Guidelines Issued for Prevention and Treatment of Stroke in Children

July 18, 2008 — The American Heart Association (AHA) Stroke Council has issued guidelines for prevention and treatment of stroke in children. The new recommendations are reported in the July 17 Online First issue of Stroke and also will be in the September print issue of Circulation.

“The purpose of this statement is to review the literature on childhood stroke and to provide recommendations for optimal diagnosis and treatment,” write E. Steve Roach, MD, from Ohio State University School of Medicine, and colleagues from the Special Writing Group of the AHA Stroke Council and the Council on Cardiovascular Disease in the Young. “This statement is intended for physicians who are responsible for diagnosing and treating infants, children, and adolescents with cerebrovascular disease.”

The AHA Stroke Council’s Scientific Statement Oversight Committee appointed a Writing Group panel including members with several different areas of expertise. The AHA Stroke Council’s Levels of Evidence grading was used to weight each of the panel’s recommendations.

The panel members, 4 expert peer reviewers, and members of the Stroke Council Leadership Committee reviewed the draft guidelines, and these were approved by the AHA Science Advisory and Coordinating Committee. The panel anticipates that the guidelines will require updating in 4 years.

The statement offers evidence-based recommendations to prevent ischemic stroke related to sickle cell disease (SCD), moyamoya disease, cervicocephalic arterial dissection, and cardiogenic embolism, as well as guidelines for evaluation and management of hemorrhagic stroke and dosing protocols for heparin and warfarin use in children. Recommendations on the evaluation and management of perinatal stroke and cerebral sinovenous thrombosis in children are also discussed.

“The treatment of stroke in infants and children is both important and understudied,” the authors write. “The issues of treatment of ischemic stroke involve both initial treatment of the acute stroke event to preserve neurological function and long-term efforts to prevent a second stroke, which occurs in 10% to 25% of children with stroke. For children with high-risk conditions such as [SCD] and congenital heart disease, efforts to prevent a first stroke also are important.”

Stroke in children should be evaluated with vascular imaging as soon as possible. In most patients, magnetic resonance angiography (MRA) is a reasonable alternative to conventional arteriography (CA), although the latter may be more accurate to image lesions of the distal arterial branches and lesions of the intracranial internal carotid artery (ICA). For vascular disorders, fat-saturated T1 imaging of the neck and/or venous imaging may improve the yield of MRA.

CA is probably justified for extracranial arterial dissections, particularly for those in the posterior circulation, and for small vessel vasculitis, which are difficult to exclude on MRA. The risk for recurrence of these conditions is high and the risk for CA is relatively low. In hemorrhagic and ischemic stroke, emergency vascular imaging should include magnetic venography (MRV) because 10% of hemorrhages in children are due to cerebral venous sinus thrombosis (CVST).

Depending on the clinical situation and setting, other imaging modalities that may be useful in evaluating children with cerebrovascular disease include cranial ultrasound, Doppler ultrasound; computed tomography, computed tomographic angiography, and computed tomographic perfusion; magnetic resonance imaging, magnetic resonance perfusion; and nuclear medicine.

Class I recommendations for children with SCD are as follows:

• Acute management of ischemic stroke from SCD should include optimal hydration and correction of hypoxemia and systemic hypotension (class I, level of evidence C).
• To lower stroke risk in children 2 to 16 years of age with abnormal transcranial Doppler (TCD), periodic transfusions to reduce the percentage of sickle hemoglobin are effective and are recommended (class I, level of evidence A).
• A regular program of red cell transfusion, along with measures to prevent iron overload, are indicated for children with SCD and confirmed cerebral infarction (class I, level of evidence B).
• Before performing CA in a patient with SCD, the percentage of sickle hemoglobin should be reduced with transfusions (class I, level of evidence C).

Class I recommendations for children with stroke and heart disease are as follows:

• Treatment for congestive heart failure is recommended and may lower the risk for cardiogenic embolism (level I, level of evidence C).
• To improve cardiac function and to lower subsequent risk for stroke, congenital heart lesions should be repaired when feasible, especially complex heart lesions with high risk for stroke (class I, level of evidence C). However, this recommendation does not yet apply to patent foramen ovale.
• In light of ongoing risk for cerebrovascular complications associated with atrial myxoma, resection is indicated (class I, level of evidence C).

Class I recommendations for evaluation and treatment of hemorrhagic stroke in children are as follows:

• To identify treatable risk factors before another hemorrhage occurs, children with nontraumatic brain hemorrhage should undergo a thorough risk factor evaluation, including standard cerebral angiography, when noninvasive tests have not been diagnostic (class I, level of evidence C).
• Appropriate factor replacement therapy is indicated for children with a severe coagulation factor deficiency. Factor replacement after trauma is indicated for children with less severe factor deficiency (class I, level of evidence A).
• Because congenital vascular anomalies are associated with risk for repeat hemorrhage, these lesions should be identified and corrected whenever it is clinically feasible, as should other treatable risk factors for hemorrhage (class I, level of evidence C).
• In children with brain hemorrhage, stabilizing measures should include optimization of respiratory effort, controlling systemic hypertension and epileptic seizures, and managing increased intracranial pressure (class I, level of evidence C).

“The incidence of stroke among children is low enough that it is difficult to plan clinical trials designed to improve therapy,” the authors conclude. “There are enough age-specific differences in the cause, manifestations, and treatment responses in individuals with stroke that we must be cautious when attempting to apply our knowledge of stroke in adults to children with stroke. Although large-scale clinical trials will be difficult to mount in children with stroke, continued research and additional experience are imperative if we are to better understand this important group of conditions.”

Some of the authors report various financial arrangements with BMS, Boehringer Ingelheim, Novartis, Sanofi-Synthelabo, Wyeth, Acuson, ATL, Nicolet, Abbott Laboratories, March of Dimes, National Institutes of Health, National Heart, Lung & Blood Institute, United Cerebral Palsy, Respironics, National Institutes of Neurological and Communicative Disorders and Stroke, and/or Stroke Association (UK).

Stroke. Published online July 17, 2008.

Circulation. 2008;117:000-000.

Learning Objectives for This Educational Activity

Upon completion of this activity, participants will be able to:

1. Describe the class I recommendations for children with sickle cell disease and for those with stroke and heart disease, according to the stroke guidelines.
2. Describe class I recommendations for evaluation and treatment of hemorrhagic stroke in children, according to the stroke guidelines.

Clinical Context

The diversity of underlying risk factors and the lack of a uniform treatment approach have complicated diagnosis and management of stroke in children, particularly since presentation is remarkably different from that in older patients. Stroke type also varies with age, with 55% of strokes in children ischemic and the remainder hemorrhagic.

The purpose of this AHA Stroke Council statement was to review the literature on stroke in children and to make recommendations for diagnosis and management whenever possible. The focus of these guidelines is on issues regarding treatment, with recommendations representing consensus opinion of the guidelines authors.

Study Highlights

• Children with stroke should have vascular imaging as soon as possible.
• MRA is a reasonable alternative to CA, except for imaging distal arterial branches and lesions of the intracranial ICA.
• Fat-saturated T1 imaging of the neck and/or venous imaging may improve the yield of MRA for vascular disorders.
• Extracranial arterial dissections (especially in the posterior circulation) and small vessel vasculitis are difficult to exclude on MRA. CA is probably justified when these conditions are suspected, because risk for recurrence of these conditions is high and the risk for CA is relatively low.
• In hemorrhagic and ischemic stroke, emergency vascular imaging should include MRV because 10% of hemorrhages in children are due to CVST.
• Other imaging modalities that may be useful in children with stroke include cranial ultrasound, Doppler ultrasound; computed tomography, computed tomographic angiography, and computed tomographic perfusion; MRI, MR perfusion; and nuclear medicine.
• Class I recommendations for children with SCD include:
  • Optimal hydration and correction of hypoxemia and systemic hypotension are indicated for acute management of ischemic stroke from SCD.
  • Children 2 to 16 years of age with abnormal TCD should have periodic transfusions to reduce the percentage of sickle hemoglobin.
  • Children with SCD and confirmed cerebral infarction should have regular red cell transfusion and measures to prevent iron overload.
  • The percentage of sickle hemoglobin should be reduced with transfusions before a patient with SCD undergoes CA.
• Class I recommendations for children with stroke and heart disease include:
  • Congestive heart failure should be treated, which may lower the risk for cardiogenic embolism.
  • Congenital heart lesions, especially complex heart lesions with high risk for stroke, but not patent foramen ovale, should be repaired when feasible to improve cardiac function and to lower subsequent risk for stroke.
  • Resection of atrial myxoma is indicated because of ongoing risk for cerebrovascular complications.
• Class I recommendations for evaluation and treatment of hemorrhagic stroke in children include:
  • Children with nontraumatic brain hemorrhage should be thoroughly evaluated (including CA) to identify treatable risk factors before another hemorrhage occurs, when noninvasive tests have not been diagnostic.
  • Children with severe coagulation factor deficiency should have appropriate factor replacement therapy, and those with less severe factor deficiency should have factor replacement after trauma.
  • Congenital vascular anomalies are associated with risk for repeat hemorrhage, and should therefore be identified and corrected whenever clinically feasible, as should other treatable risk factors for hemorrhage.
  • Stabilizing measures in children with brain hemorrhage should include optimizing respiratory effort, controlling systemic hypertension and epileptic seizures, and managing increased intracranial pressure.

Pearls for Practice

• Acute management of ischemic stroke from SCD should include optimal hydration and correction of hypoxemia and systemic hypotension. Periodic transfusions to reduce the percentage of sickle hemoglobin are recommended to lower stroke risk in children 2 to 16 years of age with abnormal TCD. For children with stroke and heart disease, treatment of congestive heart failure is recommended and may lower the risk for cardiogenic embolism. Congenital heart lesions should be repaired when feasible, especially complex heart lesions with high risk for stroke. Atrial myxoma should be resected.
• Children with nontraumatic brain hemorrhage should undergo a thorough risk factor evaluation, including standard CA, when noninvasive tests are not diagnostic. Stabilizing measures should include optimizing respiratory effort, controlling systemic hypertension and epileptic seizures, and managing increased intracranial pressure. Appropriate factor replacement therapy is indicated for children with a severe coagulation factor deficiency, and those with less severe factor deficiency should undergo factor replacement after trauma. Congenital vascular anomalies should be identified and corrected whenever clinically feasible, as should other treatable risk factors for hemorrhage.

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