Intra-Orbital Meningioma Causing Loss of Vision in Neurofibromatosis Type 2: Case Series and Management Considerations

Abstract

Objectives: Little evidence exists regarding the management of orbital meningioma causing vision loss in the setting of neurofibromatosis Type 2 (NF2). We review here our experience with patients at risk for blindness due to intra-orbital meningioma.Design/Setting/Participants/Main Outcome Measures: The charts of patients with NF2 presenting for evaluation of intra-orbital meningioma and vision impairment between 2008 and 2013 were retrospectively reviewed in accordance with institutional IRB policies. Patients with primarily extra-orbital tumors and minimal intra-orbital extension were excluded. Charts were analyzed for the presence and/or imaging progression of intra-orbital tumor, presence of other optic apparatus pathology, presence and/ or progression of vision impairment, and intervention performed (if any).Results: Seven patients with intra-orbital meningioma on MRI and bilateral vision loss and/or unilateral visual impairment due to tumor and contralateral blindness of any etiology were identified. Patients without salvageable vision in either eye were excluded (n = 3). Diagnosis of meningioma was obtained either by biopsy or based on imaging assessment.Conclusion: Conservative management of orbital tumors in NF2 may be preferred in asymptomatic patients but may not be acceptable in patients with progressive visual decline. Radiation is a reasonable option for meningiomas of the orbit and optic nerve sheath. Finally, although the benefit of cranial nerve decompression in NF2 for preservation of facial nerve and hearing has previously been established, the role of optic nerve decompression for preservation of vision in NF2 remains poorly defined.

Large Orbital Tumor in a Patient with Neurofibromatosis Type 2

“…All patients with orbital meningioma require close follow-up with serial MRI, visual field, and acuity testing. Successful strategies for preserving vision in NF2 patients with orbital meningioma include observation, microsurgical resection of tumor, or optic nerve decompression with or without radiosurgery [14]. Surgical excision is mainly discussed for symptomatic lesions.…”

Effectiveness and Safety of Robotic Radiosurgery for Optic Nerve Sheath Meningiomas: A Single Institution Series

“…The presence of bilateral tumors occurs in about 5–10% of ONSM patients, caused by overgrowth from the opposite side. However, some are independent tumors, occasionally associated with neurofibromatosis type 2 [ 1 , 2 , 8 , 9 ].…”

Imaging depth extension of optical coherence tomography in rabbit eyes using optical clearing agents

“…Because of the specific deep location, B-mode ultrasound and MRI have been commonly used for the diagnosis of orbit disease. 5,6 However, although B-mode ultrasound and MRI provide images of tumor boundaries at low resolution,…”

“…Because of the specific deep location, B-mode ultrasound and MRI have been commonly used for the diagnosis of orbit disease. 5 , 6 However, although B-mode ultrasound and MRI provide images of tumor boundaries at low resolution, they cannot provide information about the mechanism of the tumor growth, especially the neovascularization features. OCT has been used to image the structure and function of the fundus, and even the vascular of choroidal tumors, 7 but due to the limitation of imaging depth, there have been no reports of OCT imaging of intra-orbit tumor or orbital inflammation.…”

“…For optical scattering reduction to enhance light depth penetration, a tissue optical clearing (TOC) technique was proposed to reduce light scattering by using optical clearing agents (OCAs), which decrease the difference of refractive index between tissues. 6 , 12 In this technique, glycerol and glycerol-water solution, glucose, propanediol, polyethylene glycerol, polypropylene glycerol, oleic acid, and DMSO are typically used. 13 Thick tissues, even heart, liver, and bones of mouse become transparent after optical clearing treatment.…”