GPs have key role in long-term management of childhood stroke

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The new Royal College of Physicians guidelines on stroke in children will help GPs provide holistic care throughout the child’s development, as Dr Vijeya Ganesan explains

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

All children with acute stroke should be referred to, or have their management discussed with, a consultant paediatric neurologist ()
Where specialist expertise is not available locally, professionals from all disciplines are encouraged to liaise with, and obtain advice from, colleagues in specialist centres regarding the acute assessment and management of the child affected by stroke ()
The medical, social, emotional and educational needs of the child affected by stroke should be considered early and systematically assessed in a coordinated manner when planning their subsequent care (D)
The longer-term management of the child affected by stroke should be coordinated by a consultant paediatrician ()
A multidisciplinary team with expertise in the care of children with neurological conditions should be involved in the management of the child affected by stroke. Whilst this may initially be at tertiary level it is essential that the relevant secondary level child development service is involved from an early stage ()
A key worker should be appointed to coordinate the package of care, ensure its delivery and to act as a central point of contact for the family (D). The key worker and their role should be explained to the family

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.

Acute diagnosis

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

All children with a clinical presentation of stroke should be a consultant paediatrician ()
Cross-sectional brain imaging is mandatory in children presenting stroke (C)
Any new neurological symptoms or signs in children with sickle should be evaluated as potentially being due to stroke ()

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

Aspirin (5 mg/kg/day) should be given once there is radiological confirmation of arterial ischaemic stroke, except in patients with evidence of intracranial haemorrhage on imaging and those with sickle cell disease ()
In children with sickle cell disease and arterial ischaemic stroke:
- urgent exchange transfusion should be undertaken to reduce HbS to <30% and raise haemoglobin to 10-12.5 g/dl ()
- if the patient has had a neurological event in the context of severe anaemia (e.g. splenic sequestration or aplastic crisis), or if exchange transfusion is going to be delayed for more than 4 hours, urgent top-up blood transfusion should be undertaken ()
Providing there is no haemorrhage on brain imaging, anticoagulation should be considered in children with:
- confirmed extracranial arterial dissection associated with arterial ischaemic stroke ()
- cerebral venous sinus thrombosis (C)
The decision to use anticoagulation in children with arterial ischaemic stroke who have a cardiac source of embolism should be discussed with a consultant paediatric cardiologist and paediatric neurologist ()

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.

Secondary prevention

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

Patients with cerebral arteriopathy other than arterial dissection or moyamoya syndrome or those with sickle cell disease should be treated with aspirin (1-3 mg/kg/day) ()
Anticoagulation should be considered:
- until there is evidence of vessel healing, or for a maximum of 6 months, in patients with arterial dissection ()
- if there is recurrence of arterial ischaemic stroke despite treatment with aspirin ()
- in children with cardiac sources of embolism, following discussion with the cardiologist managing the patient ()
- until there is evidence of recanalisation or for a maximum of 6 months after cerebral venous sinus thrombosis ()
In children with sickle cell disease:
- regular blood transfusion (every 3 to 6 weeks) should be undertaken to maintain the HbS% <30% and the Hb between 10 and 12.5 g/dl (C)
- transfusion may be stopped after 2 years in patients who experienced stroke in the context of a precipitating illness (e.g. aplastic crisis) and whose repeat vascular imaging is normal at this time (C)
- after 3 years a less intensive regimen maintaining HbS <50% may be sufficient for stroke prevention (C)
- those who cannot receive regular blood transfusions because of allo-immunisation, auto-antibody formation, lack of vascular access or non-compliance with transfusion or chelation may be considered for treatment with hydroxyurea (C)
Children with moyamoya syndrome (including those with sickle cell disease) should be referred for evaluation to a centre with expertise in evaluating patients for surgical revascularisation (D)
Children with sickle cell disease who have had a stroke should be referred to a specialist centre for consideration of bone marrow transplantation (B)
Advice should be offered regarding preventable risk factors for arterial disease in adult life, particularly smoking, exercise and diet (D)
Blood pressure should be measured annually to screen for hypertension ()
Patients who are found to have a prothrombotic tendency should be referred to a haematologist ()

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

A named professional should take responsibility for arranging an introduction to adult health services ()
A flexible approach to the timing of this transfer needs to be considered which takes into account the young person’s readiness, current health status and links to other social transitions such as leaving school (D)
A multi-agency transition plan should be formulated for young people with special educational needs, with input from health, education, social services and the young person, to plan transition into further education, training or employment (D)
A named professional should take responsibility for coordinating the transition plan and ensuring delivery of services (D)

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

References

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.
www.agreecollaboration.org
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.