This page offers factual information for medical professionals which has been verified by the clinical teams at the two NHS retinoblastoma treatment centres in the UK – The Royal London Hospital and Birmingham Children’s Hospital. It contains advice on recognising the signs of retinoblastoma (Rb) as well as vital information regarding the care of adult patients who had Rb as a child.
There are six main warning signs of retinoblastoma – a child with Rb could have one or more of these signs. Retinoblastoma is rare but is a fast-growing cancer and early diagnosis is crucial to preserving the sight and the life of the child. Following a campaign by the Childhood Eye Cancer Trust the warning signs will now be listed as a reason to seek medical advice in all new copies of the Personal Child Health Record (red book).
What to look out for:
A child with retinoblastoma (Rb) usually shows no other signs of illness. The symptoms of Rb can be very subtle so it is extremely important to take parents’ concerns seriously when they report seeing something unusual in their child’s eye or eyes. Retinoblastoma usually, but not exclusively, affects children aged 5 and under. The six warning signs to look for are:
A white reflex (leukocoria): A white eye, white pupil or white reflection can be seen in a photograph where the flash has been used. Often one eye will have "red eye" which is normal but the other eye may look white, yellow or orange. This may be seen in just one or many photographs of the child.
A white 'reflex' or white eye/pupil may also be seen when the child is in artificial light or a darkish room. Some parents say that it looks like a cat's eye caught in light or that they think they can see the back of their child's eye, other parents say it looks like jelly. This white reflex may only be seen every so often but in some cases it is present all the time.
An absence of "red eye" in flash photographs: In a photograph where one eye has "red eye" (which is normal) the other eye may look black or looks "wrong".
A squint: A squint is the second most common sign of retinoblastoma. It is important to carry out a comprehensive red reflex test in squint cases before ruling out Rb.
Red, sore or swollen eye without infection: A child's eye may become very red and inflamed for no reason. This sign is usually linked with other signs.
A change in colour to the iris: The iris, the coloured part of the eye, can sometimes change colour in one eye, sometimes only in one area.
Deterioration in vision: A child may have deterioration in their vision or they may have had poor vision from birth. You may notice the child does not focus or fix and follow as well as they should.
A simple red reflex test can help you to determine whether a child needs to be referred for specialist examination. Click to see full size pdf of poster.
The Childhood Eye Cancer Trust's Red Reflex Test poster has been designed to inform any health professional on how to correctly perform a red reflex examination. It has been recognised by many health practitioner educators as a tool to use to teach this examination. It has also been recreated, with permission, throughout the world.
What to do next
If you are unable to confidently rule out retinoblastoma with a red reflex test NICE guidelines state an urgent referral must be made for children with:
- a white pupillary reflex (leukocoria). Pay attention to parents reporting an odd appearance in their child’s eye.
- a new squint or change in visual acuity if cancer is suspected.
- a family history of retinoblastoma and visual problems. (Screening is necessary from birth.)
Urgent referrals should be made to the local ophthalmology department. We recommend you call through to alert them to this case and to find out the speed with which their urgent referrals are seen (in some cases it can be longer than two weeks).
Supporting patients who had Rb
A percentage of Rb patients will have an increased risk of developing second primary cancers. Patients should be monitored for any worrying or persistent problems such as unexplained lumps or pains, new skin moles or changes to an existing mole.
Anyone who has had Rb should be monitored to detect and manage any long-term problems caused by the disease or treatment in order to ensure the best possible quality of life.
For more information on caring for an adult who had Rb as a child click here.
Adults who had Rb as a child may require genetic screening when planning a family. For more information on the genetic implications of retinoblastoma visit our genetics section where you will find more information for your patient on the importance of screening in some circumstances.
Any medical issues related to retinoblastoma and follow-up can be directed to the specialist teams at the Royal London Hospital or Birmingham Children’s Hospital. For more information on these specialist retinoblastoma (Rb) services follow the links below to the hospital sites and genetic screening units.
Become a professional member
For any professional whose work is somehow related to retinoblastoma or people affected by the condition. Join us.
Published articles written by the Childhood Eye Cancer Trust (CHECT)
Maxwell, P (2013) Identifying signs of retinoblastoma, Nursing Times, Vol 109 No 28
Bishop, K (2013) Retinoblastoma: the parental perspective, British Undergraduate Journal of Ophthalmology, Vol 1, Issue 1
Carter, J (2009) Recognizing the signs of retinoblastoma. Practice Nursing 20(8): 394–397.
Carter, J (2010) Supporting patients after retinoblastoma. Practice Nursing 21(1): 38-40.
Carter, J (2009) Retinoblastoma - The issues faced by adults who had retinoblastoma as a child, GP Magazine 17/04/09: 41-43.
Carter, J (2011) Retinoblastoma - The issues faced by adults who had retinoblastoma as a child, GP [online]
Avaliable at: http://www.gponline.com/Clinical/article/897922/Retinoblastoma/ [Accessed 14/07/2001]
Halford, L et al. (2008) Retinoblastoma for life. Focus - Royal College of Ophthalmologists Summer: 5-6.