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Treatment Options

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Children with retinoblastoma should be monitored with regular examination under anaesthesia (EUAs).  Due to the young age of affected children, a short acting general anaesthetic is necessary to allow a thorough examination of both eyes.

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Although the EUA takes place in Theatre (operating room), it is not itself a surgical procedure. Current treatments for retinoblastoma include laser, cryotherapy (freezing the tumour), systemic and localised chemotherapy, brachytherapy (radioactive plaque sewn directly onto the tumour), radiotherapy and enucleation (surgical removal of the eye). Treatment depends on the number, size and location of tumour(s) within the eye.  If both eyes are affected, each should be treated on its own merit. However, in many developing countries, resources and expertise are in short supply, and surgical removal of the eye is frequently the only treatment option available.

Focal treatments Chemotherapy Radiotherapy Enucleation Treatment of metastatic retinoblastoma Treatment of trilateral retinoblastoma

Focal Treatments Laser, cryotherapy and local chemotherapy injections will be given during an EUA.  Repeated treatments are usually needed to destroy tumours

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Chemotherapy Systemic chemotherapy is given in repeated cycles over 5-6 months.  Several courses of chemotherapy may be necessary.  In developed countries, a central venous catheter is usually surgically implanted in the child’s chest in order to deliver this treatment directly into a large vein.

Chemotherapy can have strong side effects.  During treatment, children are especially vulnerable to infection, fatigue and bruising due to low blood counts.  Medications and transfusions may be given to boost blood counts.

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Radiotherapy Radiotherapy used to be a standard treatment.  However, it has significant long term side effects, and the success of chemotherapy means radiotherapy is now used with much less frequency in developed countries.

A course of radiotherapy usually lasts 4-5 weeks.  Prior to treatment, a perspex mask is made to fit the child's head.  This is worn during each treatment, to protect areas outside the field of radiation. Due to their young age and the need to lie still during treatment, children receive a short acting general anaesthetic for each daily dose.

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Enucleation Sometimes, tumours may remain unresponsive to all treatments.  Others may be located very close to the optic nerve or blood vessels at the back of the eye.  In these cases, surgical removal of the eye may be the only way to prevent cancerous cells from spreading to other parts of the body.  This procedure is called enucleation.

During the surgery, an implant should be placed to replace the eye and maintain the shape of the socket. The child should then wear a conformer for approximately six weeks after enucleation, whilst the socket heals.  The conformer is usually a clear plastic shell with one or two small holes, through which antibiotic drops are administered to prevent infection.

Once the eye socket has healed, the child should be fitted for an ocular prosthesis.  This artificial eye does not enable the child to see – once the eye has been removed, vision cannot be restored.

Ocular Prosthetics are a work of art – can you tell which eye is artificial in these pictures?

The prosthesis is important for maintaining the structure of the eye socket, and preventing infection.  Artificial eyes are also hugely important for the psychological well-being of the child and his or her family. However, in developing countries, lack of resources and expertise mean the majority of children do not receive an implant or artificial eye.

In many developing countries, where resources are limited, enucleation is often the only treatment option available.  In such situations, when a child is diagnosed with tumours in both eyes, doctors sometimes offer only palliative care, even though the cancer may be curable by removing both eyes.

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Treatment of Extraocular (metastatic) Retinoblastoma When Retinoblastoma has spread outside the eye, the child will require intensive systemic and spinal chemotherapy, as well as radiotherapy and bone marrow (stem cell) transplant to give the best chance of survival.

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Treatment of trilateral retinoblastoma Trilateral retinoblastoma also requires intensive systemic chemotherapy, as well as chemotherapy injections directly into the cerebro-spinal fluid (fluid bathing the brain).  In addition, children will require bone marrow transplant, and may also require radiotherapy. In developing countries, these intensive treatments are often unavailable, or too expensive, and most doctors lack the expertise to provide effective care, As a result, most children in developing nations who have extraocular and trilateral retinoblastoma can only receive palliative care..

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