Definition

Retinoblastoma is the most frequent ferocity in the eye ball in children. Of all the tumors that usually occur in small children, retinoblastoma ranks 3rd as the most common eye tumor. The number of retinoblastoma events ranges from 1 incident per 14,000 babies born live up to 1 event per 20,000 babies born alive. Of all the retinoblastoma cases themselves, 90% appear below the age of 3.

Cause

Retinoblastoma occurs as a result of mutations or changes in a gene called RB1. Under normal conditions, this gene serves to suppress cell growth so as not to overdo it. This gene is inherited from both parents, and a new retinoblastoma occurs if these two RB1 genes experience mutations. This mutation or change in the RB1 gene causes retinal cells (layers inside the eye which function to receive light) to increase in number but out of control.

Mutations that occur in the RB1 gene can be congenital or occur during development. If the RB1 gene undergoes a congenital mutation, this mutation can be lowered so that the risk of retinoblastoma in its descendants will increase. In addition, this congenital mutation increases the risk of tumors in other parts of the body because this gene mutates throughout the body's cells. Retinoblastoma itself can occur in one or both eyes.

Risk Factors

Retinoblastoma events are no different depending on gender or race. A child is known to have a retinoblastoma around 18 months old. About 90% of all retinoblastoma cases were diagnosed under 5 years of age. Of all retinoblastoma sufferers, only 5% have a family history, while other 95% occur erratically. About 90% of all retinoblastoma cases occur in one eye and cannot be lowered. The risk of retinoblastoma in siblings or descendants from sufferers is 1%.

Symptoms

Symptoms of retinoblastoma that are most easily recognized are the cat's eye phenomenon, namely the middle part of the eye is white when exposed to light. This happens because tumor cells reflect the rays that come. Other frequent symptoms are juling eyes, red eyes accompanied by pain, inflammation of the eyelids or skin around the eyes, presence of lumps outside the eyes, decreased vision, and decreased eyeball motion.

Diagnosed

Diagnosis of retinoblastoma can begin with questions about history of illness in the family. Family history that can be related to retinoblastoma is a history of blindness, eye tumors, childhood ferocity, or a history of surgical removal of eyeballs (enucleation). In addition, doctors can ask when you will see your child's eyes white when exposed to light.

Furthermore, doctors can carry out several direct examinations. The examination starts from a sharp examination of the vision according to age, and this is done on each eye. The doctor will also shed light on the eyes to see the reflectance of the light on each eye. After that, the doctor will check the outside of the eye to find inflammation in the skin of the eyelids and the skin around the eyes. Then, the doctor can do an examination using slit lamp to find red eyes, a buildup of pus in the eye, a new blood vessel in the eye rainbow membrane, and a sign of glaukuma (visional nerve damage).

To take a look further into the eye's eye, doctors can perform an examination known as funduscopy. Before this examination is carried out, your child will be given the drug to widen the pupil (black hole in the middle of the eye) and may be given an anesthetic so that the examination can be carried out in more detail. This examination is necessary to diagnose the retinoblastoma, determine the position and magnitude of the tumor, and determine the tumor status. This examination is carried out on both eyes.

Furthermore, doctors will conduct imaging examinations with ultrasound. Ultrasound can show an image of the mass or tumor in the eyes, as well as determine the length, width, and height of the tumor. In addition, doctors will also perform MRI examinations on children, to look for tumor spread to the vision nerve. CT scans are not recommended in children with retinoblastoma, because radiation exposure can increase the risk of other ferocity if the child turns out to have a RB1 gene mutation in the body cells.

Another examination that can be done is genetic examination and tumor tissue. Genetic examination is not only carried out on children, but also on parents and siblings of children. This is necessary to find out whether the retinoblastoma experienced by the child can be lowered or not. Meanwhile, tumor tissue examination is carried out to find out where the tumor tissue came from.

Laksana Procedure

Retinoblastoma management involves many parties, namely eye specialists, pediatric cancer experts, genetics experts, and other health workers, as well as parents. Therapy that can be done on retinoblastoma can involve several ways:

• Chemotherapy. Chemotherapy is the most common therapy in retinoblastoma. Chemotherapy uses drugs that function to destroy tumors or inhibit tumor growth. The drug can be inserted through blood vessels or directly into the eye.
• Transpupillary Thermal Therapy (TTT). TTT uses heat to kill blood vessels that supply nutrients in tumors. TTT can be used simultaneously with chemotherapy or separately.
• Kriotherapy. This therapy serves to freeze the tumor, so that the tumor tissue dies.
• BrakitherapyThis therapy is carried out by placing radiation centers near body parts that require it, and aiming to destroy tumor cells.
• Radiotherapy. This therapy can be an option to destroy tumor cells, but its use must be careful because it can trigger ferocity elsewhere.
• Enucleation- Enucleation is surgery that aims to remove the eyepiece. This is done if chemotherapy fails and is at high risk of reappearing, or if the tumor attacks the front of the eye. Usually, after enucleation, the child will undergo chemotherapy if there are still tumor remnants in the tissue around the eye.

After therapy is carried out, the doctor will plan control for your child. This control aims to monitor the development of the tumor and monitor the vision function of your child.

Complications

Retinoblastoma if left unchecked can cause various complications. These complications can be in the form of the spread of tumors to other parts of the body such as the nerves of sight, the brain, bones, lymph nodes, and other tissues around the eyes. In addition, complications in the eye sphere can be in the form of removing the retinal layers, death of retinal cells, cataracts, and glaukuma. Complications on vision can be in the form of blindness. 

Prevention

Retinoblastoma cannot be prevented, but can be recognized early on. If you have a family member with a history of blindness or tumor in your eyes as a child, you can check your child with an eye specialist to do retinoblastoma screening.

When do you have to go to a doctor?

Immediately go to the doctor if you see your child's eyes white when exposed to light, or if your child has a crossing eye. These two symptoms are the most frequent retinoblastoma symptoms that parents can also recognize. These two symptoms do not only occur in retinoblastoma, but can also lead to other eye conditions that need to be treated. Retinoblastoma requires special treatment that should be done immediately before the tumor spreads to other places or before there is permanent blindness.

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