Stroke affects several hundred children in the UK each year and is among the 10 most common causes of death in childhood.1 One in 10 children with sickle cell disease will have a stroke before their 20th birthday, and a quarter will have evidence of brain injury caused by either overt or clinically ‘silent’ stroke. Many children who have a stroke have another medical condition and therefore are already vulnerable to adverse neurodevelopmental effects.2,3
There is a lack of awareness among both healthcare professionals and the public that children can be affected by stroke. An important aim of the paediatric stroke guidelines recently published by the Royal College of Physicians is to raise awareness and provide information about this condition.
Injury to the developing brain can affect the individual’s functioning for the rest of his or her life. The plasticity of the child’s brain is a concept that is widely discussed both in the popular and scientific literature. However, although the most obvious usually motor impairments resulting from childhood stroke improve to a remarkable extent in some cases, the wider consequences of acquired injury to the developing nervous system are often relatively invisible.
Such difficulties particularly affect cognition or behaviour; problems may not be apparent in childhood but may emerge later, as the demands on the individual increase. Thus, childhood stroke has potential longterm personal, social and economic consequences.
In addition to discussing the rationale for, and methodology of, the guidelines, this article will highlight some important recommendations of particular relevance to primary healthcare professionals.
Guidelines rationale and methodology
In 2000, the Royal College of Physicians published clinical guidelines for the care of adults affected by stroke. The RCP was subsequently approached by a number of individuals and professional bodies requesting information relating to childhood stroke.
A multidisciplinary working group was established, with input from the British Paediatric Neurology Association, to work in collaboration with the Clinical Effectiveness and Evaluation Unit of the RCP to produce evidence-based guidelines for the diagnosis, management and rehabilitation of childhood stroke.
The guidelines were formulated according to the principles specified by the Appraisal of Guidelines Research and Evaluation (AGREE) collaboration.4 The existing adult guidelines were used as a basis for the paediatric guidelines and specific additional issues relevant to children (for example, return to school) were also considered.
The literature was systematically searched and appraised. Members of the group brought with them their own expertise and that of their professional bodies. This was particularly important as there is a relative paucity of research evidence in this area on which to base clinical guidelines.
Recommendations were graded using the grading scheme recommended by SIGN 5 (Figure 1, below). Many of the guideline’s recommendations are in line with those of the recently published National Service Framework for Children.6
|Figure 1: Grades of recommendations and strength of evidence|
Children affected by stroke and their parents were invited to attend a structured workshop, and thematic analysis of the findings identified areas they considered important for the guidelines to address. The document was externally peer reviewed by stakeholders and appraised and approved by the Quality of Practice Committee of the Royal College of Paediatrics and Child Health.
Organisation of services
Whereas the model of adult stroke is to develop specialist stroke services, the relative rarity of childhood stroke means that existing primary, secondary and tertiary systems of child health will be involved in the care of the child affected by stroke.
A key recommendation relating to service organisation is that all children affected by acute stroke should be referred to a consultant paediatric neurologist (Box 1, below). This may entail discussion with the tertiary unit, or transfer from acute secondary care to a tertiary unit.
|Box 1: Service organisation|
Multidisciplinary assessment and coordination and provision of longterm care are usually undertaken by community child health services. The guidelines encourage any member of the multidisciplinary team to seek advice from tertiary specialists if necessary, but stress that ongoing management and coordination between health and educational services should be the responsibility of the secondary level paediatric services.
Families and carers
Stroke is completely unexpected in a child, and parents and children find the diagnosis devastating. This is compounded by the lack of awareness of childhood stroke among healthcare professionals, which often means that the family themselves must actively seek out information, treatment, rehabilitation and educational support.
There is a version of the guideline for families of children affected by stroke, which includes a list of organisations providing support and information.
The range of risk factors encountered in paediatric patients differs from those contributing to stroke risk in adults and thus a different diagnostic approach is required (Box 2, below).
|Box 2: Presentation and diagnosis|
The most common clinical presentation of acute childhood stroke is hemiparesis. Focal signs may be minimal or absent in infants. Accurate clinical assessment can be challenging, especially in very young children,and it is important to consider the diagnosis, particularly in high-risk groups, for example children with congenital heart disease.
Brain imaging is mandatory to identify the underlying pathology (ischaemia or haemorrhage) accurately; however, investigations will usually be undertaken in hospital.
Acute medical treatment
There are no clinical trials to guide acute medical management of childhood stroke. Acute treatment will usually be undertaken in hospital, but is relevant to secondary prevention and longer-term management. As yet, thrombolysis is not recommended for the treatment of acute arterial ischaemic stroke in children.
Aspirin is used in both acute and longer-term treatment of arterial ischaemic stroke in children (Box 3, below). Parents often feel concerned about this, given the recent warnings regarding use of aspirin in patients under the age of 16 years. The consensus of the working party, and indeed other expert bodies, is that the potential benefits of aspirin in this group of children outweigh the small risk of Reye’s syndrome and justify its use in this clinical context.
|Box 3: Acute medical treatment|
However, it is important that parents are aware of the existence of Reye’s syndrome and, in the event of acute illness in their child, are advised to alert medical practitioners that their child is taking aspirin.
The possibility of a stroke recurring is a major concern for children and their families. Arterial ischaemic stroke recurs in 6 to 20% of children and in over 60% of children with sickle cell disease. There is little current evidence regarding the efficacy of secondary prevention strategies,7 but this is likely to change because the need for multi-centre trials is gaining momentum (Box 4, below).
|Box 4: Secondary prevention|
Rehabilitation and return to school
There is a paucity of rehabilitation services for children with acquired brain injury in the UK; most of these children will be managed by services primarily targeted at management of children with congenital, often relatively slowly changing, conditions, most commonly cerebral palsy.
All healthcare professionals should be aware that the functional consequences of acquired brain injury are likely to change with growth and maturity. Cognition and emotion are commonly affected, but impairments of this type may be relatively invisible to professionals unless specifically sought. For example, although a third of parents say that their child’s behaviour has been affected after a stroke, behaviour is infrequently assessed.
Coordination between professionals is important to minimise duplication of appointments and assessments for the child and family. A key worker can play an important role in coordinating care.
Returning to school is a major milestone in the child’s recovery, and a positive experience is likely to enhance reintegration. Good communication is important, and a named teacher (usually the special educational needs coordinator) and a member of the community child health team should become involved before the child returns to school.
Transition of care from paediatric to adult services
The process of transition from paediatric to adult services depends on the current and future needs of the individual, and will take place between the ages of 16 and 19 years (Box 5, below).
|Box 5: Transition of care|
Children with active medical needs surveillance and management of an active condition such as cardiac disease or sickle cell disease or management of chronic disability will need to have their care transferred.
There are different models of transition, for example transfer from paediatric to adult subspecialist or coordination of transfer by the GP,8 and the most appropriate model should take into consideration the young person’s needs as well as the services available.
Primary healthcare professionals should be aware of the potentially wide-ranging effects of childhood stroke and that functional difficulties may emerge after a ‘silent’ interval.
Stroke in Childhood. Clinical guidelines for diagnosis, management and rehabilitation can be downloaded from the RCP website: www.rcplondon.ac.uk
- Fullerton HJ, Chetkovich DM, Wu YW et al. Deaths from stroke in US children, 1979 to 1998. Neurology 2002; 59: 34-9.
- Lanthier S, Carmant L, David M et al. Stroke in children: the coexistence of multiple risk factors predicts poor outcome.Neurology 2000;54: 371-8.
- Ganesan V, Prengler M, McShane MA et al. Investigation of risk factors in children with arterial ischemic stroke. Ann Neurol 2003; 53: 167-73.
- Scottish Intercollegiate Guidelines Network. SIGN 50. A guideline developers’ handbook. Edinburgh: SIGN, 2001.
- Department of Health. National Service Framework for Children, Young People and Maternity Services. London: DoH, 2004.
- Sträter R, Kurnik K, Heller C et al.Aspirin versus low-dose low-molecular-weight heparin: antithrombotic therapy in pediatric ischemic stroke patients: a prospective follow-up study. Stroke 2001; 32: 2554-8.
- Tuffrey C, Pearce A.Transition from paediatric to adult medical services for young people with chronic neurological problems. J Neurol Neurosurg Psychiatry 2003; 74: 1011-3.