Hydrocephalus: Where Have We Been? And Where Are We · PDF file•Acute Hydrocephalus...
Transcript of Hydrocephalus: Where Have We Been? And Where Are We · PDF file•Acute Hydrocephalus...
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Hydrocephalus:
Where Have We Been?
And
Where Are We Going?Garni Barkhoudarian, MD
Daniel Franc, MD, PHD, Giselle Tamula, NP, Amy Eisenberg, NP
Saturday, September 23, 2017
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Disclosures
• Consultant: VTI
• Anatomy lab receives financial or material support from:
– Karl-Storz Endoskope America, Inc
– Stryker Corporation
– Mizuho America, Inc
– Surgical West, Inc
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Objectives
1. The participants will be able to identify the different types of hydrocephalus
2. The participants will be able to recognize the different treatment modalities of hydrocephalus
3. The participants will be able to identify the signs and symptoms of hydrocephalus
4. The participants will recognize the diagnostic criteria for Normal Pressure Hydrocephalus.
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Hydrocephalus
Hydrocephalus: “Water on Brain”
• “hīdrōˈsefələs”
• Hudrokephalon (greek)
• Dysfunctional cerebrospinal fluid circulation
• AKA:
– Hydro
– HCP
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Hydrocephalus
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Ventricles
Ventricular System:
• Lateral Ventricles (2)
– Foramen of Munro
• 3rd Ventricle
– Aqueduct of Sylvius
• 4th Ventricle
– Foramen of Lushka (2)• Lateral
– Foramen of Magendi• Midline
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Choroid Plexus
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Cerebrospinal Fluid
• Produced by choroid plexus• Absorbed by arachnoid granules• ~140ml total CSF
– 25ml in ventricles
• Rate of 0.2-0.7ml/kg/hr– 25-30ml/hr– 600-700ml/day
• Relies on Na+/K+ ATP dependent pump and carbonic anhydrase
• Acetazolamide can partially decrease production of CSF
– Can cause hypercapnia that can increase CBF and possibly ICP
• Other inhibitory agents:– Halothane– Angiotensin II– Vasopressin (V1 receptor)
NO OFF SWITCH!
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Hydrocephalus
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Hydrocephalus
Categorizations:
• CSF dynamics
• Timing
• Etiology
• Intracranial Pressure
• Age of patient
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CSF Dynamics
• Obstructive Hydrocephalus
– Aqueductal Stenosis
– Cysts (colloid cysts, neuroenteric cysts)
– Tumors (cerebellar tumors, intraventricular tumors)
• Communicating Hydrocephalus
– AKA “non-obstructive hydrocephalus”
– Infection (meningitis)
– Hemorrhage (subarachnoid)
– Age (normal pressure HCP).
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Hydrocephalus Timing
• Acute Hydrocephalus
– Life threatening
– Due to hemorrhage, tumors
• Chronic Hydrocephalus
– Compensated by brain
– Delayed outcome following:• Infection
• Hemorrhage
• Congenital malformation
• Normal Pressure HydrocephalusChronic Acute
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Signs and Symptoms:
1. Headache
2. Nausea/Vomitting
3. Blurred Vision / Diplopia
4. Focal Neurological Findings1. Dilated pupil(s)
2. Hemiparesis
5. Altered Mental Status1. Confusion
2. Lethargy
3. Obtundation
4. Stupor
5. Coma
Elevated ICP
Cushing’s Reflex:
• Hypertension
• Bradycardia
• Irregular Respirations
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Monroe - Kellie Hypothesis:
(Not really a doctrine…)
1. Skull is a closed box
2. In the setting of a mass lesion:
1. Displaceable fluids will compensate first
2. Elevated intracranial pressure subsequently occurs
Monroe – Kellie
Hypothesis
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Hydrocephalus
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Presentation
Unique findings with hydrocephalus:
• Headaches (worse when lying flat)
• Papilledema
• Diplopia (Horizontal)
• Parinaud’s Syndrome
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Parinaud’s Syndrome
Due to lesion compressing “tectal plate”
1. Upgaze paralysis
2. Argyll-Robertson Pupils1. (light-near dissociation)
3. Convergence-retraction nystagmus1. With upgaze or opticokinetic drum
spinning down
4. Eyelid Retraction (Collier’s Sign)
5. Conjugate down-gaze (sun-setting sign)
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Other CSF syndromes
• Normal Pressure Hydrocephalus– Elderly population– Treatable cause of dementia
• Pseudotumor Cerebri– Symptoms of hydrocephalus (or tumor)– Normal CT or MRI– Elevated ICP (on LP)– One etiology is obesity
• “Slit Ventricle Syndrome”– In Chronically overshunted patients– Stiff ventricles (no change across wide pressure range)– CT Scans look normal or overdrained (slits)
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HCP Management
• Acute or Not?
– Should we wake up the OR team?
– Can we wait for the OR?
• Etiology
– Tumor
– Hemorrhage
• Patient status
– Hemodynamically unstable (Cushing’s response)
– Infection / Meningitis
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1. Elevation of Head > 30°
2. Hyperventilation
3. Diuresis
4. Sedation
5. Metabolic Coma
6. Systemic Hypothermia
7. Ventriculostomy
8. Surgery
HCP Management
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• Bedside Procedure– 15 minutes to place
• Rapid reduction in ICP
• Can directly measure ICP and treat by CSF drainage
Caveats:
• 30% hemorrhage rate (CT)
• 4% daily infection rate
• “Blind procedure”
• Can be dislodged– (patient / nurse / tech / family /
pet, etc….)
Ventriculostomy
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• NOT first line for Acute Hydrocephalus!
• acetazolamide (Diamox)– Carbonic Anhydrase inhibitor
– Temporary effect (equilibrates in 3-4 days)
– Can cause respiratory failure at high doses
• Mannitol– Sugar Acid
– Rapid decrease in ICP
– Lasts 3-4 hours
• Volatile Anesthetics– Halothane
– Isoflurane
• Vasopressin / Angiotensin II
Medical Management
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Internal CSF Diversion
• Ventriculoperitoneal Shunt (VP Shunt)
• Ventriculopleural Shunt (VPL Shunt)
• Ventriculoatrial Shunt (VA Shunt)
Internal CSF Bypass
• Endoscopic Third Ventriculostomy (ETV)– Bypass created in floor of 3rd Ventricle
• Torkalson Shunt– Implanted bypass between Lateral ventricle and
4th Ventricle
– Not frequently used
Surgery for HCP
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VP Shunt
• Most common type in adults
• Peritoneum can absorb liters of fluid daily
• (Total daily production of CSF ~500cc)
• Valve separates ventricular catheter and peritoneal catheter
– Fixed or Programmable
Shunts
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Ventriculopleural Shunt
• More common type in children
• Pleural space more limited
• Associated with pleural effusions
Shunts
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Ventriculoatrial Shunt
• “Backup” site if other sites fail
• No limit to CSF absorption
• Helpful for difficult to drain patients (low pressure hydrocephalus)
Shunts
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Lumboperitoneal Shunt
• Specific indications:
• Pseudotumor Cerebri
• Chronic CSF rhinorrhea– Post-traumatic
– Iatrogenic
• High Failure Rate
Shunts
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Shunts
VP Shunt VA Shunt
Pros Easier to place Avoids Abdomen
Less chance of overdrainageLow Resistance (negative pressure)
Easily Convertible
Cons Abdominal organ injury More difficult to place
Peritonitis necessitates removal Overdrainage a concern
Higher back-pressure (10mmHg)Risk of stroke during placement
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Shunt Valves
Fixed
• Distal Slit
• Fixed pressure intervening valve
Programmable
• Large Range
• High Resolution
• High Pressure
MRI-Lock
• Resistant to MRI reprogramming
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Shunt Valves
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Shunt Woes
• Hydrocephalus: Complicated Disorder
• VP Shunt: Complex Hardware (not a simple device)
• Numerous Challenges for Caregivers
1. Failure: ~10% / year (asymptotic curve)– Some reports upwards of 50% failure in 1st two years
– Tube kinks / separations
– Valve obstruction
2. Infection: 1.6-16.7%– Highest risk in 1st month after surgery
– Multiple revisions major risk factor
3. Over- / Under-drainage
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Endoscopic Third
VentriculostomyIdeal for Obstructive Hydrocephalus
• Requires relative pressure gradient for patency
• Auto-equilibrates to prevent overdrainagecomplications
• No hardware in brain
• Can still have failure rate (~20%)– Missed in ED as patients are unaware or unconscious and
caregiver does not know to evaluate for HCP
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Endoscopic Third
Ventriculostomy
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Endoscopic Colloid
Cyst Resection
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Shunt v ETV
Shunt ETV
Pros More Common Procedure No Hardware
Adjustable Pressure / Flow Little risk of OverdrainageDependent on Technology
Cons Failure Rate (10% / year) Failure Rate (2% / year)
Obstructuve HydrocephalusPost-infectious (basilar meningitis) Hydrocephalus
Abdominal Organ Injury (or cardiac valve injury) Basilar Tip Aneurysm
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Normal Pressure
HydrocephalusClinical Triad:
1. Magnetic (shuffling) Gait
2. Urinary Incontinence
3. Dementia
… In the setting of enlarged ventricles
• One of very few Treatable causes of dementia!
• Prevalence: 21.9/100,000 patients
• 3.3/100,000 (50-59yrs)
• 49.3/100,000 (60-69yrs)
• 181.7/100,000 (70-79yrs)
• 1% of all dementia patients
Leg and urination Control
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Normal Pressure
HydrocephalusDiagnosis:
1. Clinical:– Assess Gait, speed and quality
– Assess memory
2. Radiographic:– Evan’s Index >0.3
Dynamic Testing
1. High Volume Lumbar Puncture– 20-40ml (depending on patient)
2. Lumbar Drain Trial– Hourly drainage of 10-15ml CSF
– Over 3 days (in ICU)
– Daily assessments of MMSE and PT
– 84% of responders had successful VP Shunt outcomes (90% PPV)– Marmarou et.al. JNS 2005
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Normal Pressure
HydrocephalusAfter Surgery…• Generally well tolerated operation with low major
risks
• Some studies suggest upwards of 38% overall complication rate
• Need to monitor patients closely and identify target drainage pressure
• Avoid aggressive adjustments to prevent subdural hematomas
• Incremental and regimented adjustment of VP Shunt settings helpful for objective outcomes assessment
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Normal Pressure
HydrocephalusAfter Surgery…• Generally well tolerated operation with low major
risks
• Some studies suggest upwards of 38% overall complication rate
• Need to monitor patients closely and identify target drainage pressure
• Avoid aggressive adjustments to prevent subdural hematomas
• Incremental and regimented adjustment of VP Shunt settings helpful for objective outcomes assessment
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Future Directions
VP Shunts are artificial solutions to underlying pathology
• Overproduction of CSF
• Underabsorption of CSF
• Obstruction of CSF flow
Ideal goal – eliminate need for VP Shunt altogether…!
• Choroid Plexus Coagulation (CPC)
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Future Directions
• CPC + ETV 66% efficacious v ETV alone (47%)
• Most etiology demonstrates improved outcomes
• Other studies showed positive results in older children
• Potentially can apply to adults…
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Thank You
www.brain-tumor.orgJohn Wayne Cancer Institute