Information for optometrists

Retinoblastoma summary

Retinoblastoma (Rb) is the most common malignant tumour of the eye in children and accounts for 3% of all childhood cancers. It can occur either unilaterally or bilaterally and generally develops before the first five years of life.

Tumours develop in the retinal cells which are developing rapidly in early life. The process of cell development continues throughout infancy and the retina is fully developed at approximately five years of age.

It is a life threatening disease but 98% of children survive retinoblastoma in the UK. Over the past four decades, the management of this disease has evolved tremendously, changing from a deadly childhood cancer to a largely curable disease.

A swift referral pathway for a suspected case is vital to reduce loss of vision and the risk of mortality.

Research by the Childhood Eye Cancer Trust has found that optometrists are more than twice as likely to spot the signs of retinoblastoma in a child and make an urgent referral compared to GPs. This shows that optometrists can play a crucial role in ensuring a swift referral pathway for suspected cases. For more information please read our Would you recognise it leaflet.


  • Occurs in about 1:20,000 live births.
  • Between 40-50 cases are diagnosed each year in the UK.
  • Can be unilateral or bilateral.
  • No gender or race predisposition.
  • Can be heritable so adults who had Rb and wish to have children should be offered genetic counselling and testing.
  • Babies who may have inherited retinoblastoma must be screened from birth.


Early diagnosis is vital in order to save life and reduce the lifelong impact of the disease on the child. If you are unable to confidently rule out retinoblastoma with a detailed eye examination, 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 presenting with visual problems (screening is necessary from birth.)

Depending on local referral protocol, urgent referrals should be made to the local ophthalmology department, A&E or the GP stating ‘suspected retinoblastoma’.

Where referral is 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).


  • Carry out a detailed eye examination in every case of parental concern.
  • Leukocoria or strabismus are the most common signs.
  • Children with retinoblastoma usually show no other signs of illness to alert you to the condition.
  • Just because it’s rare, doesn’t mean it isn’t there.


There are several signs which could indicate retinoblastoma but it is important to remember that a child with retinoblastoma may appear systemically well.

The initial signs are confined to the eye. Leukocoria and strabismus are the most common presentations. If any child presents with one of the following, a detailed eye examination must be carried out (see also: Would you recognise it leaflet).

  • Leukocoria – (intermittent) white pupillary reflex noticed in dim lighting or a photo.
  • Strabismus – retinoblastoma must be ruled out for all cases of squint in babies and children using a red reflex test.
  • 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.
  • An absence of red reflex when doing a red reflex test.
  • Deterioration of vision in one or both eyes or poor vision from birth.
  • Nystagmus.
  • Parental history of retinoblastoma – the condition is heritable so children of an affected parent with retinoblastoma must be screened from birth.
  • Parental concern over vision or eye appearance.

Opticians’ protocol

Supported by the Royal College of Ophthalmologists and the College of Optometrists, the Opticians’ Protocol aims to cut delays in diagnosis of childhood eye cancer.

It states that all staff working in an optical practice should be aware of the main signs of Rb and offers clear information on what action to take if a parent is concerned by any of these main symptoms of Rb.

Let us know if your practice is able to adopt this protocol and help with our battle against retinoblastoma. Please email petra.maxwell@chect.org.uk

Watch our e-learning module on the Opticians’ Protocol.

Your support staff also have a key role to play – Optical Assistants’ protocol

Take five minutes to run through this simple protocol with your optical support staff such as receptionists, optical assistants and dispensing opticians in order to help expedite diagnosis of children with retinoblastoma.

Adult patients after retinoblastoma

For some people, the effects of retinoblastoma can be life-long. Read our information on supporting patients after retinoblastoma to find out more.

We also have a health professionals resources section to help you with every stage of supporting a patient affected by Rb.

For referrals and information contact the Rb teams in London and Birmingham at:

E-learning modules

Watch presentations from leading retinoblastoma consultants on presentation, referral, treatment and long term effects.