case presentation

1. DR.MUMTAZ ALI
DEPARTMENT OF NEUROSURGERY
JPMC KARACHI

2. History
 8 yrs old ,boy, student ,right handed ,resident of badin
with NKCM came to OPD with c/o of :
 headache for 6 months
 Gait distrubance for 1 month
 No significant hx of seizures , dec vision ,weakness
,urinary incontinence , dec hearing , backache

3.  Headache is started 6 months back , generalized ,
gradual , moderate to severe , heaviness ,progressive ,
continuous ,usually occur in morning .It is aggravated
by coughing , straining ,micturition and relieved by
erect posture when he walks or as day spent and pain
killers.It is associated with vomitting for 15 days which
are projectile , multiple in episodes , vomitts what ever
he eats or drinks having colour of drink and food.It is
also associated with blurring of vision in both eyes
since 15 days which is more when headache is severe in
morning then it settles.He can see all things and read
and write otherwise.

4.  Gait distrubbance is started 1 month back which is gradual
and progressive , sways on right side having multiple
episodes of Fell down on floor during walking.He has
difficulty in maintaining balance.He walks with support
since 15 days.
 It is not associated with vertigo, facial numbness fascial
asymmetry ,dec hearing change in voice and difficulty in
swallowing.
 It is not associated with anxiety , change in behaviour
,memory loss , calculation , combing ,buttoning , reading ,
writing.
 It is not associated with fever , weight loss , TB contact and
trauma

5.  No significant hx of endocrine and systemic symptoms
 No significant past medical and surgical hx , personal
hx , family hx , socioeconomic hx , Allergic hx.
 Drug hx = syp : brufen sos
 LEVEL and D/D ON HX :
 LEVEL : Posterior fossa midline neoplastic lesion
involving right cerebellum and compressing IV
ventricle that causing Hydrocephalus
 D/D :
 Medulloblastoma , astrocytoma , ependymoma

6. Clinical examination
 A young boy , well dressed ,ill looking ,well oriented
with time place and person with normal built and
height with canula in left hand ,lying comfortably on
bed with following vitals and non vitals
 Pulse : 78 bpm , BP : 120/80 mm hg , RR : 16
breaths/min , T: A/F
 Pallorness , clubbing , koilynchia dehydration , cynosis
, jaundice , lymphadenopathy , edema not present.
 No buldge on scalp palpation and scar mark of Vp
shunt presnet on head
 No thydroid swelling is palpated on deglutition

7.  Important positive findings :
 Nystagmus present
 Bilateral papilledema
 Finger nose test on right side
 Disdiadockinesia on right side
 Dec tone on right side
 Heed shin test on right side
 Rhomberg test positive
 Important negative findings :
 MME 30/30
 Primitive reflex= absent
 Pronator drift
 Lobar signs
 Viscual acuity and visual field =
normal
 Facial weakness
 Gag reflex absent
 Head titubation
 Scanning speech
 Tremors
 Pendular knee jerk
 Plantars downgoing

8. LEVEL & D/D ON C/E FINDINGS
 LEVEL : Posterior fossa midline neoplastic lesion
involving right cerebellum and compressing IV
ventricle that causing Hydrocephalus
 D/D :
 Medulloblastoma
 astrocytoma
 ependymoma

9. INVESTIGATIONS
 SPECIFIC :
 MRI Brain with contrast
 CT scan Brain
 ROUTINE :
 CBC , UCE , PT/APTT/INR , hep b & c , CXR
 RELEVANT : MRI spine with contrast

14.  HB=12
 TLC=8
 PLT=250
 Na=131
 K=3.5
 INR=1.05
 Urea=40
 Creatinine =1.3

15. Diagnosis
 Medulloblastoma and HCP

16. MANAGEMENT
 Admission
 Counselling
 Optimization
 Preop Care
 Surgery :
 HCP : VP shunt
 Definitive : Midline Suboccipital craniectomy and tumor
ressection and postopt XRT if needed
 Postop Care
 Followup
 Rehabilitation

17. Midline suboccipital craniectomy
 Shifting : After consent and counselling pt is shifted to OT
 Position : Prone and head flexion
 Pin fixation : mayfield
 Equipments : Microscope , intraoperative u/s , bipolar , cusa ,
 Neuromonitoring : V CN , VII CN ,ABR , SSEP , MEP
 Midline scalp incision: EOP to C2 spinous process
 Pericranial graft harvest: above suboccipital muscles
 Midline dissection: The ligamentum nuchae is a relatively avascular tissue
along the midline between the suboccipital muscles. It is incised, and self-
retaining retractors are placed to move the muscles laterally.
 Exposure of the subocciput: Suboccipital muscles are separated from the
occipital bone, posterior arch of C1, and superior half of the posterior arch of
C2. Dissection of C1 should be subperiosteal with blunt instruments to avoid
injury to vertebral artery and venous plexus laterally.


18.  Craniotomy:
 Removal of the posterior arch of C1: if tumor with tonsillar herniation
 Dural opening: Y-shaped fashion, to avoid the enlarged occipital and circular
venous sinuses injury
 Microscopic dissection
 CSF drainage for relaxation: cisterna magna is then opened, and CSF is
drained
 Identification of normal anatomy: cerebellar tonsils, spinal cord, obex,
arteries (PICA and vertebral artery), and cranial nerves bilaterally.
 Gentle elevation of cerebellar tonsils: to identify the floor of the fourth
ventricle. Cottonoids are placed over the floor to protect the brainstem and
spinal cord.
 Resection of the inferior vermis: required for intraventricular tumors that
extend superiorly to avoid extensive cerebellar retraction.
 Telovelar (cerebellomedullary fissure) approach: facilitate resection of
tumors that extend to the lateral cisterns and may eliminate the need for
resection of the vermis.

19.  Intraoperative ultrasound: to identify the echogenic tumor, to
verify the completion of resection , identifying the path through
the least amount of cerebellar tissue
 Tumor resection: CUSA + Bipolar + suction. Orientation of
floor of the fourth ventricle must always be maintained to avoid
injury to the brainstem. Small amounts of tumor that are clearly
attached to the brainstem or firmly attached to cranial nerves are
not resected. A small amount of tumor left behind (< 1.5 cm2 on
postoperative MRI scan) is not associated with worsened
outcome
 Hemostasis secure
 Dural closure
 Replacement of bone flap
 Standard muscle and skin closure

20. Complications
 Postop headache
 Postop edema and swelling
 Pseudomeningocele
 CSF leak
 Lower cranial nerve injury
 Brainstem injury
 Vertebral and PICA injury
 Acute HCP
 Craniotomy complications

21. Outcome
 Standard-risk patients :
 No residual tumor on post-op MRI and negative CSF results. 5-year
survival is >5%, and progression-free
 survival= 50%48,49
 Poor-risk patients :
 Bulky residual tumor > 1.5 cm2 post-op and dissemination in the brain,
spine or CSF. Worse prognosis. 5-year
 disease-free survival is 35–50%50
 Intermediate risk patients :
 An intermediate risk group probably exists, but has been poorly
characterized
 Poor prognosticator factors :
 Age < 3 years , subtotal resection , disseminated disease ,histological
differentiation , boys

22. THANKS