Hydrocephalus and endoscopy

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Maximizing Memory and Improving Ambulation Through

Endoscopic and Traditional Management of Hydrocephalus

Garni Barkhoudarian, MDDaniel Franc, MD, PHD, Sheldon Jordan, MD, PHD

Natasha Cueto, NP, Giselle Tamula, NP, Amy Eisenberg, NP

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Disclosures• Consultant: VTI• Consultant: Cerevasc Inc (data monitoringchair)• 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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Disclosures

at n

erial support from:c

• Consultant: VTI• Anatomy lab receives financial or m

– Karl-Storz Endoskope America, I– Stryker Corporation– Mizuho America, Inc– Surgical West, Inc

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Hydrocephalus

Hydrocephalus: “Water on Brain”• “hīdrōˈsefələs”• Hudrokephalon (greek)• Dysfunctional cerebrospinal fluid

circulation• AKA:

– Hydro– HCP

Hydrocephalus hicksiiTrilobite

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Intracranial 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

•

• R

– 25ml in ventricleste of 0.2-0.7ml/kg/hr– 25-30ml/hr– 600-700ml/day

elies on Na+/K+ ATP dependent pumpand carbonic anhydrase

• Acetazolamide can partially decreaseproduction of CSF– Can cause hypercapnia that can increase

CBF and possibly ICP• Other inhibitory agents:

– Halothane– Angiotensin II– Vasopressin (V1 receptor)

Ra NO OFF SWITCH!

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Hydrocephalus

Obstructive HydrocephalusCommunicating Hydrocephalus

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CSF Dynamics

• Obstructive Hydrocephalus– Aqueductal Stenosis– Cysts (colloid cysts, neuroenteric cysts)– Tumors (cerebellar tumors, brainstem

tumors, pineal tumors, intraventricular tumors)

• Communicating Hydrocephalus– AKA “non-obstructive hydrocephalus”– Infection (meningitis)– Leptomeningeal Carcinomatosis– Hemorrhage (subarachnoid)– Age (normal pressure HCP).

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Hydrocephalus Timing

• Acute Hydrocephalus– Life threatening– Due to hemorrhage, tumor

• Chronic Hydrocephalus– Compensated by brain– Delayed outcome following

• Infection• Hemorrhage• Congenital malformation• Normal Pressure Hydroceph

s

:

alus

Chronic Acute

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Signs and Symptoms:1. Headache2. Nausea/Vomitting3. Blurred Vision / Diplopia4. Focal Neurological Findings

1. Dilated pupil(s)2. Hemiparesis

5. Altered Mental Status1. Confusion2. Lethargy3. Obtundation4. Stupor5. 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 box2. In the setting of a mass

lesion:1. Displaceable fluids will

compensate first2. Elevated intracranial

pressure subsequently occurs

Monroe – Kellie Hypothesis

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Hydrocephalus

13 14 15

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ephalus:ing flat)

Presentation

Unique findings with hydroc• Headaches (worse when ly• Papilledema• Diplopia (Horizontal)• Parinaud’s Syndrome

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Parinaud’s Syndrome

s

Due to lesion compressing “tectal plate”1. Upgaze paralysis2. Argyll-Robertson Pupils

1. (light-near dissociation)3. Convergence-retraction nystagmu

1. With upgaze or opticokinetic drumspinning down

4. Eyelid Retraction (Collier’s Sign)5. Conjugate down-gaze (sun-setting sign)

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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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VentriculoPeritoneal 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

• Neuronavigation for ventricular catheter• Laparascopic Insertion of Peritoneal Catheter

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)• Complications:

– Arrhythmia– Endocarditis– Overdrainage

Shunts

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Lumboperitoneal Shunt• Specific indications:• Pseudotumor Cerebri• Lack of cranial option

– Complex cranial wounds– Small ventricles

• 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 (negativepressure)

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 valveProgrammable• Large Range• High Resolution• High PressureMRI-Lock• Resistant to MRI reprogramming

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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 ThirdVentriculostomy

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Endoscopic ThirdVentriculostomy

Ideal for Obstructive Hydrocephalus• Requires relative pressure gradient for

patency• Auto-equilibrates to prevent

overdrainage complications• 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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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 (basilarmeningitis) Hydrocephalus

Abdominal Organ Injury (or cardiac valve injury) Basilar Tip Aneurysm

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Normal Pressure Hydrocephalus

Clinical Triad:1. Magnetic (shuffling) Gait2. Urinary Incontinence3. Dementia… In the setting of enlarged ventricles

• One of very few Treatable causes ofdementia and ambulatory dysfunction!

• 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– Callosal Angle

Dynamic Testing1. High Volume Lumbar Puncture

– 30-50ml (depending on patient)2. Lumbar Drain Trial

– Hourly drainage of 10-15ml CSF– Over 3 days (in ICU)– Daily assessments of MOCA and PT– 84% of responders had successful VP Shunt

outcomes (90% PPV)– Marmarou et.al. JNS 2005

– Inpatient stay, risk of infection, DVT, PE, ICU psychosis

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JNS 2017

High-volume Lumbar puncture: 30-50mL

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After Surgery…• Generally well tolerated operation with low major

risks• Pooled mean response rate: 59% (Hebb et.al. 2001)• Some studies suggest upwards of 38% overall

complication rate• Repeat surgery rate: 22%• Need to monitor patients closely, systematically

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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65F with Normal Pressure Hydrocephalus and prior history of VP shunt without improvement• Gait Apraxia – unable to

reverse direction• VP shunt converted to VA

shunt

P age: 17 of 34 IM :17 SE:2

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Normal PressureHydrocephalus

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Arrested Hydrocephalus

“Compensated” hydrocephalus• Typically occurs in patients with

obstructive lesions

18F – Straight “A” valedictorian, now at Clemson University

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“Arrested” Hydrocephalus

• Patients can have elevated orfluctuating ICPs

• Can develop delayedneurocognitive decline

JNS 1985

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JNS 1985

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“Arrested” Hydrocephalus• 69F with progressive symptoms:

• Imbalance• Dizziness• Generalized cognitive

“slowing”• DropAttacks

• Thorough systemic and cardiacwork-up negative

• No prior comparison imaging• Neuro exam only demonstrated

tandem gait instability• No papilledema

P a g e : 1 7 o f 3 0 I M :1 7 S E :1 0

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Page: 17 of 30 IM:17 SE:10 Page: 70 of 132 IM:70 SE:17


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Fiber Tractography

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Fiber TractographyWater Imaging• All water molecules are in a

state of motion• In the body, this motion can be

restricted due to surrounding structures

• Fractional anisotrophy measures the ratio of the direction of movement of water molecules (range 0-1)

– Higher FA indicates morelinear structure

• Can “track” lengthy fiber tractswith Diffusion Tensor Imaging

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ctographyFiber Tra

P age: 1 7 of 3 0

IM :1 7 SE :1 0

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Endoscopic Third Ventriculostomy

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Obstructive Hydrocephalus

Page: 17 of 30 IM:18 SE:801

48mm42mm

Improvement of all pre-operative symptoms at 3 months

IM:17 SE:10 Page: 18 of 30

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Obstructive Hydrocephalus

Page: 70 of 132 IM:70 SE:17

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Colloid Cysts• “Congenital” non-neoplastic

cysts arising from Foramen of Monro

• Can cause obstructive hydrocephalus, headaches, cognitive decline, vision disturbances and sudden death• Sudden death 3-10%

• Open surgical or endoscopicresection when indicated

P aaggee:: 8899 ooff118866 IIMM::111188SSEE::660022

P age: 1 2 6 of 2 4 1 IM :126 SE :603

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Endoscopic Colloid Cyst Resection

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Colloid Cysts

JNS 1999

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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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Pacific Adult Hydrocephalus Center

Neurology

Daniel Franc, MD, PHD

Neurosurgery General Surgery

Melanie Goldfarb, MD

www.pacificneuro.org

Sheldon Jordan, MD, PHD Giselle Tamula,ARNP

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http://www.pacificneuro.org/

Hydrocephalus and endoscopy

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