HOW CAN RETINOBLASTOMA BE TREATED?
Depending on the institution, several experts may be involved in the discussion regarding your child’s specific treatment plan. They may include the pediatric ophthalmologist expert in the care of retinoblastoma, a pediatric oncologist, a pediatric nurse specialist/nurse practitioner and a social worker. The type of therapy offered to your child will depend on information about your child’s disease. Your team will explain if the retinoblastoma is: 1.) intraocular (within the eye) a.) affects one or both eyes b.) the stage (refer to the staging section) of the retinoblastoma in each affected eye 2.) extraocular (outside the eye) a.) disease has spread beyond the eye b.) need to determine exactly where in the body the disease has spread to. Treatment options include the following: surgery, focal therapies, chemotherapy, radiation therapy, and the availability of clinical trials. A combination of these therapies may be recommended to maximize the destruction of the retinoblastoma cells. Treatment decisions will be made collaboratively by you (the child’s parents) and the medical team. Each treatment will be discussed in more detail. It is optimal to be treated in a center with a dedicated retinoblastoma team. SURGERY Surgery is indicated for the treatment of retinoblastoma if only one eye is affected and if the tumor is large enough to cause little or no useful vision of the affected eye. It can also be used in children with bilateral disease if one eye has little or no useful vision because of tumor size. The remaining eye with lower stage disease is then treated. In some cases, both eyes are removed because vision cannot be salvaged in either eye. The operation for the removal of the eye is called enucleation and is performed as an outpatient surgical procedure. With this surgery, the entire globe (the eyeball, which resembles a marble) and part of the optic nerve is removed by the ophthalmologist in the operating room. During the procedure, the ophthalmologist places a temporary implant called a conformer inside the orbit once the globe has been removed. The implant looks like part of a small golf ball and permanently takes the place of the globe. The implant maintains the shape of the eye. Your child will be unaware that the conformer is in place; it cannot be removed, other than by surgery, and is not visible. After the surgery, your child’s eyelids may be bruised and swollen for several days, but these local side effects will go away. Within 6–8 weeks after surgery, your child will be fitted with a prosthetic (artificial) eye, which resembles a large contact lens. A special artist called an ocularist will create your child’s prosthesis by custom fitting the prosthetic eye and painting it to match your child’s remaining eye. The prosthetic eye will be placed inside the eye socket behind the eyelids and, if fitted properly, should not come out easily. It will look almost as natural as the remaining eye. It will move in coordination with the natural eye but will not have full movement. Caring for the prosthetic eye will require gentle washing at home every few weeks and scheduled appointments with the ocularist for professional cleaning and polishing. Children will need their prosthesis replaced as they grow. Children generally adapt easily to the prosthetic eye with age-appropriate explanations and support. Play therapy with young children can be especially helpful when explaining what a prosthetic eye is, how it will look and function.
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