S.K.Mohanasundari M.

Sc paediatric Nursing
Nursing Tutor
College of Nursing
AIIMS, Rishikesh
roshinikrishitha@gmail.com

OPTIC NERVE GLIOMA IN CHILD: A CASE STUDY

Abstract:

An optic nerve glioma is a type of brain tumor. There are multiple kinds of brain
tumors and glioma’s account for approximately one-third of brain tumors. They are typically
named after the kinds of cells they affect. It is a rare kind of cancer, they are considered low-
grade and do not grow as quickly as other types of brain tumors. They are found in the optic
chiasm, where the optic nerves cross, or surround, the optic nerves. They are also referred to
as optic glioma or juvenile pilocytic astrocytoma. It is rarely found in individuals over the
age of 20. It has also been associated with the genetic disorder neurofibromatosis Type 1, or
NF1. Evidence suggests that adult malignant gliomas (glioblastoma) are rare & almost
always occur in adult males with a very poor prognosis & almost certain death within one
year. Optic-nerve gliomas comprise about 1% of all intracranial tumors and Optic nerve
glioma is a slow-growing tumour, which typically affects children. 30% of patients have
associated neurofibromatosis type 1 & those have better prognosis. However, optic nerve
glioma of children is discussed in this article.
Key words: optic nerve glioma, juvenile pilocytic astrocytoma, brain tumors, Malignant
gliomas (glioblastoma) and intracranial tumors.

Introduction:
A 7 ½ year old male child was admitted in the paediatric surgical ward on 12/02/2016
with the complaints of diminished vision from past 6 months and moderate to severe head
ache on frontal area with one episode of vomiting. The child was apparently normal before 6
months, and his decreased in vision was reported by his school teacher that he is not able to
see words in blackboard. He went for routine eye check-up and suggested for improving diet
and no other treatment measures were used. The symptoms progress and the child vision
worsen with which he started banging on walls and doors and thus continuous diminishing of
vision for which he went for 2 nd ophthalmologic check-up and he was referred to PGI
Chandigarh and advised for MRI and the child was diagnosed for brain tumor. Therefore the
clint came to AIIMS OPD for the same complaints and paediatric surgeon. The child
underwent craniotomy and excision on 3rd march and the tumor was removed and culture
was sent for histopathological examination. The incision from frontal area starting from right
ear 13 sutures was made to close the incision. The conformation of diagnosis was optic nerve
glioma. Postoperatively the child was complaining for diarrhoea and head ache.

Disease condition:
Book picture Patient picture
Definition: An optic nerve glioma (also called an optic pathway glioma) is a slow-growing brain
tumor that arises in or around the optic nerve, which connects the eye to the brain. As the tumor
progresses, it presses on the optic nerve, causing a child’s vision to worsen. Blindness can occur,
but only in about 5 percent of cases. the tumor sometimes produces additional symptoms as it
grows. A low-grade form of this neoplasm, benign optic glioma, occurs most often in paediatric
patients. While these are serious tumors, they have a high cure rate.
Incidence:
 Peak incidence occurs in individuals aged 6-7years.
  Prevalence of 15% (range, 1.5–24%) Age 7 ½ years
 Benign optic glioma occur almost in children, better Sex: male
prognosis Race: Asian
 Aggressive glioma occur almost in adult, poor
prognosis.
 Genetic disorder neurofibromatosis Type 1, or NF1is
commonest type in children

 Types: • Neurofibromatosis type 1

Neurofibromatoses type 1

Optiv nerve glioma

Neurofibromatoses  type 2

Causes: Unknown
Unknown
chromosomal abnormalities/hereditary genetic disorder
 environmental or infectious causes can predispose

Pathology:
 The NF-1 product, neurofibromin has GTPase-activating protein domain with the Ras protein, which is crucial in
regulating signal transduction and cell proliferation and differentiation.

Patient with NF-1 age at high risk for developing a number of different types of tumor
because of this.

Orpital portion of the optic nerve, a glioma often appears as a fusiform

swelling

Causes nerve compression and

adjuscent structure compression

Vision loss, head ache, and

vomitting

Clinical manifestations
 Headache: due to increased intracranial pressure or Before surgery:
hydrocephalus. Head ache on frontal area
 Nausea and Vomiting: Classic projectile vomiting Vomiting
(frequently without nausea) Partial Vision loss (bitemporal
 Vision loss hemianopsia)
o Children are frequently unaware of Unusual eye movement
significant vision loss; nevertheless, this symptom After surgery:
reportedly occurs in 20-60% of paediatric patients Head ache
with craniopharyngioma at presentation. Diarrhoea
o Anterior extension to the optic chiasm can
result in a classic bitemporal hemianopsia, unilateral
temporal hemianopsia, papilledema, or
unilateral/bilateral decrease in visual acuity.
Classically, vision loss starts with a superior
temporal field cut. However, the eccentric growth of
these tumors can result in varying patterns and
severity of vision loss, including decreased acuity,
diplopia, blurred vision, and subjective visual field
deficits. Children are frequently inattentive to visual
loss, and formal testing may be required.
o Balance problem
 Seizures due to Temporal lobe involvement
 Hyperactive children with unusual eye movements
and even blindness due to extrinsic compression of
the hypothalamus.
 Endocrine deficiencies leads to
short stature, Weight gain, Lethargy, Fatigue, Cold
intolerance, Dry skin, Dry brittle hair, Slow teething,
Anorexia, Large tongue, Deep voice, Myxoedema,
Delayed puberty, memory impairment, daytime
sleepiness and growth delays
Diagnosis: History: The child natal history

 History was apparently normal.
 Physical examination with neurological exam.  General appearance: Oriented,
 Preoperative intellectual or psychological assessment. conscious, moderate body built.
 Vision testing  GCS score: Eye 4 verbal 5, and
 Serum electrolytes levels  motor 6,
 Hormonal studies  Vital signs: stable
 Skull radiography  Anthropometry: height 154cm,
 Head CT scanning weight 18kg, 1st degree
 Brain MRI malnutrition (according to
 Cerebral angiography  Gomez classification).
 Biopsies for Histological studies  Growth and development
seems to be normal. And child
was mild hyperactive and have
hurried in speech.
 Head to foot: after surgery
suture line are present, partial
visual acuity. Unusual eye
movement, pupillary dilatation,
partial optic atrophy. Extra
ocular eye movement
abnormalities. Slow teething
and deep voice, weight loss.
 No other abnormal physical
findings.
Investigations:
 Haematological investigation:
Hb: 11.3gm/dl, RBC
4.56mc/cum,TLC
7500cells/cumm,DLC-N 90%,E-
01%, L-05%, m-04%, platelet –
3.11 lacks/c/cumm, Hematocrit
34.8%.
 Hormonal studies: T3 level is
elevated.
 MRI: suggestive of possibilitiey
of Craniopharyngiomas.
 Histopathologicla
examination: suggestive of
optic nerve glioma
Treatment:  Medical management
A treatment plan must be carefully individualized for Tab Valporate 200mg OD
each patient. This need consultation and team work. (morning)
Tab Veona CR 300 mg OD
(evening)
Tab Pantop -20mg OD
Tab Sporlac 120mg TDS
 Pediatric  Surgical management
neurosurgeon Craniotomy and excision was done

Pediatric
TREATMENT Radiation
hematologist
TEAM oncologist
/oncologist

Pediatric
endocrinologist

 Observation only in presumed optic nerve glioma,

particularly with good vision on the involved side;
with careful follow up if the radiographic evidence.
 Long-term hormone replacement is the primary
medical treatment:  intranasal vasopressin
(desmopressin acetate [DDAVP]), corticosteroids,
thyroid hormones, growth hormones, and sex
hormones.
 Combination chemotherapy using actinomycin D,
vincristine, etoposide, bevacizumab and other agents
has also been reported to be effective in patients with
progressive chiasmal/hypothalamic gliomas To shrink
the tumor and stabilize vision.
 Radiation therapy as the sole treatment is
considered if the tumor cannot be resected (usually
chiasmal or optic tract lesions) and if symptoms
(particularly neurological) progress or if the tumor is
resistant to chemotherapy
 Alternative medicine (acupuncture/acupressure,
therapeutic touch, herbal medicine, etc.) to control
pain and treatment side effect.
 Surgical Care-Surgery is usually not preferred for
this type of tumor, but can sometimes relieve
symptoms and/or improve vision. Surgical excision in
case of rapid intraorbital tumor growth to isolate the
tumor from the optic chiasm and thus prevent
chiasmal invasion. The surgeon should use an
intracranial approach to obtain tumor-free surgical
margins.
 Radical surgery
 Conservative surgery alone
 Conservative surgery with postoperative radiotherapy
Complications:  TSH deficiency
 Hormonal deficiency  Diencephalic syndrome
 Cognitive difficulties, (hyperactive with unusual eye
 learning disabilities, and movements)
 impairments in growth
Prognosis and recurrence
 Variable  Not evident till date
 Optic nerve glioma recurrence may take place many
years after initial treatment.
 It usually recurs in the same place as the original
tumor, but can also occur in other parts of the brain or
spinal cord.
 Local radiation therapy is the usual treatment if the
patient has not previously been treated with this
modality.
 Chemotherapy and radiation therapy are options for
patients who have only been treated surgically.
 Child with NF-I tend to fare better with respect to
growth and visual prognosis.
 Most tumor grows slowly or having self limited
growth.
 Some tumors are more aggressive, resulting in a rapid
increase in ispilateral proptosis and visual loss.

Nursing Assessment
 Identification of risk factors for exposure to radiation or chemicals that is
carcinogenic.
 Identify the signs and symptoms are: headache, vomiting, and decreased vision or
double vision.
 Identify any changes in client behaviour.
 Observation of hemiparesis or hemiplegia.
 Changes in sensation: hyperesthesia, parasthesia.
 Observation of sensory changes: asteregnosis (not able to feel the sharp edges),
agnosia (not able to recognize objects in general), apraxia (not being able to use the
tool properly), agraphia (can’t write).
 Observation of vital signs and level of consciousness.
 Observation circumstances fluid and electrolyte balance.
 Psychosocial: personality and behavioural changes, difficulty making decisions,
anxiety and fear of hospitalization, diagnostic tests and surgical procedures, a change
in the role.
Possible Nursing diagnosis:
1. Ineffective tissue perfusion related to circulatory damage caused by a tumor
suppression.
2. Impaired sensory perception decrease visual acuity related to optic nerve
compression
3. Pain (Acute / Chronic) related to increased intracranial pressure.
Altered comfort irritability related to increased intracranial pressure
4. Fluid and electrolyte imbalance related to vomiting
5. Impaired family coping related to poor prognosis of the disease
6. Risk for injury related to poor visual acuity.
7. Risk for recurrence related to metastatic nature of the disease
 8. Potential for complications vision loss related to poor prognosis of disease
9. Potential for neurological deficit related to poor prognoses and non availability of the
chemotherapy and radiation therapy.
10. Knowledge Deficit: the condition and treatment needs related to the inability to know
the information.
Conclusion: Having cancer as a child can be socially and emotionally stressful. You or your
child may benefit from counselling or a support group. Being around peers his or her own age
can be a big support. The survival rate for optic pathway gliomas is near 90 percent. Older
children and those with neurofibromatosis 1 have better outcomes. In fact, two-thirds of
children with NF1 experience spontaneous remission of their optic pathway gliomas.
Children may suffer a smaller field of vision, which means they do not have peripheral
vision. The odds of complete blindness from these tumors, however, are less than 5 percent.
As there is chance of recurrence after treatment, follow-up visits with doctor are necessary to
check for any side effects and ensure the cancer has not returned.
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