What are the other Names for this Condition? (Also known as/Synonyms)

• Juvenile Pilocytic Astrocytoma
• Optic Nerve Glioma

What is Optic Glioma? (Definition/Background Information)

• Optic Glioma is a brain tumor that occurs in and around the optic nerves. The optic nerve connects the eye with the brain. This tumor type is common and frequent in children with a genetic condition, known as neurofibromatosis
• Optic Glioma can be unilateral (one eye is affected) or bilateral (both eyes are affected) type. This condition can affect:
  • One or both the optic nerves that carry visual information from the eye to the brain
  • The optic chiasma - the place where both the optic nerves cross each other in front of the hypothalamus of the brain
• The exact cause of Optic Glioma is unknown and is currently under investigation
• The typical symptoms associated with Optic Glioma are involuntary movement of eyeballs, vision defects, squinting, loss of peripheral or total vision in one or both eyes, memory lapses, loss of appetite, and even precocious puberty
• The treatment options for Optic Glioma may include partial or complete removal of the tumor, steroid treatment to reduce swelling, and radiation therapy. The size of the tumor and age of the child generally dictate the kind of treatment that is undertaken
• A prompt diagnosis and treatment could lead to complete recovery. However, if the diagnosis and/or treatment is delayed, permanent blindness might occur. It is therefore necessary to have routine eye exams for children, as a preventive and precautionary function

Who gets Optic Glioma? (Age and Sex Distribution)

• Young children under the age of 10 years are usually affected by Optic Glioma
• In rare cases, children under 5 years have also been reported with this condition
• Both boys and girls are equally affected
• No particular race or ethnic group is affected by Optic Glioma; this condition is observed worldwide

What are the Risk Factors for Optic Glioma? (Predisposing Factors)

• Children with neurofibromatosis (a genetic disorder) have a high risk for developing Optic Glioma
• No other significant risk factors have been identified

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Optic Glioma? (Etiology)

• The exact cause for the occurrence of Optic Glioma is not known
• Nevertheless, children with neurofibromatosis show an increased susceptibility to this condition for unknown reasons

What are the Signs and Symptoms of Optic Glioma?

The signs and symptoms of Optic Glioma include:

• Poor vision quality; loss of vision in one or both the eyes
• Involuntarily movement of the eyeballs, squinting
• Bulging out of the eyes (either unilateral or bilateral)
• Loss of peripheral vision
• Growth anomalies
• Precocious puberty

The brain tumor near or around the optic nerve may also cause:

• Sleeping during daytime
• Loss or decrease in memory
• Decreased brain function
• Delayed growth
• Loss of appetite
• Loss of body fat

How is Optic Glioma Diagnosed?

The healthcare provider may employ the following methods to help diagnose Optic Glioma:

• Complete physical examination with comprehensive medical history including family history
• Thorough neurological examination to check for
  • Vision in both the eyes
  • Changes in the optic nerve
  • Swelling or scars around the optic nerve
  • Damage to the optic disc

Other diagnostic tests and procedures may include:

• Cerebral angiography
• CT scan of the head, MRI scan of the brain
• Visual field tests
• Tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Optic Glioma?

The following are common complications that may arise due to Optic Glioma:

• The child’s endocrinological or hormone system may be affected
• Severe decrease in vision quality and complete blindness, when left untreated
• Advanced puberty

How is Optic Glioma Treated?

The goals of the treatment are to restore or improve vision quality, cure the condition, and address the symptoms. Towards this, the treatment and management of Optic Glioma will depend on the following factors:

• General health of the patient or child
• Size of the tumor

The various treatment methods may include:

• Removal of the tumor by surgery to protect the brain tissue around the tumor; else, a partial removal of the tumor when possible, so as to reduce the size of the tumor
• Radiation therapy is recommended, when surgery is not possible and/or the tumor is large in size
• Chemotherapy may be recommended, when the tumor has spread to the hypothalamus of the brain
• Corticosteroids may be prescribed to reduce swelling and inflammation

How can Optic Glioma be Prevented?

Currently, it may not be possible to prevent the formation of Optic Glioma; however, the incidence of the condition can be reduced by:

• Genetic counseling for parents of the children with neurofibromatosis
• Regular eye checkups that could help in early diagnosis of the tumor and facilitate prompt medical attention and treatment

What is the Prognosis of Optic Glioma? (Outcomes/Resolution)

• The outcome of the treatment of Optic Glioma is good, if prompt treatment is provided
• It is possible to surgically remove most tumors successfully, but there is a likelihood of tumor recurrence
• A late diagnosis and delayed treatment of Optic Glioma may result in a poor prognosis

Additional and Relevant Useful Information for Optic Glioma:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/cancer/