If the tumour is very large and has damaged the sight in the eye beyond repair, then the ophthalmologist will recommend that the eye be removed. This operation is called an enucleation. The ophthalmologist looking after the child will always do everything s/he can to preserve vision, and will only recommend enucleation if s/he feels that the other treatments would not be effective, and would put the child's life at risk.
Approximately 70% of children with unilateral Rb will require enucleation.
Children recover very rapidly following this surgery. It is not usually a particularly painful operation, and there are no stitches to be removed. Usually the child will be able to come home one or two days after the operation and the retinoblastoma nurse specialist will teach the parents how to put antibiotic drops into the socket. These drops are necessary for about two weeks, until the tissues in the socket have healed over.
Before a custom colour matched artificial eye is fitted a temporary artificial eye (conformer) is worn. It will be put in during the operation. It is worn to protect the socket whilst it is healing. Conformers can be clear or be coloured like an eye.
For more information about artificial eyes and implants click this link.
View our gallery of children and adults wearing artificial eyes.
Pathology following surgery
The decision to enucleate is always taken rapidly whenever there is any risk of spread down the optic nerve.
When an eye has to be removed, it is sent to a pathologist who will examine it under the microscope. The pathologist looks for evidence of retinoblastoma cells in the optic nerve, the deeper layers of the eye and in the front chamber of the eye. If any of these features are found, chemotherapy will be necessary.
UK statistics are showing that where chemo is needed post enucleation the vast majority of children will have had grade E tumours, with a small percentage having grade D tumours. Groups A/B/C rarely undergo enucleation. The vast majority of children in this category will safely retain their eyes following chemotherapy, possibly in conjunction with local treatments, which is why chemotherapy is the standard of care for these children. In the past five years, very few children in the UK with C eyes have required enucleation and no child with a C eye has required chemotherapy for adverse histology, that is, spread of cells beyond the eye.
If your child requires an enucleation following other treatments it is more likely than not that no further chemotherapy will be required.
When chemotherapy is necessary after enucleation, a number of tests will be done to check that the tumour has not spread outside the eye. The investigations may include blood tests, a lumbar puncture to examine the cerebrospinal fluid (CSF) which surrounds the brain and spinal cord, and a bone marrow test. In some cases, a bone scan and MRI scan of the head may be done. The paediatric oncologist will advise which tests are necessary and more information about these tests will be given to the parents.
Sometimes, when tumour cells are seen in the optic nerve, it is necessary to give both chemotherapy directly into the spinal fluid and radiotherapy to the socket, to help prevent retinoblastoma developing in the membranes covering the brain. Fortunately this is very rare.
The 'Enucleation' factsheet is downloadable here.
Click here for stories of people who have been affected by enucleation
A website for parents
Verity's Retinoblastoma Page
This site was made by one of our member, Simon Clarkson. It show's the story (with pictures) of Verity's enucleation after being diagnosed with retinoblastoma. For any parents who are worried or unsure about the enucleation process this site can put your mind at rest. The site also contains information about how Verity's parents help her to care for her artificial eye and how they told Verity's two older siblings about the diagnosis and enucleation.
The Childhood Eye Cancer Trust is not responsible for the contents or upkeep of this website.
Below is a poster which is displayed at the retinoblastoma centres. It features Verity before and after her operation and also some time after the operation doing things she enjoys years later.