Download - Hydrocephalus I
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Newborn Hydrocephalus
John Strugar M.D.
Yale University
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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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Untreated Hydrocephalus
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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Untreated Hydrocephalus
Neurologically:
Brainstem function maintained
Marked sundowning effectPressure on superior colliculi
Increased tone
Cortical blindness
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Untreated Hydrocephalus
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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Untreated Hydrocephalus: Case Report
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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Untreated Hydrocephalus: Case Report
C.M.
At OFC=80cm:
Volume: 4/3r3 = 8,646ccSurface Area= 2,037 cm2
At OFC=86cm
Volume: 10,741ccSurface Area= 2,354 cm2
E i d l
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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!