Case:

A 6-year-old female with a past medical history of immune thrombocytopenia presents to the Emergency Department (ED) for concerns of dysarthria that started the day prior to arrival. The patient’s mother denies any recent trauma, including head injury.

Vitals and Physical Exam

• Blood pressure 109/80
• Pulse 121 beats/minute
• Respiratory rate 22 breaths/minute
• Oxygen saturation 100% on room air
• Temperature 36.8ºC

Her physical exam is remarkable for a mild right-sided facial droop with forehead sparing and dysarthria.

Initial Work-Up

The patient’s ED workup shows the following:

• Point-of-care glucose: Normal
• Platelet count: 0 platelets/liter
• Hemoglobin: 9.8 g/dL
• Head CT: Frontal lobe hemorrhage

Background

Although rare, pediatric intracranial hemorrhage (ICH) contributes to almost half of all childhood strokes and can cause lifelong disability and death [1]. One 3-center prospective study on pediatric ICH noted a 9% mortality rate with ⅓ of survivors having “significant disability” at 2-year follow-up [2]. Primary predictors of adverse outcomes from pediatric ICH involve the following [2-4]:

• Hemorrhagic lesion volume
• Presence of hydrocephalus and/or herniation
• Altered mental status

Multiple studies consistently point to vascular causes such as arteriovenous malformation as a leading risk factor for spontaneous pediatric ICH followed by hematological pathologies including coagulation deficiencies [5-7].  No matter the cause, the sequelae of pediatric ICH can be devastating making early detection and immediate intervention essential for better outcomes. Unfortunately, given children often present with vague and non-specific symptoms, there is often a delay in presentation to care and in diagnosis [8]. Unfortunately, in contrast to adults, there are no set guidelines for the management of pediatric ICH despite its associated morbidity and mortality.

Clinical Findings

Although headache is the most common presenting symptom, other symptoms can vary [6,8,9]. In one study, children <6 years old were more likely to present with symptoms such as seizures and altered mental status, while children ≥6 years presented more with focal deficits, headache, vomiting, and altered mental status [9].

Table 1. Incidence rates of common symptoms and findings in pediatric patients with a spontaneous intracranial hemorrhage (adapted from Boulouis G, et al [7].) 

Differential Diagnosis

Given how rare pediatric ICH is, consider other diagnoses when a patient presents with focal deficits, altered mental status, and/or vague symptoms such as headache and weakness.

1. Bell’s palsy
2. Cerebral venous thrombosis
3. Complicated migraines
4. Drug intoxication/exposure
5. Inborn error of metabolism
6. Intracranial mass
7. Ischemic stroke
8. Metabolic derangements (hypoglycemia, hyponatremia)
9. Non-accidental trauma
10. Posterior Reversible Encephalopathy Syndrome (PRES)
11. Seizures with Todd’s paralysis

Approach for the ED Provider

Key history questions:

1. When did the symptoms start?
2. Does the child or anyone in the family have any history of bleeding disorder?
3. Have you noticed excessive bruising from minimal trauma?
4. Has the child had any recent illnesses?

Key physical exam findings:

1. Is there any bruising, gum bleeding, or signs of non-accidental trauma?
2. In infants, is the fontanelle bulging or flat?
3. Are there any focal neurologic findings such as facial droop, pupil asymmetry, etc?
4. Are there any signs of increased intracranial pressure (i.e., papilledema)?

Workup to initiate:

Emergency medicine physicians should have strong suspicion for ICH particularly in the setting of a pediatric patient presenting with acute onset of headache, vomiting, altered mental status, seizure, and/or focal deficits.

1. Emergent neuroimaging: CT or MRI is essential in order to distinguish between ischemic versus hemorrhagic causes. CT is often the first imaging study completed due to ease of access. If no acute intracranial process is noted, MRI is warranted to evaluate for ischemic stroke or other etiology.
2. Laboratory studies:
   • Point-of-care glucose
   • Comprehensive metabolic panel
   • Ammonia (if concerned for inborn error of metabolism)
   • Comprehensive blood count
   • Prothrombin time with INR, partial thromboplastin time
   • Urine drug screen (if concerned for drug exposure contributing to symptoms)

Management:

If a patient has a confirmed ICH, consultation with neurosurgery is required. Immediate transfer may be necessary if your facility does not have neurosurgical services. Further management includes:

1. Reversing coagulopathy [7,10,11]:
   • If the patient has an underlying coagulopathy, consider intravenous vitamin K and/or fresh frozen plasma.
   • Pediatric patients with hemophilia require immediate factor replacement (factor VIII or IX).
   • Patients on anticoagulation need anticoagulation reversal with the appropriate reversal agents.
2. Neuroprotective supportive measures (prevent worsening brain injury): 
   • Monitor the patient closely with frequent neurologic checks for any signs of deterioration.
   • Maintain euglycemia as hyperglycemia is associated with worse outcomes [7].
   • Maintain normothermia. Use external cooling measures or antipyretics to manage hyperthermia [10].
   • Treat clinical and subclinical seizures with antiepileptics. Consider EEG monitoring to detect subclinical status [7].  The benefits of prophylactic administration of antiepileptics is unknown in this population [10].
   • Avoid hypotension [7, 10]. There are no established guidelines for hypertension management in pediatric ICH; blood pressure goals should be discussed with the neurosurgical team and blood pressure variability should be avoided.
3. Treatment of increased intracranial pressure:  If the patient has a change in mental status or develops focal deficits, an increase in intracranial pressure should be suspected [10,11].
   • Treat hypotension, hypercapnia, and hypoxia.
   • Elevate the head of the bed to 30 degrees.
   • Ensure appropriate pain control.
   • Sedation may be necessary but be wary of resultant hypercapnia and consider intubation if patients require a lot of sedation or become too somnolent following medication.
   • In patients with acute deterioration or concern about impending herniation, consider hyperventilation if the patient is intubated and/or treatment with a hyperosmolar agent like mannitol or hypertonic saline.
   • Some patients may need acute interventions such as an external ventricular drain or operative decompression with clot removal.
   • Steroids have not been shown to be beneficial [10].

Case Resolution

The patient was transferred to a tertiary care center. Further imaging confirmed an intraparenchymal hemorrhage in the left frontal lobe and right parietal lobe with midline shift. No underlying lesions or vascular malformation were seen.

Management: The patient was admitted to intensive care and received tranexamic acid and a platelet transfusion. She was monitored by neurosurgery but no surgical interventions were needed. For her idiopathic thrombocytopenia, she received steroids and IV immunoglobulin.

Hospital Course: Her deficits and platelet count improved during her stay, and she was discharged on hospital day 5 with outpatient neurology and hematology follow-up.

Outpatient: Repeat imaging 3 weeks after discharge showed resolution of the midline shift and decrease in hemorrhage size.

Pearls

• Consider pediatric ICH in patients presenting with focal deficits, altered mental status, and/or generalized symptoms such as headache, seizures, and weakness.
• Management of pediatric ICH is focused on maintaining physiological homeostasis and preventing further brain injury.
• Call your neurosurgical team early for consultation and evaluation or transfer your patient to the appropriate tertiary care center.

Read more pediatric EM blog posts in the PEM Pearls series.

References

1. Baldovsky MD, Okada PJ. Pediatric stroke in the emergency department. J Am Coll Emerg Physicians Open. 2020;1(6):1578-1586. Published 2020 Oct 6. doi:10.1002/emp2.12275. PMID: 33392566
2. Porcari GS, Beslow LA, Ichord RN, Licht DJ, Kleinman JT, Jordan LC. Neurologic Outcome Predictors in Pediatric Intracerebral Hemorrhage: A Prospective Study. Stroke. 2018;49(7):1755-1758. doi:10.1161/STROKEAHA.118.021845 PMID: 29895534
3. Guédon A, Blauwblomme T, Boulouis G, et al. Predictors of Outcome in Patients with Pediatric Intracerebral Hemorrhage: Development and Validation of a Modified Score. Radiology. 2018;286(2):651-658. doi:10.1148/radiol.2017170152 PMID:29023219
4. Jordan LC, Kleinman JT, Hillis AE. Intracerebral hemorrhage volume predicts poor neurologic outcome in children. Stroke. 2009;40(5):1666-1671. doi:10.1161/STROKEAHA.108.541383 PMID: 19286576
5. Ciochon UM, Bindslev JBB, Hoei-Hansen CE, et al. Causes and Risk Factors of Pediatric Spontaneous Intracranial Hemorrhage-A Systematic Review. Diagnostics (Basel). 2022;12(6):1459. Published 2022 Jun 13. doi:10.3390/diagnostics12061459 PMID: 35741269
6. Al-Jarallah A, Al-Rifai MT, Riela AR, Roach ES. Nontraumatic brain hemorrhage in children: etiology and presentation. J Child Neurol. 2000;15(5):284-289. doi:10.1177/088307380001500503 PMID: 10830193
7. Boulouis G, Blauwblomme T, Hak JF, et al. Nontraumatic Pediatric Intracerebral Hemorrhage. Stroke. 2019;50(12):3654-3661. doi:10.1161/STROKEAHA.119.025783 PMID: 31637968
8. Yock-Corrales A, Mackay MT, Mosley I, Maixner W, Babl FE. Acute childhood arterial ischemic and hemorrhagic stroke in the emergency department. Ann Emerg Med. 2011; 58:156–163. doi: 10.1016/j.annemergmed.2010.10.013 PMID: 21310508
9. Lo WD, Lee J, Rusin J, Perkins E, Roach ES. Intracranial Hemorrhage in Children: An Evolving Spectrum. Arch Neurol. 2008;65(12):1629–1633. doi:10.1001/archneurol.2008.502 PMID: 19064750
10. Ferriero DM, Fullerton HJ, Bernard T, et al. Management of stroke in neonates and children. A scientific statement from the American Heart Association/American Stroke Association. Stroke. 2019;50:e51-e96. doi: 10.1161/STR.0000000000000183 PMID: 30686119
11. Tsze D and Steele D. Neurosurgical Emergencies, Nontraumatic. In: Fleisher G and Ludwig S,. eds. Textbook of Pediatric Emergency Medicine, 6e. Lippincott Willimas and Wilkins. 2010. Accessed online 5/23/2024.