7-year-old Tom presents to the emergency department with a headache and vomiting. The headache has been getting worse over two weeks and wakes him up at night. His parents are worried he might have an infection.
How common are brain tumours in children?
Brain tumours make up a quarter of all childhood cancers and sadly more children die of brain tumours than any other childhood cancer. The impact on children and their families is tragic.
How do brain tumours present?
Symptoms are often non-specific and may mimic many common childhood illnesses, such as viral infection. Headaches and other symptoms are linked to the size, speed of growth, and position of the tumour and become worse as the tumour progresses. Sadly, deaths occur due to delayed presentations with raised intracranial pressure.
How long does it take to get a diagnosis?
The longer it takes for a diagnosis, the worse the outcome is. The median total diagnostic interval (TDI) – the time from the start of symptoms to the confirmation of diagnosis – is 14.4 weeks in the UK. This is three times longer than in the US. Although the reasons for this are not clear, it may bed related to disease awareness, coupled with availability of imaging, and delayed referral times in the UK.
What is HeadSmart?
HeadSmart was created due to the prolonged TDI. The aim is to separate out children whose parents can be reassured, those who need urgent review, and those that need imaging. The public awareness campaign, decision support tool, and associated guideline are a partnership between the Children’s Brain Tumour Research Centre in Nottingham, the Royal College of Paediatrics and Child Health, and the Brain Tumour Charity. The purpose of HeadSmart‘s population-based approach is to raise awareness, improve early recognition, and thus shorten the TDI for children with brain tumours.
How was HeadSmart developed?
HeadSmart was developed in a three-step process. Step one looked at all relevant studies in the form of a systematic review and meta-analysis. The second step was a workshop incorporating the opinions of the multi-disciplinary team, the patient and their families. The final stage was a Delphi consensus process. This is a series of questionnaires with expert feedback designed to come to an agreed outcome.
The systematic review and meta-analysis included 148 papers. The reviewer’s used rigorous exclusion criterion to ensure studies contained sufficient data and only involved children. There were some limitations. Some used symptom clusters rather than using individual symptoms. This made pooled comparison difficult. The Delphi process allowed a structured and controlled discussion between experts reducing bias. While there were some limitations, the large analysis and structured approach to consultation showed the patterns of presentation and facilitated the creation of consensus guidelines.
What are the red flags in children?
Parents and carers of children presenting to the Emergency Department may be reluctant to tell you that they are worried about a brain tumour. It might be better to explicitly ask, “Is there anything in particular that you are worried about?” Often they can be reassured and the patient discharged with appropriate information, sign-posting and a safety net. If you are considering the diagnosis then this should prompt a thorough history and examination. Red flags are shown in the figure below.
Certain predisposing factors increase the risk of brain tumours and lower the threshold for referral and investigation. These include a personal or family history of brain tumours, leukaemia, sarcoma or early onset breast cancer, prior radiation to the brain, or neurocutaneous conditions such as tuberous sclerosis.
How should we investigate children with red flags?
Children needing imaging should have an MRI or contrast CT with MRI as the imaging of choice. The HeadSmart Quick Reference provides further details and guides decisions based upon head circumference, growth and endocrine changes, as well as behavioural changes.
Specific red flags mandate imaging.
HeadSmart helps to determine which patients need imaging and recommends that this is carried out, with a report available, within 4 weeks. The need for sedation for imaging should not be a reason for delay. The decision to image as an inpatient, or as an outpatient (within the four week timeframe), will be dependent on the clinical situation as well as local resources.
- Failing to reassessing children with migraine or changing tension headache
- Failing to assess vision
- Not doing a thorough assessment if there is a change in behaviour
- Not considering the diagnosis in children with persistent nausea and vomiting
- Not considering diabetes insipidus in children with excessive thirst or urination
- Thinking that vomiting is due to infection without any supporting findings
- Thinking that abnormal balance or funny walking is because of middle ear disease when there are no supporting findings
Has HeadSmart made a difference?
The impact of HeadSmart on total diagnostic interval was explored in a 2015 study involving 18 children’s cancer centres. Between 2011 and 2013, the TDI has almost halved, from 14.4 weeks to 6.7 weeks post-HeadSmart.
Although this study is limited in its cross-sectional design, a randomised control trial would not neither feasible nor ethical. Lack of more recent data is another limitation. Increasing public and professional awareness, coupled with the introduction of community and hospital champions, and clinician training is likely to have further reduced the TDI. Further research is needed.
As clinicians we should be using the HeadSmart tool. The public-awareness campaign has struck the balance between raising awareness whilst avoiding public alarm, and the tool has utility in helping to reassure and provide a safety net for families. The tool greatly aids in helping to identify red flags and support decision making on imaging, whilst evidence has shown there has been a measurable change in overall TDI since the introduction of HeadSmart. We should recognise that the more people who are aware and the more the tool is used, the better the outcome for children and their families.
Take home messages
- Use HeadSmart’s decision support tool and guidelines
- Be aware of headache red flags and other symptoms of brain tumours
- Refer urgently when there are concerns
- Arrange imaging appropriately
- Avoid the common pitfalls
- Provide a safety net and information leaflet for children being discharged from the emergency department. You may also want to signpost to the symptoms diary.
Informed by the HeadSmart decision tool, a CT head with contrast is organized in the emergency department. Tom has a brain tumour. He is admitted to the ward and later starts chemotherapy.
Cancer Research UK. Childhood cancer incidence statistics. 2016. Accessed online at https://www.cancerresearchuk.org/health-professional/cancer-statistics/childrens-cancers/incidence#heading-Three on 09/04/2022
HeadSmart Be Brain Tumour Aware. A new clinical guideline from the Royal College of Paediatrics and Child Health with a national awareness campaign accelerates brain tumor diagnosis in UK children–“HeadSmart: Be Brain Tumour Aware”. Neuro Oncol. 2016;18(3):445-454. doi:10.1093/neuonc/nov187
HeadSmart Be Tumour Aware. HeadSmart Decision Support Tool. Accessed online at https://www.headsmart.org.uk/clinical/decision-support-tool/ on 09/04/2022
HeadSmart Be Tumour Aware. HeadSmart Symptoms Diary. Accessed online at https://assets.headsmart.org.uk/live/media/filer_public/70/7c/707c7fdf-1cad-4a23-ac77-d01666f4a800/symptoms_diary_a4_4pp_interactive_rgb_nb.pdf on 09/04/2022
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Raucci U, Della Vecchia N, Ossella C, et al. Management of Childhood Headache in the Emergency Department. Review of the Literature. Front Neurol. 2019;10:886. doi:10.3389/fneur.2019.00886
Royal College of Paediatric and Child Health. The Brain Pathways
Guideline: a guideline to assist healthcare professionals in the assessment of children who may have a brain tumour; 2017. Accessed online at https://www.headsmart.org.uk/clinical/clinical-guideline/
The brain tumour charity. Sophie’s Smile. Accessed online at https://www.thebraintumourcharity.org/get-involved/our-supporter-groups/supporter-groups/groups/the-sophies-smile-fund/
The brain tumour charity. A cure can’t wait. Accessed online at https://www.thebraintumourcharity.org
Wilne, S. et al. Presentation of childhood CNS tumours: a systematic review and meta-analysis. The Lancet Oncology, 2007. 8(8): 685-695.