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«The Diagnosis of Brain Tumours in Children An evidence-based guideline to assist healthcare professionals in the assessment of children presenting ...»

-- [ Page 1 ] --

The Diagnosis of Brain

Tumours in Children

An evidence-based guideline to assist healthcare

professionals in the assessment of children

presenting with symptoms and signs that may be

due to a brain tumour

Quick Reference Guide

endorsed by the RCPCH

Version 3: March 2011

Contents of Quick Reference Guide

1. Guideline background, aim and scope p.1

2. Guideline summary p.3

3. Best practice p.4 Best practice – consultation p.4 Best practice – referral p.4 Best practice – imaging p.4 Best practice – feedback p.5

4. Predisposing factors p.6 p.7

5. Presentation and assessment of children who may have a brain tumour Presenting signs and symptoms p.7 History p.8 Assessment p.8 Summary table of presentation and assessment of children who may p.9 have a brain tumour

6. Signs and symptoms p.10 Headache p.10 Nausea and vomiting p.11 Visual symptoms and signs p.12 Motor symptoms and signs p.14 Growth and development p.16 Behaviour p.17 Statements in a pink box advise on indications for imaging Statements in a black box advise on presentations frequently associated with diagnostic difficulty

7. Further information p.18

1. Guideline background, aim and scope:

This quick reference guide summarises the recommendations in the “The Diagnosis of Brain Tumours in Children” guidelines. The complete guideline including methodology, evidence base and references can be viewed at and downloaded from www.rcpch.ac.uk/bpp.

Background Approximately 450 children are diagnosed with a brain tumour each year in the UK. Brain tumours are the commonest cause of cancer related death, with an annual mortality of nine per million (80 to 100 children annually in the UK).

60% of survivors are left with life-altering disability. It can be difficult for healthcare professionals to recognise when a child presents with the symptoms and signs of a brain tumour. Childhood brain tumours are relatively rare and have a very varied presentation. The symptoms and signs that precede diagnosis are diverse, fluctuate in severity and differ according to the tumour location and the developmental stage of the child. Many of the initial symptoms and signs of brain tumours are non-specific and mimic other more common and less serious disorders.

Children with brain tumours are frequently unwell for prolonged period before the diagnosis is made. In the UK, the median symptom interval (time between symptom onset and diagnosis) for childhood brain tumours is between 2.5 to 3 months, this is longer than that experienced by children in other countries. A prolonged symptom interval childhood CNS tumours is associated with an increased risk of life-threatening and disabling neurological complications at presentation and a worse cognitive outcome in survivors. It has a detrimental effect on professional relationships with patients and their families, and their subsequent psychological well-being.

The Diagnosis of brain tumours in children guideline was written to support healthcare professionals in the recognition and assessment of children and young people presenting with symptoms and sign that could be due to a brain tumour. It aims to reduce prolonged symptom interval experienced by many UK children diagnosed with a brain tumour.

Aim of the guideline

The guideline advises on the following:

1. The symptoms and signs that may occur in children with brain tumour

2. Assessment of children presenting with these symptoms and signs

3. Indications and waiting times for imaging children with these symptoms and signs

-1Scope Patient inclusion criteria The guideline is applicable to all children aged 0-18 years who present with symptoms and / or signs that could result from a brain tumour and are being reviewed by a healthcare professional.

Guideline users The guideline is intended to support the assessment and investigation by healthcare professionals of children who may have a brain tumour.

The guideline has been developed following careful consideration of the available evidence and has incorporated professional expertise via a Delphi consensus process. Healthcare professionals should use it to support their decision making when assessing children who may have an intracranial tumour. It does not however override the responsibility of a healthcare professional to make decisions appropriate to the condition of individual children.

There are 76 recommendations in total with 21 grade B recommendations.

Levels of evidence and grading of recommendations are explained below and are taken from SIGN, Scottish Intercollegiate Guideline Network (2000) [19].

–  –  –

Parents and their carers should be asked explicitly about their concerns in any consultation.

Strength of evidence 4 Recommendation grade D If a parent / carer expresses concerns about a brain tumour this should be reviewed carefully. If a brain tumour is unlikely the reasons why should be explained and arrangements made for review within 4 weeks.

Strength of evidence 4 Recommendation grade D If the patient, parent / carer and healthcare professional are not fluent in a common language an interpreter must be used for the consultation (www.languageline.co.uk).

Strength of evidence 4 Recommendation grade D Low parental educational level, social deprivation and lack of familiarity with the UK healthcare system may be associated with diagnostic delay. A lower threshold for investigation and referral may be appropriate in these situations.





Strength of evidence 4 Recommendation grade D Referral A primary healthcare professional who has a high index of suspicion regarding a possible brain tumour should discuss their concerns with a secondary health care professional the same day.

Strength of evidence 4 Recommendation grade D A child referred from primary care in which the differential diagnosis includes a possible space occupying lesion should be seen within two weeks.

Strength of evidence 4 Recommendation grade D Imaging A child in whom CNS imaging is required to exclude a brain tumour (potential diagnosis but low index of suspicion) should be imaged within 4 weeks.

–  –  –

MRI is the imaging modality of choice for a child who may have a brain tumour.

Strength of evidence 2++ Recommendation grade B If MRI is not available a contrast enhanced CT should be performed.

Strength of evidence 2++ Recommendation grade B Imaging results should be interpreted by a professional with expertise and training in central nervous system MR and CT imaging in children.

Strength of evidence 4 Recommendation grade D The need to sedate or anaesthetise a child for imaging should not delay imaging by more than 1 week.

Strength of evidence 4 Recommendation grade D Feedback Patients and their families should receive the provisional results of CNS imaging within 1 week of the investigation.

Strength of evidence 4 Recommendation grade D

-5Predisposing factors:

The following are all associated with an increased risk of childhood brain tumours. Their presence may lower the threshold for referral and

investigation:

–  –  –

-6Presentation and assessment of a child

with a potential brain tumour:

Presenting symptoms and signs The following symptoms and signs are all associated with childhood brain tumours. Their presence should alert the clinician to this possibility.

–  –  –

Symptoms and signs in childhood brain tumours may occur singularly or in combination.

Strength of evidence 2+ Recommendation grade C History

–  –  –

The initial symptoms of a brain tumour frequently mimic those that occur with many common childhood conditions Strength of evidence 2+ Recommendation grade C Symptoms frequently fluctuate in severity – resolution and then recurrence does not exclude a brain tumour Strength of evidence 4 Recommendation grade D Presentation depends upon the age of the child Strength of evidence 2++ Recommendation grade B A normal neurological examination does not exclude a brain tumour Strength of evidence 2+ Recommendation grade C

–  –  –

NOTE:

The initial symptoms of a brain tumour frequently mimic those that occur with many common childhood conditions Strength of evidence 2+; Recommendation grade C Symptoms frequently fluctuate in severity – resolution and recurrence does not exclude a brain tumour Strength of evidence 4; Recommendation grade D Presentation depends upon the developmental age of the child Strength of evidence 2++; Recommendation grade B A normal neurological examination does not exclude a brain tumour Strength of evidence 2+; Recommendation grade C

–  –  –

Consider a brain tumour in any child presenting with a new persistent headache. (A continuous or recurrent headache lasting for more than 4 weeks should be regarded as persistent) Strength of evidence 2++ Recommendation grade B Brain tumour headaches can occur at any time of the day or night Strength of evidence 2+ Recommendation grade C Children aged younger than 4 years, or those with communication difficulties, are frequently unable to describe headache; their behaviour e.g. withdrawal, holding head may indicate a headache.

Strength of evidence 4 Recommendation grade D In a child with a known migraine or tension headache a change in the nature of the headache requires reassessment and review of the diagnosis.

Strength of evidence 3 Recommendation grade D Delayed diagnosis has been associated with failure to reassess a child with migraine or tension headache when the headache character changes.

Strength of evidence 3 Recommendation grade D

CNS IMAGING (within a maximum of 4 weeks) REQUIRED FOR:

Persistent headaches that wake a child from sleep Strength of evidence 4 Recommendation grade D Persistent headaches that occur on waking Strength of evidence 4 Recommendation grade D A persistent headache occurring at any time in a child younger than 4 years Strength of evidence 4 Recommendation grade D

–  –  –

Early specialist referral for consideration of underlying causes including CNS causes is required for a child with persistent nausea and / or vomiting. (Nausea and / or vomiting that lasts for more than two weeks should be regarded as persistent) Strength of evidence 2++ Recommendation grade B CNS imaging (within a maximum of four weeks) is required for persistent vomiting on awakening (either in the morning or from a day time sleep). N.B. exclude pregnancy where appropriate.

Strength of evidence 4 Recommendation grade D

Delayed diagnosis has been associated with:

Attributing persistent nausea and vomiting to an infective cause (in the absence of corroborative findings e.g. contact with similar illness, pyrexia, diarrhoea).

Strength of evidence 3 Recommendation grade D

CNS IMAGING (within a maximum of 4 weeks) REQUIRED FOR:

Persistent vomiting on awakening (either in the morning or from a day time sleep) NB: exclude pregnancy where appropriate.

Strength of evidence 4 Recommendation grade D

–  –  –

Consider a brain tumour in any child presenting with a persisting visual abnormality. (Any visual abnormality lasting longer than 2 weeks should be regarded as persistent) Strength of evidence 2++ Recommendation grade B

Visual assessment must include assessment of:

–  –  –

If the assessing healthcare professional is unable to perform a complete visual assessment the child should be referred for assessment.

Strength of evidence 4 Recommendation grade D Children referred for visual assessment with symptoms or signs suggestive of a brain tumour should be seen within two weeks of referral.

Strength of evidence 4 Recommendation grade D Community optometry should refer any child with abnormal eye findings suggestive of a possible brain tumour directly to secondary care.

Strength of evidence 4 Recommendation grade D Consideration should be given to the appropriate place of assessment.

If appropriate community optometry expertise is not available, pre

–  –  –

Delayed diagnosis has been associated with:

Failure to fully assess vision in a young or uncooperative child Strength of evidence 4 Recommendation grade D Failure of communication between community optometry and primary and secondary care Strength of evidence 4 Recommendation grade D

CNS IMAGING (within a maximum of 4 weeks) REQUIRED FOR:

–  –  –

Reduction in visual acuity not attributable to an ocular cause Strength of evidence 4 Recommendation grade D Visual field reduction not attributable to an ocular cause Strength of evidence 4 Recommendation grade D

–  –  –

Consider a brain tumour in any child presenting with a persisting motor abnormality. Any motor abnormality lasting longer than two weeks should be regarded as persistent.

Strength of evidence 2++ Recommendation grade B Brain tumours may cause a deterioration or change in motor skills; this may be subtle e.g. change in hand or foot preference, loss of learned skills (computer games).

Strength of evidence 3 Recommendation grade D

Motor system assessment must include observation of:

–  –  –

Delayed diagnosis has been associated with:

Attributing abnormal balance or gait to middle ear disease in the absence of corroborative findings Strength of evidence 3 Recommendation grade D

–  –  –

Abnormal gait and / or coordination (unless local cause) Strength of evidence 4

CNS IMAGING (within a maximum of 4 weeks) REQUIRED FOR:



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