(May 19, 2009 - Insidermedicine) In this video, Dr. Carol Shields MD, a preeminent authority on ocular tumors, discusses what she would do if she had a child with a white pupil. Dr. Shields is Associate Director of the Ocular Oncology Service at Wills Eye Institute. Dr. Shields is also Professor of Ophthalmology at Thomas Jefferson University.

If I had a child with a white pupil

There are many causes of white pupil. I’ll divide them into normal and abnormal eye problems.

A patient who comes in with a white pupil, perhaps if it is a child who comes in with a white pupil, can come in with an absolutely normal eye. If a photograph is taken of the eye with the eye about fifteen degrees off the visual axis (that is to say off to the side) you might get a white pupil because you are imaging the optic nerve. This would be a normal eye exam and I know this from personal experience because that happened with one of our children.

There are problems that can cause white pupil. Beginning with the front of the eye, the cornea, some congenital corneal scars or opacities can lead to a white pupil, or a cataract can lead to a white pupil. If we move further back in the eye, any abnormality in the vitreous whether it be blood, or infection, or inflammation can cause a white pupil. Finally, retinal problems can lead to white pupil. We call white pupil leukocoria. Some of the retinal problems that lead to leukocoria include retinal detachment, and then the one that we all fear, retinoblastoma. Retinoblastoma is the most serious cause of leukocoria in children.

 Whom should I see and how urgent is referral?

 If you have a child with a white pupil, I would see the local ophthalmologist or the local pediatric ophthalmologist. I would tell them on the phone that your child has a white pupil, and I think it’s relatively urgent to see them within a week.

What tests will my ophthalmologist perform?
 

Your ophthalmologist will start out by taking a vision on the child, and dilating the eyes. It is very critical that they dilate the eyes to look into the back of the eye. We have an instrument that we use to looks at the front of the eye – it’s called a slit lamp biomicroscope – and the doctor will be looking for cataract and inflammation in front of the eye. Again, most importantly, they should dilate the eye to look at the retina to see if there is a retinal detachment or if there is a retinoblastoma.

How is retinoblastoma diagnosed?

Retinoblastoma is a cancer of the eye. Many cancers in the body require a biopsy to confirm diagnosis. This is not so with retinoblastoma. Retinoblastoma requires a good eye examination, with the ophthalmologist recognizing the clinical features of this tumor. 

The diagnosis of retinoblastoma is established by clinical examination, consisting of dilated examination with the ophthalmologist putting the equipment on their head, using their lenses, and looking into the back of the eye. Most people will also confirm the diagnosis by taking a photograph of the back of the eye, and also doing an ultrasound. An ultrasound is a test where you place a probe on the eye, and using sound waves you can identify whether there is calcium in the mass that you see in the eye. Retinoblastoma is one of the few cancers in the eye that produces calcium. When you do an ultrasound you establish the size of the tumor, its location, how extensive it is, and one important point – if there is calcium or not. If there is calcium then you essentially have a confirmed diagnosis. 

What treatments are available?

Our goals when we manage retinoblastoma are three-fold:

1)    We have to make decisions to save the child’s life. Retinoblastoma is 100% fatal if a child has an active tumor and it’s not treated, so our first goal is to save the life of the child

2)    Our second goal is to save at least one eye for that child.

3)    Our third goal is to maximize the child’s vision.

We have to balance all three of these goals when we make a decision on how to treat retinoblastoma. Basically, the options at our disposal are eye removal, chemotherapy, radiotherapy, and if the tumor is really small, laser treatments or cryotherapy (which is a freezing treatment). The management of retinoblastoma depends on the extensiveness of the tumor within the eye.

What is the prognosis, and how should my child be followed?

In the United States, the systemic prognosis for retinoblastoma is excellent. 95-99% of children in the United States survive retinoblastoma. It is not so in other parts of the world. In undeveloped countries, they’re still struggling with survival of the patient because of late diagnosis, and about 50% of children in countries that are undeveloped survive this cancer.

 Another subject or issue to discuss is the ocular survival, that is to say if you start chemotherapy, what is the chance that the eye will survive, or remain, or not need to be removed? A lot of this depends on the stage of the eye the time of detection.

• If you have a group A eye, that eye is going to be saved.

• If you have a group B or C eye, there is 90-95% chance that the eye will be saved.

• If you have a group D eye, There is about a 50-50 chance that the eye will be saved with chemotherapy.

• If you have a group E eye, the chance is very low, it is about a 20-30% chance that the eye will be saved with chemotherapy, but there are a lot of new ideas emerging for the management of retinoblastoma.

In Summary

If I had a child with a white pupil, the first thing I would do is to call my ophthalmologist, and ask if I could have an appointment within a week or two weeks because of the potential seriousness of the problem.  I would hope that it was just the optic disk or a normal eye giving a funny reflex, I would worry that it might be retinoblastoma, and if it were retinoblastoma, the first thing I would do is I’d ask my ophthalmologist what major centre is close to me, where I can have my child examined by experienced ocular oncologists and treated.

Unequivocally, I would go to a centre for retinoblastoma. I would not have treatment locally at home. I would go to that centre and I would listen to the physician regarding the classification of the tumor in one eye, or both eyes, feeling good if the class were group A, B or C, worrying if it were group D or E, and knowing all along that my main goal is to save my child’s life, and hopefully save one eye, and hope down the road the vision could be protected.