Pediatrics

Hemorrhagic stroke

OVERVIEW: What every practitioner needs to know

Are you sure your patient has hemorrhagic stroke? What are the typical findings for this disease?

Hemorrhagic stroke refers to spontaneous (nontraumatic) intracranial hemorrhage (ICH) of several types, including intracerebral or parenchymal hemorrhage (ICH), intraventricular hemorrhage, and subarachnoid hemorrhage (SAH).

The most common symptoms, usually occurring together with sudden onset and rapid progression are headache, depressed mental status, and focal neurologic deficit, and in severe cases rapid progression to coma or herniation syndromes.

Additional common associated symptoms include vomiting, seizures, meningismus, and fever (with SAH).

What other disease/condition shares some of these symptoms?

Diseases that mimic ICH are numerous and diverse and cover the following broad categories of neurologic disease:

Trauma: contusion, concussion, subdural hematoma, epidural hematoma

Malignancy: solid tumor, carcinomatous meningitis

Infection: meningitis, encephalitis, abscess

Rheumatologic: systemic lupus erythematosus, Behçet disease

Cerebrospinal fluid disorders: hydrocephalus, pseudotumor cerebri

Vascular: cerebral venous thrombosis, hypertensive encephalopathy, primary central nervous system vasculitis

Toxic, metabolic: hyperammonemia, hypernatremia

Migraine: hemiplegic migraine, acute confusional migraine, ophthalmoplegic migraine

Demyelinating: acute disseminated encephalomyelitis

What caused this disease to develop at this time?

Vascular malformations account for the vast majority of cases, are of several types, and may coexist in the same patient:

Arteriovenous malformations (AVMs)

Aneurysm—may be isolated or associated with other vasculopathy such as sickle vasculopathy or systemic/genetic arteriopathies or arteritides

Cerebral cavernoma

Vein of Galen malformation

Hereditary hemorrhagic telangiectasia (HHT)

Incontinentia pigmenti–cerebral hemorrhage when it occurs in this disease usually presents in the newborn period

Hemorrhagic venous infarction is most typical of cerebral venous thrombosis

Mycotic aneurysm from infective endocarditis

Tumor embolism, especially cardiac myxoma

Bleeding diathesis, including neonatal vitamin K deficiency, hemophilia, liver failure, consumptive coagulapathy, iatrogenic, thrombocytopenia

Malignancy—hemorrhagic degeneration of tumor

Postradiation necrosis

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

Basic hematologic profile for all cases:

  • Complete blood count and platelet count

  • Coagulation profile, including prothrombin time/international normalized ratio and partial thromboplastin time

Selected additional studies depending on history and examination, and suspicion of other organ dysfunction or underlying chronic disease:

  • Comprehensive metabolic panel if concern for liver or kidney disease

  • Screening rheumatologic panel (erythrocyte sedimentation rate, lupus panel) if inflammatory disease suspected

  • Blood cultures if mycotic aneurysm suspected

  • Genetic diagnostic studies if specific genetically determined vascular syndromes suspected (e.g., familial cerebral cavernoma, Alagille syndrome, HHT)

Would imaging studies be helpful? If so, which ones?

Computed tomography (CT) of the head: Start with this, as it is rapid, widely available, and provides sensitive and specific diagnostic confirmation of the presence of hemorrhage. This is the necessary first step in surgical triage and identifies hydrocephalus, which is a common acute complication of ICH. Desire to avoid radiation should not stand in the way of obtaining CT of the head urgently. This is the best and quickest way to identify possible neurosurgical emergency.

Magnetic resonance imaging (MRI) of the brain: After initial diagnosis of ICH on CT of the head, MRI is commonly the next best study to sensitively and specifically characterize the cause of the hemorrhage and the extent and nature of parenchymal injury. For example, cerebral cavernoma is best diagnosed with MRI and is not diagnosed by other vascular imaging modalities. Advanced susceptibility-weighted sequences best identify multiple small foci of hemorrhage, an important clue to cardioembolic etiology, and will diagnose cerebral venous thrombosis as the cause of hemorrhage.

Vascular imaging: This modality is noninvasive and may be incorporated with MRI in the form of MR angiography (MRA), or with CT (CT angiography [CTA]), as a prelude to catheter angiography and to help plan the timing and approach to surgical intervention. MRI and CTA are insensitive to small aneurysms (<3 mm) and will miss small AVMs. MRA is often preferred over CTA to avoid radiation exposure and because MRI is often obtained for many other reasons.

Catheter angiography: This imaging modality is needed for surgical planning and for definitive diagnosis of suspected AVMs and aneurysm. It should be performed in all cases of ICH not attributable to bleeding diathesis in which MRI/MRA fails to identify an underlying vascular anomaly. Timing of angiography is usually a surgical decision and is planned in coordination with surgeon, intensivist, and interventional radiologist. Repeated angiography may be needed at 6-12 weeks for SAH if initial study was normal.

Cranial ultrasonography: In newborns, cranial ultrasonography is useful only as a screening modality, with the advantages of being a bedside technique. It is insufficient for definitive diagnosis and surgical planning.

Transcranial Doppler: This is sometimes obtained as a baseline study to follow for signs/symptoms of vasospasm in cases of aneurysmal SAH. This technique for this indication is not widely used in pediatric hospitals and is difficult technically in small children, in whom normative data is often lacking.

If you are able to confirm that the patient has hemorrhagic stroke, what treatment should be initiated?

Acute treatment, supportive

Airway, Breathing, Circulation—in patients with depressed mental status who are rapidly deteriorating or have herniation syndrome, it is necessary to secure the airway and control ventilation; ensure adequate vascular access, and support perfusion/oxygenation with fluids and pressors as needed.

Fluids, metabolic—maintain normoglycemia and normovolemia, ensure normal acid-base balance and electrolyte status

Hematologic—normalize coagulation status with blood products or fresh frozen plasma as needed; transfuse to normalize hematocrit if needed

Thermoregulation—prevent and aggressively treat hyperthermia

Anticonvulsant therapy—treat seizures aggressively to prevent the risk of worsening intracranial hypertension or precipitating rebleeding. Continuous bedside electroencephalographic (EEG) monitoring may be required in the obtunded or comatose patient or if the patient is treated with neuromuscular blockade. The use of anticonvulsant agents prophylactically in the absence of any clinical or electrographic seizures is controversial, is not proved to be beneficial in children, and is associated with poorer outcome in adults with ICH.

Aspiration precautions—nothing by mouth pending dysphagia screen

Deep vein thrombosis precautions

Pain management

Acute treatment, definitive, brain-directed

Coordinated between intensivist and neurosurgeon, and neurointerventional radiologist

Control intracranial hypertension—one or more strategies may be needed, including external ventricular drainage, hematoma evacuation, hemicraniectomy, hyperosmolar therapy (3% saline)

In SAH, surveillance for vasospasm is needed (careful clinical examinations, possibly transcranial Doppler), and prophylaxis with a calcium channel blocker (nimodipine) for 14-21 days is favored by many neurosurgeons

Surgical and/or endovascular treatment of vascular anomaly—aneurysm clipping or embolization, AVM resection or embolization, cavernoma resection

Most vein of Galen malformations are treated with embolization

Nonoperable lesions may be amenable to radiation therapy—gamma knife or proton beam

After acute treatment: start rehabilitation therapies in the intensive care unit as soon as possible and facilitate transfer to inpatient rehabilitation if indicated as soon as medical status allows.

Longer=term treatment

AVMs may recur/regrow and are associated with significant risk of recurrent hemorrhage. Follow-up catheter angiography is usually necessary at regular intervals through age 18 years. MRA and CTA are inadequate to detect small AVMs.

Anticonvulsant treatment: published data are insufficient to guide decisions on duration of treatment with anticonvulsant medication. One option is to treat for 3-6 months, allowing for completion of initial surgical intervention and rehabilitation. Factors affecting this decision include presence of residual or untreated AVM or aneurysm, side effects of medication, and presence of significant epileptiform changes on EEG.

Strong psychosocial support for patient and family are necessary, ideally linked to community-based long-term rehabilitation services and individually tailored school reentry programming.

What are the adverse effects associated with each treatment option?

Options for definitive treatment of underlying vascular anomalies that cause ICH each have risks versus benefits.

Surgical resection of AVM or aneurysm may be curative and lifesaving by preventing rebleeding, but have risks due to collateral damage to normal vessels and adjacent brain. Estimated risk of recurrent hemorrhage from untreated AVM or aneurysm is 4%/year. Some AVMs are located in regions that are not accessible without causing major neurologic injury.

Embolization for AVM or aneurysm has the advantage of being less invasive and thus less likely to cause damage to normal adjacent brain when compared with surgery. It does still put normal vessels at risk, and it has some risk of rupture and hemorrhagic complications. It is rarely curative when used alone for AVM.

Radiation treatments for AVMs have the advantage of causing less damage to adjacent normal brain when compared with surgery. They can be curative but are less likely to completely cure lesions and require 12-18 months to accomplish the intended benefit. Many patients experience radiation toxicity.

What are the possible outcomes of hemorrhagic stroke?

Outcomes after ICH vary widely. Cohort studies report outcomes as follows:

Mortality: case fatality rates range from 7%-50%, with a median of about 25%, in studies dating from the 1970s-2004. In the most recent cohort studies with aggressive surgical management, mortality is at the lower end of this range.

Neurologic status of survivors: survival with "good" outcome is reported in 30%-50% of patients. In the most recent studies, residual impairments are mostly in the mild to moderate range.

Additional long-term morbidities include chronic headache and epilepsy, although rates of these problems are not well characterized.

Factors associated with greater risk of poor outcome include large volume of hemorrhage (>2% of total brain volume) and depressed mental status on presentation.

What causes this disease and how frequent is it?

Estimated incidence of primary ICH is 1-2/100,000/year in North America

ICH accounts for approximately half of all cases of childhood stroke

Neonates account for about 20%-30% of all cases

Boys are affected more frequently than girls, 60% compared with 40%

Underlying causes include AVM 40%, coagulopathies 20%, cavernoma 10%, aneurysm 10%, other 20%

Genetic syndromes or known mutations contribute to a minority of cases, including the following entities:

Coagulopathies—hemophilia

Autosomal dominant cerebral cavernomatous malformation (CCM)

HHT

Alagille syndrome

Microcephalic primordial dwarfism with cerebral arteriopathy/aneurysms

Hereditary angiopathy with nephropathy, aneurysm, and muscle cramps

How can this disease be prevented?

There are no proven or widely accepted means to screen and treat prophylactically for conditions that predispose to ICH. Most cases of AVM and aneurysm are isolated and nonsyndromic and are asymptomatic until they present with hemorrhage. In patients with known genetic syndromes associated with aneurysms, AVMs, or CCMs, there is no consensus or proven strategy to perform surveillance imaging or presymptomatic surgical or endovascular therapy. Such decisions are made on a case-by-case basis under the direction of individual practitioners.

What is the evidence?

There have been no case control studies or clinical trials of interventions for the treatment of ICH in children. Existing evidence on epidemiology, risk factors, clinical presentation, treatment, and outcome is found in small case series, retrospective single-center cohort studies, and a few prospective cohort studies. Selected references are given below.

Roach, ES, Golomb, MR, Adams, R. "American Heart Association Stroke Council; Council on Cardiovascular Disease in the Young. 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. vol. 39. 2008. pp. 2644-91.

Jordan, LC, Hillis, AE. "Hemorrhagic stroke in children". Pediatr Neurol. vol. 36. 2007. pp. 73-80.

Jordan, LC, Johnston, SC, Wu, YW. "The importance of cerebral aneurysms in childhood hemorrhagic stroke: a population-based study". Stroke. vol. 40. 2009. pp. 400-5.

Beslow, LA, Licht, DJ, Smith, SE. "Predictors of outcome in childhood intracerebral hemorrhage: a prospective consecutive cohort study". Stroke. vol. 41. 2010. pp. 313-18.

Lo, WD, Lee, J, Rusin, J. "Intracranial hemorrhage in children: an evolving spectrum". Arch Neurol. vol. 65. 2008. pp. 1629-33.

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