Enucleation: Making the Decision
There is no safe way to surgically remove retinoblastoma without removing the entire eye.
Enucleation is the surgical removal of the entire eye. Your child’s ophthalmologist will perform this surgery, only after the team has fully discussed it with you.
Recommendation to remove your child’s eye will be based on tumour size and position, risk to life, age, health of the other eye, and chance of cure with other therapies.
When Enucleation is Recommended
Surgery is the best treatment when:
- Only one eye is affected, and it has advanced cancer – eyes staged IIRC group D and TNMH cT2b require aggressive therapy that often continues for several years, with lower chance of salvage using current therapies.
- There is no hope of saving useful vision.
- The cancer threatens, or has already spread to, the optic nerve, outer layers of the eye (choroid and sclera), or front of the eye (anterior chamber). Any eye staged IIRC group E and TNMH cT3 is a threat to life.
- Pressure in the eye is high (glaucoma), indicating high risk tumour in the front part of the eye.
- Bleeding or cataract prevents thorough examination of the retina, with a risk that new tumour activity will grow undetected.
- Other treatments have failed to destroy the cancer.
- No other eye-saving treatment is available.
Around the world, enucleation is the most effective life-saving therapy for retinoblastoma. Surgery is done to prevent cancer spreading beyond the eye and killing the child.
Retinoblastoma that spreads beyond the eye has very poor chance of cure, even with the most sophisticated intensive therapies.
Primary Treatment for Unilateral Retinoblastoma
When a child has a large tumour in only one eye, with normal vision in the other, enucleation is the best option to protect the whole child. Eye salvage therapy is prolonged and intensive, with many unpleasant side effects and potential complications, ongoing risk of relapse inside and outside the eye, and low chance of successfully saving vision or preserving the eye.
These aggressive treatments can hinder natural child development, and have negative physical and emotional effects that affect the entire family and may continue throughout life. Surgery allows the child to heal quickly and move forward in life, cancer-free. The cosmetic outcome of immediate enucleation with a good artificial eye is also usually far better than with months or years of invasive therapies.
Many parents seek a second opinion before surgery, and this is very reasonable. Always seek a second opinion with the full involvement of your child’s doctors as they will be able to support you in this process, provide vital clinical information for the second opinion team, and help prevent unnecessary delays in care.
Do not delay treatment more than a week or two, or keep seeking second opinions hoping you will find an alternative treatment. Your child’s cancer is potentially fatal. The longer you delay, the greater the risk becomes. If two doctors agree on the proposed surgery, please respect their opinion – they are trying to save your child’s life.
You have the right to refuse surgery. However, if the doctors feel this puts your child’s life at risk, they may request a court order for treatment. Your child’s welfare as a complete person is paramount. If legal intervention is sought, both you and the doctors will have the opportunity to share all concerns during the court’s decision making process.
Not An Easy Decision
Even though surgery is the best lifesaving care for children, this is often not an easy decision for parents and extended family. The Rb team at St. Jude Children’s Research Hospital, together with some of their patient families and survivors, made this video to help other families on the decision making process and journey to recovery.
Questions to Ask the Ophthalmologist
- Why is enucleation being suggested?
- Are there any other options to save or restore my child’s sight?
- What risk do these options pose to my child’s life?
- When will the surgery take place?
- How long will the surgery take?
- How many eyes with retinoblastoma have you removed?
- What type of implant will you use and why?
- Will my child be admitted overnight?
- When and where will the patch be removed?
- How soon will you receive the pathology results?
- How will I be told what the results are?
- When will my child receive an artificial eye?
- How does the artificial eye process work?