Visually Impairing Conditions

Optic Nerve Glioma

For whom is this information intended?
The information contained in this document is intended for use primarily by parents, other members of the family and older children with visual impairment. The information will also be of use to interested health professionals, carers and teachers.

The purpose of each information document
The purpose of the information is to explain:

This document is written with the minimum use of medical terms and jargon. It is impossible to avoid all medical terms but where we have used them we have attempted to explain them as clearly as we can. Although the information is intended to describe most aspects of the condition each child is different and there will always be exceptions to the rule. As far as we can determine these pages are true and accurate and have been written in good faith. Inevitably there will be some mistakes. We apologise for this.

What this information is not for
This document is not a substitute for a consultation with a Health Professional and should not to be used as a means of diagnosing a condition. It is not a means to access a 'second opinion' particularly in any medico-legal cases. We hope the information will help you.

After reading the information we hope you will:

  • Have a better understanding of the condition
  • Know what tests and treatments are normally available
  • Know when to seek professional advice
  • Be able to discuss the condition in a more informed way
  • Make the most of consultations with carers, teachers and health professionals
  • Be reassured and more able to cope

Due to staffing limitations we are not able to offer telephone or email advice to parents of children who are not notified with VI Scotland.

Authors
These pages represent the consensus of opinion of many different people who include Parents of Visually Impaired Children, Visually Impaired Children themselves, Community Paediatricians, Ophthalmologists, Educationalists and Psychologists. The main author and person responsible for their content is Dr Andrew Blaikie who is an Ophthalmology Research Fellow with Visual Impairment Scotland and a member of the Royal College of Ophthalmologists.

We are very keen that parents and children feedback to VI Scotland about these information documents.
Visual Impairment, Scotland Scottish Sensory Centre, The University of Edinburgh, Holyrood Road, Edinburgh EH8 8AQ
Telephone Number: 0131 651 6078
Fax Number: 0131 651 6502
Textphone: 0131 651 6067
Email: viscotland@ed.ac.uk

Medical information on Optic Nerve Glioma

What we see is made in the brain from signals given to it by the eyes
What we see is in fact made in the brain. The brain makes sight from signals given to it by the eyes.

What is the normal structure of the eye?
The eye is made of three parts.

  • A light focusing bit at the front (cornea and lens).
  • A light sensitive film at the back of the eye (retina).
  • A large collection of communication wires to the brain (optic nerve).

A curved window called the cornea first focuses the light. The light then passes through a hole called the pupil. A circle of muscle called the iris surrounds the pupil. The iris is the coloured part of the eye. The light is then focused onto the back of the eye by a lens. Tiny light sensitive patches (photoreceptors) cover the back of the eye. These photoreceptors collect information about the visual world. The covering of photoreceptors at the back of the eye forms a thin film known as the retina. Each photoreceptor sends its signals down very fine wires to the brain.

The wires joining each eye to the brain are called the optic nerves. The optic nerves join about half way between the eye and brain, and then split up again. The join is called the optic chiasm. At the join, signals from the 'nose' side of each eye’s visual world swap sides and continue travelling along the opposite side from where they started. The two optic nerves then join on to the brain. The brain is split into two halves, right and left. This means all the signals from the visual world on the child’s right hand side are now travelling in the left side of the brain. It also means that all the signals from the visual world on the child's left hand side are now travelling in the right half of the brain.

The information then travels to the many different special vision parts of the brain. The main bit of the brain that works vision is at the back of the head. It is called the occipital lobe. The joined up path that signals travel down from retina to optic nerve then optic chiasm then occipital lobe is called the visual pathway. There are two visual pathways, one on the right side of the brain and another on the left. All parts of both visual pathways need to be present and working for us to see normally.

What is an Optic Nerve Glioma?

A Glioma is a benign tumour of nerve tissue. A tumour is a condition where a part of the body grows more than it would normally. Benign means that bits of the tumour will not normally break off and spread to other parts of the body. It may, with time however grow and get bigger. The optic nerve is made of nerve tissue. If such a tumour affects the optic nerve then it is called an Optic Nerve Glioma. Most children present with this condition around nine years of age. Some may present much earlier, before they even start school. It usually only affects one eye. If a child also has a condition called Neurofibromatosis both eyes may become affected.

How does an Optic Nerve Glioma affect a child's eyes and vision?

Optic Nerve Glioma may cause:

  • Blurred vision
  • Squint
  • Prominence of the eye

Blurred vision
A glioma may cause gradual damage to the optic nerve. Slowly, overtime this will cause the optic nerve to stop working. This will lead to blurred vision in that eye. Young children will not usually notice this. This is because they will still have good vision in their other eye. If both eyes are affected or the other eye already has reduced vision the child is more likely to notice a problem sooner. Children may often only complain of blurred vision when the vision in their only remaining good eye becomes blurred. Parents and teachers may also suspect, by the way a child acts that vision maybe impaired. If they discuss their concerns with the Family Doctor an assessment can be arranged.

Prominence of the eye
The glioma grows behind the eye. As it gets bigger it may push the eye forward. Parents may notice that one of their child's eyes is bigger and more noticeable than the other. This is called proptosis and is a common symptom of Optic Nerve Glioma.

Squint
Squint is when a child's eyes do not appear to be looking in the same direction. As a Glioma grows and gets bigger it may push the eye upwards and forwards. This leads to a squint. If this happens children rarely complain of double vision. Parents or teachers may however notice the squint. Children with a squint should always be examined in the eye clinic.

Children with Neurofibromatosis are more likely to develop a Glioma of both optic nerves
There is a rare condition in which children are more likely to develop a glioma of both optic nerves. The condition is called Neurofibromatosis. Many children with the condition have a parent or brother and sister with this condition as well. Quite often however a child may be the first in the family to be affected. As many as half of all children with a glioma of both optic perves will also have Neurofibromatosis. In this condition children usually develop small growths of nerve tissue and skin.

How is the diagnosis made?

Parents may notice that one of their child's eyes is sticking out further than the other (proptosis) or that their child is squinting. Parents may also suspect, by the way their child acts, that their child's vision is impaired. Children may sometimes complain that their vision is blurred. If parents discuss their concerns with their Family Doctor an assessment can be arranged.

A school nurse or optometrist may notice the blurred vision during a vision screening examination. They can organise an examination at the eye clinic.

At the eye clinic an eye doctor and orthoptists can assess the child's eyes and vision. If an Optic Nerve Glioma is suspected other tests can be done. A head scan often helps to confirm the diagnosis.

What can be done to treat the glioma?

Many children with Optic Nerve Glioma do not need any treatment. This is because many gliomas grow so slowly that they do not cause any further problems. Children who also have Neurofibromatosis tend to have very slow growing gliomas that are less likely to cause visual loss. Regular checks are still needed at the clinic. Scans can help to see if the glioma is getting any bigger.

If the glioma is getting bigger and causing new problems then treatment may help. Problems that might develop include:

  • More obvious pushing forward of the eye (proptosis)
  • Increasingly blurred visual loss
  • Further growth backwards that may affect the optic chiasm (and vision in the other eye)

If these problems develop there are three kinds of treatment that can help. Any decision on treatment is discussed fully with the child and their parents. Most treatments can have side effects. The benefit to the child must always be weighed against any possible damaging side effects of the treatment.

Surgery
A surgeon might try to remove the glioma and the optic nerve by an operation. This will result in total visual loss on this side. Other parts of the brain might be damaged during an operation including the optic chiasm. This might cause visual loss in the other eye. By doing this operation it is hoped that all the Glioma is removed and that it will not recur causing new problems.

Radiotherapy
Often X-ray treatment is recommended. This is called radiotherapy. X-rays are normally used to take pictures of patient's lungs and broken bones. When concentrated and focused on to a glioma they can help slow down and stop its growth. This may cause damage to the optic nerve leading to further visual loss.

Chemotherapy
Sometimes pills to be taken or drugs injected into a child's veins are also recommended. This is called chemotherapy.

How can parents, family, friends and teachers make a difference?
We use our vision to get around, learn new things and to meet other people and make friends. It is important to be aware of your child’s own special problems with vision.

Problems at school may be due to some of the reading books being hard to see. This often means it takes longer and more effort to do the work. If the size of print is increased most children will find schoolwork easier. If a Low Visual Aid (LVA) or spectacles have been prescribed then try to encourage their use. This will help the child see more clearly. It will also help the vision parts of the brain to grow and develop.

It is worth watching carefully to find out what the smallest toys are that a child can see and play with. Then try to only play with toys that are the same size or bigger.

Recognising facial expressions can often be difficult. It is worth trying to find out at what distance facial expressions can be seen and responded to. Then always try to talk and smile from within this distance. This helps a child to learn what facial expressions mean and to copy them. If the child has visual field loss try to place objects in the part of the child's vision that is working.

Infants and young children need to learn about the world around them. Home visiting teachers, physiotherapists and occupational and speech therapists may all add to the child's care and education. It is important to continue the programmes that they recommend. If the child is involved in family activities vision can improve and new skills can develop.

Even if a child has very poor vision many useful and practical things can be done to improve the ability of the child to get around, interact with other children and learn.