Retinoblastoma:

Your child’s eye surgeon must have informed you that your child had ‘Retinoblastoma’ for which immediate treatment is necessary. Retinoblastoma is a cancerous growth that occurs in the eye.

The tumors are of two types. The cancerous tumor can spread to other parts of the body, ultimately causing death. The other ‘benign’ form of tumor does not spread like a cancerous one and is thus relatively safer. Retinoblastoma is a cancerous tumor needing immediate attention.

Retinoblastoma can present as a white reflex at the pupil of the eye (Cat’s eye like appearance) or as a squint. Rarely if ignored in the early stage, it can cause protrusion of the eyeball.
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What causes a Retinoblastoma?

As in most cancers, we still do not know why some children develop Retinoblastoma. Retinoblastoma can however occur in families due to a genetic defect and children born in such families are definitely at risk. However, most children who develop this tumor do not have other affected family members.

Usually Retinoblastoma affects only one eye of the child. Rarely both eyes of the child may be affected, particularly if other family members (either parents or brother and sister) are affected.

If either parent is affected, there is approximately 40% chance of future offspring developing Retinoblastoma. Hence future children of the family where a parent and a child are affected should be checked by an ophthalmologist immediately after birth. For this reason, your ophthalmologist may advice examination of the parents’ eyes.

The risk of the future children developing Retinoblastoma can be assessed in some cases by doing a ‘genetic analysis’ of the blood. If necessary, a test can also be done on the pregnant mother to find out if the fetus has the risk for tumor.

SYMPTOMS:

The most common symptom of Retinoblastoma is white pupillary reflex. In an eye affected with Retinoblastoma, pupil will appear white or pinks instead of red when light shines into it. This can be seen by child’s parents after taking a flash photograph, especially if the pupils are of distinct colors. This can also be noticed by the child’s doctors during a routine eye check-up.

Lazy eye- The eyes of the child do not seem to look in the same direction. Lazy eye in child mainly occurs due to the mild weakness of the muscles which control the eyes, but Retinoblastoma is also one of the exceptional causes.

The other symptoms include:

Pain in the eyes
Redness in the white part of the eye.
Eyeball is larger than normal.
Colored part of the eye and pupil look cloudy.
Each iris is of different colors
TREATMENT:
Most cancers are treated by:

Removing the affected part so that it does not spread to other part of the body and cause death
Using radiation treatment
Giving intravenous medicines (chemotherapy)
Using laser or cryo treatment
Retinoblastoma is also treated based on these broad principles

As an initial step, your ophthalmologist may advice ‘examination under general anaesthesia”, where the child is examined in detail after giving a short anaesthesia. This is essential to examine the child’s eyes completely and plan treatment. This examination under general anaesthesia may have to be repeated at every visit of the child to assess the treatment response.

Your doctor might have already briefed you about the tentative plan of treatment for your child. He may have advised ‘Enucleation’, that is removal of the eye if the tumor is very advanced. Enucleation is usually advised when the tumor is very large and the eye does not have any vision. Removing this sightless eye will decrease the chances of the tumor spreading to other parts of the body and causing death. Most children adapt to this procedure quite well and grow up normally with one eye. They can attend regular school and be as normal as any other child. To make the child appear normal, your doctor will fit a ‘cosmetic shell’ in place of the removal eye, 6 weeks later. Some movement of this cosmetic eye can be expected though it cannot match the normal eye.

Subjecting to child enucleation is a traumatic experience. However it is in the child’s own interest. Your child’s life may be in danger if the eye is not removed.

Eye saving treatment may be tried if the eye with the tumor has vision. If the tumor is small, your doctor may advice ‘Laser treatment, Cryo treatment or Transpupillary Thermotherapy’, which are treatments that can be performed during examination of the child under anaesthesia.

If the tumor is large in an eye with vision or if it is the only remaining eye of the child, then doctor may advice “Radiation or Chemotherapy”. You will be referred to a cancer hospital for these treatments. Though the treatment is done at the cancer hospital, it is your ophthalmologist who will assess the response to treatment and hence you should come back to your ophthalmologist for follow-up. If chemotherapy is done, is had to be followed-up by Laser or Cryo treatment which will be done by your ophthalmologist.
FAQs:
What are the possible side effects of treatment?

Laser or Cryo treatments are usually safe but problems like bleeding in to the eye or retinal detachment may rarely occur.

Radiation may lead to late cataract formation, improper growth of face bones and teeth. Rarely other tumors can occur in later life especially in a child with familial form of disease.

Chemotherapy has so far been found to be safe except for short term complication like loss of hair, weight, vomiting, susceptibility and infection. Hair that is lost grows back and the child gains weight after stopping the chemotherapy.

Will my child be cured?

It is every parent’s and ophthalmologist’s wish that both the eyes and life of the child with Retinoblastoma is cured. However, all Retinoblastomas do not respond well to treatment. If the tumor does not respond adequately to eye saving treatment, your doctor may advice removal of the eye in the interest of saving the child’s life.

However, with the latest advances like chemotherapy, many children who would have lost their eyes earlier retain them now.

It is very essential that the advised treatments are carried out and the child brought back for periodic follow-up as advised by your ophthalmologist. You must bring the child back for review 4-6 weeks later (or as advised by your ophthalmologist) to assess the response of the tumor to treatment and also to repeat the treatment if necessary. Even if your child’s eye has been enucleated, you may still have to come once in 3 months in the first year, 6 monthly in the second and yearly once for the first 5 years at least. Children with tumor in both the eyes may have to be followed for longer periods of time.
Article by
Sankara eye care Hospitals