OPTIC NERVE

GLIOMA
CASE SUMMARY
 Eaint Kyi Phyu, 8 yr old girl, a single child of non-consenguineous parents
 Known case of Rt optic nerve glioma and epilepsy
 History of drop attacks and divergent squint (Rt) with impaired vision since 4 yr ago
 Other manestations – delay in gross motor, speech and weak in school performance
 History of delivery by El LSCS but one-week post-date as well as NNJ on Day-2 old with
Phototherapy for 7 days
CASE SUMMARY
 Correction of Rt divergent squint done 3 and half yr ago but with CT result of Rt ONG and
then it had been observed
 epilepsy has been managing with AED since then
 4 months ago, drop attacks has been still presented and MRI(brain) as well as EEG were done
 MRI revealed Rt ONG and Rt frontal, temporal slow waves on EEG
 Parents went back home although there was a plan to admit for further management
 Epilepsy is treated with 3 AEDs (Topirol, Zeptol, Encorate)
CASE SUMMARY
 Referral to Opthalmology, Paediatric Oncology and Neurosurgery
 Ophthalmologist suggested to observe but neurosurgeon will review ONG on new MR
imaging with consideration for surgery if possible
 Oncologist will give opinion after foreign consultation
 Rt and mid-central region Epileptiform discharges on rechecked EEG
OPTIC NERVE GLIOMAS
 the most common tumours of the optic nerve
 More than 90 % of primary optic nerve tumours are either benign gliomas of childhood or
optic nerve sheath meningiomas
 comprise about 1 % of all intracranial tumours
OPTIC NERVE TUMOURS ARE
CLASSIFIED AS
 those arising from the optic nerve matter or
 those arising from the surrounding sheath
TWO DIFFERENT TYPES—
 the juvenile benign pilocytic astrocytoma and
 the malignant glioblastoma of adulthood
BENIGN TUMOURS
 usually present in the first decade of life
 almost always unilateral
 more frequently in females
 the incidence may be sporadic or sometimes familial
 most of the patients presenting with optic nerve gliomas have neurofibromatosis type 1 (NF-1)
 varying levels of incidence of NF-1 among patients of optic nerve glioma: 10-70 %, whereas
the incidence of optic nerve glioma in patients with NF-1 varies from 8 to 31 %
CLINICAL FEATURES
 Proptosis is usually gradual, painless
 on rare occasions, may present with acute loss of vision
 Optic disc swelling or pallor, visual acuity loss, visual field loss and relative afferent pupillary
defect are seen due to compressive effects of the tumour
 eventually causes optic nerve atrophy because of pressure effects on the nerve fibres as well as
the nutrient arteries
CLINICAL FEATURES
 Primary and secondary strabismus is seen, along with restriction of extra ocular muscle
motility
 Chronic compression of the central retinal vein can cause central retinal vein occlusion
(CRVO) resulting venous stasis retinopathy, optociliary shunt vessels are seen
CLINICAL FEATURES
 Chiasmal gliomas may present with slow bilateral visual loss associated with bitemporal field
defects, optic disc changes and strabismus
 Endocrinal disturbances may also be seen in patients with chiasmatic gliomas
 Sporadic optic nerve glioma more often present with symptoms of raised intracranial pressure
owing to their spread beyond the chiasm
 the tumours associated with NF-1 in whom precocious puberty is more commonly seen
DIAGNOSIS
DIAGNOSIS
 based on clinical presentation and imaging
 all children with NF-1 younger than 8 years of age should undergo a thorough annual
ophthalmological examination
 but baseline ‘screening’ neuroimaging or visual evoked potentials of asymptomatic children
with normal visual examinations is not warranted
DIAGNOSIS
 Most tumours present with progressive visual loss and variable proptosis
 ability to recognize systemic features of NF-1 is important in early diagnosis of optic nerve
gliomas
NEUROIMAGING
 helps in diagnostic dilemmas such as differentiation between meningiomas and gliomas
 diagnosing infiltrative optic neuropathies such as leukaemia or lymphoma
 CT
 the tumour appears as an iso-to hypoattenuating fusiform enlargement of the optic nerve
 sometimes with kinking or tortuosity of its course
 Less commonly, it is an eccentric or discrete mass arising from the nerve
 can also reveal subtle erosions and enlargement of the optic canal and the rare fine
calcifications
 Although intrinsic contrast with intraconal fat on CT allows for evaluation of the optic nerve
AXIAL NONCONTRAST CT IMAGE SHOWS AN
ISODENSE LOBULATED INTRACONAL MASS
ALONG THE LEFT OPTIC NERVE THAT CAUSES
LEFT PROPTOSIS
 MR IMAGING
 The modality of choice to evaluate intracranial extent, including extension to the optic chiasm,
hypothalamus and beyond
 MR imaging protocols using coronal and axial thin section T1-weighted and fat-saturated T2-
weighted images are useful, as is additional evaluation with paramagnetic contrast agents
 MR IMAGING
 The size and course of the nerve are best evaluated on T1-weighted images without fat
saturation

 Axial unenhanced T1-weighted image shows a fusiform, isointense mass along the right optic
nerve. The normal hyperintense intraconal fat provides intrinsic contrast to delineate the mass.
 MR IMAGING
 The tumour is iso-to hypointense to the optic pathway on T1weighted images and slightly
hyperintense on T2weighted images, with rare areas of haemorrhage and calcification.
 Variable patterns of enhancement are seen with intravenous gadolinium
 MR IMAGING
 Two architectural
 In the diffuse type, which grows predominantly within the optic nerve parenchyma, the nerve
is enlarged and the surrounding subarachnoid space is effaced

 Postcontrast fat suppressed T1-weighted axial image at the same level shows intense
enhancement in the mass
 MR IMAGING
 In the other form, enhancing tumour in the subarachnoid space compresses the normal-sized
minimally enhancing nerve
 Cystic tumours show enhancement of the wall of the cyst
 Enhancement in optic nerve gliomas is associated with a more aggressive behaviour
 Extension to the chiasm through the optic canal produces a characteristic dumbbell-shaped
configuration
SOME IMAGING FINDINGS OF OPTIC
PATHWAY BETWEEN CHILDREN WITH AND
WITHOUT NEUROFIBROMATOSIS
 Bilateral optic nerve tumours are pathognomonic for NF-1
 Tumours isolated to the optic nerve are more commonly seen in patients with NF-1, while
intracranial extension is more common without NF-1
 The tumour causes nerve enlargement without altering its configuration in most patients with
NF-1 but in only a small minority without NF-1
 Tumour diameter and volumes tend to be greater in non-NF-1 cases Cytic components are rare
in NF-1. Hydrocephalus is almost exclusively seen in non-NF-1 cases
TREATMENT OF
OPTIC NERVE
GLIOMAS
 A TEAM EFFORT
MANAGEMENT AMONG
 ophthalmologist
 radiologist and
 oncologist
 as not only diagnosis but also subsequent treatment and follow-up involves all the three
clinicians
 OBSERVATION
 The natural history of childhood optic nerve gliomas is almost always benign
 most tumours grow slowly in a self-limited manner and some even spontaneously regress
 most patients with unilateral optic nerve gliomas, particularly those with NF-1, be followed at
regular intervals both clinically and with neuroimaging without intervention unless there is
documented visual deterioration
 CHEMOTHERAPY
 particularly important for the youngest patients
 should be the primary treatment modality for ONG in children under age 3 years
REGIMEN FOR LOW-GRADE
GLIOMAS
 10-week induction phase, followed by 48 weeks of maintenance carboplatin/vincristine
 resulting in a progression-free survival (PFS) of 75 % at 2 years and 68 % at3 years
 Children aged 5 years or younger had a notably more favourable overall rate of response
SECOND-LINE THERAPY
 TPCV (6-thioguanine, procarbazine, CCNU and vincristine)
 Risks associated with chemotherapy are also substantial like renal toxicity, myelosuppression,
peripheral neuropathy, ototoxicity, etc
FRACTIONATED STEREOTACTIC
RADIOTHERAPY (FSRT)
 is now emerging as one of the preferred treatment modalities
 FSRT was safe and well tolerated in all patients
 5-year survival rate after FSRT was 90 %
 compared to conventional techniques, FSRT has the potential of sparing the pituitary gland in
chiasmatic lesions
RISK
 neurocognitive sequelae
 endocrine impairment
 late vascular effects like Moya Moya syndrome (especially in NF-1) and
 second malignancies
INDICATIONS
 Children above 5 years of age who have significant visual or neurological impairment at
presentation or
 Who have clinical or radiological progression while on close observation or
 Under 5 years of age who progress on chemotherapy
SURGICAL EXCISION
 Only if there is cosmetically unacceptable proptosis
 definite radiologically documented tumour enlargement or
 extension (not involving the optic chiasm) or
 a combination of these
 In the presence of good vision, surgery carries the risk of vision loss
CASE REPORT
 A 7½ year old male child, apparently normal before 6 months
 with the complaints of diminished vision from past 6 months
 moderate to severe frontal head ache with one episode of vomiting
 his visual alteration was reported by his school teacher that he is unable to read words
on blackboard
 progressive diminishing of vision made his parents to take him to ophthalmologic
checkup twice and he was referred to tertiary care centre
CASE REPORT
 Unusual eye movement, pupillary dilatation, partial optic atrophy and extra ocular eye
movement abnormalities
 MRI: suggestive of possibilities of Craniopharyngiomas
 Craniotomy and excision was done
 No post-operative radiotherapy was given to the child
 Histo-pathological examination: suggestive of optic nerve glioma
CASE REPORT
CASE REPORT
 After one month of hospitalization, the child was discharged with minimal deficit in activities
of daily living
 The child was progressed positively according to the expectations
 Supportive treatment and therapies have been provided
 The child recovered completely and was discharged after one month of surgery
THANK YOU