Optic nerve glioma (or optic glioma), a form of glioma which affects the optic nerve, is often one of the central nervous system manifestations of neurofibromatosis 1.Optic gliomas are usually pilocytic tumors, and can involve the optic nerve or optic chiasm. Optic gliomas are usually associated with neurofibromatosis type 1 in 30% of people with the condition.Optic nerve gliomas have low mortality but extremely high prevalence of vision loss & eye-bulging exophthalmos) in children. As of 2014, approximately 1000 cases had been reported. Diagnosis
Optic nerve gliomas are diagnosed using magnetic resonance imaging (MRI) and CT scans. The tumor adopts a fusiform appearance, appearing wider in the middle and tapered at the ends. Enlargement of the optic nerve along with a downward kink in the mid-orbit is usually observed. While CT scans allow for optic nerve evaluation, MRI allows for intracranial evaluation to observe if the tumor has extended to other regions such as the optic chiasm & hypothalamus. Treatment
The main goal of treating optic gliomas is to preserve vision for as long as possible. The tumor’s slow & self-limiting growth indicates that it is not immediately problematic in most benign cases, with long-term studies showing that people with optic glioma may still have stable functional vision without intervention. As a result, the first & preferred course of action is usually observation of optic glioma over time.Once the first signs of visual deterioration and/or tumor progression are observed, interventional treatments will then commence. These include radiation therapy, [[chemotherapy and surgical excision.
Individuals with the disorder need to be advised on its inheritance, complications, and preventative measures that can be taken to avoid bleeding and/or thrombosis. Since >80% of individuals may develop bleeding or thrombosis complications of the disorder, asymptomatic individuals diagnosed with hydposyfibrinogenemia are best handled at a specialized center in order to benefit from multidisciplinary management.Measures to prevent and/or treat complications of hypodysfibrinogenemia should be tailored to the personal and family history of the individual by a specialized center. Individuals with a personal or family history of bleeding are considered to be of low risk of bleeding when their functional fibrinogen levels are >1 gram/liter for major surgery, >0.5 gram/liter for minor surgery, >0.5 to 1-2 gram/liter for spontaneous bleeding (depending on its severity), >0.5 to > 1 gram/liter for the first two trimesters of pregnancy, and >1 to <2 gram/liter for the last trimester of pregnancy and postpartum period. Functional fibrinogen below these levels should be treated preferably with fibrinogen concentrate or if not available, fibrinogen-rich cryoprecipitate or plasma to attain low risk levels of functional fibrinogen. Antifibrinolytic drugs such as tranexamic acid or (ε-aminocaproic acid) may be considered as an alternative preventative or therapeutic treatments in cases of minor surgery, dental extractions, mucosal bleeding, or other episodes of mild bleeding. In individuals with a personal or family history of thrombosis, should be considered for long-term anticoagulation drugs such as low molecular weight heparin, coumadin, or rivaroxaban. References


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