The first comprehensive guidelines for the diagnosis and management of stroke in children are intended to provide a wide range of clinicians responsible for treating cerebrovascular disease in infants and children with evidence- and consensus-based recommendations, according to the American Heart Association.
The recommendations, written by a group of experts from the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young, have been released online.
“Only a few centers in the country have a high level of expertise in dealing with stroke in children, and these guidelines share this concentrated knowledge with physicians who don't have access to that expertise,” committee chair Dr. E. Steve Roach said in an interview.
One important message in the statement is that stroke in children is far more common than is generally realized. Data from the National Hospital Discharge Survey from 1980 to 1998 suggested that the overall risk of stroke from birth through 18 years is 13.5/100,000, and the rate of hemorrhagic stroke for term infants is 6.7/100,000 per year. Other recent investigations found that neonatal stroke occurs in about 1 in 4,000 live births, with about of those 80% being ischemic.
“Strokes actually are twice as common as brain tumors in children,” said Dr. Roach, chief of neurology at Nationwide Children's Hospital and professor of pediatric neurology, Ohio State University, both in Columbus.
Strokes in children differ from those in adults in that few are associated with atherosclerosis, but they are similar in that once the stroke has occurred, no medicine can reverse it, Dr. Roach said. “However, an aggressive approach to finding out the cause of the stroke is your best chance for preventing [subsequent] strokes and the cumulative pileup of brain damage that will determine whether that child grows into a normally functioning adult.”
Among the causes and risk factors for stroke cited in the statement are sickle cell disease and cervicocephalic arterial dissection (Circulation 2008 [doi:10.1161/strokeaha.108.189696
The guidelines offer detailed recommendations on primary and secondary stroke prevention in sickle cell disease. Management of acute ischemic stroke should include optimal hydration and correction of hypoxemia and hypotension. Periodic transfusions are recommended for children aged 2-16 years with abnormal transcranial Doppler findings, and those with a confirmed cerebral infarction should be on a program of red cell transfusion with measures to prevent iron overload.
In sickle cell disease with acute cerebral infarction, exchange transfusion with the goal of reducing sickle hemoglobin to less than 30% of total hemoglobin is “reasonable,” and hydroxyurea may be considered for children who are unable to continue on long-term transfusion.
Cervicocephalic arterial dissection is described as an important but underrecognized cause of stroke in children. For extracranial cervicocephalic arterial dissection, it is reasonable to institute unfractionated heparin or low-molecular-weight heparin as a bridge to oral anticoagulation. Anticoagulant therapy can continue for 3-6 months or longer for patients with recurrent symptoms, according to the guidelines.
For hemorrhagic stroke, recommendations include noninvasive testing and standard cerebral angiography if needed, along with stabilizing measures such as controlling hypertension and seizures and managing increased intracranial pressure. Surgical evacuation of a supratentorial intracerebral hematoma is not usually recommended, although in certain selected patients with developing brain herniation or very high intracranial pressure, surgery may be helpful.
With cerebral venous sinus thrombosis (CVST) in children, anticoagulation is reasonable, with the exception of neonates. “Until there is more evidence of safety and effectiveness, anticoagulation is not appropriate for most neonates with CVST,” the authors wrote, adding that it may be considered in the context of severe prothrombotic disorders, multiple emboli, or radiologic evidence of propagating CVST despite supportive care.
Some recommendations, such as those that suggest using anticoagulation only for neonates with some evidence of progression of venous sinus thrombosis, are likely to cause controversy, said Dr. Heather J. Fullerton, who directs the pediatric stroke and cerebrovascular disease center at the University of California, San Francisco.
Nonetheless, “these are landmark comprehensive guidelines” for clinicians who have “struggled with how to manage these patients in the absence of more evidence,” said Dr. Fullerton, who was not a member of the writing group. The guidelines are at www.americanheart.org/presenter.jhtml?identifier=3003999