Hydrocephalus I

7/29/2019 Hydrocephalus I

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Newborn Hydrocephalus

John Strugar M.D.

Yale University


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y

Topics

Epidemiology

Physiology

PathogenesisClinical Features

Prognosis

Management

Untreated Hydrocephalus: case presentations

Economic Factors


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Epidemiology

Swedish population based studies:

Incidence rose from 0.48/1,000 live births in1967-70 to 0.81/1000 live births in 1979-82

Reflection of premature IVH survival

CSF shunt prevalence in U.S. about twice the

incidence at 1.2/1000 children (about125,000 in the US)


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Physiology

CSF production by the choroid plexus

Rate of CSF production:

Adults ~ 20cc/hrInfants ~ variable with age and weight

Total CSF volume:

Adults ~150 ccInfants ~ 50 cc


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CSF Flow


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Pathogenesis of Hydrocephalus

Imbalance between CSFproduction and absorption:

1. obstruction of CSF pathways(OBSTRUCTIVE/NON-COMMUNICATING)

2. impaired venous absorption

(COMMUNICATING)3. oversecretion of CSF (choroid

plexus papilloma-rare)

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Obstructive/ Non-CommunicatingHydrocephalus

OBSTRUCTION

CSF ABSORPTION

CSF VOLUME

VENTRICULAR PRESSURE

VENTRICULAR DILATATION


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Communicating Hydrocephalus

impaired venousabsorption

typically due toinflammation orplugging of thesubarachnoid villi

dilation of entireventricular system


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Etiology of Hydrocephalus

Congenital

Neural tube defects

Isolated

X-linked

CNS malformations(Dandy Walker,aqueductal stenosis)

Syndromic (Cruzons,Pfeiffer)

Intrauterine infection

Acquired

CNS infections

TumorsSubarachnoidhemorrhage

Intraventricular

hemorrhage


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Genetics of Hydrocephalus

43 mutants/locilinked to hereditaryhydrocephalus havebeen identified inanimal models andhumans

9 genes identified inanimal modes

1 gene in humans

Genes identifiedcode for cytokines,growth factors andcellular signalpathways duringearly braindevelopment

Pathways not wellunderstood inhumans


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Sequential Apoptosis inArhinencephalic Mutant Mice

Neonatal Pdn/Pdn mice exhibit:

Absence of corpus callosum

Absence of anterior commissureNo olfactory bulbs

Abnormal gyri formation on cerebral hemisphere

Hydrocephalus

Abnormal apoptosis of developing fetal brainmay be lead to a subset of hydrocephalicoffspring


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Posthemorrhagichydrocephalus

Premature infants:Walls of vessels lack

mature elementsSupply blood torapidly dividinggerminal matrix

Hemodynamic surges

pose highest risk infirst 72 hours of life

Papiles grading criteria:

Grade 1: bleed ingerminal matrix w/oextension

Grade 2: IVH up to 50%of ventricle w/o dilitation

Grade 3: IVH greaterthan 50% of ventriclewith dilitation

Grade 4:

intraparenchymal

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Posthemorrhagichydrocephalus

Grade 1

Grade 2

Grade 3

Grade 4

3 weeks

later, notecyst.

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Posthemorrhagic hydrocephalus

Among low weight


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Clinical Features in NewbornHydrocephalus

Signs and symptoms result from increasedICP and dilatation of the ventricles

Vomiting and poor PO intake

Changes in behavior, irritability (headache)

Lethargy and drowsiness

Loss of milestones


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Physical Findings

Cushings triad (HR, BP, irregularRR)

Excessive head growth

Full or distended anteriorfontanelle

Frontal bossing

dilated and prominent scalp veins

3rd and 6th CN compression-diplopia

Papilledema

Spasticity of extremities

Perinaud syndrome


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Perinaud SyndromeVertical gaze abnormalities

Downward gaze preference ("setting sun sign")

Primary position upbeat or downbeat nystagmus

Impaired convergence and divergence

Excessive convergence tone

Convergence-retraction nystagmus

Skew deviation often with the higher eye on the side ofthe lesion

Alternating adduction hypertropia or alternating

adduction hypotropiaBilateral upper eyelid retraction (Collier's "tucked lid"sign)

Bilateral ptosis

Pupillary abnormalities

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Prognosis

Distinguish outcome of IVH from HCFor neonates with IVH, grade correlates with

outcomeEstimates (based on combined published data) ofserious deficits:

Grade 1: 5%

Grade 2: 14%Grade 3: 35%

Grade 4: 90%

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Prognosis

IVH and PVL (periventricularleukomalacia) were

independent predictors ofpoor outcome.Grade 4 long term outcome(8-18yrs):

60% mortality78% survivors IQ > 2 S.D.bellowmean

All had spasticity

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Management of Hydrocephalus

Surgical: Shuntone-way valvesystem

VentriculoatrialVentriculopleural

ventriculoperitoneal

Medical

diureticsfibrinolysis

serial lumbarpunctures


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Medical Therapy

Serial lumbarpunctures

Except in cases ofrapid expansion ofventricles

10cc/kg/tap

Diuretics (not as

effective):acetazolamide,furosemide

Fibrinolytics: noreduction in shunt

rate noted.Ventricular irrigationfor 72 hours


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Surgical Treatment

Place a temporarysubgaleal reservoir toallow frequent taps,

without going throughthe cortical mantledaily.

Generally done until the

infant weighs at least2,000g.

Clears high protein level

Shunting


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Outcomes

Early 20th century:

Monroe and Magendie described the CSF pathways

Dandy and Blackfan (1914): understanding of the underlyingpathophysiology of hydrocephalus.

Early surgical treatments had published mortality of 45-53%.

1956 introduction of the Holter valve

Overall mortality rate dropped to less than 20% at 10 yearsInfection is the primary cause of mortality

5 year mortality in modern series are 2-4%


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OutcomesUntreatedHydrocephalus

Survival is POOR!~ 50% of diebefore age 3

~ 80% die before

reaching 10 yearsof age

TreatedHydrocephalus

Markedly improvedoutcome

90-95% survival


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Outcomes: treated hydrocephalus

Published series dependent outcomes:50% of patients have IQ above 80

More severely patients die, thus data is favorable

20% of patients have IQ between 60-8030% of patients have IQ bellow 60

50% experience normal schooling

In shunted patients verbal cognitive skills are betterthan non-verbal

Half the children have behavioral disorders(especially in those with IQ


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Outcome variables

Ventricular dilatation isonly marginally realatedto lower IQ

Site of obstruction doesnot influence outcome

50-65% of aqueductalstenosis pateints arenormal at 10 years

28% presented withhypothalamic-pituitarydysfunction

Prognosis of congenitalHC is worse thanpostnatal HC

Prognosis of HC withmyelomeningocele isbetter than otheretiologies


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Prognosis and timing of surgery

When surgeryperformed before 6

months of age:60% have IQ>80.

Number of revisionsnot determinant of

prognosis

When surgery isperformed after 2 yearsof age:

29% have IQ>80

Aqueductal stenosispatients may presentlater in life

Shunt=3rd

ventriulostomy results,although vent size islarger in latter.


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Prognosis

Prognosis is based on past observations,and thus is a probability not a certainty.


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Shunting

Third Ventriculostomy

Indicated in aqueductal stenosis

Healthy appearing cerebral cortex

on MRIEnlarged frontal horns and 3rdvent.: access to floor of ventricle.

VentricularShunting: all other

patients


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Shunting

Proximal: ventricular

Anti-siphon,

unidirectional flowvalves

Pressure control

Variable pressure

valves


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Shunting

Failure Rate:First 2 years: up to 50%

Causes:

Obstruction in up to 31% of shuntsInfection: 8%

Overdrainage: 3.5%

Loculations: 6%

Position of ventricular cath related to failure ratesOccipital horn has hazard ration of 0.45 compared to tip found inbody of ventricle.

Cath tips surrounded by CSF (vs. slit vents) have a hazzard ratio of0.21

Meningomyelocele increase risk of failure


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Shunting

In shunted patients ventricular size decreases forup to one year, then stabilizes, regardless of

shunt type (pressure or volume):


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Untreated Hydrocephalus

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Does not denote arrestedhydrocephalus

Includes all causes of hydrocephalus

Communicating and non-communicating

Exceedingly rare

Developed countries: shunting

mandatoryUndeveloped countries: lack ofancillary care leads to early death

Developing countries: supportive

care facilitates prolonged survival


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Neurologically:

Brainstem function maintained

Marked sundowning effectPressure on superior colliculi

Increased tone

Cortical blindness

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Life limiting complication: Scalp breakdown


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Scalp ulceration: pathophysiology

Sustained prolongedpressure

Loss of pain sensibility orinability to move

Friction: sustained forceapplied at angle

Microthrombi/cold spot


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Scalp ulceration

Microvascular trauma can occur at pressures of100-500 mm Hg, inducing multiple

microthrombi, anoxia and cellular death.Ulcers may thus develop 2-4 days afterpressure isrelieved as the prolonged ischemia of the encirclingthrombi causes necrosis.


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Skin ulcers:prevention

NutritionAbsorption and other metabolic demands of organism areimportant co-factors.

Fever increases metabolic demand

Anticoagulants and antiplatelet agentsMovement

Recurrent reactive hyperremia will induce callus and thusresistance to ulceration

Turning is the traditional method and can be on aschedule (2-4 hours) or as needed.

Patient supports to distribute weight evenly


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Skin ulcers treatment

Debridment of necrotictissue

Moist dressings, occlusivedressing.

In failing skin: one can put

anything on the ulcerexcept the patient.


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Untreated Hydrocephalus: Case Report

Case L.I. (D.O.B. 18.12.05):

6.04.06: OFC=46.5cm, total wt.=4000g,

scalp erythema20.09.06: OFC=71.5cm, ulcer grade IIIwith necrosis


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Case Report

L.I.

At OFC=46.5cm:

Volume: 4/3r3 = 1,698cc or approx 1.7kg

Surface Area= 688 cm2

At OFC=71.5cm

Volume: 6,173cc or approx. 6.2kg

Surface Area= 1,627 cm2


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Case C.M. (D.O.B. 31.03.06):

08.01.07: OFC=80cm, scalp errythema,

weight 15kg14.05.07: OFC-86cm, scalp grade IIulceration


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C.M.

At OFC=80cm:

Volume: 4/3r3 = 8,646ccSurface Area= 2,037 cm2

At OFC=86cm

Volume: 10,741ccSurface Area= 2,354 cm2

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Estimated scalppressures: L.I.

Assumption that most of the weightdistributed across a 6cm diameter (surfacearea-28.26cm2)

Initial pressure: weight of head=1,700gAverage constant pressure= 44mmHg

Final calculated pressure: weight of head 6,000g

Average constant pressure= 156mmHg


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Estimated scalp pressures: C.M.

Assumption that most of the weight distributed across a 6cmdiameter (surface area-28.26cm2)

Initial pressure: weight of head= 8,646g

Average constant pressure= 227mmHgFinal calculated pressure at weight of head= 10,741

Average constant pressure= 282mmHg


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Scalp pressure

Scalp pressures at timeof fulminant ulceration:

L.I.= 156 mmHgC.M.= 282 mmHg

Pressures are above

possible systolic BP.


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Untreated hydrocephalus

Reported case of brain rupture(extrusion of brain and CSFthrough scalp):

Results from marked tensionhydrocephalus

Enlargement of vents occur atfrontal and occipital horns

Spontaneous ventriculostomy,usually through parieto-occipitalregion

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Hydrocephalus and Economic Development

Romania and the treatment of complexmedical diseases

Requires both professional expertise and

technology


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Hydrocephalus and EconomicDevelopment

Major disadvantage of beingin the Developing or

Adaptor column of nationsis that it is relatively MOREexpensive on a macro-

economic level to provide themedical technology.


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Multumesc!

Hydrocephalus I

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