Retinoblastoma

Retinoblastoma is a cancer of the retina, the light-sensitive layer at the back of the eye [1][2]. It starts when cells in the developing retina grow out of control, because of a change in a gene called RB1.

• It is rare, but it is the most common eye cancer in children.
• Most children are diagnosed before age 5, and many before age 2.
• It can affect one eye or both eyes.

The good news: with early diagnosis and modern treatment, almost all children survive, and many keep useful vision [1][3].

Image to add

Two side-by-side drawings of a child's face in a flash photo: one with normal red-eye in both pupils, one with a white glow in one pupil. Alt text: "Drawing comparing a normal red reflex in both eyes with a white reflex in one pupil, a warning sign of retinoblastoma."

Most children with retinoblastoma have no family history, and nothing the parents did caused it.

About 1 in 3 children have an inherited (heritable) form [3]. This is more likely if:

• both eyes are affected
• a parent, brother or sister has had retinoblastoma

If retinoblastoma runs in your family:

• Babies and young children in the family (brothers, sisters, and the children of someone who had it) need regular eye checks from birth.
• Genetic testing can show who carries the gene change, so checks can focus on the children who need them [3].
• Tell your doctor about the family history during pregnancy or as soon as your baby is born.

Signs to look out for [1][2]:

• A white pupil (leukocoria): the black centre of the eye looks white or pale, or glows white in flash photos, instead of red or black. This is the most common sign.
• A new squint: an eye that turns in or out.
• A red, sore or swollen eye without an obvious reason.
• Poor vision: your child may bump into things or not follow objects.
• A change in the colour of the iris (the coloured part) in one eye.

Many of these signs have other, less serious causes. But they should always be checked by an eye doctor.

At the clinic, the doctor will put in drops to widen the pupils and look at the back of the eye. If retinoblastoma is suspected, your child will be referred to a specialist team, and may have an ultrasound or MRI scan and an examination under anaesthetic.

Most white reflections in photos turn out to be something harmless. But because early treatment makes such a difference, do not wait and see.

Retinoblastoma is treated by specialist eye cancer centres, and the plan depends on the size of the tumour and whether one or both eyes are affected [1][2][3]. The aims are to save life first, then to save the eye and as much sight as possible.

Treatments include:

• Chemotherapy: medicine to shrink the cancer. It can be given into a vein, into the artery that supplies the eye, or as an injection into the eye.
• Laser or freezing treatment (cryotherapy) for smaller tumours.
• Radiotherapy, sometimes with a small radioactive plate placed on the eye.
• Removing the eye (enucleation): sometimes the safest option, especially for large tumours. Children adapt very well, and an artificial eye gives a natural look.

After treatment, your child will need regular check-ups for several years. Children with the inherited form have a higher risk of some other cancers later in life, so they are followed up long term [3].

Early diagnosis matters: retinoblastoma is highly curable when it is found while still inside the eye [4].

Where to get help in Brunei

If you are worried about your child's eyes, you can walk in to any Community Eye Clinic at your health centre. No referral is needed. Tell the staff about any white glow in the pupil, so your child can be seen quickly.