3. History of present illness:
• Known case of craniopharyngioma since last 8
months.
• Was apparently well 1 year back when she
developed on and off headache over the
bitemporal region.
• She had similar episode of headache the day she
arrived to the hospital since last one hour.
• Acute onset, severe, continuous, sharp stabbing ,
with no aggravating or relieving factor or
radiation.
• Associated with projectile vomiting (8-10
episodes). Vomitus contained food particles.
4. • No history of loss of consciousness, seizure
activity.
• No neurological deficits.
• No fever, visual defects, sudden weight gain,
increased frequency of micturition.
5. Past history:
• Diagnosed in National Institute
Of Neurological And Allied Science, Bansbari.
• Was advised to undergo resection of the tumor
mass.
• Due to poor economic status she did not undergo
surgery.
• Multiple episodes of dull aching headache,
bitemporal region which used to last for 10-15
mins, moderate severity, acute onset with no
radiation.
6. • Associated with 1-2 episodes of projectile
vomiting. No aggravating factor but it was
relieved by vomiting and NSAIDs.
• She used to sleep for 3-4 hours after the
episode of headache
7. • Birth history:
A. Antenatal: antenatal period was uneventful
B. Natal: home delivery, cried immediately after
birth
C. Postnatal: postnatal period was uneventful
• Developmental history:
Normal development according to milestones for
age of the patient
8. • Immunization history:
The child was immunized according to EPI schedule.
• Family history:
No history of similar illness in the family or other
significant diseases
• Treatment history:
She was not under any medication for the
condition.
9. General examination
• Child was conscious, oriented to time, place ,
person.
• Well built, comfortably lying on bed.
• There was no pallor, icterus, cyanosis,
lymphadenopathy, edema, or signs of
dehydration
Vitals:
• Pulse:102 b/m regularly regular
• BP: 90/60 mmHg
• RR: 40 breaths/min
• Temperature: 98 degree F measured at Rt Axilla
10. Neurological examination
• GCS: E4V5M6
• Pupils: reactive, round and direct and consensual
light reflex was present bilaterally
• Fundus examination: bilaterally papilloedema
present.
• Cranial nerves grossly intact
• Motor examination: normal tone of upper and
lower limbs
• Power:5/5 over upper and lower limbs
11. • Reflexes: normal and no involuntary
movements present
• Sensory function: normal
• No signs of cerebellar dysfunction present
• No neck stiffness present
12. Investigations
Before surgery:
CBC: TLC: 21.08 X 103 /cu. mm
N:92,L 7
Haemoglobin: 10.5gm/dl
Platelet count: 440X 103 /cu. mm
S. sodium: 141 mEq/l
S. Pottassium: 3.4 mEq/l
S. urea: 20 mg/dl
S. creatinine: 0.6 mg/dl
Normal bleeding and clotting time
PT/ INR:15.3 seconds/1.14
13. • CT scan: enhancing mass in suprasellar region
with calcification and mild ventriculomegaly
• MRI brain: a large mass within sella and supra-
sellar cistern causing obstructive hydrocephalus
and periventricular seepage of CSF (4.1X 4.3X 4.3)
in size.
15. Intraoperative management
• NIBP, ECG, oxygen saturation and capnography
monitoring
BASELINE VITALS
• BP: 100/60 mmHg, SpO2:97%, HR:110 beats/min
• Premedication: Inj. FENTANYL 50 mcg IV
• Preoxygenation was done. Induced with Inj.
PROPOFOL 90mg IV. Inj. VECURONIUM 2.5mg IV
given.
• Direct laryngoscopy was done. PVC endotracheal
tube of 5.5 mm internal diameter tube was used.
Cuffed confirmed and fixed.
16. • Maintained with O2, nitrous oxide, sevoflurane,
Inj. VECURONIUM 0.5+ 0.5+0.5+ 0.5 mg iv and Inj.
FENTANYL 25 mcg+ 25mcg given.
• Inj. Mannitol 2gm iv given.
• Inj. Phenytoin 375mg IV via infusion over half an
hour.
• Total blood loss 300ml, 250ml of lost blood was
supplemented using whole blood.
• Inj. NS was used in intraoperative period. Total
fluid given was 1400ml
• Urine output 1500ml
• Duration of surgery 6 hours
17. • Intraoperative vitals:
• BP:80/50-100/60 mmHg
• SpO2: 97-100%
• HR: 120-50
• After the procedure, Inj. Ondansetron 2mg IV, Inj
Paracetamol 350 mg IV stat given. Reversed with
Inj. Neostigmine 1mg and Inj. Glycopyrrolate 0.2
mg IV. Suctioned and extubated.
18. • The patient underwent right pterional
craniotomy and partial excision of
craniopharyngioma with external ventricular
drain, under an emergency basis.
19. Post operative
• Vitals: (post extubation)
• BP= 100/60 mmHg
• HR= 105 beats/min
• SpO2= 97% in room air
• GCS: 15/15
20. Immediate post operative period
• Polyuria , on 1st post operative day 900-500 ml/hr
was on a negative balance of 200-150ml/hour
• Supplemented with ½ NS
• S. Na:155m Eq/l and S. pottasium:5.6mEq/l urine
specific gravity: 1.005-1.010
• Range of S.sodium (165-141) mEq/l
• Range of S.potassium (5.6-2.4) mEq/l
• Serum osmolality and urine osmolality: test were
no available
• GRBS was taken every 4 hrly
• Maintained on T. Carbamazepine
21. • Central line was kept on the 2nd post operative
day via subclavian vein. Fluid management done
using CVP.
• Patient was still having negative fluid balance in
2nd post operative day
• Due to unavailability of desmopressin.
• Inj. Vasopressin was started at rate of
0.1mU/kg/hr.
• Started to have positive fluid balance of around
150 ml/hr.
• Vasopressin stopped after 30hrs.
• Negative fluid balance till 3rd post op day
resolved.
24. • Develops from:
1. Ectodermal outpocketing (primitive oral
cavity) RATHKE’S POUCH
2. Infundibulum (downward extension of
diencephalon)
Cells in anterior wall of Rathke’s pouch :
adenohypophysis
Small extension of this lobe, pars tuberalis along
infundibulum surrounding it
Posterior wall develops into pars intermedia
26. Anatomic overview
• Hypophysis cerebri
• Master of endocrine orchestra
• Lies on hypophyseal fossa/ sella turcica/ pituitary
fossa
• Roofed by diaphragmatic sella
• Stalk of pituitary pierces diaphragma sellae and
attached above to the floor of third ventricle
27.
28.
29. Arterial supply
• Branches of internal carotid artery
• Venous drainage
Veins on surface of
gland drain to dural
venous sinuses
30. Craniopharyngioma
• Rare embryonic malformation of sellar and
parasellar area with low histological grade of
non glial intra cranial tumor.
• From remnants of Rathke’s pouch along a line
of nasopharynx to diencephalon (Rathke’s/
hypophyseal tumor)
• 1-2% of all intracranial childhood tumors
• Solid or mixed (solid-cystic tumors)
• Males = Females
• Bimodal age distribution
33. Pre operative evaluation and
management
• Assessment of endocrine function: correction
prior if possible.
• Detailed neuropthalmic examination
• Peri tumoral edema and increased ICP
controlled
• Hydrocephalus: permanent/ temporary shunt
• Aspiration of large cystic component prior
surgery
• Volume status and electrolyte disbalance.
35. Complications of surgery
• Recurrance
• Long term:
1. Pituitary deficiencies
Transient post surgical diabetic insipidus (80-
100%)
Permanent post surgical diabetes insipidus (40-
93%)
2. Visual deficits
3. Neurological sequalae: hemiparesis, epilepsy,
cranial nerve deficits
36. 4. Hypothalamic dysfunctions: obesity,
behavioural change, disturbed circadian rhythm,
sleep irregularities, day time sleepiness,
dysregulation of body temperature, HR, BP,
thirst
5. Obesity and eating disorder
37. Central diabetes insipidus
• Due to deficiency of synthesis, release of
arginine vasopressin(AVP) or both
• Diagnosis:
Polyuria and polydipsia
( urine volume >1500ml/kg/day at birth, 100-
110 ml/kg/day up to 2 years, 40-50ml/kg/day
after 2 year)
Urine osmolality <300mosmol/kg in 24 hour
urine specimen
S. sodium >145mEq/l
38. • Urine osmolality < plasma osmolality
• Water deprivation test and desmopressin trial
39. • Severe damage to hypothalamus or its tract by
neurosurgery or trauma results in TRIPHASIC
RESPONSE
• Initial POLYURIC phase: within 24 hours, lasts
4-5 days
• Next 6-11 days: ADH slowly released from
degenerating posterior pituitary , transient
SIADH
• Permanent Diabetes insipidus: after posterior
pituitary stores are depleted
40. Treatment
• Volume replacement for transient CDI with iv
fluids (0.45%or 0.9%)saline
• Low dose desmopressin (5-10 mcg intranasally
or 50-100mcg orally or 0.1-0.2mcg s.c. or i.m.)
• Continous aq vasopressin infusion 0.1
mU/kg/h
Masster of endocrine orchestra: produces number of hrmones which control many other endocrine glands of the body
5-15 yrs (adamantinomatous): epithelial remnants of Rathke’s pouch or craniopharyngeal duct
50-75yrs(papillary)metaplasia of squamous epithelial cells of pituitary stalk and pars distalis.
Headache d/t traction on pain sensitive structures, obstructive hydrocephalus from tumor compression of 3rd ventricle/ meningeal irritation by escaped cyst contents
Neuropthalmic exam: determine whether there is compression of optic pathways and establish presurgical baseline
Depends on location of the tumor
Intrasellar: trans sphenoidal
Pterional craniotomy: far superior extension into 3rd ventricle requiring opening of lamina terminalis or approach above through the foramen of monro
Large volume with dilute urine
Polyuric: inhibition of adh d/t hypothalamic dysfunction
2. Excessive water intake and hyponatremia
Most of the cases do not develop permanent DI d/t less severe inj, begins after 24-48 hrs then resolves in next 1st week
Not all progress through 3 phases
Some transisent hyponatremia without preceeding polyuria