Optic nerve glioma (ONG) is the most predominant type of neoplasm of the optic nerve [1]. These tumors are frequently unilateral and have a predilection for females [1]. They occur sporadically although familial cases do develop in patients with neurofibromatosis 1 (NF1) as these diseases are strongly associated [1] [2]. Furthermore, most ONGs occur in children while three-fourth of sufferers experience symptoms within the first decade and 90% by the age of 20 years [3].

The clinical presentation includes decreased visual acuity and function, progressive proptosis, and swelling of the optic disc [1] [4]. Strabismus is another common feature [4]. There is no pain associated. Some cases will suffer from acute blindness and worsening proptosis secondary to hemorrhage [5]. Note that not all ONGs produce complaints as some are detected incidentally during routine screening in those with NF1 [4].

Complications

Chronic compression of the central retinal vein can result in central retinal vein occlusion (CRVO) and other severe eye pathologies [1].

Physical exam

Fundoscopy reveals pallor and swelling of the optic disc and impaired acuity [4] [6]. Moreover, the eye exam demonstrates an abnormal pupillary reflex as well as visual field deficits. The latter reflects the affected optic nerve segments. For example, the involvement of the portion anterior to the optic chiasm results in a visual loss in the ipsilateral eye. Additionally, a defect in the optic chiasm causes bitemporal hemianopsia whereas a damaged segment posterior to the chiasm leads to homonymous hemianopsia.

Proptosis is apparent and may appear cosmetically disfiguring [6]. Eye movements may also be affected [6].

Patients with NF-1 will exhibit skin lesions in addition to other features.

• Turkish

  History Meanings of "optic nerve glioma" in Turkish English Dictionary : 1 result(s) Category English Turkish Optics 1 Optics optic nerve glioma optik sinir gliomu Pronunciation of optic nerve glioma Term Options [tureng.com]

• Unilateral Proptosis

  A 2 and a half year-old boy with neurofibromatosis developed unilateral proptosis, decreased visual acuity, and optic disk edema. [ncbi.nlm.nih.gov]

  Gradual, painless, unilateral proptosis associated with loss of vision and an afferent pupillary defect is a common presentation. Proptosis often is non-axial, with temporal or inferior dystopia. [eyewiki.aao.org]

• Flushing

  We present the case of a 16-year-old boy who underwent total resection of an optic nerve glioma beginning proximally at the chiasm and ending distally flush with the globe. After resection, heavy bipolar cautery was applied. [ncbi.nlm.nih.gov]

• Cesarean Section

  She delivered a healthy baby by cesarean section. A left frontotemporal craniotomy was then performed. Incision of the lateral surface of the left optic nerve revealed clotted blood from the left optic nerve and the left side of the chiasm. [ncbi.nlm.nih.gov]

• Papilledema

  He had papilledema in his remaining eye. At exploration, a cerebellar astrocytoma and a neuroglial hamartoma were removed. [ncbi.nlm.nih.gov]

  […] performed to see the entire extent of the lesion since it could appear to be more extensive than it is on T2-weighted images secondary to edema Differential Diagnosis Sarcoidosis Infiltration by leukemia or lymphoma Optic neuritis Perineural hematoma Papilledema [eyefacialplasticsurgery.com]

  […] performed to see the entire extent of the lesion since it might appear to be more extensive than it is on T2-weighted images secondary to edema Differential Diagnosis Sarcoidosis Infiltration by leukemia or lymphoma Optic neuritis Perineural hematoma Papilledema [drelizabethnguyen.com]

  […] performed to see the entire extent of the lesion since it may possibly appear to be more extensive than it is on T2-weighted images secondary to edema Differential Diagnosis Sarcoidosis Infiltration by leukemia or lymphoma Optic neuritis Perineural hematoma Papilledema [eyeplastics.com]

  Definition / general Relatively rare Slow growing tumor within orbital segment of optic nerve Usually ages 0 - 9 years with symptoms of minimal exophthalmos, optic nerve atrophy or papilledema Associated with neurofibromatosis type 1 Radiology description [pathologyoutlines.com]

• Frontal Headache

  A 15-year-old girl presented with a 3-year history of frontal headaches and a 5-month history of blurred vision in the right eye. [ncbi.nlm.nih.gov]

• Unable to Walk

  After being unable to walk or practice her favorite activity, dancing, for nearly a year, she was diagnosed at age 10 with a cancer no doctor... more [alexslemonade.org]

Those coming with the above clinical manifestations should be considered for optic nerve glioma. Additionally, children with NF-1 should be screened for this tumor. The workup will include a thorough history, assessment of the symptoms, a detailed ophthalmic and general physical exam, and the appropriate studies.

Imaging

Magnetic resonance imaging (MRI) is the preferred diagnostic tool for optic nerve gliomas. This modality is more effective than computed tomography (CT) in characterizing the intraorbital tumor and the extent of its growth as it broadens into the chiasm [1] [7] [8]. CT scanning typically depicts lesions confined within the orbit. Note that both MRI and CT show an enlarged optic canal if present, although this finding does not necessarily indicate an intracranial extension.

MRI with gadolinium enhancement and fat saturation characterizes the extent of the lesion. Moreover, T1-weighted tumors are described as hypointense to isointense [9] [10] whereas T2-weighted masses are revealed as isointense or hyperintense.

Very importantly, an ONG appears differently on imaging in patients with NF1 than it does in isolated cases [1]. When associated with NF1, the lesion can expand into subarachnoid and subdural spaces [11]. In individuals without NF1, imaging tends to feature diffuse fusiform expansion of the optic nerve with a bend or kink [1] [12].

1. Miller NR. Primary tumours of the optic nerve and its sheath. Eye (Lond). 2004;18(11):1026-37.
2. Listernick R, Louis DN, Packer RJ, Gutmann DH. Optic pathway gliomas in children with neurofibromatosis type 1: consensus statement from the NF1 Optic Glioma Task Force. Ann Neurol. 1997;41(2):143–149.
3. Chutorian AM, Schwartz JF, Evans RA, Carter S. Optic gliomas in children. Neurology. 1964;14:83–95.
4. Tow SL, Chandela S, Miller NR, Avellino AM. Long-term prognosis in children with gliomas of the anterior visual pathway. Pediatr Neurol. 2003;28(4):262–270.
5. Sharma A, Mohan K, Saini JS. Haemorrhagic changes in pilocytic astrocytoma of the optic nerve. Orbit 1990; 9(1): 29–33.
6. Chen A, Yoon MK, Haugh S, et al. Surgical management of an optic nerve glioma with perineural arachnoidal gliomatosis growth pattern. J Neuroophthalmol. 2013;33(1):51-53.
7. Aoki S, Barkovich AJ, Nishimura K, et al. Neurofibromatosis types 1 and 2: cranial MR findings. Radiology. 1989;172(2):527-534.
8. Hendrix LE, Kneeland JB, Haughton VM, et al. MR imaging of optic nerve lesions: value of gadopentetate dimeglumine and fat-suppression technique. AJNR Am J Neuroradiol. 1990;11(4):749-754.
9. Glass LR, Canoll P, Lignelli A, Ligon AH, Kazim M. Optic nerve glioma: case series with review of clinical, radiologic, molecular, and histopathologic characteristics. Ophthal Plast Reconstr Surg. 2014;30(5):372-376.
10. Shofty B, Mauda-Havakuk M, Weizman L, et al. The effect of chemotherapy on optic pathway gliomas and their sub-components: A volumetric MR analysis study. Pediatr Blood Cancer. 2015;62(8):1353-1359.
11. Stern J, Jakobiec FA, Housepian EM. The architecture of optic nerve gliomas with and without neurofibromatosis. Arch Ophthalmol. 1980; 98: 505–511.
12. Brodsky MC. The 'pseudo-CSF' signal of orbital optic gliomas on magnetic resonance imaging: a signature of neurofibromatosis. Surv Ophthalmol. 1993; 38(2):213–218.