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Jeff Edwards

Gillian Lieberman, MD

Hydrocephalus in Children:Hydrocephalus in Children:

Diagnostic Imaging andDiagnostic Imaging andRadiological CharacteristicsRadiological Characteristics

January 2002

Jeff Edwards, Harvard Medical School, Year III



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AgendaAgenda

Introduction to the Patient

Macrocephaly

Basics of Hydrocephalus

Diagnostic Imaging of Hydrocelphalus

Ultrasound, CT, MR

Shunts


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The PatientThe Patient

6 yo boy with macrocephaly, whichmanifested in the first year of life asexcessive head growth, accompaniedby reduced activity and poor feeding.

Other significant medical hx includes

cognitive delay, osteogenesisimperfecta type 2, restrictive lungdisease.

Birth hx: born at 34 wk premature byc-section with subsequent 25 day NICUcourse significant for apneic andbradycardic spells

No family hx of large headsUsed with permission of

patients mother


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MacrocephalyMacrocephaly in Infant or Childin Infant or Child

Defined as a head circumference more than 2 SDabove the mean for age and sex

Excessive rate of head growth over time suggests

increased intracranial pressure Most often caused by hydrocephalus, extra-axial fluid

collections, or neoplasms

Macrocephaly with normal head growth rate

suggests familial macrocephaly or true

megalencephaly


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DDxDDx ofof MacrocephalyMacrocephaly

Causes ExamplesPseudomacrocephaly,

pseudohydrocephalus, catch-up growth

Growing premature infant, recovery

from malnutrition, congenital heart

disease

Increased intracranial pressure

with dilated ventricles

with other mass

Progressive hydrocephalus, subduraleffusion, hydrancephaly

Arachnoid cyst, porencephalic cyst,brain tumor

Benign familial macrocephaly

(idiopathic external hydrocephalus)

Benign enlargement of subarachnoidspaces, congenital communicatinghydrocephalus, benign subduralcollections of infancy


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DDxDDx ofof MacrocephalyMacrocephaly (cont(contd)d)

Megalencephaly

with neurocutaneous disorder

with gigantism

with dwarfism

metabolic

lysosomal

other leukodystrophy

Benign familial (see above)

Neurofibromatosis, tuberous sclerosis

Soto syndrome

Achondroplasia

Mucopolysaccharidoses, Krabbesdisease, Ganglioside storage disease

Metachromatic leukodystrophy

Canavan spongy degeneration

Thickened skull Fibrous dysplasia (bone), hemolyticanemia (marrow), sicklemia,thalasemia

Modified from Hay, WW, et al. Current Pediatric Diagnosis & Treatment.

15th ed. New York: Lange Medical Books/McGraw Hill, 2001, p. 669.


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Workup ofWorkup of MacrocephalyMacrocephaly

History including family medical hx

Physical exam Neurological exam

Plotting measurement of head circumference for age and sex

Palpation of anterior fontanelle (if not closed) and of head forasymmetries or ridges

Listen for bruits in neck and head

In infants, transillumination of skull in a darkened room may reveal

subdural effusions, hydrocephalus, hydranencephaly, or cystic defect

Imaging studies

Depending on hx, possible labs include toxoplasmosis serum

antibody levels, lumbar puncture, subdural tap


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NarrowedNarrowed DDxDDx for our Patientfor our Patient

Hydrocephalus

Space-occupying lesion (e.g., tumor or cyst)

Extra-axial fluid collection (e.g., subduraleffusion)

Growing premature infant


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Pathophysiology of HydrocephalusPathophysiology of Hydrocephalus

Imbalance of CSF formation andabsorption, resulting in an excess of CSFwith subsequent increase in intracranial

pressure

CSF basics

Normal CSF production 02.-035 mL/min with amajority produced by the choroid plexus

Total volume of CSF in an adult ~ 120 mL


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CSF Circulation & Ventricular SystemCSF Circulation & Ventricular System

From Nolte, J. The Human Brain: An Introduction to ItsFunctional Anatomy. 4th ed. St. Louis: Mosby, 1999.


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EpidemiologyEpidemiology

Incidence of congenital hydrocephalus is 2-3 per 1,000 live births

Incidence of acquired hydrocephalus is not

known

About 100,000 shunts are implanted each

year in the developed countries

Incidence is equal in males and females


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Symptoms of Hydrocephalus inSymptoms of Hydrocephalus inInfantsInfants

Poor feeding

Irritability

Reduced activity

Vomiting

J ff Ed d


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Signs in InfantsSigns in Infants

Macrocephaly with excessive rate of head growth

Dysjunction of sutures

Dilated scalp veins

Tense/bulging fontanelle

Setting-sun sign: Eyes are deviated downward, theupper lids are retracted, and superior sclerae may

be visible.

Lower limb spasticity and hypertonia

Papilledema often not present in infants

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Symptoms in ChildrenSymptoms in Children

Altered behavior

Slowing of mental capacity or decreased level ofconsciousness

Headaches (initially in AM)

Neck pain (2 to tonsilalar herniation)

Vomiting (worse in AM)

Blurred vision (2 to papilledema)

Double vision (2 to Abducens nerve palsy)

Stunted growth and sexual maturation from third ventricledilatation obesity, precocious or delayed onset ofpuberty

Difficulty in walking 2 to spasticity

J ff Ed d


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Signs in ChildrenSigns in Children

Papilledema optic atrophy and vision loss (if increasedintracranial pressure (ICP) goes untreated)

Failure of upward gaze (2 to pressure on tectal plate through thesuprapineal recess)

Macewen sign: A "cracked pot" sound is noted onpercussion of the head.

Unsteady gait (2 to spasticity in lower extremities)

Macrocephaly (sutures are closed, but chronic ICP will lead toprogressive abnormal head growth)

Uni- or bilateral sixth nerve palsy

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Classifications of HydrocephalusClassifications of Hydrocephalus

Congenital v. Acquired

Communicating v. Noncommunicating

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Congenital HydrocephalusCongenital Hydrocephalus

Most common category in children

Usually present during infancy

Hydrocephalus presenting after age 6 months is

less likely to be congenital, and neoplasm mustbe excluded

Jeff Edwards


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Congenital CausesCongenital Causes

Aqueduct (of Sylvius) stenosis

due to malformation (10% of all cases in newborns)

Arnold-Chiari I &II, Vein of Galen, Klippel-Feil

Postinfectious: toxoplasmosis, cytomegalic inclusiondisease, rubella, syphilis

Obstruction of foramina of Luschka and Magendie

Dandy-Walker malformation (2-4% of newborns)

Agenesis of foramen of Monro

Bickers-Adams syndrome

Achondroplasia

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Acquired CausesAcquired Causes

Mass lesions

account for 20% of all cases in children

usually tumors (eg, medulloblastoma, astrocytoma), but cysts, abscesses,or hematoma also cause

Intraventricular hemorrhage

2 to prematurity, head injury, or rupture of a vascular malformation.

Infections Meningitis, especially bacterial, Mumps, cysticercosis

Increased venous sinus pressure

2 to achondroplasia, craniostenoses, or venous thrombosis.

Iatrogenic Hypervitaminosis A.

Idiopathic

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Communicating HydrocephalusCommunicating Hydrocephalus

Overproduction of CSF (rare)

Choroid plexus papillomas (more common of the two)

Diffuse villous hyperplasia of choroid plexus

Extraventricular (not between ventricles andsubarachnoid space) obstruction by tumor,hemorrhage, infection, vascular abnormality, orstructural abnormality

Possible sites: cerebellar subarachnoid space, basalcisterns, tentorial hiatus (Chiari malformation,achondroplasia) cerebral subarachnoid space

Jeff Edwards


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NoncommunicatingNoncommunicating HydrocephalusHydrocephalus

An intraventricular obstruction by tumor, hemorrhage,

infection, vascular abnormality, or structural abnormality

Most common cause is aqueductal stenosis, often in

association with Chiari II

Common sites of obstruction

Lateral ventricle

Foramina of Monro Third ventricle

Aqueduct

Fourth Ventricle

Jeff Edwards


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Fetal Diagnosis ofFetal Diagnosis of VentriculomegalyVentriculomegaly

Primarily by obstetric ultrasound

Defined as an atrium of a lateral ventricle largerthan 11 mm

However, ultrasound cannot confirm whether

ventriculomegaly is result of hyrdocephalus or loss ofperiventricular brain tissue in which the vacant space is

passively filled with CSF

May be detected as early as latter part of 1sttrimester. Around 20-24 weeks, abnormal dilation

of ventricles is more clearly detectable.

Jeff Edwards


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Postnatal DiagnosisPostnatal Diagnosis

Diagnosis of hydrocephalus is made whenthe ventricles are enlarged in the absence ofcerebral atrophy or dysgenesis

Radiological Modalities Plain film

Ultrasound

CT

MR

Jeff Edwards


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Structural characteristics ofStructural characteristics of

hydrocephalushydrocephalus

Dilation of temporal and frontal horns of thelateral ventricles (often first sign)

Enlargement of anterior or posterior

recesses of third ventricle

Narrowing of mamillopontine distance

Narrowing of ventricular angle

Effacement of cortical sulci

= will return to point again

Jeff Edwards


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Hydrocephalus v. Cerebral AtrophyHydrocephalus v. Cerebral Atrophy

Enlargement of temporal horns commensurately

with the bodies of the lateral ventricles is probablymost sensitive and reliable sign in thedifferentiation of hydrocephalus from atrophy

In atrophy, there is less dilatation of the temporal hornsthan the lateral ventricles bodies

If sylvian fissures are enlarged, dilated temporal hornsare not reliable sign

Large or rapidly enlarging head suggestshydrocephalus

Small or diminishing head circumference suggestsatrophy

Jeff Edwards


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Hydrocephalus v. Cerebral AtrophyHydrocephalus v. Cerebral Atrophy

Ventricular Angle (left) tends to be smaller in hydrocephalus

(shown above) than in atrophy. Frontal horn radius (right) tends

to be larger in hydrocephalus than in atrophy.

Courtesy of Dr. Nedda Hobbs and Childrens Hospital Boston Film Library

Jeff Edwards


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Plain Films and HydrocephalusPlain Films and Hydrocephalus

Prior to newer modalities, diagnosis was made byskull films showing:

split sutures

disproportionate craniofacial ratio bulging of the anterior fontanel

erosion of the dorsum sellae

hammered silver appearance of calvarium

Jeff Edwards


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Ultrasound and HydrocephalusUltrasound and Hydrocephalus

If anterior fontanlle is open, intracranial structures including

ventricles, parenchyma, and vessels are readily visualized in thecoronal and sagittal planes.

From Kirks, DR. Practical Pediatric Imaging: Diagnostic

Radiology of Infants and Children. 3rd

ed. Philadelphia:Lippincott-Raven Publishers: 1998, p. 66.

Used to evaluate for

ventricular size,parenchymal and

intraventricular

hemorrhage,

extracerebral fluid

collections, cystic

lesions, and solid

parenchymal masses.

Jeff Edwards


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Normal UltrasoundNormal Ultrasound

Anterior to posterior coronal images of the patient at age 10 days (study

done to look for intraparaenchymal or intraventricular hemorrhage)Courtesy of Dr. Nedda Hobbs and Childrens Hospital, Boston Film Library

Jeff Edwards

illi i b


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Anterior coronal image of patient at

10 days

Superior aspect

of orbits

Frontal lobe

Courtesy of Dr. Nedda Hobbs and Childrens Hospital, Boston Film Library

Jeff Edwards

Gilli Li b MD


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More posterior coronal image of

patient at 10 days

Interhemispheric

fissure

Corpuscallosum Frontal horn of

lateral ventricle

Sylvian

fissure


Jeff Edwards

Gilli Li b MD


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Body of lateralventricle

Corpus

callosum

Sylvianfissure Area of

thalamus and

third ventricle



patient at 10 days

Jeff Edwards

Gilli Li b MD


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Patient at 10 days

Sagittal Right Parasagittal


Jeff Edwards

Gillian Lieberman MD


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Patient at 10 days

Sagittal


Corpus callosum

Third

ventricle

Fourth

ventricle

Thalamus

Pons

Jeff Edwards



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Patient at 10 days

Right Parasagittal


Body of lateralventricle

Jeff Edwards



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Abnormal UltrasoundAbnormal Ultrasound

Anterior to posterior coronal images of

patient at age 14 months


Jeff Edwards



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Anterior coronal image of patient at 14

months

Superior

aspect of orbitDilated

frontal horns

of lateralventricles


Jeff Edwards



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patient at 14 months

Dilated

frontal horns

of lateral

ventricles

Dilated

temporal

horn of

lateralventricle

Dilated

temporal

horn of

lateralventricle


Jeff Edwards



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Dilated

bodies of

lateralventricles Dilated

temporal

horns of

lateralventricles

Dilated

third

ventricle



patient at 14 months

Jeff Edwards



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Patient at 14 months

Sagittal Right Parasagittal Left Parasagittal


Jeff Edwards



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Sagittal


Dilated

thirdventricle

Dilated body of

lateral ventricle

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Right Parasagittal


Dilated

body of

lateral

ventricle

Dilated frontal

horn of lateral

ventricle

Dilatedtemporal

horn of

lateral

ventricle

Jeff Edwards



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Comparison of Ultrasound SectionsComparison of Ultrasound Sections

Coronal images of patient at 10 days

Coronal images of patient at 14 months

Jeff Edwards



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Comparison of Ultrasound SectionsComparison of Ultrasound Sections

Patient at 14 monthsSagittal Right Parasagittal

Patient at 10 days

Jeff Edwards



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CT/MRI Findings inCT/MRI Findings inAcuteAcute

HydrocephalusHydrocephalus

Temporal horns are preferentially dilated antero-

posterially

Size > 2 mm

Normally, temporal horns are slit-like and barely visible

Ballooning of frontal horns of lateral ventricles and

third ventricle (ie, "Mickey mouse" ventricles)

Periventricular interstitial edema

Sylvian and interhemispheric fissures are not visible

Jeff Edwards



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CT/MRI Findings inCT/MRI Findings inAcuteAcute

Hydrocephalus (contHydrocephalus (contd)d)

Fourth ventricle is usually normal in size

Ratio between largest width of the frontal horns

and the internal diameter from inner-table to

inner-table at this level should be greater than 0.5

Ratio of largest width of frontal horns to maximal

biparietal diameter > 30%

Upward bowing of corpus callosum on sagittal

MRI

Jeff Edwards



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CT/MRI Findings inCT/MRI Findings in ChronicChronic


Temporal horns may be less prominent than inacute hydrocephalus

Third ventricle may herniate into sella turcica,which may be eroded

Corpus callosum may be atrophied (best appreciatedon sagittal MRI)

With long-standing untreated hydrocephalus,white matter will undergo irreversibledemyelination

Jeff Edwards



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PeriventricularPeriventricular Interstitial EdemaInterstitial Edema

Transependymal CSF resorption from ventricularlumen to the parenchyma

On CT, appears as hypodensity in periventricularregion with indistinct ventricular margins

On MR, appears as rim of prolonged T1 or T2relaxation times surrounding lateral venticles

proton density image or a fluid attenuated inversionrecovery (FLAIR) image is much more sensitive

Seen particularly at the superlateral angles of thefrontal horns

Not seen in neonates or young infants (immaturebrain has normally high water content)

Jeff Edwards



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PeriventricularPeriventricular Interstitial EdemaInterstitial Edema

Courtesy of Dr. R. Michael Scott and Childrens Hospital, Boston Film Library

T2-weighted image of 5 yo boy who presented with 3 weeks

of bifrontal headache, morning vomiting, and blurred vision

Jeff Edwards



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Computed Tomography andComputed Tomography and


Pros: widely available, rapid, compatiblewith life support devices, often requires no

patient sedation

Should be performed with and without

contrast

Jeff Edwards



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CTCT



Jeff Edwards



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CTCT



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CTCT



Expansion ofextra-axial

CSF spaces

Brachycephaly

Enlarged frontal

horns + third

ventricle =

Mickey Mouse

ventriclesGray-whitematter junction

is intact

Note: no evidenceof periventricular

interstitial edema

Widened sulci

with overalleffacement of

sulci

Enlarged

temporal horns

Jeff Edwards



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Comparison ofComparison of CTsCTs at Similar Levelsat Similar Levels

of Sectionof Section

PatientNormal adult

Normal from Mori, K. MRI of the Central Nervous System: A Pathology

Atlas. Tokyo: Springer-Verlag, 1991, p. 15.

Jeff Edwards



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CTCT

Patient at 23 months s/p Ventriculoperitoneal

(VP) shunt placement


Flattening of posterior occiput

Jeff Edwards



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Patient 2: MRI andPatient 2: MRI and HyrdocephalusHyrdocephalus

11 yo girl who presented with three days of headache and

nausea/vomiting


Jeff Edwards



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MRIMRI

11 yo girl who presented with three days of headache and

nausea/vomiting

Courtesy of Dr. R. Michael Scott and Childrens Hospital Boston, Film Library

Decompressed left

lateral ventricle

Slight periventricular

edema

Shift of midline

structures to left

Dilated right

lateral ventricle

Jeff Edwards



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MRIMRI

Same 11 yo girl with non-enhancing hyperintense mass

most consistent with colloid cyst


Jeff Edwards



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Comparison of MRIs at SimilarComparison of MRIs at Similar

Levels of SectionLevels of Section

Our 11 yo girlNormal 11 yo girl

Normal from Bisese, JH, Wang, AM. Pediatric Cranial MRI: An Atlas of

Normal Development. New York: Springer-Verlag, 1994, p.94

Jeff EdwardsGillian Lieberman, MD


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MRIMRI

Same 11 yo girl s/p colloid cyst removal one

day prior


Postoperative

edema in left

thalamus

Right

pneumocephalu

and small

subdural

hematoma

causing mass

effect on right

frontal lobe



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Comparison of MRIsComparison of MRIs

11 yo girl with colloid cyst

Before surgery After surgery

Ventricles

are mildly

dilated butimproved

from the

prior study




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Patient 3: MRIPatient 3: MRI

5 yo boy who presented with 3 weeks of bifrontal

headache, morning vomiting, and blurred vision

Stenotic aqueduct




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Comparisons of MRIsComparisons of MRIs

5 yo boywith stenotic

aqueduct

Stenotic aqueduct

Normal adult

Normal from Truwit, CL, Lempert, TE. High Resolution Atlas of Cranial Neuroanatomy.

Baltimore: Williams & Wilkins. 1994, p2



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ShuntsShunts

Principle of shunting is to establish a communicationbetween the CSF (ventricular or lumbar) and a

drainage cavity (peritoneum, right atrium, pleura)

In principle, a shunt is a plastic tube less than 1/8of an inch thick that allows one-directional flow of

CSF by responding to pressure differencesbetween the ventricle and the cavity to which theshunt terminates.

There is a valve system that regulates the flow aswell as a reservoir, which can be felt through theskin. This reservoir allows for sampling of CSF by

needle aspiration.



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ShuntsShunts

CSF is simply absorbed in the drainage

cavity

Ventriculoperitoneal (VP) shunt is themost common

lateral ventricle is the usual proximal

location

advantage is that the need to lengthen the

catheter with growth may be obviated by

using a long peritoneal catheter

Like all foreign bodies, shunts canmalfunction or become infected

Only about 25% of patients with

hydrocephalus are treated successfullywithout shunt placement

From

http://www.cinn.org/conditio

ns/hydrocephalus.html

Diagram of VP and VA

shunts



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Common Shunt ComplicationsCommon Shunt Complications

Ventricular end Blockage

Disconnection

Migration

Hemorrhage

Infection

Isolated or trapped fourth

ventricle Secondary craniosynostosis

Calvarial thickening

Slit ventricle syndrome

Atrial end Thrombosis

Infection

Peritoneal end Infection (peritonitis,

adhesions)

CSF pseudotumor,

encystment



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Patient 4 Shunt SeriesPatient 4 Shunt Series

Plain films used to aid in confirming proper location of shunt

3 yo boy s/p VP shunt placementCourtesy of Dr. Ron Becker and Childrens Hospital, Boston Film Library



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CSF ShuntCSF Shunt

ScintigraphyScintigraphy

In different patient

evaluated for possible

shunt malfunction, there

is normal progression of

tracer down the shunt

catheter, with free

spillage into theperitoneal cavity by 15

min. There is no reflux

into the ventricles.

From Vreeland, TH, Wallis, J. Diagnosis: Normal CSF shunt scintigraphy.

http://gamma.wustl.edu/cs001te117.html

Normal transit time ~ 10-20 min. Transit time > 30 min is abnormal.



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ReferencesReferences

Barkovich, AJ. Pediatric Neuroimaging. 3rd ed. Philadelphia: LippincottWilliams & Wilkins, 2000.

Barr, LL. Neonatal Cranial Ultrasound. Rad Clin N Am 1999; 37: 1127-46.

Bisese, JH, Wang, AM. Pediatric Cranial MRI: An Atlas of NormalDevelopment. New York: Springer-Verlag, 1994.

Chicago Institute of Neurosurgery and Neuroresearch. Hydrocephalus.http://www.cinn.org/conditions/hydrocephalus.html

Goetz. Textbook of Clinical Neurology. 1st ed. W. B. Saunders Company,1999.

Hay, WW, et al. Current Pediatric Diagnosis & Treatment. 15

th

ed. New York:Lange Medical Books/McGraw Hill, 2001.

Heilman, KM, Watson, RT, Greer, M. Handbood for Differential Diagnosis ofNeurologic Signs and Symptoms. New York: Appleton-Century-Crofts, 1977.

Hord, ED. Hydrocephalus. http://www.emedicine.com/neuro/topic161.html



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References (contReferences (cont

d)d)

The Hydrocephalus Association.http://www.hydroassoc.org/information/index.html

Kirks, DR. Practical Pediatric Imaging: Diagnostic Radiology of Infants andChildren. 3rd ed. Philadelphia: Lippincott-Raven Publishers: 1998.

Mori, K. MRI of the Central Nervous System: A Pathology Atlas. Tokyo:Springer-Verlag, 1991.

Nolte, J. The Human Brain: An Introduction to Its Functional Anatomy. 4th

ed.St. Louis: Mosby, 1999.

Rumack, CM, Johnson, ML. Perinatal and Infant Brain Imaging: Role ofUltrasound and Computed Tomography. Chicago: Year Book MedicalPublishers, 1984.

Truwit, CL, Lempert, TE. High Resolution Atlas of Cranial Neuroanatomy.Baltimore: Williams & Wilkins. 1994.

Vreeland, TH, Wallis, J. Diagnosis: Normal CSF shunt scintigraphy.

http://gamma.wustl.edu/cs001te117.html



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AcknowledgementsAcknowledgements


Pamela Lepkowski

Lolita Lewis

Nedda Hobbs, MD

R. Michael Scott, MD

Ron Becker, MD

Daniel Saurborn, MD

Alexandru Bageac, MD

Michael Stella, MD

Larry Barbaras

Cara Lyn Damour

The Patient and his Mom

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