CRANIOPHARYNGIOMA
IN CHILD: A CASE STUDY
Abstract:
Craniopharyngiomas are histologically benign neuroepithelial tumors
of the CNS that are predominately observed in children aged 5-10 years. People
may present with bitemporal
inferior quadrantanopia leading
to bitemporal hemianopsia, as the
tumor may compress the optic
chiasm These
tumors arise from squamous cell embryologic rests found along the path of the
primitive adenohypophysis and craniopharyngeal duct. Although histologically
benign, these tumors frequently recur after treatment. In addition, because
they originate near critical intracranial structures (eg, visual pathways,
pituitary gland, hypothalamus), both the tumor and complications of curative
therapy can cause significant morbidity. These characteristics have led to
various treatment approaches, and disagreement continues regarding optimal
treatment in children with this disease. Other
names are Rathke pouch tumors,
hypophyseal duct tumors, or adamantinomas. Evidence suggests that adult
craniopharyngiomas are histologically and biologically different from paediatric
craniopharyngiomas; however, only childhood craniopharyngiomas are discussed in
this article.
Key words:
Craniopharyngiomas, benign, quadrantanopia, bitemporal hemianopsia,
neuroepithelial tumors, primitive
adenohypophysis and craniopharyngeal duct.
Introduction: baby X 7 years old male child
was admitted in the paediatric surgical ward on 23/02/2016 with the complaints
of diminished vision from past 6 months and moderate to sever head ache on
frontal area with one episode of
vomiting. Thge 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 checkup 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 2nd
opthalmalogic check up and he was refered to PGI chandighar and advised for MRI
and the child was diagnosed for brain tumor. Therefore the clint came to AIIMS
OPD for the same complaints and pediatric 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 Craniopharyngiomas. Postoperatively the child was
complaining for diarrhoea and head ache.
Disease condition:
Book picture
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Patient picture
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Definition: Craniopharyngiomas
are benign neuroepithelial tumors of the CNS. These tumors arise from
squamous cell embryologic rests found along the path of the primitive
adenohypophysis and craniopharyngeal duct. Craniopharyngiomas are the most common
childhood tumor
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Incidence:
·
Craniopharyngiomas are relatively rare, representing 6-10%
of intracranial malignancies in children and adolescents (approximately 2-3
cases per 1,000,000 children).
·
a slight male preponderance has been historically reported.
·
Peak incidence occurs in individuals aged 5-14 years.
·
Neonatal craniopharyngiomas are rare.
·
Higher incidence rates have been observed in Asia and Africa.
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Age
7 year
Sex:
male
Race:
Asian
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Causes:
Unknown
chromosomal abnormalities
environmental or infectious causes can
predispose
|
Unknown
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Pathophysiology
 Due to chromosomal abnormalities like deletions,
translocations, and increased copy numbersTumors arise from cellular remnants of
the Rathke pouch, which is an embryologic structure that forms both the
infundibulum and anterior lobe of the pituitary gland.
These tumors have been identified extensively in
suprasellar, parasellar, and ectopic locations. Typically, the
tumors arise within the sella or adjacent suprasellar space.
Tumor mass compresses the adjacent normal intracranial
structures.
Obstructing cerebral spinal fluid (CSF) pathways (ie, third
ventricle, Monro foramen) and causing hydrocephalus and increased
intracranial pressure that leads to headaches, nausea, and projectile
vomiting.
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Clinical manifestations
·
Headache: due to increased intracranial
pressure or hydrocephalus.
·
Vomiting: Classic projectile vomiting
(frequently without nausea)
·
Vision loss
o
Children
are frequently unaware of significant vision loss; nevertheless, this symptom
reportedly occurs in 20-60% of pediatric patients with craniopharyngioma at
presentation.
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.
·
Seizures due to Temporal
lobe involvemen
·
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, Myxedema, Delayed puberty.
|
Before surgery:
Head
ache on frontal area
Vomiting
Partial
Vision loss (bitemporal hemianopsia)
Unusual
eye movement
After surgery:
Head
ache
Diarrhoea
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Diagnosis:
·
History
·
Physical examination
·
Preoperative intellectual or psychological assessment
·
Serum electrolytes levels
·
Hormonal studies
·
Skull radiography
·
Head CT scanning
·
Brain MRI
·
Cerebral angiography
·
Histological
studies
|
·
History: The child
natal history was apparently normal.
·
General
appearance: Oriented, conscious, moderate body built.
·
GCS score: Eye 4
verbal 5, and motor 6,
·
Vital signs: stable
·
Anthropometry: height
154cm, weight 18kg, 1st degree malnutrition (according to Gomez
classification).
·
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
Craniopharyngiomas.
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Treatment:
·
Long-term hormone replacement is the primary medical
treatment: intranasal vasopressin (desmopressin acetate [DDAVP]),
corticosteroids, thyroid hormones, growth hormones, and sex hormones.
·
There is no role of chemotheraphy in craniopharingioma.
Immunostimulatory therapies with interferon and intracystic/intratumoral
injection of chemotherapeutic agents (eg, bleomycin) are occasionally used in
cases of recurrent disease. (
Bleomycin (Blenoxane), Interferon alfa 2a (Roferon-A))
·
Intracavitary irradiation (brachytherapy) also shows no
clear outcome
·
Repeated Surgical management for recurrent
craniopharyngiomas after radiation therapy also shows fewer prognoses.
Surgical Care
·
Radical surgery
·
Conservative surgery alone
·
Conservative surgery with postoperative radiotherapy
|
·
Medical management
Tab
valporate 200mg OD (morning)
Tab
veona CR 300 mg OD (evening)
Tab
pantop -20mg OD
Tab
sporlac 120mg TDS
·
Surgical management
Craniotomy
and excision was done
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Complications:
·
Vision
loss
·
Growth
hormone deficiency, (35-95%).
·
Thyroid-stimulating
hormone deficiency (21-42%),
·
adrenocorticotropic
·
Hormone
deficiency, (21-62%)
·
Antidiuretic
hormone deficiency,
·
Luteinizing
hormone or follicle-stimulating hormone deficiency (38-82%)
Diencephalic
syndrome
|
·
TSH
deficiency
·
Diencephalic
syndrome (hyperactive with unusual eye movements)
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Prognosis:
·
There
is 10 years of survival rates of 86-100% among patients
who underwent gross total resection
·
Subtotal
resection or recurrence treated with surgery and radiation therapy carry
10-year overall survival rates of 57-86%.
·
The
perioperative mortality rate after primary surgical intervention has been
estimated to be 1.7-5.4%. However, the mortality rate after re-resection for
recurrent disease can be as high as 25%.
·
Almost
all patients with craniopharyngioma ultimately suffer from chronic
endocrinologic morbidities. And significant neurologic morbidities such as
vision loss, ataxia, behavioral problems, cognitive disabilities, and sleep
disorders
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·
Not
evident till date
·
Expected
to have poor prognosis in future
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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 hemiparese 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
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:
Even though it is a common childhood
tumor the treatment mortalities for this kind of tumor is still not clear.
There is no role of chemotherapy in curing tumor. Radiation therapy and
surgical interventions also shows no complete cure. Repeated surgeries needed
for recurrent cases. This need consultation and team work of Pediatric neurosurgeon, Radiation oncologist,
Pediatric endocrinologist and Pediatric hematologist/oncologist.
Reference:
1.
Suraj
G “The Short Textbook Of Paediatric Nursing” 11th edition, Jaypee
publications, 565-566.
2.
Archar’s
“ Textbook of Paediatric Nursing” 4th edition, universities press,
534,541,543
3.
Nelson,
“Textbook of Paediatrics” 10th edition, volume 1, Elsevier
publication, 1746,1752
4.
Karin
R, Beth B “ paediatric Acute Care” Jones and Bartlett publication, 375, 637
5.
Nanda
nursing interventions
