The treatment of retinoblastoma is customized for each patient. The treatment depends upon the age of the child, the involvement of one or both eyes, and whether or not the cancer has spread to other parts of the body. Almost all parents choose some form of treatment for their child. No treatment may mean that the child would die. Goals of treatment from most to least important ranking are: saving life, maintaining the eye and vision, and preserving cosmetic appearance.

Enucleation (ee-nuke-lee-a-shon) is the most common form of treatment for retinoblastoma. During an enucleation, the eye is surgically removed. This is necessary because it is the only way to remove the cancer completely. It is not possible to remove the cancer from within the eye without removing the entire eye. This can be done for some other eye cancers, but is dangerous and may even contribute to the spread of the cancer for retinoblastoma patients.

The removal of the eye is done under general anesthesia (the child is asleep). A parent may accompany the child to the operating room on the day of surgery and may be present until the child is anesthetized. The anesthesiologist will place a mask over the child's mouth and nose. Children usually fall asleep within a minute or two after breathing the anesthesia gases.

In the operation, the entire eye is removed along with a long piece of optic nerve as one specimen, and is sent to a pathologist for examination under a micrscope.

The eyebrow, lids, and muscles of the eye are all left in place. Blinking, tearing, and movement of the brow are not affected from this surgery. The operation takes less than an hour and is not painful. Children go home the same day and are usually examined in the office on the following day when post-operative instructions and care are explained.

A ball of plastic, rubber, or coral is placed where the eye had been so there is no cavity or hole. After the socket heals, it will look like the tissue on the inside of the lip. The child is fit for a prosthesis or false eye approximately 3 weeks after the operation. The prosthesis is made of plastic by an ocularist (an artist/technician) to look exactly like the other eye. Since the extraocular muscles are not attached to the prosthesis, it does not move as well as a natural eye. The prosthetic eye tends to move better up and down than it does side to side. And, of course, the prosthetic eye does not see. Unfortunately, there is currently no way to transplant or replace an entire eye.

When both eyes are involved, sometimes the more involved or "worse" eye is enucleated, while the other eye may be treated with one of the vision-preserving treatments, such as external-beam radiation, plaque therapy, cryotherapy, laser treatment, and chemoreduction which are described below.

continued . . .


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