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Hydrocephalus After Hemispherectomy And Other Resective Procedures

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. 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
  2. 2. No conflicts of interest 2
  3. 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 3
  4. 4. 4 Anatomy of the ventricular system
  5. 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 5
  6. 6. Where is CSF produced? • Choroid Plexus – 50-85% Extrachoroidal sources – up to 30% • Ependymal layer • Brain parenchyma • Spinal subarachnoid spaces 6
  7. 7. Where is CSF absorbed? • Arachnoid villi – primary site Other sites • Lymphatic drainage • Brain capillaries • Choroid Plexus 7
  8. 8. Physiology 8 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)
  9. 9. 9
  10. 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 10
  11. 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 11
  12. 12. Hydrocephalus • Due to increased production of CSF • Due to obstruction in fluid pathways • Due to impaired absorption 12
  13. 13. Anatomic hemispherectomy 13 • Highest chance for blood loss • Highest risk of hydrocephalus • Most certain procedure to ensure disconnection • Indicated for persistent seizures after functional hemispherectomy
  14. 14. Hydrocephalus following an anatomic hemispherectomy 14 In up to a third of patients
  15. 15. Hydrocephalus • Due to increased production of CSF • Due to obstruction in fluid pathways • Due to impaired absorption 15
  16. 16. Evolution of hemispherectomy techniques 16 • Smaller craniotomy • Reduced operative time • Reduced blood loss • Decreased risk of infection • Decreased risk of developing hydrocephalus
  17. 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 17
  18. 18. “Baby’s Soft spot” 18 Head circumference chart Detecting hydrocephalus
  19. 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 19
  20. 20. Symptoms and Signs in Older Children 20 • Headaches • Learning problems • Nausea and Vomiting • Drowsiness • Loss of coordination and difficulty walking • Swelling of the optic nerve • Inability to look up
  21. 21. Diagnosing hydrocephalus 21 CT Ventricular shunt Cavity shunt
  22. 22. Natural history of untreated hydrocephalus • ½ untreated patients die • ½ survive with “arrested hydrocephalus” • Of these, 15% have normal cognitive and neurologic findings 22
  23. 23. Shunt components 23 Ventricular catheter Valve Distal Catheter
  24. 24. Ventriculoperitoneal shunt 24 Journal of Neurosurgery: Pediatrics Alternative sites •Pleural cavity •Atrium
  25. 25. Types of valves 25
  26. 26. Types of valves 1. Differential Pressure Valves • Constant Differential Pressure regardless of flow 1. Flow-regulated Valves – constant flow • Constant flow regardless of differential pressure 1. Anti-siphon devices 2. Programmable valves 26 Source: Drake et al. 1998
  27. 27. Shunt complications • 3 types: • Mechanical failure – improper placement, migration, obstruction, fracture, disconnection • Infection/skin problems • Functional failure (inadequate flow rate of a functioning shunt) • 3 origins: • Shunt • Patient • Surgeon 27
  28. 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 28
  29. 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 29
  30. 30. Three types of shunt patients 30
  31. 31. 31
  32. 32. Endoscopic Treatment 32
  33. 33. Is one better than the other? 33 Kestle et al. Childs Nerv Syst (2009)
  34. 34. Addition of Choroid Plexus Cauterization 34
  35. 35. Are we able to predict success? 35 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. 36 Results of ETV and CPC Weil, A.G., Westwick, H., Wang, S. et al. Childs Nerv Syst (2016)
  37. 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. 37
  38. 38. How should we measure success? • Number of operations? • Quality of life • Neuropsychological • Education/Job • Social interactions 38
  39. 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 39
  40. 40. 40 Resources http://www.hydroassoc.org/ https://medlineplus.gov/hydrocephalus.html

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