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==History==
==History==
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). Acute visual loss due to hemorrhage into the tumor is uncommon. 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).
Most frequently optic pathway glioma patients present in the first decade with a median age of 8.8 years. They present with slowly progressive visual loss which is followed later by proptosis. Acute visual loss due to hemorrhage into the tumor is rare.  
==Common Symptoms==
==Common Symptoms==
Decreased vision (63%) is usually evident and can be documented with visual field examination if the child is old enough. Eventually mass effects will also occur, with proptosis and even intracranial sequelae including symptoms of raised intracranial pressure, focal neurological deficits and hydrocephalus from distortion of the midbrain. Involvement of the hypothalamus may result in polyuria or polydipsia.<ref>{{Cite web | title =Radiopedia Optic nerve glioma clinical presentation| url =http://radiopaedia.org/articles/optic-nerve-glioma }}</ref>  
Decreased vision (63%) is usually evident and can be documented with visual field examination if the child is old enough. Eventually mass effects will also occur, with proptosis and even intracranial sequelae including symptoms of raised intracranial pressure, focal neurological deficits and hydrocephalus from distortion of the midbrain. Involvement of the hypothalamus may result in polyuria or polydipsia.<ref>{{Cite web | title =Radiopedia Optic nerve glioma clinical presentation| url =http://radiopaedia.org/articles/optic-nerve-glioma }}</ref>  
The symptoms are due to the tumor growing and pressing on the optic nerve and nearby structures. Symptoms may include:<ref>{{Cite web | title =Medline Plus optic nerve glioma symptoms| url =https://www.nlm.nih.gov/medlineplus/ency/article/001024.htm }}</ref>
 
*Involuntary eyeball movement
The symptoms of optic pathway gliomas usually develop over a period of months to years and usually depend upon the location of the tumor.
*Outward bulging of one or both eyes
*Proptosis- It is the presenting symptom.
*Squinting
*Impaired vision-In patients with chiasmal lesions, it is the presenting symptom.
*Vision loss in one or both eyes that starts with the loss of peripheral vision and eventually leads to blindness
*Obstructive hydrocephalus may also be noted as presenting symptom.
*The child may show symptoms of diencephalic syndrome, which includes:
*Other less frequent symptoms are:
** Unilateral visual impairment.
**Involuntary eye ball movement
**Squinting
Hypothalamic gliomas may present with symptoms of the diencephalic syndrome, which include:
**Daytime sleeping
**Daytime sleeping
**Decreased memory and brain function
**Decreased memory and brain function
**Delayed growth
**Delayed growth
**Loss of appetite and body fat
**Loss of appetite and body fat
*Precocious puberty- Due to involvement of hypothalamic-pituitary-gonadal axis precocious puberty may occur in 39 % of children with chiasmal optic nerve gliomas and NF-1.<ref name="pmid7869193">{{cite journal| author=Habiby R, Silverman B, Listernick R, Charrow J| title=Precocious puberty in children with neurofibromatosis type 1. | journal=J Pediatr | year= 1995 | volume= 126 | issue= 3 | pages= 364-7 | pmid=7869193 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7869193  }} </ref> In 10 to 20 percent of patients with optic pathway gliomas endocrinopathies due to hypothalamic extension are present.
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 16:36, 29 September 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

History and Symptoms

History

Most frequently optic pathway glioma patients present in the first decade with a median age of 8.8 years. They present with slowly progressive visual loss which is followed later by proptosis. Acute visual loss due to hemorrhage into the tumor is rare.

Common Symptoms

Decreased vision (63%) is usually evident and can be documented with visual field examination if the child is old enough. Eventually mass effects will also occur, with proptosis and even intracranial sequelae including symptoms of raised intracranial pressure, focal neurological deficits and hydrocephalus from distortion of the midbrain. Involvement of the hypothalamus may result in polyuria or polydipsia.[1]

The symptoms of optic pathway gliomas usually develop over a period of months to years and usually depend upon the location of the tumor.

  • Proptosis- It is the presenting symptom.
  • Impaired vision-In patients with chiasmal lesions, it is the presenting symptom.
  • Obstructive hydrocephalus may also be noted as presenting symptom.
  • Other less frequent symptoms are:
    • Unilateral visual impairment.
    • Involuntary eye ball movement
    • Squinting

Hypothalamic gliomas may present with symptoms of the diencephalic syndrome, which include:

    • Daytime sleeping
    • Decreased memory and brain function
    • Delayed growth
    • Loss of appetite and body fat
  • Precocious puberty- Due to involvement of hypothalamic-pituitary-gonadal axis precocious puberty may occur in 39 % of children with chiasmal optic nerve gliomas and NF-1.[2] In 10 to 20 percent of patients with optic pathway gliomas endocrinopathies due to hypothalamic extension are present.


References

  1. "Radiopedia Optic nerve glioma clinical presentation".
  2. Habiby R, Silverman B, Listernick R, Charrow J (1995). "Precocious puberty in children with neurofibromatosis type 1". J Pediatr. 126 (3): 364–7. PMID 7869193.

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