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.

• Pallor

  Clinical manifestations include decreased visual acuity; EXOPHTHALMOS; NYSTAGMUS, PATHOLOGIC; STRABISMUS; pallor or swelling of the optic disc; and INTRACRANIAL HYPERTENSION. The tumor may extend into the optic chiasm and hypothalamus. [rareomics.healx.io]

  Physical exam Fundoscopy reveals pallor and swelling of the optic disc and impaired acuity. Moreover, the eye exam demonstrates an abnormal pupillary reflex as well as visual field deficits. The latter reflects the affected optic nerve segments. [symptoma.com]

  Initial signs and symptoms of malignant gliomas include severe retro-orbital pain, unilateral or bilateral vision loss, and, typically, massive swelling and hemorrhage of the optic nerve head (although disc pallor may also be observed with posterior lesions [eyewiki.aao.org]

  The finding of optic nerve pallor or other visual problems an MRI scan. An MRI scan is extremely sensitive in the diagnosis of an optic nerve or visual pathway glioma. [childrensnational.org]

• Inflammation

  Differentiating between optic nerve neoplasm and inflammation may be difficult. On occasion, the classic clinical finding of pain with eye movement and the radiographic finding of enlargement and enhancement of the optic nerve may be misleading. [ncbi.nlm.nih.gov]

  Corticosteroids may be prescribed to reduce swelling and inflammation during radiation therapy, or if symptoms return. The outlook is very different for each person. Early treatment improves the chance of a good outcome. [nlm.nih.gov]

  Corticosteroids may be prescribed to reduce swelling and inflammation during radiation therapy, or if symptoms return. [pennstatehershey.adam.com]

  If growth compresses the artery, ischaemia and necrosis with inflammation can ensue. [patient.info]

• Weakness

  Seizures are a very common symptom of these gliomas (affecting 50 percent to 80 percent of patients), as well as headache, weakness, or problems with speech. Oligodendrogliomas typically have a better prognosis than most other gliomas. [hopkinsmedicine.org]

  The first was weakness in one foot caused by neuropathy, a condition of the peripheral nervous system that can cause pain, numbness, and muscle weakness. The second, more obvious side effect was hair loss. [mskcc.org]

  […] or numbness of the arms and/or legs Facial weakness Double vision Symptoms can develop slowly and subtly and may go unnoticed for months. [braintumor.org]

  Diagnosis of these lesions is usually made on the basis of an MRI scan done because of central brain problems such as weakness, unsteadiness and seizures. [childrensnational.org]

• Short Stature

  Involvement of the hypothalamus may result in polyuria/polydipsia 4, as well as obesity, sexual precocity and endocrine dysfunction (e.g. short stature). Diencephalic symptoms include a change in alertness and hyperactivity. [radiopaedia.org]

• Fever

  CASE REPORT History and Examination A 27-year-old male presented with progressive, painless loss of vision in right eye, associated with vomiting and headache for one and a half months, without accompanying loss of consciousness or fever. [surgicalneurologyint.com]

• Vascular Disease

  The fundus of the affected eye initially may appear normal, but most patients rapidly develop evidence of occlusive vascular disease involving the optic disc, including venous stasis and oedema ( Figure 5a ). [nature.com]

• Strabismus

  Strabismus is another common feature. There is no pain associated. Some cases will suffer from acute blindness and worsening proptosis secondary to hemorrhage. [symptoma.com]

  Abstract A 4-month-old infant came to the department of Ophthalmology, King Chulalongkorn Memorial Hospital with right eye proptosis, strabismus, and no vision. She was diagnosed as optic nerve and chiasm glioma. [ncbi.nlm.nih.gov]

  Clinical manifestations include decreased visual acuity; EXOPHTHALMOS; NYSTAGMUS, PATHOLOGIC; STRABISMUS; pallor or swelling of the optic disc; and INTRACRANIAL HYPERTENSION. The tumor may extend into the optic chiasm and hypothalamus. [rareomics.healx.io]

• Unilateral Blindness

  Thus, the main threat of this tumour is eventual unilateral blindness, which may take years or even decades. [nature.com]

• Papilledema

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

  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]

  […] 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]

• Hyperactivity

  Diencephalic symptoms include a change in alertness and hyperactivity. In large intracranial tumors symptoms of raised intracranial pressure, focal neurological deficits and hydrocephalus from distortion of the midbrain may also be encountered. [radiopaedia.org]

  The latter, classically leading to the diencephalic syndrome (emaciation, pallor, and hyperactivity), is seen in up to 20% of patients under 3 years of age. [thamburaj.com]

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].

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]

In this section Treatment Effects of treatment Optic pathway glioma is also called a low grade glioma. Gliomas are tumours that originate from brain cells called astrocytes. [royalmarsden.nhs.uk]

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]

Prognosis A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. [braintumour.ca]

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]

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]

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]

Jump to navigation Jump to search Optic Pathway Glioma Epidemiology [ edit ] Low grade astrocytic tumors Prevalence 2% of cerebral gliomas 5% of childhood brain tumors 90% in children Associated with neurofibromatosis 30% of OPG patients have NF1 stigmata [en.wikibooks.org]

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]

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]

There are no treatments with demonstrated effectiveness in curing optic gliomas or preventing the vision loss that may be associated with them. [healio.com]

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