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The diagnosis of brain tumours in children: a guideline for healthcare professionals

Referral from primary care

  • High risk of tumour—SAME DAY referral to secondary care
  • Lower risk*—specialist assessment within 2 weeks 

Imaging

  • High risk of tumour—URGENT CNS imaging
  • Lower risk*—CNS imaging within 4 weeks

*Lower risk=CNS tumour in differential diagnosis, low index of suspicion

Consider a brain tumour in any child presenting with 

  • Headache 
  • Nausea and/or vomiting 
  • Visual signs and symptoms: 
    • reduced visual acuity and/or fields 
    • abnormal eye movements 
    • abnormal fundoscopy 
  • Motor symptoms and signs:
    • abnormal gait
    • abnormal co-ordination
    • focal motor weakness 
  • Growth and endocrine symptoms:
    • growth failure (weight/height)
    • delayed, arrested or precocious puberty
    • galactorrhoea 
    • primary/secondary amenorrhea 
  • Increasing head circumference 
  • Behavioural change 
  • Diabetes insipidus 
  • Seizures (see www.nice.org.uk/guidance/qs27
  • Altered consciousness (see www.rcpch.ac.uk/system/files/protected/page/Decon%20guidelines.pdf

Assess these children with 

  • History: associated symptoms, any predisposing factors 
  • Examination of: 
    • visual system 
    • motor system 
    • height and weight 
    • head circumference (<2 years of age) 
    • pubertal status 

IF TWO OR MORE SYMPTOMS—they need a SCAN 

Ask about common predisposing factors 

  • Personal or FH of brain tumour, sarcoma, leukaemia, or early onset breast cancer 
  • Neurofibromatosis 
  • Tuberous sclerosis 
  • Other familial genetic syndromes 

Assessment pitfalls 

  • Initial symptoms of brain tumour can mimic other common illnesses 
  • Symptoms frequently fluctuate—resolution then recurrence does not exclude a brain tumour 
  • A normal neurological examination does not exclude a brain tumour 
  • Language difficulties—use interpreter 

Headaches 

  • Consider a brain tumour in any child with a new, persistent headache 
  • Headache in isolation, unlikely to be a brain tumour 
  • Brain tumour headaches occur at any time of day 
  • Children aged younger than 4 years may not be able to describe a headache—observe behaviour 

CNS imaging required with 

  • Persistent headache that wakes a child from sleep 
  • Persistent headache that occurs on waking 
  • Persistent headache in a child under 4 years of age
  • Confusion or disorientation with a headache 
  • Persistent headache with 1 or more other symptoms 

Common pitfalls 

  • Failure to reassess a child with a migraine or tension headache when the headache character changes 

Persistent=continuous or recurrent headache present for more than 4 weeks 

Nausea and/or vomiting 

  • Consider a brain tumour in any child with persistent nausea and/or vomiting 
  • Head circumference should be measured and plotted in children under 2 years of age with persistent vomiting 

CNS imaging required with 

  • Persistent vomiting on waking (NB: exclude pregnancy where appropriate) 
  • Persistent nausea/vomiting with one or more other symptom 

Common pitfalls 

  • Failing to consider a CNS cause for persistent nausea and vomiting 

Persistent=nausea and/or vomiting present for more than 2 weeks 

Visual signs and symptoms 

  • Consider a brain tumour in any child with persistent§ visual abnormality 
  • Visual assessment requires assessment of: 
    • visual acuity 
    • eye movements 
    • pupil responses 
    • optic disc appearance 
    • visual fields (≥ 5 years of age) 
  • Pre-school and unco-operative children should be assessed by hospital eye service within 2 weeks of referral 
  • Parent concern alone warrants referral for visual assessment 

CNS imaging required with 

  • Papilloedema
  • Optic atrophy
  • New onset nystagmus 
  • Reduction in visual acuity not due to refractive error 
  • Visual field reduction
  • Proptosis
  • New onset paralytic squint
  • Visual symptom with 1 or more other symptom

Common pitfalls 

  • Failure to fully assess vision—REFER IF NECESSARY
  • Failure of communication between community optometry and primary and secondary care

§ Persistent=visual abnormality present for more than 2 weeks 

Head circumference 

  • Consider a brain tumour in any child under two years with an increasing head circumference outside the normal range in comparison to their height and weight 
  • Careful assessment of other signs and symptoms of a brain tumour should be undertaken in these babies 

CNS imaging required with 

  • Rapid rate of head circumference growth crossing centiles 
  • Increasing head circumference with any other associated symptoms 

Common pitfalls 

  • Failing to measure and monitor head circumference in a baby or young child with persistent vomiting 

Motor symptoms and signs 

  • Consider a brain tumour in any child with persistingl motor abnormality 
  • Motor assessment requires history or observation of: 
    • sitting and crawling in infants 
    • walking and running 
    • handling of small objects 
    • handwriting in school age children 
  • Brain tumours can cause a loss or change in motor skills and this can be subtle e.g. ability to play computer games 

CNS imaging required with 

  • Regression in motor skills 
  • Focal motor weakness 
  • Abnormal gait/co-ordination (unless local cause) 
  • Bells palsy with NO improvement within 4 weeks 
  • Swallowing difficulties (unless local cause) 
  • Head tilt/torticollis (unless local cause) 
  • Motor symptom with one or more other symptom 

Common pitfalls 

  • Attributing abnormal gait/balance to middle ear disease with no corroborating findings 
  • Failure to identify swallowing difficulties and aspiration as a cause of recurrent chest infections 

l Persistent=motor abnormality present for more than 2 weeks 

Growth and endocrine 

  • Consider a brain tumour in any child with any combination of growth failure, delayed/arrested puberty and polyuria/polydipsia 
  • Early specialist assessment if required for:
    • precocious puberty/delayed or arrested puberty
    • growth failure
    • galactorrhoea
    • primary or secondary amenorrhoea

CNS imaging required with 

  • Growth or endocrine symptom with 1 or more other symptoms

Common pitfalls 

  • Failing to consider a CNS cause in children with weight loss and vomiting
  • Failure to consider diabetes insipidus in children with polyuria and polydipsia

Behaviour 

  • Consider a brain tumour in any child with new onset lethargy, mood disturbance, withdrawal or disinhibition 

Common pitfalls 

  • Failing to consider a physical cause for behavioural symptoms 

Presenting symptoms of brain tumours by sub-specialty 

Supratentorial tumours can cause change in personality, mood or disinhibition. They can also cause symptoms of anorexia. A brain tumour needs to be considered as part of the differential diagnosis

Psychiatry  

  • Anorexia   
  • Behavioural change   
  • Depression 
  • Psychosis  

Central tumours such as optic pathway glioma are slow growing and will present with progressive visual symptoms that may present to an ophthalmologist

Ophthalmology 

  • Papilloedema 
  • Decreased visual acuity 
  • Nystagmus/Parinauds 
  • Diplopia 
  • Squint 
  • Visual field defect 
  • Blindness 
  • Ptosis 
  • Proptosis 
  • Ocular palsies 
  • Ophthalmoplegia 

Head tilt or torticollis can be caused by a posterior fossa tumour. These symptoms may present to ENT specialists as head tilt and torticollis have other common ENT causes 

Ear, nose and throat  

  • Dizziness  
  • Vertigo  
  • Torticollis  
  • Head tilt  
  • Hearing loss  
  • Tinnitus

A young child with hydrocephalus caused by a brain tumour will have an increasing head circumference and developmental delay or regression

Community paediatrics 

  • Developmental delay  
  • Developmental regression  
  • Increasing head circumference  

A child with hydrocephalus caused by a brain tumour will have persistent vomiting. In infants where the sutures are not yet fused there will be no other signs of hydrocephalus aside from macrocephaly

Gastroenterology  

  • Nausea and vomiting  
  • Abdominal pain  
  • Reflux  
  • Failure to thrive  
  • Dysphagia  

A supratentorial cortical tumour will present with focal neurological signs such as weakness

Neurology  

  • Seizures  
  • Motor weakness  
  • CN palsies  
  • Ataxia/cerebellar  
  • Focal neurological deficits  

Central tumours such as a craniopharyngioma are slow growing and will present with abnormal growth or precocious or delayed puberty. These children may also have visual symptoms

Endocrinology 

  • Growth problem 
  • Hypo-pit/pituitary dysfunction 
  • Diabetes insipidus 
  • Precocious or delayed puberty 
  • Menstrual irregularities 
  • Galactorrhoea 
  • Gynaecomastia 
  • Cushing’s 
  • Obesity/weight gain 

Recurrent respiratory infections can occur secondary to aspiration caused by a bulbar palsy

Respiratory  

  • Recurrent chest infections  
  • Apnoeas  

full guideline available from…

www.headsmart.org.uk

HeadSmart. The diagnosis of brain tumours in children: a guideline for healthcare professionals. Quick reference poster. January 2017.

HeadSmart. Presenting symptoms of brain tumours by sub-specialty. Sub-specialty poster. January 2017.

First included: April 2018.