- Email: [email protected]
Evaluation, Identification, and Management of Pediatric Strokes in the Emergency Department Using a Pathway Algorithm
CLINICAL
EVALUATION, IDENTIFICATION, AND MANAGEMENT OF PEDIATRIC STROKES IN THE EMERGENCY DEPARTMENT USING A PATHWAY ALGORITHM Authors: Victor Jerome Hicks Jr, MSN, APN, and Lisa M. Black, PhD, RN, CNE, Carson City and Reno, NV
Earn Up to 9.0 CE Hours. See page 211.
Clinical Scenario
On Christmas Day 2009, a 22-month-old male infant presented to a local children's emergency department accompanied by his mother. The patient's mother reported to the emergency staff that before their arrival to the emergency department that day, the patient seemed less steady on his feet and had fallen several times with no apparent reason. The mother reported that the patient had been taking oral amoxicillin for about 48 hours to treat acute otitis media. The mother stated that her son had also had acute influenza with a maximum temperature of 39.8°C (103.6°F) that was controlled with oral antipyretics. The child had no history of seizures or head trauma. Approximately 1 hour before ED presentation, the child was noted to be unable to use his arms to rise to a standing support position. When assisted to a standing position, the patient was noted to have significant weakness in the left lower extremity, left facial droop, and confusion. There was also noticeable muscle spasm to the left arm, hand, leg, knee, and foot. Initial ED hematology and chemistry studies were found to be within normal limits. Although the parents expressed concern that the diagnostic workup was incomplete, the child was discharged from the emergency department with a diagnosis of synovial joint Victor Jerome Hicks Jr is Family Nurse Practitioner CHNIII, State of Nevada Health Division, Carson City, NV. Lisa M. Black is Assistant Professor, Orvis School of Nursing, University of Nevada, Reno, NV. For correspondence, write: Victor Jerome Hicks Jr, MSN, APN, 1728 Back Country Ct, Reno, NV 89521; E-mail: [email protected]. J Emerg Nurs 2013;39:132-7. Available online 5 February 2013. 0099-1767/$36.00 Copyright © 2013 Published by Elsevier Inc. on behalf of Emergency Nurses Association. http://dx.doi.org/10.1016/j.jen.2012.11.013
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pain for which over-the-counter nonsteroidal anti-inflammatory drugs were prescribed. Five days later, the patient's symptoms had not resolved. The child was taken to see his primary pediatrician, who immediately noted persistent leftsided deficits. The next day, the patient underwent magnetic resonance imaging, which showed an acute arterial ischemic infarct. Later the same day, the patient underwent an echocardiogram to rule out cardiac etiology, which was negative. The patient was ultimately diagnosed with arterial vasculitis, which resulted in an ischemic multifocal infarct of the right basal ganglia. Problem
Childhood stroke is rare, with an estimated incidence of 13 in 100,000, 1 and misdiagnosis of ischemic events in children is common. 1-3 Lack of awareness about pediatric strokes within the health care community causes delays in quick evaluation and proper diagnosis, which limits treatment options. Delayed diagnosis leads to irreversible damage to cerebral tissues, greater long-term deficits, and other adverse sequelae. Together, these factors dramatically increase the resources required to treat adverse sequelae of childhood stroke. This article aims to increase awareness about the incidence and prevalence of pediatric stroke and to provide a uniform, evidence-based treatment algorithm for children who present with acute neurologic compromise. This treatment algorithm is intended to provide emergency nurses with increased confidence when caring for a child with a suspected cerebral infarct. Introduction and Background PATHOPHYSIOLOGY AND EPIDEMIOLOGY OF ADULT STROKE
As defined by the World Health Organization, stroke is a clinical disorder of rapidly developing centralized or
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comprehensive deterioration of brain function lasting longer than 24 hours with no indication of it originating from a cause outside the vascular system. 1,4-6 Stroke is classified into 2 major categories, ischemic or hemorrhagic, both of which decrease cerebral blood flow and increase the potential for cell death. Arterial ischemic stroke and cerebral venous thrombosis result in decreased or total blockage in venous blood flow to the brain. Intracerebral hemorrhage and nontraumatic subarachnoid hemorrhage cause a disruption in normal cerebral blood flow resulting in a decrease in oxygen-rich blood to brain cells. 7 Adult strokes account for the United States' third leading cause of death and is a leading cause of functional impairment. It is reported that nearly 800,000 Americans have a cerebral infarct each year, and stroke accounts for 1 of every 18 deaths in the United States. 8 With strokes having such high rates of morbidity and mortality, increased awareness and education on warning signs and the primary etiologies (ie, arrhythmias and arteriosclerotic arteriopathies) 9 have led to faster presentation times and improved end outcomes. This increased awareness about strokes has only begun to be realized in the field of pediatric neurology. EPIDEMIOLOGY OF PEDIATRIC STROKE
Pediatric stroke is more serious than once thought. As common as pediatric brain tumors, pediatric stroke ranks among the top 10 causes of death in children aged under 18 years. 7,10-12 With the recent advances in technology and increased awareness, it is likely that the incidence of pediatric strokes is higher than what has been documented. Consensus in the pediatric stroke literature on numbers of reported cases annually ranges between 2 and 3 per 100,000 children, with some literature reporting a stroke incidence as high as 13 per 100,000 children. 11-14 Given the complexity of etiologic presentations within the field of pediatric stroke, researchers have begun to classify pediatric stroke according to the age of the child. This allows for investigation into plausible originating sources and etiologies, whether from maternal fetal sources or from other non-maternal factors. Neonatal strokes are those that occur within the first month of life. 5 Neonatal strokes are of significant importance because disruption in the development of the brain's vascular system causes significant mental retardation and other neurologic sequelae. Ischemic or hemorrhagic events within the brain between 28 weeks of gestation and 28 days after birth are defined as perinatal strokes. 5,15 Strokes occurring from day 30 of life through the age of 18 years are classified as pediatric or childhood strokes. 13,15 Breaking down the ages in which pediatric strokes occur allows researchers to study various theories as
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to how and why strokes occur at specific points in the development process. PATHOPHYSIOLOGY OF PEDIATRIC STROKE
Difficulties arise in the identification and evaluation of pediatric stroke in that children often present with symptoms that differ from those seen in the adult population. 3 Risk factor variance, differing clinical presentations, and the absence of a standardized approach to evaluation and treatment, 5 coupled with differing etiologies from the adult stroke cohort, often result in misdiagnosis and mismanagement. 9 Thrombotic events account for approximately 85% of all stroke events witnessed in children. 14 Other pathologic findings are observed within the cerebral arteries in 80% of children with acute ischemic stroke (AIS). AIS in the pediatric population generally occurs between the ages of 1 and 5 years. 13 Traumatic head injuries make up most cases before age 1 and after age 5. Although a large percentage of stroke cases have underlying embolic causes related to cardiac malformations, many root causes are unknown. No preexisting or identifiable risk factors can be seen in 10% to 30% of pediatric stroke victims. 7 Common illnesses with stroke-like mimickers including migraine, encephalitis, tumors, and postictal paralysis are often linked to neurologic deficits seen in children, 16,17 which convolutes appropriate management. Seizures and altered level of consciousness are relatively frequent in pediatric stroke patients, particularly in neonates and children aged under 4 years. 12,16,17 This increased prevalence of seizure activity in pediatric stroke points to the occurrence of seizures as a presenting sign that is unique to the pediatric stroke population. Current or recent infection with the varicella virus has been shown to increase the risk of a child having a stroke. Miravet et al 18 found that one-third of the children in their study cohort who had an AIS were currently infected with or had recently recovered from varicella. Likewise, Lynch et al 15 noted that among children aged between 6 months and 10 years who were diagnosed with a stroke, up to 30% had been infected with the varicella virus within the previous year. Thus a thorough history and evaluation of possible stroke etiologies are essential in children presenting with acute neurologic compromise. PEDIATRIC STROKE AWARENESS
Lack of pediatric stroke awareness has led to delayed medical evaluation and treatment of children with acute symptoms. A further delay takes place once a child presents for medical evaluation. The time delay from symptom presentation to medical triage averaged 1.7 hours in the community setting. 16
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Presentation from the community setting is relatively rapid because many patients ultimately diagnosed with stroke were in need of emergency care because of another illness or injury, such as sickle cell disease, seizure disorders, and accidents causing trauma to the head. 12,16,19 Treatment delays were far longer for children who were hospitalized at the time of symptom onset. Hospitalized children are often afflicted with illnesses or receiving medications that may limit the ability to thoroughly assess neurologic function and may mask the onset of a new stroke. 16 Thus the time interval from symptom presentation to evaluation was approximately 12.7 hours in hospitalized children. 16 Variability in time to presentation for medical evaluation underscores the argument that awareness and education within the public and medical community are lacking. Although some guidance has been formulated to facilitate rapid pediatric stroke evaluation and treatment, much work remains to be performed. Rapid evaluation and management of children presenting with acute neurologic compromise are of critical importance. A uniform recognition and treatment algorithm that allows clinicians to effectively manage the pediatric stroke patient is essential to mitigate the potentially devastating consequences of a delayed diagnosis. STROKE SCALES
Many tools have been introduced to help evaluate and determine whether an adult is having a cerebral vascular accident. These tools have to some degree shaped how clinicians evaluate, identify, and manage childhood strokes. Unfortunately, equal attention has not been given to pediatric stroke management, resulting in low awareness levels within both the general and medical communities. Common themes in pediatric stroke evaluation draw from successes seen in adult stroke management. Reconstruction of adult stroke tools has been used with some success. Much work, however, needs to be performed to formulate a specific model geared toward the pediatric patient. The National Institutes of Health Stroke Scale (NIHSS) has been used to correctly assess adult patients presenting with signs and symptoms of stroke. The tool scores patients on 15 areas including various cognitive, language, and motor skills to determine the probability and severity of stroke. The NIHSS assessment takes only approximately 10 minutes to complete. 7 The Children's Medical Center Dallas has used an adapted version of the NIHSS, called the Ped-NIHSS, to assess potential pediatric victims aged as young as 4 months. 20 The Royal College of Physicians (RCP) has published guidelines for treating pediatric stroke patients 21 and has
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provided recommendations for the use of anticoagulants in pediatric patients with no pre-existing medical conditions. The RCP recommendations include administration of 5 mg of aspirin per kilogram of body weight. 22 The American College of Chest Physicians (ACCP) has also published guidelines on the use of anticoagulants in children. 23 The ACCP recommends a more aggressive approach than the RCP in the absence of contraindications (ie, cardiac disease, sickle cell disease, or neck vessel dissection). The ACCP recommends administration of unfractionated heparin, low–molecular weight heparin, or 1 to 5 mg of aspirin per kilogram of body weight per day until stroke-related etiologies stemming from cardiac malformations, dysrhythmia, or vessel dissection can be evaluated and excluded. 22 Prehospital and ED assessment tools have also been adapted to assess the pediatric patient with suspected stroke symptoms. Instruments including the Face Arm Speech Test (FAST) and Recognition of Stroke in the Emergency Room (ROSIER) have been used with success to correctly identify stroke in the adult population. 24 The FAST prehospital tool assesses for the 3 primary symptoms that indicate stroke in the adult victim: facial weakness, arm weakness, and speech disturbance. The FAST is potentially of great benefit to the assessment of children with suspected stroke, because children most commonly present with facial droop, unilateral hemiparesis, and changes in ability to communicate. 25 The ROSIER tool has also been shown to quickly identify stroke in the emergency setting. The ROSIER uses a 7-item scale in which elements of the FAST combined with patient demographics, blood pressure, blood glucose concentrations, seizure activity, and loss of consciousness are assessed. 24,25 More research is needed to definitely assess the utility of these scales in the pediatric population, but both offer positive attributes that could be of benefit in the pediatric population. 24 Although the various evaluation tools of treatment guidelines available in the literature offer a great deal toward the management of pediatric stroke, a singular, evidencebased treatment algorithm specifically geared toward management of the pediatric stroke patient does not currently exist in the medical literature. A comprehensive yet easy-to-follow algorithm takes the guesswork out of the diagnostic and management processes. The aim of this algorithm is to collectively present the best evidence of how to most effectively treat the pediatric patient presenting with a potential cerebral infarction. This algorithm, in conjunction with a specialized pediatric stroke whip-into-action team (P-SWAT), modeled after popular rapid response teams, assists clinicians in swift identification and management of pediatric stroke.
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Pediatric patient presents to ED
Positive FAST / ROSIER screen
Negative FAST / ROSIER screen Obtain a history, PMH, family history, and conduct an examination (FAST screen and ROSIER) Consider alt diagnosis (stroke remains a possibility)
Exclude: Trauma, hypoglycemia, HTN
contrast CT of head to rule out bleed
Remember ABC’s Make pt NPO Establish IV Obtain initial labs (CBC /diff, lytes, BUN, Dimer, Type / screen and hold)
CT scan result for hemorrhage
Negative
Anticipate CT Angiogram
Positive Neurosurgery for rtPA or clot extraction consideration w / in 3 hours of last seen well
Negative Emergent w / in 1 hour anticipate an MRI / MRA with diffusion weighted images. Order as r / o Stroke
Positive
Correct fluid balance. Want to be euvolemic Optimize Mean Arterial Pressure (MAP) Neurological checks every 15 minutes x 4 the every hour x 6
diagnosed and rtPA and / or clot extraction is contraindicated consider platelet and hemoglobin abnormality correction as ordered by the provider
Anticipation of Neurosurgery involvement
Anticipate orders to correct coagulation abnormalities as indicated
Anticipate possible administration of Heparin
Once patient is able to be moved send to ICU or appropriate care unit
FIGURE D-SIERRA. CT Scan, computed tomography scan; MRA, magnetic resonance angiogram; MRI, magnetic resonance imaging; rtPA, recombinant tissue plasminogen activator. Adapted from references 5 and 20-25.
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Discussion of Dylan's Stroke Identification Evaluation and Rapid Response Algorithm
Dylan's Stroke Identification Evaluation and Rapid Response Algorithm (D-SIERRA) (Figure) provides the emergency nurse with an evidence-based treatment guideline for pediatric patients presenting to the emergency department within 6 hours of presentation of neurologic symptoms. The algorithm can also be adjusted to evaluate and test children who present to the emergency department more than 6 hours after having been observed to be symptom free. 20 The D-SIERRA incorporates evidence drawn from the Ped-NIHSS, AHA, RCP, and ACCP treatment protocols into a singular treatment algorithm pathway that incorporates age-adjusted adaptations from the FAST and ROSIER stroke evaluation tools. Application of the algorithm begins upon presentation to the emergency department, whether from the street or through emergency medical services transport. The emergency nurse obtains a focused family, medical, and symptom history, which is crucial in making an initial decision as to the possibility of stroke. In particular, the nurse should identify whether the patient has a history of cardiac disease, oncologic diagnosis, hematologic disorders, recent infection, sickle cell disease, or identified structural abnormalities that could affect cerebral circulation. 7 The FAST and ROSIER assessments will be completed as a critical decision point at this step. History of trauma, hypoglycemia, and blood pressure must be assessed as possible causes for altered neurologic status. Simultaneously, the emergency nurse must closely monitor the patient's vital signs, maintain a NPO (nothing by mouth) status, and ensure that the child has a patent large-bore intravenous line in a proximal vessel. Decision making at this step will guide the clinician down 1 of 2 routes. If the patient's history, physical examination, and FAST and ROSIER assessments are negative, stroke remains a possibility, but alternate diagnoses should be considered. If initial assessment suggests that the child may be having a stroke, the emergency nurse activates the P-SWAT, composed of an emergency practitioner or physician, pediatric neurologist, pediatric emergency nurse, pharmacist, radiologist, phlebotomist, and nursing supervisor. 26 Additional personnel in the P-SWAT may include a neonatal intensive care nurse for patients aged less than 30 days, as well as counseling services to support the family unit through the triage and decision-making process. The emergency nurse should anticipate that surgical personnel may be alerted to the child's condition in the event that surgical intervention becomes necessary.
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A noncontrast computed tomography (CT) scan would be expected next to rule out intracranial bleeding. Once intracranial hemorrhage is excluded, the nurse should prepare to correct fluid imbalances, optimize the mean arterial pressure, and conduct ongoing neurologic evaluation. In the event that CT identifies intracranial hemorrhage, neurosurgery will be consulted, and the emergency nurse should anticipate orders to correct coagulation abnormalities as indicated. 22 If the CT scan is negative for hemorrhage, CT angiography would be the next probable course of action to assess for obstructions in cerebral blood flow. If CT angiography shows cerebral perfusion abnormalities, the emergency nurse should anticipate that neurosurgery will be contacted and that the administration of recombinant tissue plasminogen activator or clot extraction may take place in cases when evaluation occurs within 3 hours of the time that the child was last seen well. If the findings are negative, the emergency nurse should be prepared for the ordering of magnetic resonance imaging/magnetic resonance angiography in an attempt to identify the location of cerebral compromise. In cases of non-hemorrhagic stroke where tissue plasminogen activator or clot extraction is contraindicated, the emergency nurse would expect that any underlying platelet and hemoglobin abnormalities would be identified and corrected if possible. Clotting profiles may be measured to identify deficiencies in natural clotting inhibitors such as protein C, protein S, and antithrombin III. Drugs such as anticoagulants, corticosteroids, or aspirin may be ordered by the provider at this point and administered by the emergency nurse caring for the child. 5,22,23 If a potential stroke victim presents to the emergency department more than 6 hours after symptom onset, the D-SIERRA is still followed, except tissue plasminogen activator and clot extraction are not beneficial. Implementation of a comprehensive algorithm will allow cerebral infarctions to be rapidly identified, which will allow for more expedient treatment. Decreased time to diagnosis will reduce long-term sequelae and preserve neurologic function. Although the D-SIERRA incorporates a large number of evidence-based guidelines, it is not exhaustive. The D-SIERRA serves to increase awareness of pediatric stroke and to promote timely and accurate evaluation of children with possible cerebral infarction.
Conclusion
Pediatric stroke is a reality. The child described at the beginning of this article was my son, Dylan. The D-SIERRA presented in the Figure combines the most common
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themes discussed in the literature pertaining to the identification and treatment of pediatric stroke. Adoption of a comprehensive stroke treatment algorithm allows every child who passes through the doors of an emergency department the opportunity to receive accurate and prompt evaluation, diagnosis, and treatment of a pediatric stroke. Rapid evaluation and timely treatment preserve brain tissue and potentially prevent irreparable neurologic damage, thus enhancing the child's long-term treatment prognosis. REFERENCES
12. Kirkham F, Sebire G, Steinlin M, Strater R. Arterial ischaemic stroke in children. Review of the literature and strategies for future stroke studies. Thromb Haemost. 2004;92(4):697-706. 13. Gorchynski J, Herrick J, Cortes E. Acute ischemic stroke in a pediatric patient. West J Emerg Med. 2008;9(4):225-7. 14. Sachdev A, Sharma R, Gupta D. Cerebrovascular complications in pediatric intensive care unit. Indian J Crit Care Med. 2010;14(3):129-40. 15. Lynch JK, Hirtz DG, DeVeber G, Nelson KB. Report of the National Institute of Neurological Disorders and Stroke workshop on perinatal and childhood stroke. Pediatrics. 2002;109:116-23. 16. Rafay MF, Pontigon AM, Chiang J. Delay to diagnosis in acute pediatric arterial ischemic stroke. Stroke. 2009;40(1):58-64.
1. Ciccone S, Cappella M, Borgna-Pignatti C. Ischemic stroke in infants and children: practical management in emergency. Stroke Res Treat. 2011;2011:736965.
17. Srinivasan J, Miller SP, Phan TG, Mackay MT. Delayed recognition of initial stroke in children: need for increased awareness. Pediatrics. 2009;124(2):e227-34.
2. Goodman S, Pavlakis S. Pediatric and newborn stroke. Curr Treat Options Neurol. 2008;10(6):431-9.
18. Miravet E, Danchaivijitr N, Basu H, Saunders DE, Ganesan V. Clinical and radiological features of childhood cerebral infarction following varicella zoster virus infection. Dev Med Child Neurol. 2007;49(6):417-22.
3. Hartman AL, Lunney KM, Serena JE. Pediatric stroke: do clinical factors predict delays in presentation? J Pediatr. 2009;154(5):727-32. 4. Aho K, Harmsen P, Hatano S, Marquardsen J, Smirnov VE, Strasser T. Cardiovascular disease in the community: results of a WHO collaborative study. Bull World Health Organ. 1980:28. 5. Roach ES, Golomb MR, Adams R. 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):2644-91. 6. The Royal College of Physicians. Stroke in childhood: clinical guidelines for diagnosis, management and rehabilitation. http://bookshop. rcplondon.ac.uk/contents/f98c6540-a541-4bed-837d-ef293ac458bf. pdf. Accessed December 22, 2011. 7. Ciceri EF, Cuccarini V, Chiapparini L, Saletti V, Valvassori L. Paediatric stroke: review of the literature and possible treatment options, including endovascular approach. Stroke Res Treat. 2011;2011:781612. 8. Roger VL, Go AS, Lloyd-Jones DM. Executive summary: heart disease and stroke statistics—2012 update: a report from the American Heart Association. Circulation. 2012;125(1):188-97. 9. Fox CK, Fullerton HJ. Recent advances in childhood arterial ischemic stroke. Curr Atheroscler Rep. 2010;12(4):217-24. 10. Beslow LA, Jordan LC. Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations. Childs Nerv Syst. 2010;26(10): 1263-73. 11. Jordan LC. Assessment and treatment of stroke in children. Curr Treat Options Neurol. 2008;10(6):399-409.
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19. Abram HS. Childhood Strokes: evaluation and management. http:// www.dcmsonline.org/jax-medicine/1998journals/november98/ childhoodstrokes.htm. Accessed October 13, 2012. 20. Wolfson's Children's Hospital. Pediatric Stroke Guidelines. 2011. pedsjax.files.wordpress.com/.../wolfson-stroke-guidelines-2012.doc. Accessed January 31, 2012. 21. The Royal College of Physicians. Information for parents and families of children affected by stroke. http://bookshop.rcplondon.ac.uk/contents/ 520d33d7-9532-48d8-8cd7-37a9978e6d0d.pdf. Accessed December 22, 2011. 22. DeVeber G, Kirkham F. Guidelines for the treatment and prevention of stroke in children. Lancet Neurol. 2008;7(11):983-5. 23. Monagle P, Chalmers E, Chan A, et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133(6 suppl):S887-968. 24. Yock-Corrales A, Babl FE, Mosley IT, Mackay MT. Can the FAST and ROSIER adult stroke recognition tools be applied to confirmed childhood arterial ischemic stroke? BMC Pediatr. 2011;11:93. 25. The Royal College of Physicians. Stroke: national clinical guideline for diagnosis and initial management of acute stroke and transient ischaemic attack (TIA).http://bookshop.rcplondon.ac.uk/contents/9c4488ac-d8f143d8-b9da-b801441bcdfb.pdf. Accessed December 22, 2011. 26. Krock AB, Massaro L. Facilitating ED evaluation of patients with acute ischemic stroke. J Emerg Nurs. 2008;34(6):519-22.
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EVALUATION, IDENTIFICATION, AND MANAGEMENT OF PEDIATRIC STROKES IN THE EMERGENCY DEPARTMENT USING A PATHWAY ALGORITHM Authors: Victor Jerome Hicks Jr, MSN, APN, and Lisa M. Black, PhD, RN, CNE, Carson City and Reno, NV
Earn Up to 9.0 CE Hours. See page 211.
Clinical Scenario
On Christmas Day 2009, a 22-month-old male infant presented to a local children's emergency department accompanied by his mother. The patient's mother reported to the emergency staff that before their arrival to the emergency department that day, the patient seemed less steady on his feet and had fallen several times with no apparent reason. The mother reported that the patient had been taking oral amoxicillin for about 48 hours to treat acute otitis media. The mother stated that her son had also had acute influenza with a maximum temperature of 39.8°C (103.6°F) that was controlled with oral antipyretics. The child had no history of seizures or head trauma. Approximately 1 hour before ED presentation, the child was noted to be unable to use his arms to rise to a standing support position. When assisted to a standing position, the patient was noted to have significant weakness in the left lower extremity, left facial droop, and confusion. There was also noticeable muscle spasm to the left arm, hand, leg, knee, and foot. Initial ED hematology and chemistry studies were found to be within normal limits. Although the parents expressed concern that the diagnostic workup was incomplete, the child was discharged from the emergency department with a diagnosis of synovial joint Victor Jerome Hicks Jr is Family Nurse Practitioner CHNIII, State of Nevada Health Division, Carson City, NV. Lisa M. Black is Assistant Professor, Orvis School of Nursing, University of Nevada, Reno, NV. For correspondence, write: Victor Jerome Hicks Jr, MSN, APN, 1728 Back Country Ct, Reno, NV 89521; E-mail: [email protected]. J Emerg Nurs 2013;39:132-7. Available online 5 February 2013. 0099-1767/$36.00 Copyright © 2013 Published by Elsevier Inc. on behalf of Emergency Nurses Association. http://dx.doi.org/10.1016/j.jen.2012.11.013
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pain for which over-the-counter nonsteroidal anti-inflammatory drugs were prescribed. Five days later, the patient's symptoms had not resolved. The child was taken to see his primary pediatrician, who immediately noted persistent leftsided deficits. The next day, the patient underwent magnetic resonance imaging, which showed an acute arterial ischemic infarct. Later the same day, the patient underwent an echocardiogram to rule out cardiac etiology, which was negative. The patient was ultimately diagnosed with arterial vasculitis, which resulted in an ischemic multifocal infarct of the right basal ganglia. Problem
Childhood stroke is rare, with an estimated incidence of 13 in 100,000, 1 and misdiagnosis of ischemic events in children is common. 1-3 Lack of awareness about pediatric strokes within the health care community causes delays in quick evaluation and proper diagnosis, which limits treatment options. Delayed diagnosis leads to irreversible damage to cerebral tissues, greater long-term deficits, and other adverse sequelae. Together, these factors dramatically increase the resources required to treat adverse sequelae of childhood stroke. This article aims to increase awareness about the incidence and prevalence of pediatric stroke and to provide a uniform, evidence-based treatment algorithm for children who present with acute neurologic compromise. This treatment algorithm is intended to provide emergency nurses with increased confidence when caring for a child with a suspected cerebral infarct. Introduction and Background PATHOPHYSIOLOGY AND EPIDEMIOLOGY OF ADULT STROKE
As defined by the World Health Organization, stroke is a clinical disorder of rapidly developing centralized or
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comprehensive deterioration of brain function lasting longer than 24 hours with no indication of it originating from a cause outside the vascular system. 1,4-6 Stroke is classified into 2 major categories, ischemic or hemorrhagic, both of which decrease cerebral blood flow and increase the potential for cell death. Arterial ischemic stroke and cerebral venous thrombosis result in decreased or total blockage in venous blood flow to the brain. Intracerebral hemorrhage and nontraumatic subarachnoid hemorrhage cause a disruption in normal cerebral blood flow resulting in a decrease in oxygen-rich blood to brain cells. 7 Adult strokes account for the United States' third leading cause of death and is a leading cause of functional impairment. It is reported that nearly 800,000 Americans have a cerebral infarct each year, and stroke accounts for 1 of every 18 deaths in the United States. 8 With strokes having such high rates of morbidity and mortality, increased awareness and education on warning signs and the primary etiologies (ie, arrhythmias and arteriosclerotic arteriopathies) 9 have led to faster presentation times and improved end outcomes. This increased awareness about strokes has only begun to be realized in the field of pediatric neurology. EPIDEMIOLOGY OF PEDIATRIC STROKE
Pediatric stroke is more serious than once thought. As common as pediatric brain tumors, pediatric stroke ranks among the top 10 causes of death in children aged under 18 years. 7,10-12 With the recent advances in technology and increased awareness, it is likely that the incidence of pediatric strokes is higher than what has been documented. Consensus in the pediatric stroke literature on numbers of reported cases annually ranges between 2 and 3 per 100,000 children, with some literature reporting a stroke incidence as high as 13 per 100,000 children. 11-14 Given the complexity of etiologic presentations within the field of pediatric stroke, researchers have begun to classify pediatric stroke according to the age of the child. This allows for investigation into plausible originating sources and etiologies, whether from maternal fetal sources or from other non-maternal factors. Neonatal strokes are those that occur within the first month of life. 5 Neonatal strokes are of significant importance because disruption in the development of the brain's vascular system causes significant mental retardation and other neurologic sequelae. Ischemic or hemorrhagic events within the brain between 28 weeks of gestation and 28 days after birth are defined as perinatal strokes. 5,15 Strokes occurring from day 30 of life through the age of 18 years are classified as pediatric or childhood strokes. 13,15 Breaking down the ages in which pediatric strokes occur allows researchers to study various theories as
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to how and why strokes occur at specific points in the development process. PATHOPHYSIOLOGY OF PEDIATRIC STROKE
Difficulties arise in the identification and evaluation of pediatric stroke in that children often present with symptoms that differ from those seen in the adult population. 3 Risk factor variance, differing clinical presentations, and the absence of a standardized approach to evaluation and treatment, 5 coupled with differing etiologies from the adult stroke cohort, often result in misdiagnosis and mismanagement. 9 Thrombotic events account for approximately 85% of all stroke events witnessed in children. 14 Other pathologic findings are observed within the cerebral arteries in 80% of children with acute ischemic stroke (AIS). AIS in the pediatric population generally occurs between the ages of 1 and 5 years. 13 Traumatic head injuries make up most cases before age 1 and after age 5. Although a large percentage of stroke cases have underlying embolic causes related to cardiac malformations, many root causes are unknown. No preexisting or identifiable risk factors can be seen in 10% to 30% of pediatric stroke victims. 7 Common illnesses with stroke-like mimickers including migraine, encephalitis, tumors, and postictal paralysis are often linked to neurologic deficits seen in children, 16,17 which convolutes appropriate management. Seizures and altered level of consciousness are relatively frequent in pediatric stroke patients, particularly in neonates and children aged under 4 years. 12,16,17 This increased prevalence of seizure activity in pediatric stroke points to the occurrence of seizures as a presenting sign that is unique to the pediatric stroke population. Current or recent infection with the varicella virus has been shown to increase the risk of a child having a stroke. Miravet et al 18 found that one-third of the children in their study cohort who had an AIS were currently infected with or had recently recovered from varicella. Likewise, Lynch et al 15 noted that among children aged between 6 months and 10 years who were diagnosed with a stroke, up to 30% had been infected with the varicella virus within the previous year. Thus a thorough history and evaluation of possible stroke etiologies are essential in children presenting with acute neurologic compromise. PEDIATRIC STROKE AWARENESS
Lack of pediatric stroke awareness has led to delayed medical evaluation and treatment of children with acute symptoms. A further delay takes place once a child presents for medical evaluation. The time delay from symptom presentation to medical triage averaged 1.7 hours in the community setting. 16
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Presentation from the community setting is relatively rapid because many patients ultimately diagnosed with stroke were in need of emergency care because of another illness or injury, such as sickle cell disease, seizure disorders, and accidents causing trauma to the head. 12,16,19 Treatment delays were far longer for children who were hospitalized at the time of symptom onset. Hospitalized children are often afflicted with illnesses or receiving medications that may limit the ability to thoroughly assess neurologic function and may mask the onset of a new stroke. 16 Thus the time interval from symptom presentation to evaluation was approximately 12.7 hours in hospitalized children. 16 Variability in time to presentation for medical evaluation underscores the argument that awareness and education within the public and medical community are lacking. Although some guidance has been formulated to facilitate rapid pediatric stroke evaluation and treatment, much work remains to be performed. Rapid evaluation and management of children presenting with acute neurologic compromise are of critical importance. A uniform recognition and treatment algorithm that allows clinicians to effectively manage the pediatric stroke patient is essential to mitigate the potentially devastating consequences of a delayed diagnosis. STROKE SCALES
Many tools have been introduced to help evaluate and determine whether an adult is having a cerebral vascular accident. These tools have to some degree shaped how clinicians evaluate, identify, and manage childhood strokes. Unfortunately, equal attention has not been given to pediatric stroke management, resulting in low awareness levels within both the general and medical communities. Common themes in pediatric stroke evaluation draw from successes seen in adult stroke management. Reconstruction of adult stroke tools has been used with some success. Much work, however, needs to be performed to formulate a specific model geared toward the pediatric patient. The National Institutes of Health Stroke Scale (NIHSS) has been used to correctly assess adult patients presenting with signs and symptoms of stroke. The tool scores patients on 15 areas including various cognitive, language, and motor skills to determine the probability and severity of stroke. The NIHSS assessment takes only approximately 10 minutes to complete. 7 The Children's Medical Center Dallas has used an adapted version of the NIHSS, called the Ped-NIHSS, to assess potential pediatric victims aged as young as 4 months. 20 The Royal College of Physicians (RCP) has published guidelines for treating pediatric stroke patients 21 and has
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provided recommendations for the use of anticoagulants in pediatric patients with no pre-existing medical conditions. The RCP recommendations include administration of 5 mg of aspirin per kilogram of body weight. 22 The American College of Chest Physicians (ACCP) has also published guidelines on the use of anticoagulants in children. 23 The ACCP recommends a more aggressive approach than the RCP in the absence of contraindications (ie, cardiac disease, sickle cell disease, or neck vessel dissection). The ACCP recommends administration of unfractionated heparin, low–molecular weight heparin, or 1 to 5 mg of aspirin per kilogram of body weight per day until stroke-related etiologies stemming from cardiac malformations, dysrhythmia, or vessel dissection can be evaluated and excluded. 22 Prehospital and ED assessment tools have also been adapted to assess the pediatric patient with suspected stroke symptoms. Instruments including the Face Arm Speech Test (FAST) and Recognition of Stroke in the Emergency Room (ROSIER) have been used with success to correctly identify stroke in the adult population. 24 The FAST prehospital tool assesses for the 3 primary symptoms that indicate stroke in the adult victim: facial weakness, arm weakness, and speech disturbance. The FAST is potentially of great benefit to the assessment of children with suspected stroke, because children most commonly present with facial droop, unilateral hemiparesis, and changes in ability to communicate. 25 The ROSIER tool has also been shown to quickly identify stroke in the emergency setting. The ROSIER uses a 7-item scale in which elements of the FAST combined with patient demographics, blood pressure, blood glucose concentrations, seizure activity, and loss of consciousness are assessed. 24,25 More research is needed to definitely assess the utility of these scales in the pediatric population, but both offer positive attributes that could be of benefit in the pediatric population. 24 Although the various evaluation tools of treatment guidelines available in the literature offer a great deal toward the management of pediatric stroke, a singular, evidencebased treatment algorithm specifically geared toward management of the pediatric stroke patient does not currently exist in the medical literature. A comprehensive yet easy-to-follow algorithm takes the guesswork out of the diagnostic and management processes. The aim of this algorithm is to collectively present the best evidence of how to most effectively treat the pediatric patient presenting with a potential cerebral infarction. This algorithm, in conjunction with a specialized pediatric stroke whip-into-action team (P-SWAT), modeled after popular rapid response teams, assists clinicians in swift identification and management of pediatric stroke.
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Pediatric patient presents to ED
Positive FAST / ROSIER screen
Negative FAST / ROSIER screen Obtain a history, PMH, family history, and conduct an examination (FAST screen and ROSIER) Consider alt diagnosis (stroke remains a possibility)
Exclude: Trauma, hypoglycemia, HTN
contrast CT of head to rule out bleed
Remember ABC’s Make pt NPO Establish IV Obtain initial labs (CBC /diff, lytes, BUN, Dimer, Type / screen and hold)
CT scan result for hemorrhage
Negative
Anticipate CT Angiogram
Positive Neurosurgery for rtPA or clot extraction consideration w / in 3 hours of last seen well
Negative Emergent w / in 1 hour anticipate an MRI / MRA with diffusion weighted images. Order as r / o Stroke
Positive
Correct fluid balance. Want to be euvolemic Optimize Mean Arterial Pressure (MAP) Neurological checks every 15 minutes x 4 the every hour x 6
diagnosed and rtPA and / or clot extraction is contraindicated consider platelet and hemoglobin abnormality correction as ordered by the provider
Anticipation of Neurosurgery involvement
Anticipate orders to correct coagulation abnormalities as indicated
Anticipate possible administration of Heparin
Once patient is able to be moved send to ICU or appropriate care unit
FIGURE D-SIERRA. CT Scan, computed tomography scan; MRA, magnetic resonance angiogram; MRI, magnetic resonance imaging; rtPA, recombinant tissue plasminogen activator. Adapted from references 5 and 20-25.
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Discussion of Dylan's Stroke Identification Evaluation and Rapid Response Algorithm
Dylan's Stroke Identification Evaluation and Rapid Response Algorithm (D-SIERRA) (Figure) provides the emergency nurse with an evidence-based treatment guideline for pediatric patients presenting to the emergency department within 6 hours of presentation of neurologic symptoms. The algorithm can also be adjusted to evaluate and test children who present to the emergency department more than 6 hours after having been observed to be symptom free. 20 The D-SIERRA incorporates evidence drawn from the Ped-NIHSS, AHA, RCP, and ACCP treatment protocols into a singular treatment algorithm pathway that incorporates age-adjusted adaptations from the FAST and ROSIER stroke evaluation tools. Application of the algorithm begins upon presentation to the emergency department, whether from the street or through emergency medical services transport. The emergency nurse obtains a focused family, medical, and symptom history, which is crucial in making an initial decision as to the possibility of stroke. In particular, the nurse should identify whether the patient has a history of cardiac disease, oncologic diagnosis, hematologic disorders, recent infection, sickle cell disease, or identified structural abnormalities that could affect cerebral circulation. 7 The FAST and ROSIER assessments will be completed as a critical decision point at this step. History of trauma, hypoglycemia, and blood pressure must be assessed as possible causes for altered neurologic status. Simultaneously, the emergency nurse must closely monitor the patient's vital signs, maintain a NPO (nothing by mouth) status, and ensure that the child has a patent large-bore intravenous line in a proximal vessel. Decision making at this step will guide the clinician down 1 of 2 routes. If the patient's history, physical examination, and FAST and ROSIER assessments are negative, stroke remains a possibility, but alternate diagnoses should be considered. If initial assessment suggests that the child may be having a stroke, the emergency nurse activates the P-SWAT, composed of an emergency practitioner or physician, pediatric neurologist, pediatric emergency nurse, pharmacist, radiologist, phlebotomist, and nursing supervisor. 26 Additional personnel in the P-SWAT may include a neonatal intensive care nurse for patients aged less than 30 days, as well as counseling services to support the family unit through the triage and decision-making process. The emergency nurse should anticipate that surgical personnel may be alerted to the child's condition in the event that surgical intervention becomes necessary.
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A noncontrast computed tomography (CT) scan would be expected next to rule out intracranial bleeding. Once intracranial hemorrhage is excluded, the nurse should prepare to correct fluid imbalances, optimize the mean arterial pressure, and conduct ongoing neurologic evaluation. In the event that CT identifies intracranial hemorrhage, neurosurgery will be consulted, and the emergency nurse should anticipate orders to correct coagulation abnormalities as indicated. 22 If the CT scan is negative for hemorrhage, CT angiography would be the next probable course of action to assess for obstructions in cerebral blood flow. If CT angiography shows cerebral perfusion abnormalities, the emergency nurse should anticipate that neurosurgery will be contacted and that the administration of recombinant tissue plasminogen activator or clot extraction may take place in cases when evaluation occurs within 3 hours of the time that the child was last seen well. If the findings are negative, the emergency nurse should be prepared for the ordering of magnetic resonance imaging/magnetic resonance angiography in an attempt to identify the location of cerebral compromise. In cases of non-hemorrhagic stroke where tissue plasminogen activator or clot extraction is contraindicated, the emergency nurse would expect that any underlying platelet and hemoglobin abnormalities would be identified and corrected if possible. Clotting profiles may be measured to identify deficiencies in natural clotting inhibitors such as protein C, protein S, and antithrombin III. Drugs such as anticoagulants, corticosteroids, or aspirin may be ordered by the provider at this point and administered by the emergency nurse caring for the child. 5,22,23 If a potential stroke victim presents to the emergency department more than 6 hours after symptom onset, the D-SIERRA is still followed, except tissue plasminogen activator and clot extraction are not beneficial. Implementation of a comprehensive algorithm will allow cerebral infarctions to be rapidly identified, which will allow for more expedient treatment. Decreased time to diagnosis will reduce long-term sequelae and preserve neurologic function. Although the D-SIERRA incorporates a large number of evidence-based guidelines, it is not exhaustive. The D-SIERRA serves to increase awareness of pediatric stroke and to promote timely and accurate evaluation of children with possible cerebral infarction.
Conclusion
Pediatric stroke is a reality. The child described at the beginning of this article was my son, Dylan. The D-SIERRA presented in the Figure combines the most common
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themes discussed in the literature pertaining to the identification and treatment of pediatric stroke. Adoption of a comprehensive stroke treatment algorithm allows every child who passes through the doors of an emergency department the opportunity to receive accurate and prompt evaluation, diagnosis, and treatment of a pediatric stroke. Rapid evaluation and timely treatment preserve brain tissue and potentially prevent irreparable neurologic damage, thus enhancing the child's long-term treatment prognosis. REFERENCES
12. Kirkham F, Sebire G, Steinlin M, Strater R. Arterial ischaemic stroke in children. Review of the literature and strategies for future stroke studies. Thromb Haemost. 2004;92(4):697-706. 13. Gorchynski J, Herrick J, Cortes E. Acute ischemic stroke in a pediatric patient. West J Emerg Med. 2008;9(4):225-7. 14. Sachdev A, Sharma R, Gupta D. Cerebrovascular complications in pediatric intensive care unit. Indian J Crit Care Med. 2010;14(3):129-40. 15. Lynch JK, Hirtz DG, DeVeber G, Nelson KB. Report of the National Institute of Neurological Disorders and Stroke workshop on perinatal and childhood stroke. Pediatrics. 2002;109:116-23. 16. Rafay MF, Pontigon AM, Chiang J. Delay to diagnosis in acute pediatric arterial ischemic stroke. Stroke. 2009;40(1):58-64.
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19. Abram HS. Childhood Strokes: evaluation and management. http:// www.dcmsonline.org/jax-medicine/1998journals/november98/ childhoodstrokes.htm. Accessed October 13, 2012. 20. Wolfson's Children's Hospital. Pediatric Stroke Guidelines. 2011. pedsjax.files.wordpress.com/.../wolfson-stroke-guidelines-2012.doc. Accessed January 31, 2012. 21. The Royal College of Physicians. Information for parents and families of children affected by stroke. http://bookshop.rcplondon.ac.uk/contents/ 520d33d7-9532-48d8-8cd7-37a9978e6d0d.pdf. Accessed December 22, 2011. 22. DeVeber G, Kirkham F. Guidelines for the treatment and prevention of stroke in children. Lancet Neurol. 2008;7(11):983-5. 23. Monagle P, Chalmers E, Chan A, et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133(6 suppl):S887-968. 24. Yock-Corrales A, Babl FE, Mosley IT, Mackay MT. Can the FAST and ROSIER adult stroke recognition tools be applied to confirmed childhood arterial ischemic stroke? BMC Pediatr. 2011;11:93. 25. The Royal College of Physicians. Stroke: national clinical guideline for diagnosis and initial management of acute stroke and transient ischaemic attack (TIA).http://bookshop.rcplondon.ac.uk/contents/9c4488ac-d8f143d8-b9da-b801441bcdfb.pdf. Accessed December 22, 2011. 26. Krock AB, Massaro L. Facilitating ED evaluation of patients with acute ischemic stroke. J Emerg Nurs. 2008;34(6):519-22.
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