Pediatric Stroke Part 2: Rehabilitation Management and Treatments, Cutting Edge Concepts, and Gaps in the Evidence

Disease/Disorder

See Pediatric Stroke Part One

Essentials of Assessment

See Pediatric Stroke Part One

Rehabilitation Management and Treatments

Available or current treatment guidelines

Evidence-based research on pediatric stroke management remains limited. Guidelines established by the American Heart Association (AHA) and the Canadian Stroke Best Practice Recommendations have been augmented recently by the Australian clinical consensus guideline in 2021.

The AHA released “Considerations for Clinical Practice” for pediatric rehabilitation in 2019 that supports:

• “Age-appropriate rehabilitation and therapy programs are appropriate for children after a stroke.”
• “Psychological assessment to document cognitive and language deficits is useful for planning therapy and educational programs after a child’s stroke.”
• “Constraint therapy should be considered in children with unilateral hand dysfunction after arterial ischemic stroke.”
  • Constraint therapy can also be applied for upper extremity strengthening.
• “Long-term follow-up is required for children with stroke to assess for development of new cognitive, physical, and emotional concerns that may occur over time as children grow into deficits.”¹

These recommendations complement the 2015 Canadian Stroke Best Practice Recommendations which support:

Level A Evidence:  Constraint-Induced Movement Therapy for upper limb impairment

Level B Evidence:  Initial assessment by medical professionals should be done quickly to determine stroke severity and rehabilitation needs. Provide acute and rehabilitation stroke care in a specialized pediatric unit. Offer outpatient support to children who have a change in functional status and would benefit from more rehabilitation services at any time during their recovery.

Regarding the rehabilitation team, care should be given by health professionals who have experience in pediatric stroke care, regardless of the location of services to decrease complication risk. This team should include physicians, occupational therapists, physical therapists, speech-language pathologists, nurses, social workers, psychologists, and dietitians.

Additionally, there is often guilt associated with pediatric strokes. Therefore, it is recommended to provide education to reduce maternal guilt or parental blame. It is encouraged to offer families support and information about changes in physical needs and dependency of the child.

Level C Evidence:  Individualized rehabilitation plans and annual developmental updates as the patient progresses. Given many assessments were developed for adults after stroke, current standardized and validated assessment tools relating to functional activity limitations, impairment due to the stroke, restrictions in role participation, changes in mood and behavior, and environmental restriction receive a Level C recommendation. It is recommended that children have rehabilitation that is meaningful, engaging, age-appropriate, task-specific, goal-oriented, repetitive, and progressively adapted to enhance motor control and return of sensorimotor function. Children should participate in training that encourages the use of the affected limb undergoing functional tasks and that mimics daily life activities appropriate for the child’s developmental stage.

For strength and range of motion, the upper limb should be placed in a wide range of positions that are safe in the child’s visual field. Rehabilitation interventions that can be used for the upper limbs include Functional Electric Stimulation, Mirror Therapy, Botulinum Toxin Type A, Repetitive Transcranial Magnetic Stimulation, or Surgical Interventions. Similarly, for the lower limb, range of motion exercises, gait training, and physical activity are recommended, in addition to the use of other rehabilitation therapies such as Ankle-Foot Orthoses, Botulinum Toxin Type A, or Surgical Interventions 

Adaptive devices as well as special equipment can be used for the long-term management of pediatric stroke, such as customized hand and wrist splints or wheelchair trays. The orthotic pieces can be customized for the child paired along with a plan to monitor for effectiveness. Continued evaluation for educational and vocational needs should be considered. School-aged children should receive educational rehabilitation and support services, along with an individualized educational plan.²

Recently, the Australian Clinical Consensus Guideline in 2021 further explored specific aspects of rehabilitation in childhood stroke supporting:

The elaboration that a child’s individualized therapy and educational programs should encompass a thorough assessment of deficits and may involve multiple team members.

Criteria to consider when transferring a child from an acute setting hospital to subacute rehabilitation care are: medical stability, change in function that could benefit from rehabilitation, and rehabilitation goals. Considerations for transfer from subacute rehabilitation to long-term community care include: when child’s safety is ensured, goals will be better addressed in the community, and family readiness at home.

Group therapy should be considered in addition to standard individualized therapy. It is most appropriate when goals are generic among enough children, target social and communication skills, or focus on common physical skills (e.g., running or riding a bike). Individualized therapy is appropriate when goals are specific to the child, in cases with cognitive dysfunction, frequent modification to therapy, or when privacy is required.

Environmental adaptations (e.g. utensils, home modifications, Velcro) are needed to improve activities of daily living. Repetitive transcranial magnetic stimulation (rTMS) is a feasible intervention to improve upper limb strength.

Graded sensory exposure and the use of vision to provide sensory feedback about limb position are strategies that should be considered to address sensory difficulties. Additionally, a comprehensive multidisciplinary pain management approach that encompasses medications, prevention strategies, and psychological support should be implemented when appropriate.

Dietary changes, food consistency modification, and techniques (e.g., chin tuck, head rotation, double swallow) should be considered in dysphagia or poor nutritional intake. Nasogastric tubes or gastrostomy should be used to manage severe dysphagia only.

Articulation or phonological therapy should be considered to improve speech intelligibility. Augmentative and alternative communication may include Dynavox, picture boards, gestures, or electronic tablets. Compensation techniques (e.g., written instructions and schedules) may benefit working memory, short-term memory, and recall.

Parents, teachers, and the community should be educated on the impact of stroke on emotional and behavioral function. Individualized strategies should be developed to encourage the integration and participation of patients with childhood stroke. Return to school must be a graded process that includes adjustments to the school curriculum and an Individual Learning Plan.³

At different disease stages

New onset/acute

Starts with supportive care that includes hydration, management of hypoxia, hypotension, seizures, and close monitoring for signs of increased intracranial pressure.

As an initial imaging study, magnetic resonance (MR) can be done if available within 1 hour of arrival in the hospital. Intravenous thrombolysis can be offered within 4.5 hours of symptom onset in patients aged two years or more and a PedNIHSS score between 4 and 24. Imaging must confirm the absence of hemorrhage and normal or minimally ischemic parenchyma on computed tomography (CT) or MR evidence of acute ischemia. There also must be arterial occlusion or severe stenosis on CT with contrast (CTA) or MR angiography (MRA).⁴

Timely decisions for thrombolytic therapies can be improved by getting neurology and pediatric hematology or a stroke consult service involved early on. It is essential to highlight that the results of adult stroke trials testing antithrombotic treatments cannot be directly used for children due to different thrombus formation mechanisms. Anticoagulation in children with arterial ischemic stroke (AIS) is based on perceived mechanisms for arterial thrombosis, including cerebral vasculopathy, sickle cell disease, and in situ thrombosis.⁵

In 2010, the National Institute of Neurological Disorders and Stroke (NINDS) funded the first prospective treatment trial in acute pediatric stroke, the Thrombolysis in Pediatric Stroke (TIPS) trial. This study was defunded because it was unable to recruit enough pediatric ischemic stroke patients.⁶ However, the former TIPS sites have continued to identify patients who would have been eligible for TIPS and have treated children with tissue-type plasminogen activator (tPA) using the TIPS protocol with a low risk of intracranial hemorrhage.⁷ tPA is low risk in children at doses of 0.9 mg/kg with a max dose of 90 mg.⁷

Heparin is not used widely in children with perinatal AIS, although children with severe pro-thrombotic disorders or with cardiac or multiple systemic thrombi may benefit. Recommendation for neonates with a first arterial ischemic stroke and a documented cardioembolic source suggest anticoagulation with unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH). ⁵ However, there is insufficient evidence in neonates or infants to make any recommendation regarding mechanical thrombectomy in children younger than 12 months.⁸

Mechanical thrombectomy is a treatment option for ischemic stroke due to a large vessel occlusion in patients aged 1-18 years. 

Even though surgical evacuation of hemorrhage is not typically indicated, this may be useful in select patients with persistently increased intracranial pressure refractory to medical management.

Markedly low platelet counts, and factor deficiencies should be corrected. Vitamin K deficiency may be an issue in areas of the world where vitamin K is not routinely administered to newborns, in infants with biliary atresia, or in babies whose mothers ingested warfarin, phenytoin, or barbiturates during pregnancy.

Sub-acute, chronic/stable

Sickle cell patients with confirmed infarct require a long-term red blood cell transfusion program for secondary prevention. If transfusions are not possible, they may receive hydroxyurea. Even though chronic transfusions carry risks, stopping chronic transfusions leads to an increased risk of stroke in this patient population.⁹

Congenital vascular abnormalities should be treated with surgery, endovascular repair, or radiosurgery when possible. In patients with Moyamoya (a congenital syndrome of cerebral arterial malformation), surgical revascularization is the primary treatment.¹

Heparin therapy can be started in patients with extracranial arterial dissection or cardioembolism and later transitioned to warfarin. The duration of therapy is three to six months for dissection and one year or longer for cardioembolic causes (depending on whether the causative lesion can be corrected). 

Oral contraceptives are associated with cerebral sinovenous thrombosis. Other forms of birth control should be considered in this patient population.¹

Folic acid can decrease the risk of sequelae in those with elevated homocysteine levels. Diet changes and folate, vitamin B6, and vitamin B12 supplementation are recommended. ¹

Even though there is little information about the long-term use of prophylactic therapies such as LMWH in neonates, it is known that this population has a low recurrence risk for stroke. Therefore, aspirin and anticoagulation with LMWH are not indicated. However, they can be used if there is a high risk of recurrent AIS, such as thrombophilia or congenital heart disease.¹

Rehabilitation strategies

It includes using orthotics, adaptive equipment, and medical management of spasticity. It is crucial to consider the entire family when starting a rehabilitation program since parental guilt or blame can be seen in some cases. Among goals, can have education and vocational rehabilitation as well as goals similar to that of an adult.²

An Individualized Education Plan (IEP) should be made when required. It is important to provide information about resources available through the school, and how to request a 504 plan or an IEP. This plan should specify educational adaptations, educationally related therapy services, and nursing services to be provided by the school.²

Coordination of care

Multidisciplinary teams remain the standard of care for pediatric stroke patients. The critical care team, neurology, hematology, trauma service, neurosurgery, care coordination, and physiatry may be involved in the acute care setting.

In the rehabilitation setting, physiatry, pediatrics, physical therapy, occupational therapy, speech therapy, neuropsychology, dietician, child life therapies, recreational therapies, care coordinators, social workers, nursing staff, teachers, and services that evaluated the patient during acute management (to follow up on any pending health concerns) may be involved.

Patient & family education

Most sources indicate cautious optimism is warranted; a good balance of honesty about probable long-term deficits and overall strong potential for recovery should be provided.

The Children’s Hemiplegia and Stroke Association (http://www.chasa.org/) is an excellent starting point for families to meet others with similar experiences, learn about stroke rehabilitation, and find events and activities for children who have had a stroke. 

Emerging/unique interventions

Several validated measures have been used to document outcomes, but not all are specific to pediatric stroke. The Pediatric Stroke Outcomes Measure, Pediatric Stroke Recurrence and Recovery Questionnaire, and the PedNIHSS were developed specifically for this population.¹⁰

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Remember to keep stroke in the differential diagnosis for children with neurologic complaints, headache, or vomiting. Misdiagnosis or delayed diagnosis is common.¹¹

Inpatient Rehabilitation (IPR) has been shown to significantly improve functional outcomes, especially in motor and cognitive function, with hemorrhagic strokes having better outcomes than ischemic strokes.

Provide education to primary physicians and parents of pediatric stroke survivors. 

Encourage consideration for early referrals. Currently, it takes pediatric stroke survivors a median time of 78 days from the onset of symptoms to IPR admission.¹²

Pediatric stroke survivors were found to have worse academic achievement in the future, specifically in mathematics.¹³ Recommend neuropsychology screen given the potential for difficulties with higher-level learning that are overlooked early on.¹⁴

It is recommended that children receive an individualized education plan to meet the needs of that child.²

The severity of neurological impairment, sex (girls rate their quality of life lower than boys), younger age at stroke onset, cognitive function, self-esteem, and family factors have all separately been associated with poorer quality of life. 

The presence of arteriopathy in children is the most important predictor of recurrent arterial ischemic stroke, with rates of >65% reported.¹⁵

A recent study of Cerebral Palsy patients suggests that children can choose functional and developmentally appropriate goals for themselves. This study suggests that children should be able to define their own goals in rehabilitation and may be helpful when treating pediatric stroke patients.¹⁶

Cutting Edge/Emerging and Unique Concepts and Practice

Robotic therapies to improve upper extremity function have been tested in adult stroke patients and in children with cerebral palsy. Expansion to the pediatric stroke population is a logical next step. Newer robotic therapies like virtual reality and electromechanical assisted gait training, can be useful in children. A recent review showed most effective methods are CIMT, forced use therapy, repetitive transcranial magnetic stimulation, functional electrical stimulation, and robotics.¹⁷

Implanted brain-computer interface is an emerging technology in adults and a recent pilot study showed benefit in improving limb function.¹⁸

Gaps in the Evidence-Based Knowledge

Extensive pediatric research in the field of pediatric stroke is lacking due to relative low incidence of stroke in the pediatric population. Some of the reasons include the difference in clinical presentation based on symptoms and age, making the diagnosis more challenging. There are various risk factors (e.g. sepsis, dehydration, hypoxia, congenital heart disease, sickle cell disease) that can lead to pediatric stroke, and the low prevalence making it difficult to perform large studies.⁴

Mechanical thrombectomy reports have some inconsistency and may not be as beneficial as some reports imply.⁸

The gene ADAMTS encodes for parts of the extracellular matrix and recent evidence suggests that some of the genes, like ADAMTS12, can lead to increased risk of pediatric stroke.¹⁹ Other genes that may affect pediatric stroke include: ANCC6 and COL4A1. These studies suggest that there are a number of genetic variances that can increase likelihood of pediatric stroke.²⁰

Early studies showed Pediatric Stroke is an infrequent, but important, complication of COVID-19. ²¹ Case reports have described pediatric strokes 3-4 weeks after a COVID-19 infection, but the overall effect is unknown.²²

References

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7. Amlie-Lefond C, Shaw DWW, Cooper A, Wainwright MS, Kirton A, Felling RJ, Abraham MG, Mackay MT, Dowling MM, Torres M, Rivkin MJ, Grabowski EF, Lee S, Kurz JE, McMillan HJ, Barry D, Lee-Eng J, Ichord RN. Risk of Intracranial Hemorrhage Following Intravenous tPA (Tissue-Type Plasminogen Activator) for Acute Stroke Is Low in Children. Stroke. 2020 Feb;51(2):542-548. doi: 10.1161/STROKEAHA.119.027225. Epub 2019 Dec 17. Erratum in: Stroke. 2020 Feb;51(2):e46. PMID: 31842706
8. Bhatia K, Kortman H, Blair C, Parker G, Brunacci D, Ang T, Worthington J, Muthusami P, Shoirah H, Mocco J, Krings T. Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series. J Neurosurg Pediatr. 2019 Aug 9:1-14. doi: 10.3171/2019.5.PEDS19126. Epub ahead of print. PMID: 31398697.
9. Adams RJ, Brambilla D; Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005 Dec 29;353(26):2769-78. doi: 10.1056/NEJMoa050460. PMID: 16382063.
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16. Metzler MJ, Haspels E, Brunton L, Andersen J, Pritchard L, Herrero M, Hodge J, Kirton A. Goals of children with unilateral cerebral palsy in a brain stimulation arm rehabilitation trial. Dev Med Child Neurol. 2020 Dec 24. doi: 10.1111/dmcn.14763. Epub ahead of print. PMID: 33368181.
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19. Witten A, Rühle F, de Witt M, Barysenka A, Stach M, Junker R, Nowak-Göttl U, Stoll M. ADAMTS12, a new candidate gene for pediatric stroke. PLoS One. 2020 Aug 20;15(8):e0237928. doi: 10.1371/journal.pone.0237928. PMID: 32817637; PMCID: PMC7446847.
20. Grossi A, Severino M, Rusmini M, Tortora D, Ramenghi LA, Cama A, Rossi A, Di Rocco M, Ceccherini I, Bertamino M; Gaslini Stroke Study Group. Targeted re-sequencing in pediatric and perinatal stroke. Eur J Med Genet. 2020 Nov;63(11):104030. doi: 10.1016/j.ejmg.2020.104030. Epub 2020 Aug 18. PMID: 32818659.
21. Beslow LA, Linds AB, Fox CK, Kossorotoff M, Zuñiga Zambrano YC, Hernández-Chávez M, Hassanein SMA, Byrne S, Lim M, Maduaka N, Zafeiriou D, Dowling MM, Felling RJ, Rafay MF, Lehman LL, Noetzel MJ, Bernard TJ, Dlamini N; International Pediatric Stroke Study Group. Pediatric Ischemic Stroke: An Infrequent Complication of SARS-CoV-2. Ann Neurol. 2020 Dec 17. doi: 10.1002/ana.25991. Epub ahead of print. PMID: 33332607.
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Original Version of the Topic

Edward Hurvitz, MD and Alecia Daunter, MD. Pediatric Stroke. 9/20/2013

Previous Revision(s) of the Topic

Rajashree Srinivasan, MD and Saylee Dhamdhere MD, UTSW. Pediatric Stroke. 8/8/2017

Sathya Vadivelu, DO, Joshua Kaseff, MS. Pediatric Stroke. 4/20/2021

Author Disclosures

Michael Nguyen, MD, MPH
Nothing to Disclose

Catherine Velasquez-Ignacio, MD
Nothing to Disclose

Mara Martínez-Santori, MD, MS
Nothing to Disclose

Josselyne Orbegoso, BS
Nothing to Disclose

Alyssa Miller, BS
Nothing to Disclose

Elizabeth Chan, MD
Nothing to Disclose