Newborn Hydrocephalus

John Strugar M.D. Yale University

Topics
Epidemiology Physiology Pathogenesis Clinical Features Prognosis Management Untreated Hydrocephalus: case presentations Economic Factors

Epidemiology
Swedish population based studies:
Incidence rose from 0.48/1,000 live births in 1967-70 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 (about 125,000 in the US)

Physiology
CSF production by the choroid plexus Rate of CSF production: Adults ~ 20cc/hr Infants ~ variable with age and weight Total CSF volume:
Adults ~150 cc Infants ~ 50 cc

CSF Flow

Pathogenesis of Hydrocephalus
Imbalance between CSF production and absorption: 1. obstruction of CSF pathways (OBSTRUCTIVE/NONCOMMUNICATING) 2. impaired venous absorption (COMMUNICATING) 3. oversecretion of CSF (choroid plexus papilloma-rare)
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Obstructive/ Non-Communicating Hydrocephalus

OBSTRUCTION
CSF ABSORPTION CSF VOLUME VENTRICULAR PRESSURE

VENTRICULAR DILATATION

Communicating Hydrocephalus
impaired venous absorption typically due to inflammation or plugging of the subarachnoid villi dilation of entire ventricular system

Etiology of Hydrocephalus
Congenital
Neural tube defects Isolated X-linked CNS malformations (Dandy Walker, aqueductal stenosis) Syndromic (Cruzons, Pfeiffer) Intrauterine infection

Acquired
CNS infections Tumors Subarachnoid hemorrhage Intraventricular hemorrhage

Genetics of Hydrocephalus
43 mutants/loci linked to hereditary hydrocephalus have been identified in animal models and humans 9 genes identified in animal modes 1 gene in humans Genes identified code for cytokines, growth factors and cellular signal pathways during early brain development Pathways not well understood in humans

Sequential Apoptosis in Arhinencephalic Mutant Mice

Neonatal Pdn/Pdn mice exhibit:
Absence of corpus callosum Absence of anterior commissure No olfactory bulbs Abnormal gyri formation on cerebral hemisphere Hydrocephalus

Abnormal apoptosis of developing fetal brain may be lead to a subset of hydrocephalic offspring

Posthemorrhagic hydrocephalus
Premature infants:
Walls of vessels lack mature elements Supply blood to rapidly dividing germinal matrix Hemodynamic surges pose highest risk in first 72 hours of life

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Papiles grading criteria: Grade 1: bleed in germinal matrix w/o extension Grade 2: IVH up to 50% of ventricle w/o dilitation Grade 3: IVH greater than 50% of ventricle with dilitation Grade 4: intraparenchymal

Posthemorrhagic hydrocephalus
Grade 1 Grade 4

Grade 2

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Grade 3

3 weeks later, note cyst.

Posthemorrhagic hydrocephalus
Among low weight <1,500g, incidence of IVH is 22%. PVD seen in:
Grade Grade Grade Grade 1: 2: 3: 4: 4% 11% 76% 71%

One large study (Murphy, mean gestation 26.8 weeks), incidence of posthemorrhagic vent dilitation: 25%.
38% 48% 18% 34% arrested spont. treated w/o sx. died shunted

Clinical Features in Newborn Hydrocephalus

Signs and symptoms result from increased ICP and dilatation of the ventricles
Vomiting and poor PO intake Changes in behavior, irritability (headache) Lethargy and drowsiness Loss of milestones

Physical Findings
Cushings triad (HR, BP, irregular RR) Excessive head growth Full or distended anterior fontanelle Frontal bossing dilated and prominent scalp veins 3rd and 6th CN compressiondiplopia Papilledema Spasticity of extremities Perinaud syndrome

Perinaud Syndrome

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Vertical 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 of the lesion Alternating adduction hypertropia or alternating adduction hypotropia Bilateral upper eyelid retraction (Collier's "tucked lid" sign) Bilateral ptosis Pupillary abnormalities

Prognosis
Distinguish outcome of IVH from HC
For neonates with IVH, grade correlates with outcome Estimates (based on combined published data) of serious deficits:
Grade Grade Grade Grade 1: 2: 3: 4: 5% 14% 35% 90%
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Prognosis
IVH and PVL (periventricular leukomalacia) were independent predictors of poor outcome.
Grade 4 long term outcome (8-18yrs):
60% mortality 78% survivors IQ > 2 S.D. bellow mean All had spasticity
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Management of Hydrocephalus
Surgical: Shunt
one-way valve system Ventriculoatrial Ventriculopleural ventriculoperitoneal

Medical
diuretics fibrinolysis serial lumbar punctures

Medical Therapy
Serial lumbar punctures
Except in cases of rapid expansion of ventricles 10cc/kg/tap

Fibrinolytics: no reduction in shunt rate noted. Ventricular irrigation for 72 hours

Diuretics (not as effective): acetazolamide, furosemide

Surgical Treatment
Place a temporary subgaleal reservoir to allow frequent taps, without going through the cortical mantle daily.
Generally done until the infant weighs at least 2,000g. Clears high protein level

Shunting

Outcomes
Early 20th century:
Monroe and Magendie described the CSF pathways Dandy and Blackfan (1914): understanding of the underlying pathophysiology 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 years
Infection is the primary cause of mortality 5 year mortality in modern series are 2-4%

Outcomes
Untreated Hydrocephalus
Survival is POOR! ~ 50% of die before age 3 ~ 80% die before reaching 10 years of age

Treated Hydrocephalus
Markedly improved outcome 90-95% survival

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 60-80 30% of patients have IQ bellow 60

50% experience normal schooling In shunted patients verbal cognitive skills are better than non-verbal Half the children have behavioral disorders (especially in those with IQ<60 40% of severely disabled patients have epilepsy

Outcome variables
Ventricular dilatation is only marginally realated to lower IQ Site of obstruction does not influence outcome
50-65% of aqueductal stenosis pateints are normal at 10 years
28% presented with hypothalamic-pituitary dysfunction

Prognosis of congenital HC is worse than postnatal HC Prognosis of HC with myelomeningocele is better than other etiologies

Prognosis and timing of surgery

When surgery performed before 6 months of age:
60% have IQ>80.

When surgery is performed after 2 years of age:

29% have IQ>80 Aqueductal stenosis patients may present later in life
Shunt=3rd ventriulostomy results, although vent size is larger in latter.

Number of revisions not determinant of prognosis

Prognosis

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

Shunting
Third Ventriculostomy
Indicated in aqueductal stenosis Healthy appearing cerebral cortex on MRI Enlarged frontal horns and 3rd vent.: access to floor of ventricle.

Ventricular Shunting: all other patients

Shunting
Proximal: ventricular Anti-siphon, unidirectional flow valves Pressure control Variable pressure valves

Shunting
Failure Rate:
First 2 years: up to 50% Causes:
Obstruction in up to 31% of shunts Infection: 8% Overdrainage: 3.5% Loculations: 6%

Position of ventricular cath related to failure rates

Occipital horn has hazard ration of 0.45 compared to tip found in body of ventricle. Cath tips surrounded by CSF (vs. slit vents) have a hazzard ratio of 0.21

Meningomyelocele increase risk of failure

Shunting
In shunted patients ventricular size decreases for up to one year, then stabilizes, regardless of shunt type (pressure or volume):

Untreated Hydrocephalus

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Untreated Hydrocephalus
Does not denote arrested hydrocephalus Includes all causes of hydrocephalus Communicating and noncommunicating Exceedingly rare Developed countries: shunting mandatory Undeveloped countries: lack of ancillary care leads to early death Developing countries: supportive care facilitates prolonged survival

Untreated Hydrocephalus
Marked tension hydrocephalus Open fontanelles Open sutures Expanding skull: OFCs can reach beyond 90cm Retarded body growth

Untreated Hydrocephalus
Neurologically:
Brainstem function maintained Marked sundowning effect
Pressure on superior colliculi

Increased tone Cortical blindness

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Untreated Hydrocephalus
Life limiting complication: Scalp breakdown

Scalp ulceration: pathophysiology

Sustained prolonged pressure Loss of pain sensibility or inability to move Friction: sustained force applied at angle Microthrombi/cold spot

Scalp ulceration
Microvascular trauma can occur at pressures of 100-500 mm Hg, inducing multiple microthrombi, anoxia and cellular death.
Ulcers may thus develop 2-4 days after pressure is relieved as the prolonged ischemia of the encircling thrombi causes necrosis.

Skin ulcers: prevention

Nutrition
Absorption and other metabolic demands of organism are important co-factors. Fever increases metabolic demand

Anticoagulants and antiplatelet agents Movement

Recurrent reactive hyperremia will induce callus and thus resistance to ulceration

Turning is the traditional method and can be on a schedule (2-4 hours) or as needed. Patient supports to distribute weight evenly

Skin ulcers treatment

Debridment of necrotic tissue Moist dressings, occlusive dressing. In failing skin: one can put anything on the ulcer except the patient.

Untreated Hydrocephalus: Case Report

Case L.I. (D.O.B. 18.12.05):
6.04.06: OFC=46.5cm, total wt.=4000g, scalp erythema 20.09.06: OFC=71.5cm, ulcer grade III with necrosis

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

Untreated Hydrocephalus: Case Report

Case C.M. (D.O.B. 31.03.06):
08.01.07: OFC=80cm, scalp errythema, weight 15kg 14.05.07: OFC-86cm, scalp grade II ulceration

Untreated Hydrocephalus: Case Report

C.M. At OFC=80cm:
Volume: 4/3r3 = 8,646cc Surface Area= 2,037 cm2

At OFC=86cm
Volume: 10,741cc Surface Area= 2,354 cm2

Estimated scalp pressures: L.I.

Assumption that most of the weight distributed across a 6cm diameter (surface area-28.26cm2)
Initial pressure: weight of head=1,700g
Average constant pressure= 44mmHg

Final calculated pressure: weight of head 6,000g

Average constant pressure= 156mmHg

Estimated scalp pressures: C.M.

Assumption that most of the weight distributed across a 6cm diameter (surface area-28.26cm2)
Initial pressure: weight of head= 8,646g
Average constant pressure= 227mmHg

Final calculated pressure at weight of head= 10,741

Average constant pressure= 282mmHg

Scalp pressure
Scalp pressures at time of fulminant ulceration:
L.I.= 156 mmHg C.M.= 282 mmHg

Pressures are above possible systolic BP.

Untreated hydrocephalus
Reported case of brain rupture (extrusion of brain and CSF through scalp):
Results from marked tension hydrocephalus Enlargement of vents occur at frontal and occipital horns Spontaneous ventriculostomy, usually through parieto-occipital region
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Hydrocephalus and Economic Development

Romania and the treatment of complex medical diseases
Requires both professional expertise and technology

Hydrocephalus and Economic Development

Major disadvantage of being in the Developing or Adaptor column of nations is that it is relatively MORE expensive on a macroeconomic level to provide the medical technology.

Multumesc!