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Optic glioma

Every year, approximately 10-15 children in the Netherlands are diagnosed with opticus glioma. The disease occurs in children of all ages. Optic glioma is a low-grade glioma in the optic nerve, the junction of the optic nerves and/or the optic nerve pathways to the back of the brain.

The brochure entitled Low-grade gliomas contains comprehensive information about this disease. The most important information is provided below.


Causes

Gliomas are caused by a developmental error in a (progenitor) cell of the supporting cells of the brain. Probably something is changed in the DNA in the nucleus of the cell. The cause of that change is unknown. Some children have neurofibromatosis type I, a condition whereby tumors can develop in the nervous system.

 

Symptoms

Children with an optic glioma almost always have vision problems: either their vision is blurred or it is reduced. Some children have eye movement disorders or misaligned eyes. A tumor at the junction of the optic nerves can flatten the pituitary gland and or the hypothalamus, resulting in hormone disorders such as restricted growth, early puberty or obesity.

How are opticus gliomas diagnosed?

An MRI scan is performed as well as a thorough eye test. Sometimes a biopsy is performed.

Treatment

Children with an opticus glioma are treated according to a protocol drawn up by national and international experts. The treatment can consist of chemotherapy, surgery and in some instances radiotherapy, and depends, among other things, on the symptoms, the location, the age of the child and the possible occurrence of neurofibromatosis I. For some children, treatment is put on hold, in which case they are closely monitored, undergo regular MRI scans and get regular eye check-ups. The brain tumor working group, comprising all the specialists involved, carefully considers what is best for your child: wait and see or more treatment. If follow-up treatment is required, it is usually chemotherapy. If the tumor does not respond well to chemotherapy, surgery can be an option. It is not possible to remove the whole tumor without causing blindness or hormone deficiency. The aim of the treatment is to get the tumor under control. Older children sometimes receive radiotherapy.

Chance of recovery

Because an optic glioma cannot be removed completely, a residual tumor is almost always left behind. It usually stabilizes over time, but it does mean that your child has a chronic illness.

Questions?

Children with an optic glioma are treated in the neuro-oncology department.