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Optic Nerve Glioma

Optic Gliomas

Optic nerve glioma is the most common type of tumor of this nerve. While it occurs in the pediatric and adult populations, clinical manifestations typically become apparent in childhood. If symptomatic, signs include decreased visual acuity, proptosis, strabismus, and swelling of the optic disc. This tumor is strongly associated with neurofibromatosis 1 (NF1) but can exist alone. The diagnosis is based on a detailed history, eye and general exam, and imaging.


Presentation

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.

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]
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]
  • We report a 14-y-old girl with colonic polyposis and unilateral proptosis for whom an excisional biopsy of the orbital lesion was performed. Histopathological evidence of juvenile pilocytic astrocytoma grade 1 was detected.[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]
Progressive Visual Loss
  • Patient 1 is a 17-year-old girl who presented with acute onset of pain and rapidly progressive visual loss in the right eye. Patient 2 is a 38-year-old man who presented with painless progressive visual loss in the left eye.[ncbi.nlm.nih.gov]
  • Patients with optic pathway gliomas most frequently present in the first decade with a median age of 6.5 years, with slowly progressive visual loss, followed later by proptosis (although this sequence may occasionally be reversed).[eyewiki.aao.org]
  • They may involve the optic nerve and/or the chiasm, with slowly progressive visual loss. With third ventrical or midbrain invasion, mortality may be as high as 50%. The left eye is proptotic.[atlasophthalmology.net]
Rubeosis Iridis
  • Slit lamp examination revealed rubeosis iridis, a swollen pale optic disc, and vitreous hemorrhage. After medical treatment, the IOP decreased to 34 mmHg, and no pain was reported.[ncbi.nlm.nih.gov]
Progressive Loss of Vision
  • They may also result in progressive loss of vision in one or both eyes. The cause of optic glioma is unknown. Tumors may occur at any age but the incidence is higher in children.[prep4usmle.com]
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 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]
  • […] 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]
  • 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]
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]

Workup

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].

Treatment

  • The treatment of optic nerve glioma requires a multi-disciplinary approach where all treatment options may have to be implemented in a highly individualized manner.[ncbi.nlm.nih.gov]
  • We discuss the treatment strategy in pediatric patients with orbital ONG associated with NF type 1.[ncbi.nlm.nih.gov]
  • There is controversy over the best treatment for these patients.[ncbi.nlm.nih.gov]
  • The treatment modalities for neurofibromatosis type 1-associated optic gliomas include chemotherapy, radiation therapy, and surgical excision.[ncbi.nlm.nih.gov]
  • Given the scarcity of reports on this subject, the authors support more extended studies of the CyberKnife for the effective treatment of this relatively common childhood tumor.[ncbi.nlm.nih.gov]

Prognosis

  • RESULTS: "En bloc" removal in patients with optic nerve glioma led to complete visual deficit but ensures excellent long-term prognosis.[ncbi.nlm.nih.gov]
  • In general, patients with NF-1 have a better visual prognosis and their optic nerve gliomas rarely cause progressive vision loss beyond the age of 12.[healio.com]
  • Patients with an optic glioma and neurofibromatosis type 1 have a better prognosis than those who do not have neurofibromatosis.[thamburaj.com]
  • Optic gliomas of adulthood seem to comprise a distinct entity with an extremely poor prognosis.[jpgmonline.com]
  • They also have a more favorable prognosis compared with all other astrocytoma types. The most common site of juvenile pilocytic astrocytomas is the cerebellum.[neurosurgery.ucla.edu]

Etiology

  • After discussing the different possible etiologies to the wedge-shaped defects, the disruption of the nerve fiber layer due to the chorioretinal lesion was considered the most plausible cause.[ncbi.nlm.nih.gov]
  • Clinical Symptoms decreased visual acuity minimal proptosis restricted, decreased eye movement strabismus Etiology neoplasm of the optic nerve sometimes referred to as juvenile pilocytic astrocytoma Optic nerve glioma in adults are glioblastoma Optic[eyefacialplasticsurgery.com]
  • Clinical Symptoms decreased visual acuity minimal proptosis restricted, decreased eye movement strabismus Etiology neoplasm of the optic nerve may also be called juvenile pilocytic astrocytoma Optic nerve glioma in adults are glioblastoma Optic nerve[drelizabethnguyen.com]
  • Some hypotheses could be made about the possible etiologies for this case. Sector scotomas associated with posterior uveitis such as toxoplasmosis have already been described.[scielo.br]

Epidemiology

  • Histology of Optic Nerve Glioma usually slowly growing tumour (pilocytic astrocytoma WHO grade I Epidemiology of Optic Nerve Glioma 80% occur in first decade of life very rarely occur in adulthood neurofibromatosis type I as known risk factor Symptoms[hirntumorhilfe.org]
  • Diagnosis: Optic nerve glioma associated with Neurofibromatosis Type 1 EPIDEMIOLOGY of NF1 Rare condition (1 per 3-4,000 people) characterized by hamartomas of neural crest origin Autosomal dominant inheritance Variable expressivity, 100% penetrance Half[eyerounds.org]
  • Epidemiology OPGs account for 3-5% of all brain tumors in children. Approximately 75% of OPGs are diagnosed within the first decade, and 60% are diagnosed before 5 years of age. The mean age of presentation is 8.8 years.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • The association of optic glioma and other intracranial neoplasms in patients with NF1 suggests that there are fundamental pathophysiological differences between patients with and without optic glioma [4].[wikigenes.org]

Prevention

  • She was treated with chemotherapy to prevent further extension of her sight-threatening tumour. Three years after chemotherapy her growth hormone hypersecretion has resolved although she has gone on to develop precocious puberty.[ncbi.nlm.nih.gov]
  • To date no consensus has been reached regarding the therapeutic approach and prevention of visual impairment in these patients.[ncbi.nlm.nih.gov]
  • They bring together our team of multidisciplinary experts to deliver a coordinated approach to neuroscience, from research and prevention to treatment and post-treatment care.[neuro.northshorelij.com]
  • There are no treatments with demonstrated effectiveness in curing optic gliomas or preventing the vision loss that may be associated with them.[healio.com]
  • Most kids do well with treatment, and further vision loss usually is prevented. All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.[kidshealth.org]

References

Article

  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.

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Last updated: 2019-07-11 21:27