion.
  • MRI gradient echo and T2 susceptibility-weighted imaging are helpful for acute and remote hemorrhage.
  • The most important study to obtain when considering ICH in the differential diagnosis because of its relative convenience, speed, and low false-negative rate
  • Acute intracerebral blood demonstrates increased density; between 1 and 6 weeks becomes isodense with adjacent brain parenchyma. Acute ICH may appear isodense if hemoglobin <8-10 g/dL.
  • Epidural hemorrhage: biconvex, lens-shaped hemorrhage, displacing gray-white matter
  • Subdural hemorrhage: crescent-shaped hemorrhage; bilateral subdural hemorrhage frequent in nonaccidental injury
  • CT/MRI/conventional cerebral angiography and venography: for evaluation of vascular lesions, including congenital vascular anomalies, vasculopathies, venous sinus thrombosis
  • Head ultrasound: Intraventricular hemorrhage in infants warrants serial head ultrasound exams to rule out hydrocephalus.

Differential Diagnosis

  • Ischemic stroke/transient ischemic attack
  • Brain tumor
  • Headache (migraine, primary thunderclap)
  • Metabolic derangements (hyper/hyponatremia, hypoglycemia)
  • Encephalitis
  • Meningitis
  • Seizure with post-ictal Todd paralysis

Treatment

Medication

  • Correction of coagulopathies (suggested by PT, PTT, platelet abnormalities) should be done promptly and may require hematology evaluation. Therapies include vitamin K, fresh frozen plasma, cryoprecipitate, or platelet infusion. Monitor for fluid overload, especially in patients with cardiac disease.
  • Management of elevated blood pressure: Blood pressure should be maintained within normal parameters for age with a target of 50th-95th percentile according to age and height. Nicardipine drip (1 Ојcg/kg/min) or labetalol (0.2 mg/kg IV push over 2-3 minutes, repeat every 15 minutes PRN)
  • Management of seizures: fosphenytoin or levetiracetam 20 mg/kg IV load + maintenance for clinical or electrographic seizures. No evidence for seizure prophylaxis in ICH.
  • Acyclovir therapy if herpes simplex type 1 encephalitis is considered
  • Recombinant factor VIIa is FDA-approved in children with hemophilia who have systemic bleeding and are resistant to factor VIII therapy.
  • Corticosteroids NOT recommended; may result in harmful hyperglycemia

Additional Treatment

  • Temperature management: maintain normothermia. Fever associated with worse neurologic outcome in adults
  • Maintain normoglycemia and euvolemia
  • Urgent neurosurgical consultation
  • Management of elevated ICP: head of bed elevated to 30 degrees, maintain adequate analgesia and sedation, consider invasive ICP monitoring and aggressive therapies (mannitol, hypertonic saline, hyperventilation) in consultation with neurosurgery and PICU
  • Consider continuous EEG monitoring for seizures.
  • Elevated intracranial pressure may necessitate surgical hematoma evacuation or decompressive hemicraniectomy.
  • Aneurysms or AVMs may require neurosurgical or neurointerventional treatment.
  • Admission to pediatric/neurointensive care unit
  • Urgent management of coagulopathy, hypertension, seizures, elevated ICP as listed earlier
  • Patients with altered mental status to monitor for elevated ICP
  • Patients with focal neurologic deficits for workup and rehabilitation
  • Patients with seizures for continuous EEG monitoring and anticonvulsant management
  • Patients with hypertension for management
  • Patients with coagulopathy for correction
  • Patients requiring neurosurgical or neurointerventional management

Ongoing Care

Follow-up Recommendations

Prognosis

  • Children may develop long-term focal or cognitive deficits or seizures.
  • Often, good neurologic recovery is possible.
  • Predictors of poor neurologic outcome: infratentorial location, GCS ≤7 at admission, aneurysm, age <3 years, underlying hematologic disorder

Complications

  • Increased intracranial pressure and brain herniation syndromes
  • Hydrocephalus
  • Vasospasm secondary to blood and breakdown products of erythrocytes
  • Seizures
  • Motor, visual, and cognitive deficits
  • Death (5-54%, pooled data 25%)

Additional Reading

  • Jordan пїЅLC, Hillis пїЅAE. Hemorrhagic stroke in children. Pediat Neurology. 2007;36(2):73-80. пїЅ[View Abstract]
  • Lo пїЅWD, Lee пїЅJ, Rusin пїЅJ, et al. Intracranial hemorrhage in children. An evolving spectrum. Arch Neurol. 2008;65(12):1629-1633. пїЅ[View Abstract]
  • Proust пїЅF, Toussaint пїЅP, Garni пїЅri пїЅJ, et al. Pediatric cerebral aneurysms. J Neurosurg. 2001;94(5):733-739. пїЅ[View Abstract]
  • Roach пїЅES, Golomb пїЅMR, Adams пїЅR, et al. Management of stroke in infants and children: a scientific statement from a special writing group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke. 2008;39(9):2644-2691. пїЅ[View Abstract]
  • Squier пїЅW. Shaken baby syndrome: the quest for evidence. Dev Med Child Neurol. 2008;50(1):10-14. пїЅ[View Abstract]

Codes

ICD09

  • 432.9 Unspecified intracranial hemorrhage
  • 853.00 Other and unspecified intracranial hemorrhage following injury without mention of open intracranial wound, unspecified state of consciousness
  • 747.81 Anomalies of cerebrovascular system
  • 852.40 Extradural hemorrhage following injury without mention of open intracranial wound, unspecified state of consciousness
  • 286.9 Other and unspecified coagulation defects

ICD10

  • I62.9 Nontraumatic intracranial hemorrhage, unspecified
  • S06.300A Unsp focal TBI w/o loss of consciousness, init
  • Q28.2 Arteriovenous malformation of cerebral vessels
  • S06.4X0A Epidural hemorrhage w/o loss of consciousness, init encntr
  • D68.9 Coagulation defect, unspecified

SNOMED

  • 1386000 Intracranial hemorrhage (disorder)
  • 450410005 intracranial hemorrhage following injury (disorder)
  • 234142008 Cerebral arteriovenous malformation (disorder)
  • 43216008 Extradural hemorrhage following injury without open intracranial wound (disorder)
  • 64779008 Blood coagulation disorder (disorder)

FAQ

  • Q: What is the annual risk of hemorrhage in children with known AVM?
  • A: The estimated annual hemorrhagic risk is 2-4%. In 25% of patients, the hemorrhage is fatal.
  • Q: How often should an asymptomatic child at risk for AVM be screened, and with what imaging modality?
  • A: Depending on the risk of aneurysm in a given condition, screening with MRA every 1-5 years is reasonable.