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Retinoblastoma: would you recognise it?

27th September 2018

A guest blog by Petra Maxwell, Information & Research Manager at the Childhood Eye Cancer Trust (CHECT).

Petra Maxwell, Information & Research Manager, Childhood Eye Cancer Trust

Petra Maxwell, Information & Research Manager, Childhood Eye Cancer Trust

Take five minutes during National Eye Health Week (24-30 September 2018) to learn the main signs and symptoms of this rare condition. Your swift action could save a child’s life.

The mother of a 10-week old baby shows you a photo she took recently, in which a white glow can be observed in one of the baby’s eyes, with the more normal ‘red eye’ effect in the other. She asks you if it is anything to be concerned about. What do you do?

In most cases this will merely be the result of the camera flash reflecting off the optic nerve, and nothing more. However, for one child a week in the UK, this could be a warning sign that the child has developed the rare and aggressive eye cancer retinoblastoma.

Retinoblastoma (Rb) affects babies and children mainly under the age of five[i], in either one or both eyes. Although this cancer has a very high survival rate of over 98% in the UK, many children live with the consequences of a delayed diagnosis. Late diagnosis for a child with Rb can mean loss of one or both eyes, life with an artificial eye, a visual impairment or, in some cases, complete blindness. Over half of children diagnosed with Rb will need their eye removed to save their life.

As the key professional in early years, health visitors play an important role in this referral pathway.

The signs and symptoms of Rb are often very subtle and confined to the eye, and most often detected by parents [ii]. The child usually appears well in themselves, so it is important to pay attention to parental concern about their child’s eyes

Signs and symptoms

The two main symptoms of retinoblastoma are:

  • A white glow in the pupil or a white reflection in the pupil in flash photographs.
Image courtesy of CHECT

Image courtesy of CHECT

  • A new squint. Between 2012 and 2017, 33% of children diagnosed with Rb had a squint as a symptom.[iii]
Image courtesy of CHECT

Image courtesy of CHECT

Other symptoms include:

  • A change in the colour of the iris or part of the iris
  • Inflammation, redness or increased pressure in or around the eye without an infection.
  • Deterioration of vision in one or both eyes.
  • Nystagmus (wandering eyes)
  • Parental history of retinoblastoma – the condition can be heritable so children of an affected parent with retinoblastoma must be screened from birth
  • Parental concern over vision or eye appearance.

Referral

If parents mention any of the signs and symptoms listed above the child must be examined urgently to rule out retinoblastoma.

Local referral protocols vary: in most cases health visitors would refer the child to their GP or optometrist. It is crucial that parents are made aware of the need for their child’s eyes to be examined as soon as possible. This examination must include a red reflex test. If the GP is unable to perform the red reflex test to rule out Rb or has any concerns, then the child must be referred to an ophthalmologist urgently.

The speed of referral is vitally important, as a swift referral can reduce the long-term impact of the disease and treatment of the baby or child. If a parent continues to have concerns about their child’s eye, the child should be taken back to the GP / optometrist.

Why not take 30 seconds to test your knowledge of the signs and symptoms of retinoblastoma?

To learn more about retinoblastoma visit www.chect.org.uk/hv

Petra Maxwell, Information & Research Manager at the Childhood Eye Cancer Trust (CHECT)

References

[i] Draper G, Sanders B, Brownbill P, Hawkins M (1992) Patterns of risk of hereditary retinoblastoma and applications to genetic counselling.  Br J Cancer 66(1): 211-9

[ii] Wallach M, Balmer A, Munier F et al (2006). Shorter time to diagnosis and improved stage at presentation in Swiss patients with retinoblastoma treated from 1963 to 2004. Paeditrics 118(5): e1493-8

[iii] Childhood Eye Cancer Trust (2018) Pathways to Diagnosis 2012 – 2017