Optic nerve sheath meningioma
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [1] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Overview
Optic nerve sheath meningiomas (ONSM) are rare benign tumors of the optic nerve. 60-70% of cases occur in middle age females, and is more common in older adults (mean age 44.7 years). It is also seen in children, but this is rare. The tumors grow from cells that surround the optic nerve, and as the tumor grows, it compresses the optic nerve. This causes loss of vision in the affected eye. [2]
It is typically a slow growing tumor, and has never been reported to cause death. However, there is concern that the tumor can grow into the brain and cause other types of neurological damage. In some patients, the tumor grows so slowly that treatment is not necessary. Standard treatments are observation, surgery, radiation therapy, and combinations of the above. [3]
Incidence
About 1-2% of all meningiomas are optic nerve sheath meningiomas. Meningiomas have an incidence of ~4.18/100,000 persons each year. Thus, ~10,000 meningiomas are diagnosed in the US each year; corresponding to ~100 cases of ONSM each year in the US. The actual number of meningiomas is likely much higher as it is very common in elederly women. ONSM comprises about 2% of orbital tumors, and about 10% of optic nerve lesions.[4]
Neurofibromatosis type II (NF-2) affects around 9% of ONSM patients, where the incidence in the general population is around 0.03-0.05%. Thus NF-2 is felt to be a risk factor for the development of ONSM.[5]
Diagnosis
Usually diagnosis can be achieved by an MRI of the brain or orbits when the patient presents with chronic visual loss, optic disc atrophy, and retinocilliary shunt vessels (the so called Hoyt-Spencer triad).[6]
Treatment
Most ophthalmologists will not advocate any treatment unless visual loss is present and ongoing. Reports of patients with ONSM having no change in their vision for multiple years are not uncommon. If loss of vision occurs, radiation therapy will improve vision in about 1/3rd of cases, and preserve vision in about 1/3rd of cases. Surgery has traditionally been associated with rapid deteroriation of vision. However, newer surgical techniques may prove better for the treatment of ONSM.[7][8][9]
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
