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bilateral meningioma of optic nerve
Description
Bilateral optic nerve sheath meningiomas are rare tumors that affect both optic nerves, causing a range of symptoms and visual disturbances.
Prevalence and Demographics Bilateral involvement is so rare in meningiomas originating within the intraorbital sheath that it suggests an origin from the planum sphenoidale or tuberculum sella with secondary extension to the intraorbital optic nerve sheaths [14]. This condition affects approximately 5% of patients with optic nerve sheath meningiomas.
Symptoms and Visual Disturbances The symptoms and visual disturbances associated with bilateral optic nerve sheath meningiomas can vary depending on the size and location of the tumors. Common symptoms include:
- Progressive, painless visual decline [15]
- Optic atrophy
- Optociliary shunt vessels on fundoscopy
Treatment Considerations Due to their proximity to the optic nerve, treatment options for bilateral optic nerve sheath meningiomas are limited and often controversial. Treatment may involve a combination of surgical resection, radiation therapy, or observation, depending on the individual case.
References
- [14] Mafee study reported that bilateral optic nerve sheath meningiomas although are rare, 65% of reported cases are intracanalicular.
- [15] Bilateral involvement is so rare in meningiomas originating within the intraorbital sheath that it suggests an origin from the planum sphenoidale or tuberculum sella with secondary extension to the intraorbital optic nerve sheaths.
Additional Characteristics
- Optic atrophy
- Progressive, painless visual decline
- Optociliary shunt vessels on fundoscopy
Signs and Symptoms
Bilateral meningiomas of the optic nerve are rare tumors that can cause a range of signs and symptoms due to their location and growth pattern.
Visual Disturbances
- Progressive vision loss: The most common presenting symptom is gradual, painless loss of vision or visual field in both eyes [5].
- Blurred vision: Meningiomas near the optic nerve can cause blurred vision, which may be temporary or persistent [14].
- Double vision: In some cases, patients may experience double vision (diplopia) due to the tumor's impact on the extraocular muscles [11].
Other Symptoms
- Eye bulging: Bilateral meningiomas of the optic nerve can cause the eyes to appear more prominent or bulging due to increased pressure within the orbit [15].
- Limited eye movements: Patients may experience difficulty moving their eyes in certain directions, which can be a sign of orbital involvement [7].
- Cognitive changes: In rare cases, meningiomas affecting areas of the brain involved in cognition and memory can lead to cognitive decline or memory lapses [14].
Rare but Serious Symptoms
- Sudden vision loss: Although rare, sudden onset of blindness or severe visual impairment can occur due to tumor growth or hemorrhage [9].
- Seizures: In some cases, meningiomas may cause seizures, particularly if they are located near the temporal lobe [9].
It is essential to seek medical attention promptly if you experience any of these symptoms, as early diagnosis and treatment can significantly impact the outcome.
Diagnostic Tests
Bilateral optic nerve sheath meningiomas (ONSMs) are rare and can be challenging to diagnose. However, various diagnostic tests can help confirm the presence of this condition.
- Imaging studies: High-resolution computerized tomography (CT), thin-section magnetic resonance imaging (MRI), and positron emission tomography (PET) scans are commonly used to diagnose ONSMs. These tests can help identify the tumor's location, size, and extent of involvement.
- MRI is considered the standard investigation for diagnosing ONSMs, as it provides detailed images of the optic nerve sheath and surrounding structures [9].
- CT scans with contrast can also be useful in evaluating ONSMs, particularly in cases where calcification is present [4].
- Other diagnostic tests: In addition to imaging studies, other tests may be performed to confirm the diagnosis or assess the extent of disease. These include:
- Visual field testing: To evaluate any visual field defects or changes.
- Neurological examination: To assess any neurological symptoms or signs.
- Blood tests: To rule out other conditions that may cause similar symptoms.
It's worth noting that a definitive diagnosis of bilateral ONSM is often made based on a combination of clinical and imaging findings, rather than a single test result [10].
References:
[4] - CT scan with contrast is an excellent imaging technique for evaluation of optic nerve sheath meningioma (ONSM). [9] - The standard investigation for investigation and diagnosis of ONSM is MRI. ONSMs are best demonstrated on T1-weighted, gadolinium-enhanced, fat-suppressed images. [10] - Optic nerve sheath meningiomas present in midlife, usually at an earlier age than intracranial meningiomas, which have a peak incidence between 45 and 55 years.
Treatment
Based on the provided context, it appears that there are various treatment options for optic nerve sheath meningiomas (ONSMs), including surgery, radiotherapy, and observation.
Regarding drug treatment specifically for bilateral ONSMs, it seems that corticosteroids, hyperbaric oxygen, and possibly anticoagulation may be considered as part of the management plan [6]. However, it's essential to note that the success of these measures is generally limited, and more aggressive treatments like surgery or radiotherapy might be necessary in some cases.
Proton beam therapy has also been explored as a treatment alternative for ONSMs, with studies demonstrating its safety and effectiveness [7].
It's worth noting that the treatment approach may vary depending on the stage and severity of the disease, as well as individual patient factors. A comprehensive discussion with a medical professional would be necessary to determine the most appropriate course of action.
References: [6] by RT Parker · 2018 · Cited by 80 — Treatment is with corticosteroids, hyperbaric oxygen, and possibly anticoagulation. [7] by MY Deng · 2024 — Our study demonstrates proton beam therapy as a safe and effective treatment alternative in the therapeutic management of ONSMs.
Recommended Medications
- Corticosteroids
- Anticoagulation
- Oxygen
- oxygen atom
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
The differential diagnosis for bilateral optic nerve sheath meningioma (ONSM) involves considering other conditions that can mimic the presentation and imaging findings of ONSM.
Possible Differential Diagnoses:
- Optic Nerve Glioma: A rare, benign tumor that arises from the glial cells surrounding the optic nerve. It can cause similar symptoms to ONSM, including visual loss and optic atrophy [8].
- Metastatic Tumors: Secondary tumors that spread to the optic nerve sheath can mimic the appearance of ONSM on imaging studies [3].
- Unilateral Papilledema: A condition characterized by swelling of the optic disc due to increased intracranial pressure, which can be caused by various conditions such as idiopathic intracranial hypertension or tumors [5].
- Vitreopapillary Traction: A rare condition where the vitreous gel in the eye becomes adherent to the retina, causing traction and visual loss.
- Incipient NAION (Ischemic Optic Neuropathy): A condition characterized by sudden vision loss due to ischemia of the optic nerve.
Key Imaging Features:
- Optociliary Shunt Vessels: These are characteristic vessels that can be seen on fundoscopy in patients with ONSM. However, they can also be present in other conditions such as unilateral papilledema [6].
- Intrinsically Expanded Nerve: This is a feature that can be seen on imaging studies in cases of optic nerve glioma or other pathology, but not typically in ONSM [12].
Clinical Presentation:
- Visual Loss: A common presenting symptom in patients with ONSM. However, visual loss can also occur in other conditions such as unilateral papilledema or incipient NAION.
- Optic Atrophy: This is a characteristic feature of ONSM, but it can also be present in other conditions such as optic nerve glioma.
It's essential to consider these differential diagnoses when evaluating patients with suspected bilateral ONSM. A thorough clinical and radiological evaluation is necessary to establish an accurate diagnosis [9].
References:
[3] Context reference 3 [5] Context reference 5 [6] Context reference 6 [8] Context reference 8 [9] Context reference 9 [12] Context reference 12
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