1. Moderator / ICU: Jeremy Barnett MD Neurology: Janet Tamai MD
Kingston Hospital Grand Rounds Pediatric Brain Tumor Presenting as Dizziness & Otitis Media
Moderator / ICU: Jeremy Barnett MD
ED: Amy Gutman MD
Neurology: Janet Tamai MD

2. Case Presentation ~ Chief Complaint
8 yo male presents to the ED with the complaint of weakness and dizziness

3. Initial Presentation
8 yo M, JS, presents at 0840 to room 19 via private vehicle
Nurse immediately came to Core 1 asking me to see patient rather than wait for 0900 MD
Patient laying limp on the stretcher, with mother at bedside. Maintaining airway, but clearly struggling to move & communicate
Mother states that he had been diagnosed with an ear infection, but today “was dizzy, couldn’t walk & was wobbly”

4. History of Present Illness
Mother reports 4 days ago child vomited once, dry heaving yesterday but otherwise acting / eating “normally” except for complaining of dizziness
Mother called PCP yesterday, who advised them to start antihistamines due to history of similar episodes with ear infections / congestion
Mother also called child’s neurologist / autism specialist who stated “don’t worry about JS’s symptoms”, but would see him in the office if he worsened
Last night child having difficulty walking, unable to stand unassisted, & unable to stand this am due to “dizziness”

5. Case Presentation ~ SHx, FHx, PMH
Social:
Non-smoking home
2nd grade
Family History
No pertinent family history
Allergies
NKDA
Medications
Risperdal, lorazepam
PMH
Full-term
Autism, OM

6. Review Of Systems (Per Mother)
General: Anxious, toxic, ill appearing & unable to communicate
HEENT: Ear “fullness”, nasal congestion, no visual complaints
Pulm: (-) SOB, DOE, PND
CV: (-) CP, palpitations
GI: (+) vomited once yesterday
Skin: (-) rashes, lesions
Neuro: Cannot ambulate, “can’t move or feel” left side, “dizzy”

7. Physical Examination General: CV: GI: Vitals: Neuro: Skin: HEENT:
Awake & interactive. Appears “scared”, responds to staff, maintains airway
Vitals:
T 98.3 oral, HR 98, Resp 16, Wt 35.7kg BP 124/71, Pulse Ox 100% ra
Skin:
Warm, dry, (-) rash, (-) temp differential
HEENT:
(-) swelling, tenderness, LAD, tongue deviation. TMs unremarkable
Pulm:
CTA AF BL, no distress
CV:
S1S2 RRR (-) MRG
GI:
Soft NT ND (+) BSAQ
Neuro:
Mild right eye deviation which corrects to center. PERRLA at 3 mm = BL & briskly reactive; otherwise CN 2-12 intact
LUE grip 3/5, LE BL 5/5
(-) Babinski BL
Falls to left side when tries to ambulate
(+) vertical nystagmus BL 5 beat

8. What Do You Want To Do Next?
What Is Your Differential?
What Do You Want To Do Next?

9. Labs CBC: Unremarkable CMP: Unremarkable Coags: Unremarkable
Urinalysis: Ordered, Not collected
Cardiacs: Unremarkable
ESR: Pending at time of transfer; slight elevation
Lactic Acid: 22 (elevated)
ED Lyme: Ordered, not sent

10. Non-Contrast Head CT Midline Falx Frontal Horns of Lateral Ventricles
3rd Ventricle
Basal Cistern
Midline Falx

12. Non-Contrast Head CT
Diffuse low density in left cerebellum consistent with acute infarct.
Slight deviation of 4th ventricle to the right side. No acute hemorrhage. Supratentorial area, brainstem unremarkable, calvarium intact.
Impression: Acute infarct in left cerebellum

13. ED Course
Case discussed with Dr P, pediatric neurologist at Albany Medical Center who advised on acute ED management
Suspected underlying cerebellar mass due to CT appearance
Asked us to not wait for MRI, & rapidly transport
Interventions:
Oxygenation & airway protection
NS IVF pediatric bolus
Keppra 250 IV
Signal 9 transfer
Notified patient’s pediatric neurologist & pediatrician

14. Albany Medical Center Course
“JS is an 8 yo male admitted from an outside hospital for neurosurgical evaluation in regards to left cerebellar infarct, hydrocephalus & swelling of the posterior fossa.”
“The patient was taken emergently to the OR for suboccipital craniectomy & partial resection of the left cerebellum and dural augmentation, as well as a external ventricular drain for hydrocephalus. The patient tolerated the procedure well.”
Pathology: Glioblastoma

15. Albany Medical Center Course
“The most recent CT scan re-demonstrated the evolving infarct within the left cerebellum with slightly improved areas of trace pneumocephalus and hemorrhage.”
“No new hemorrhage identified…there was noted to be a slight decrease in the size of the anterior 3rd ventricle in comparison to the prior CT scan 1 month earlier”.
MRI showed a cerebellar infarct, appearing as a bright signal on the diffusion-weighted image

16. Differential diagnosis in children
Vertigo
Differential diagnosis in children

17. Vertigo Definition: Need to differentiate between
A sensation of movement when there is none. Commonly a spinning sensation, but can be any type of movement
Need to differentiate between
“Dizziness”
Ataxia
Weakness
Confusion

18. Pediatric Dizziness & Vertigo
1st reported by Harrison in 1962
Uncommon pediatric complaint
>560,000 pediatric encounters over a 4-year period showed prevalence of 0.4% (dizziness), 0.03% (peripheral vertigo), & 0.02% (central vestibulopathy)
History & exam challenged by vocabulary, distractibility & compliance
Common impression that symptoms secondary to lack of coordination or behavioral
Remarkable compensation for vestibular deficits that in adults results in disequilibrium and / or ADL limitations

19. Essential History in the Dizzy Patient
True vertigo present?
Onset pattern?
Symptom duration?
Auditory symptoms?
Associated neurological symptoms?
Associated symptoms?
PMH?
Medications?
Social?
Family?

20. Neurologic Exam Keep a look out for red flags!
Inspection for alertness, GCS, AVPU
Developmental: cognitive, behavioral, dysmorphia
Cranial nerves, especially III, IV, VI
Eyes: nystagmus, diplopia
Hearing: tinnitus, hearing loss
Coordination:
Gait, limb ataxia, cerebellar signs
Motor / Sensory:
Focal weakness or sensory changes
Peripheral Nerves

21. Eye Movements Internuclear ophthalmoplegia:
Medial longitudinal fasciculus (MLF) Lesion
Cranial nerve 3, 4 or 6 palsy
Cn 3: Oculomotor
Cn 4: Trochlear
Cn 6: Abducens
Gaze palsy:
Pontine lesion
Ophthalmoplegia

22. Nystagmus Fast uncontrollable eye movements
Vertical vs horizontal/rotatory nystagmus
Congenital nystagmus
Acquired
Brainstem lesion
Peripheral lesion
Other (i.e. medication)

23. Vertigo Differential Diagnosis
Migraine
Seizures
Aura
Infection
Peripheral: otitis media, labyrinthitis
Central: meningitis, encephalitis
Head trauma
Structural lesions
AVMs, Tumors, Arnold-Chiari
Drug overdose
i.e. NSAIDs, anticonvulsants
Benign paroxysmal vertigo of childhood
Recurrent vertigo, normal in between episodes, self limited after years
Benign paroxysmal positional vertigo
Rare in children
Stroke:
Hypercoagulable state, i.e. Sickle cell disease, genetic/inherited conditions, elevated homocysteine
Trauma (i.e. dissection)
Congenital heart disease

24. Central vs Peripheral Vertigo
Symptom
Central
Peripheral
Nausea
Mild-Moderate
Severe
Imbalance
Moderate-Severe
Moderate
Hearing Loss
Rare
Common
Tinnitus
Possible
Oscillopsia
Mild
Neurological
Compensation
Slow
Rapid
Nystagmus
Pure vertical
Unchanged with fixation
No fatigability
No latency
Mixed torsional, vertical/horizontal
Dampens with fixation
Fatigability (BPPV)
Latency post maneuver (BPPV)

25. Pediatric Vertigo Etiologies
Diversity of etiologies present with complaint of “vertigo” or “dizziness”
Detailed history & otoneurological exam essential
Most common etiology is migraine
Accompanies vertigo, but dominates clinical picture
Migraine in 35% of vertigo pediatric pts vs 6% adults
Though 10% children meet International Headache Society criteria for migraine, vestibular symptoms occur in about 25%

26. Migraine Family history Symptoms Car sickness Imaging normal
Can occur even in 1-2 year olds, but difficult to diagnose until older
Symptoms
Throbbing head pain
Nausea, vomiting
Photophobia
Sonophobia
Visual aura
Focal symptoms
Vertigo

27. Google, fine art America; imagesofdisease.blogspot.com
Multiple Sclerosis
Demyelinating CNS disorder
Most commonly in young adults, but can occur in children
2-5% of adult patients had symptoms prior to age 18
Google, fine art America; imagesofdisease.blogspot.com

28. Multiple Sclerosis on MRI
Google, fine art America; imagesofdisease.blogspot.com

29. Common Pediatric Tumors
~70% occur in posterior fossa
Brainstem or cerebellum
Astrocytoma
Ependymoma
Medulloblastoma or primitive neuroectodermal tumor (PNETs)
Imgarcade.com

30. Normal astrocytes comminfo.rutgers.edu
Cell Types
Three types of normal glial cells can produce tumors
Astrocytes produce astrocytomas (including glioblastomas)
Oligodendrocytes produce oligodendrogliomas
Ependymal cells produce ependymomas
Tumors that display a mixture of different cells are “mixed gliomas”
Normal astrocytes comminfo.rutgers.edu

31. Pilocytic Astrocytoma (Juvenile Pilocytic Astrocytoma)
Most common Grade 1 astrocytomas
Typically do not spread
Considered “most benign” (noncancerous) of all the astrocytomas
Other Grade 1 astrocytomas:
Cerebellar astrocytoma
Desmoplastic infantile astrocytoma
Based on pathology
Pathologyoutlines.com

32. Pilocytic Astrocytoma on MRI
Commonly see solid & cystic components
Neuropathology-web.org

33. Diffuse Astrocytoma Also called Low-Grade or Astrocytoma Grade II
Types:
Fibrillary
Gemistocytic
Protoplasmic
Tend to invade surrounding tissue and grow at a relatively slow rate
More common in the cerebral hemispheres

34. Fibrillary Astrocytoma
Fibrillary astrocytoma is the most common diffuse low grade astrocytoma
Cystic appearance

35. Anaplastic Astrocytoma
Grade III tumor
These rare tumors require more aggressive treatment than lower grade tumors
Brain.mgh.harvard.edu; Emedicine.medscape.com

36. Grade IV Astrocytoma ~ Glioblastoma
Previously called “Glioblastoma Multiforme,” “Grade IV Glioblastoma,” and “GBM”
Two types of astrocytoma Grade IV
Primary, or de novo
Most common form, very aggressive
Secondary
Originate as a lower-grade tumor & evolve into a grade IV tumor
Radiologyteacher.com

37. Brainstem Glioma
Named for location, not tissue type from which they originate
Glioblastoma
Astrocytoma
Hemorrhage and necrosis (seen in the bottom image) indicates Grade IV
Brain tumor foundation of canada website;
Neuropathology-web.org

38. Ependymoma
Arise from ependymal cells that line the ventricles of the brain and the center of the spinal cord
30% diagnosed in children under age 3
Grades 1-3
Grade 1: Subependymomas
Grade 1: Myxopapillary ependymomas
Grade 2: Ependymoma
4 subtypes
Grade 3: Anaplastic ependymoma
abta.org

39. Medulloblastoma / Primitive Neuroectodermal Tumors (PNET)
Always located in the cerebellum
Unusual to spread outside brain & spinal cord
However, as CSF “seeding” common, need to image entire neuroaxis to identify drop metastasis and leptomeningeal spread
>70% of all pediatric medulloblastomas are diagnosed in children under age 10

40. Medulloblastoma
cancerwall.com//nature.com

41. PNET / Primitive Neuroectodermal Tumor
Pathology identical to medulloblastoma, but occurs primarily in cerebrum
Pineoblastoma, polar spongioblastoma, medulloblastoma, medulloepithelioma
Very rare
Treatment
Biopsy & resection/debulking if possible
Radiation
Chemotherapy
Supportive care

42. Suboccipital Craniectomy
Performed to remove a tumor or hematoma from beneath the skull
Suboccipital approach allows exploration of posterior fossa & associated cranial nerves
5th CN cut to treat trigeminal neuralgia
8th CN (vestibular branch of the vestibulocochlear) cut to surgically treat Ménière's disease
9th CN cut to treat glossopharyngeal neuralgia
Remove acoustic neuromas from auditory canal

43. Duraplasty provides additional room for cerebellar tonsils at craniocervical junction, to close dura & prevent CSF leakage
Primary dural closure difficult in many neurosurgical cases
Options for dural grafting include monolayer collagen & bilayer collagen sponges

45. External Ventricular Drain (EVD)
CSF circulates around brain & spinal cord then reabsorbed via arachnoid villi
Pediatric ventricular system produces CSF at ~0.35mls/kg/hr via choroid plexus of lateral ventricles
Normal ICP is 0-20 mmHg 
EVD is a temporary system allowing CSF drainage from ventricles to an external system

46. EVD Monitoring
EVD pressure transducer maintained at the same horizontal level as the ventricles for reliable interpretation
Laser level device should be in line with the patient's Foramen of Monro
If patient supine, level EVD system to the ear tragus
If the patient upright, level EVD to the mid sagittal line (between eyebrows)

47. EVD Appearance on CT Scan

48. References
Cohen-Kerem MR et al. Vertigo in Children and Adolescents: Characteristics and Outcome. ScientificWorldJournal. 2012; 2012: ePub 2012 Jan 3. 
Tintinelli. Emergency Medicine – A comprehensive Study Guide. “Pediatric Neurological Emergencies”
UpToDate.com. “Vertigo”, “Pediatric Dizziness”, “Pediatric Stroke”
Yang WC, Chen CY, Wu KH, Wu HP. Acute onset of dizziness caused by a cavernous malformation lateral to the fourth ventricle: a case report. Pediatr Neonatol 2011 Apr;52(2):113-6.
Reed Group MDGuidelines. “Suboccipital Craniectomy”
Litvack ZN, West GA, Delashaw JB, Burchiel KJ, Anderson VC. Dural augmentation: part I-evaluation of collagen matrix allografts for dural defect after craniotomy. Neurosurgery. 2009 Nov;65(5):890-7; discussion 897.
Albanese, J; Leone M; Alliez JR; Kaya JM; Antonini F; Alliez B; Martin C (2003). "Decompressive craniectomy for severe traumatic brain injury: Evaluation of the effects at one year". Critical Care Medicine 31 (10): 2535–2538.
Hejazi, N; Witzmann A; Fae P. "Unilateral decompressive craniectomy for children with severe brain injury. Report of seven cases and review of the relevant literature". European Journal of Pediatrics 161 (2): 99–104. 
Agannathan, J; Okonkwo DO; Dumont, AS. "Outcome following decompressive craniectomy in children with severe traumatic brain injury: a 10-year single-center experience with long-term follow up". Journal of Neurosurgery Pediatrics. April  (4): 268–275. 
Adelson. P., et al. Intracranial pressure monitoring. Paediatric Critical Care. 4(3) (suppl.):S28-S
Tippett N.  Intracranial pressure (ICP) monitoring and extraventricular drains (EVD). Bayside Health, Alfred ICU:
Photo Search: wikipedia, google, bing
Muralidharan R.External ventricular drains: Management and complications. Surg Neurol Int May 25;6(Suppl 6):S271-4.
Lau T, Reintjes S, Olivera R, van Loveren HR, Agazzi S. C-shaped Incision for Far-Lateral Suboccipital Approach: Anatomical Study and Clinical Correlation. J Neurol Surg B Skull Base Mar;76(2):  
Klimo P Jr, Nesvick CL, Broniscer A, Orr BA, Choudhri AF. Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas.J Neurosurg Pediatr Oct 16:1-9. 
Hopkinsmedicine.org
Medscape.com
Klimo P Jr, Nesvick CL, Broniscer A, Orr BA, Choudhri AF. Malignant brainstem tumors in children, excluding diffuse intrinsic pontine gliomas.. J Neurosurg Pediatr Oct 16:1-9.
PDQ Pediatric Treatment Editorial Board. Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®): Health Professional Version. PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); Sep 24.
Aghayan Golkashani H, Hatami H, Farzan A, Mohammadi HR, Nilipour Y, Khoddami M, Jadali F. Tumors of the Central Nervous System: An 18-Year Retrospective Review in a Tertiary PediatricReferral Center. Iran J Child Neurol Summer;9(3):24-33.
Assina R, Meleis AM, Cohen MA, Iqbal MO, Liu JK. Titanium mesh-assisted dural tenting for an expansile suboccipital cranioplasty in the treatment of Chiari 1 malformation. J Clin Neurosci Sep;21(9):
Robertson SC, Lennarson P, Hasan DM, Traynelis VC. Clinical course and surgical management of massive cerebral infarction. Neurosurgery Jul;55(1):55-61; discussion 61-2.
Tomita T, Grahovac G. Cerebellopontine angle tumors in infants and children. Childs Nerv Syst Oct;31(10):

49. Patient Follow-Up EVD removed 2 weeks after initial surgery
As of 3 months after the initial presentation, patient with improved speech, motor function and ambulation
No evidence of metastatic spread
Weaned off TPN, tolerating po
Unclear if any long-term cognitive insults, but is able to ambulate & has regained strength on his left side

50. Summary
Pediatric brain tumor is a rare cause of dizziness, but needs to be considered if the history & exam suggestive of a significant or focal neurological deficit
A through history & physical exam is often a better diagnostic “tool” than any radiologic testing
Thank you for your attention today ~ any questions?