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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
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Case Presentation ~ Chief Complaint
8 yo male presents to the ED with the complaint of weakness and dizziness
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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”
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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”
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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
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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”
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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
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What Do You Want To Do Next?
What Is Your Differential? What Do You Want To Do Next?
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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
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Non-Contrast Head CT Midline Falx Frontal Horns of Lateral Ventricles
3rd Ventricle Basal Cistern Midline Falx
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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
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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
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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
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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
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Differential diagnosis in children
Vertigo Differential diagnosis in children
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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
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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
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Essential History in the Dizzy Patient
True vertigo present? Onset pattern? Symptom duration? Auditory symptoms? Associated neurological symptoms? Associated symptoms? PMH? Medications? Social? Family?
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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
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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
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Nystagmus Fast uncontrollable eye movements
Vertical vs horizontal/rotatory nystagmus Congenital nystagmus Acquired Brainstem lesion Peripheral lesion Other (i.e. medication)
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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
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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)
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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%
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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
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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
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Multiple Sclerosis on MRI
Google, fine art America; imagesofdisease.blogspot.com
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Common Pediatric Tumors
~70% occur in posterior fossa Brainstem or cerebellum Astrocytoma Ependymoma Medulloblastoma or primitive neuroectodermal tumor (PNETs) Imgarcade.com
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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
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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
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Pilocytic Astrocytoma on MRI
Commonly see solid & cystic components Neuropathology-web.org
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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
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Fibrillary Astrocytoma
Fibrillary astrocytoma is the most common diffuse low grade astrocytoma Cystic appearance
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Anaplastic Astrocytoma
Grade III tumor These rare tumors require more aggressive treatment than lower grade tumors Brain.mgh.harvard.edu; Emedicine.medscape.com
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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
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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
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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
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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
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Medulloblastoma cancerwall.com//nature.com
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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
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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
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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
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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
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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)
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EVD Appearance on CT Scan
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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):
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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
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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?
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