Dr. Aria Fallah, a fellowship trained pediatric neurosurgeon who practices at UCLA Mattel Children’s Hospital and Ronald Reagan UCLA Medical Center and Assistant Professor of Neurosurgery and Pediatrics at the David Geffen School of Medicine at UCLA, discusses hydrocephalus after hemispherectomy and other procedures at the 2017 Pediatric Epilepsy Surgery Conference and Family Reunion.
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Hydrocephalus After Hemispherectomy And Other Resective Procedures
1. Aria Fallah, MD, MSc, FRCSC FAANS, FAAP
Assistant Professor
Department of Neurosurgery
Hydrocephalus after hemispherectomies and other
resective surgeries
Pediatric Epilepsy Surgery Conference and Family Reunion
3. Objectives
• Overview of the brain's ventricular system and CSF
physiology
• Explain the Monroe Kellie hypothesis in the context of
hydrocephalus
• Diagnosis of hydrocephalus
• Discuss management options for hydrocephalus
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5. CSF production
• For each drop of CSF produced , the same amount
should be absorbed
• Infants and children produce
0.33 ml/kg/hr
• Adult = 125mL ; 20 mL in the ventricle
• In General 15-20mL CSF is made hourly
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6. Where is CSF produced?
• Choroid Plexus – 50-85%
Extrachoroidal sources – up to 30%
• Ependymal layer
• Brain parenchyma
• Spinal subarachnoid spaces
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7. Where is CSF absorbed?
• Arachnoid villi – primary site
Other sites
• Lymphatic drainage
• Brain capillaries
• Choroid Plexus
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8. Physiology
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Skull is a closed box, “mostly inelastic”
Monroe-Kellie doctrine:
•Sum of volumes of brain, CSF and intracranial blood
is constant and incompressible
•Increase in volume can lead to significant raise in ICP
•Brain has limited compliance
Alexander Monroe
(physicist)
10. What is Hydrocephalus?
•An imbalance between the production and absorption of
cerebral spinal fluid (CSF)
•This results in an increased volume and an increase in
intracranial pressure
•*May or may not be associated with a change in the
ventricular size
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11. FACTS
• Hydrocephalus is Common
• Affects 1 or 2 of every 1000 babies born
• Most common reason for neurosurgery in children
• There is No Cure
• No effective medical therapy
• The only effective treatments are surgical
• It is often a life-long condition
• Children with hydrocephalus require long-term follow-up
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12. Hydrocephalus
• Due to increased production of CSF
• Due to obstruction in fluid pathways
• Due to impaired absorption
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13. Anatomic hemispherectomy
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• Highest chance for blood loss
• Highest risk of hydrocephalus
• Most certain procedure to ensure
disconnection
• Indicated for persistent seizures after
functional hemispherectomy
15. Hydrocephalus
• Due to increased production of CSF
• Due to obstruction in fluid pathways
• Due to impaired absorption
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16. Evolution of hemispherectomy techniques
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• Smaller craniotomy
• Reduced operative time
• Reduced blood loss
• Decreased risk of
infection
• Decreased risk of
developing
hydrocephalus
17. Decreasing the risk of hydrocephalus following
hemispherectomy
• Disconnective hemispherectomy as opposed to
resective hemispherectomy
• Decreasing blood loss at the time of surgery
• Decreasing the amount of blood inside the ventricular
system
• Aggressive drainage of CSF following hemispherectomy
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19. Symptoms and Signs in Infants
• Abnormally increase in head size
• Drowsiness
• Irritability
• Poor feeding / Vomiting
• Breath-holding Spells, Slow heart rate, Bulging
and Tense Fontanel
• Head circumference growing faster than
expected
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20. Symptoms and Signs in Older Children
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• Headaches
• Learning problems
• Nausea and Vomiting
• Drowsiness
• Loss of coordination and difficulty walking
• Swelling of the optic nerve
• Inability to look up
22. Natural history of untreated hydrocephalus
• ½ untreated patients die
• ½ survive with “arrested hydrocephalus”
• Of these, 15% have normal cognitive and neurologic findings
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28. Complications
• Early complications: hematoma, bowel injury
• Infection: most commonly in first 3 months, 90% of
shunt infections within 6 months
• Most common: Proximal shunt obstruction
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29. Management of shunt malfunction
• Confirmation of diagnosis
• CT scan/FAST MRI, shunt series
• Shunt tap
• Site of malfunction
• Rule out infection – fever, abdominal pain, CRP, ESR
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35. Are we able to predict success?
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with choroid plexus
cauterization
ETV Success Score
Warf BC, et al. J Neurosurg Pediatr 5:143-148, 2010Kulkarni AV, et al.. J Pediatr 155:254-259.e251, 2009
36. 36
Results of ETV and CPC
Weil, A.G., Westwick, H., Wang, S. et al. Childs Nerv Syst (2016)
37. Prognosis in hydrocephalus
• Neurologic and intellectual disabilities depend on:
• Cause of hydrocephalus
• Thickness of the brain and corpus callosum
• Requirement for a shunt
• Presence of other brain anomalies
• Associated conditions: IVH, infection, etc. may play a larger role
than the hydrocephalus alone.
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38. How should we measure success?
• Number of operations?
• Quality of life
• Neuropsychological
• Education/Job
• Social interactions
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39. Conclusion
• Hydrocephalus is a deceptively complex disorder
• Careful consideration of the cause of hydrocephalus is
required in all cases
• This may affect decision making, family counseling,
prognosis and outcome
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Most common disorder treated by pediatric neurosurgeons
Over 30,000 shunts are placed annually in the united states.
CSF constantly produced and absorbed.
Siphon resisting valves prevent against siphoning. (Delta and other types of valves)