dr mohammad shehadeh salam hirbawi n.
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Dr. Mohammad Shehadeh Salam Hirbawi. Tumours. Optic nerve glioma. Slow-growing. Pilocytic astrocytoma which typically affects children. girls more often than boys. Approximately 30% of patients have associated nurofibromatposis-1 and in these patients the prognosis is better.

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optic nerve glioma
Optic nerve glioma
  • Slow-growing.
  • Pilocytic astrocytomawhich typically affects children.
  • girls more often than boys.
  • Approximately 30% of patients have associated nurofibromatposis-1 and in these patients the prognosis is better.
slide3

Presentation:

  • Most frequently in the first decade of life(median age 6.5 years).
  • Slowly-progressive visual loss, followed later by proptosis.
  • Although this sequence may occasionally be reversed.
  • Acute loss of vision as a result of haemorrhage into the tumour is uncommon.
slide4

Signs:

  • Proptosis often with inferior dystopia.
  • The optic nerve head is initially swollen and subsequently becomes atrophic.
  • Opticociliary collaterals and central retinal vein occlusion are ossasionally seen.
  • Intracranial spread to the chiasm and hypothalamus may develop.
slide6

CT:

  • shows a fusiform enlargement of the optic nerve with a clear cut margin produced by the intact dural sheath.
slide7

MR:

  • Shows gliomas.
  • may also show intracranial extension.

Treatment:

  • May not be required in patient with no evidence of growth,Good vesion and no cosmetic deformity.
  • surgical excision with preservation of the globe is required for large or growing tumours that are confined to the orbit particularlly if vision poor and proptosis significant.
  • Radiotherapy may be combined with chemotherapy for tumours with intracranial extension.
slide8

Prognosis:

  • Variable.
  • Some tumours have an indolent course with little growth.
  • Others may extent intracranialy and threaten life.
slide9

NB:

  • malignant gliomas are rare & almost always occur in adults. the prognosis is very poor with almost certain death within one year of diagnosis.
optic nerve sheath meningioma
Optic nerve sheath meningioma
  • Arise from meningothelial cells of the arachnoid villy surroundings the intraorbital or less commonly the intracanalicular portion of the optic nerve.
  • In some cases the tumour merely encircles the optic nerve whilst in others it invades the nerve by growing along the fibrovascularpialseptae.
  • Primary optic nerve sheath meningiomas typically affect middle-aged women.
slide11

(c) axial CT of small tumor shows slight thickening & calcification

(d)axial T2-weighted MR in a more advanced case shows fusiform enlargement of the optic nerve & proptosis

slide12

Presentation:

  • With gradual unilateral visual impairment .
  • Transient obscurations of vision may be the presenting symptom.

Signs:

  • The classical triad is:

Visual loss,optic atrophy,opticociliary shunt vessels.

slide13

However, the simultaneous occurrence of all three signs in one individual is uncommon.

  • The sequence of involvement is as follows:
  • Optic nerve dysfunction & chronic disc swelling followed by atrophy.
  • Opticocilliary collaterals found in about 30% of cases, regress as optic atrophy supervenes.
  • Restrictive motility defects, particularly in up-gaze, because the tumour may splint the optic nerve.
  • Proptosis caused by intraconal spread-usually develops after the onset of visual loss.
slide14

NB:

  • this sequence is the opposite of that seen with tumours outside the dural sheath, in which proptosis develops long before optic nerve compression.

CT:

  • Shows thickening & calcification of the optic nerve.

MR :

  • More clearly detects smaller tumours & those around the optic canal.
slide15

Treatment:

  • May not be required in middle-aged patients with slow-growing tumours because the prognosis is good.
  • Surgical excision is required in young patients with aggressive tumours, particularly if the eye is also blind.
  • Fractionated stereotactic radiotherapy may be appropriate in selscted cases or as adjunctive treatment following surgery.

Prognosis:

  • Is good in adults, although the tumour may be more aggressive & sometimes fatal in children.