‹ Blog / Children's Eyecare

Understanding Retinoblastoma

May 17th 2024

It’s World Retinoblastoma Awareness Week from 12th May – 18th May 2024.

Retinoblastoma is a rare type of eye cancer in young children, affecting one or both eyes, and usually developing in children under the age of five. It is the most common malignant tumour of the eye in children and accounts for 3% of all childhood cancers.

This cancer develops in the retina, the light-sensitive lining at the back of the eye. The retina is crucial for vision as it converts light into signals that are sent to the brain for visual recognition.

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.

As opticians, we play a critical role in the early detection of eye conditions like retinoblastoma. Regular eye exams for children are essential. If you notice any unusual signs or symptoms in your child’s eyes, don’t hesitate to seek professional advice or contact us to book an appointment. Early intervention can save sight and lives.

Signs and Symptoms

Early detection of retinoblastoma is vital. The Childhood Eye Cancer Trust (CHECT) have put together this useful leaflet which lists the main symptoms.

Here are some common signs to watch for:

White Glow in the Pupil

You might see a white glow in the eye, sometimes described as a ‘cat’s eye’, a white pupil or white reflection in a photo where a flash has been used, or when your child is in artificial light or a darkish room. This may only be seen once but in some cases it’s present all the time. There are other, more common causes of this – read more about what causes white eye.

Squint

A squint, where the eyes do not look in the same direction, can sometimes be a symptom of retinoblastoma. In many cases, it’s nothing more than a squint but all children should be checked out just in case.

Red, sore or swollen eye

Persistent redness, swelling, or soreness can be a symptom.

Different Coloured Irises

The coloured part of the eye (iris) may appear different in each eye.

Vision Problems

Children may show signs of poor vision, such as difficulty focusing or following objects with their eyes.

No Red Eye

In a photo where one eye has “red eye” (which is normal), the other eye may look black. This can be a sign that something is not right.

Diagnosis and Treatment

Early diagnosis can significantly improve the chances of successful treatment. If you’re worried that your child is showing a symptom of retinoblastoma take them to your GP, a local optician/optometrist or an ophthalmology (eye specialist) department to have their eyes examined as soon as possible.

Chemotherapy

Chemotherapy (drug treatment) is usually used in the first stage of treatment for patients with bilateral retinoblastoma and can be used for both small and large tumours. It involves using powerful drugs to target and kill cancer cells. These drugs can be administered in various ways:

  • Systemic Chemotherapy: Administered orally or through an injection into a vein, allowing the medication to travel throughout the body.
  • Local Chemotherapy: Delivered directly to the eye to minimise side effects on the rest of the body.

Chemotherapy can shrink tumours, making other treatments like laser therapy or cryotherapy more effective. It may also be required after an eye has been removed by surgery (enucleation), if there are signs that the tumour had involved the nerve, the deeper layers of the eye or the front chamber of the eye.

Laser

Laser therapy uses focused light to treat retinoblastoma. The laser precisely targets and destroys the blood vessels supplying the tumour, effectively cutting off its blood supply and causing the cancer cells to die. This method is typically used for smaller tumours and can be repeated if necessary.

Proton beam therapy

Proton beam therapy uses a high energy beam of protons rather than X-rays to deliver a dose of radiotherapy. The beams stop once they hit the target, rather than carrying on through the body. It can be a more effective form of radiotherapy as it causes minimal damage to surrounding tissue and a reduced risk of late side-effects.

Cryotherapy

Cryotherapy involves freezing cancer cells to kill them. A small probe is placed on the outside of the eye, near the tumor, and extreme cold is applied. This process can be repeated over several sessions to ensure all cancerous cells are destroyed. Cryotherapy is usually used for smaller tumours located at the edges of the retina.

Radiation Therapy

Radiation therapy uses high-energy rays to target and destroy cancer cells. There are two main types:

  • External Beam Radiation: Directs radiation from outside the body onto the tumour. It is typically reserved for cases where other treatments have not been successful.
  • Brachytherapy (Plaque Radiotherapy): Places a small radioactive plaque directly on the eye near the tumour. This localised approach helps minimise exposure to surrounding healthy tissue.

Surgery

In severe cases, where the tumour is large or not responsive to other treatments, surgery may be necessary. The most common surgical procedure is called enucleation, which is the removal of the affected eye. While this is a drastic measure, it is sometimes required to prevent the cancer from spreading. After enucleation, most children can be fitted with an artificial eye that looks and moves naturally.

Treatments can be tailored to the individual needs of the child, depending on the size, location, and extent of the tumour. Early diagnosis and a combination of these treatments can lead to successful management and cure of retinoblastoma. Always consult with a specialist to determine the best treatment plan for your child.

Living with Retinoblastoma

While a diagnosis of retinoblastoma can be daunting, advancements in medical treatments have significantly improved outcomes. Early detection and treatment are crucial. Children treated for retinoblastoma typically undergo regular follow-ups to monitor for any recurrence or complications.

Support and Resources

Caring for a child with retinoblastoma can be challenging, but various resources and support networks are available to help families navigate this journey. Organisations such as the Childhood Eye Cancer Trust (CHECT) offer invaluable support, information, and advocacy for affected families.

For more information and support, you can visit the Childhood Eye Cancer Trust (CHECT) website.

Stay informed and vigilant, and remember that timely medical care is the key to effectively managing retinoblastoma.