Lisa McLeod HMS III

Gillian Lieberman, MD

Use of MRI in Evaluating Use of MRI in Evaluating Fetal Fetal VentriculomegalyVentriculomegaly

Lisa McLeod, Harvard Medical School Year IIILisa McLeod, Harvard Medical School Year IIIGillian Lieberman, MDGillian Lieberman, MD

January 2004

http://bidmc.harvard.edu/content/departments/radiology/files/fetalatlas/default.htm

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Lisa McLeod HMS III

Gillian Lieberman, MD

Objectives:Objectives:

Review Review basic basic fetal CNS development and fetal CNS development and neuroanatomyneuroanatomy

Discuss Discuss DDxDDx of of ventriculomegalyventriculomegaly documented on fetal documented on fetal ultrasoundultrasound

Illustrate the use of fetal MRI in differentiating these diagnosIllustrate the use of fetal MRI in differentiating these diagnoses es and its impact on managementand its impact on management

Identify pros and cons of Ultrasound and MRI for fetal surveyIdentify pros and cons of Ultrasound and MRI for fetal survey

Future directions of use of fetal MRI in diagnosis of etiology oFuture directions of use of fetal MRI in diagnosis of etiology of f ventriculomegalyventriculomegaly

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Lisa McLeod HMS III

Gillian Lieberman, MD

Landmarks of fetal brain Landmarks of fetal brain development visible by MRIdevelopment visible by MRI

GlialGlial Cell MigrationCell MigrationVisible @ 22 weeks GAVisible @ 22 weeks GACells migrate from Cells migrate from ventricular periphery ventricular periphery toward cortical ribbontoward cortical ribbonT2 T2 HypointenseHypointense

SulcationSulcation/Ventricles/VentriclesAgyricAgyric (exc. (exc. SylvianSylvian) until ) until 24 weeks24 weeksPhysioPhysio Hydrocephalus Hydrocephalus resolves from 14 weeks resolves from 14 weeks Both T2 Both T2 HyperintenseHyperintense

Axonal Maturation/Axonal Maturation/MyelinationMyelinationCaudalCaudal--cephalic/Dorsalcephalic/Dorsal--ventralventralT2 T2 HypointenseHypointense

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Lisa McLeod HMS III

Gillian Lieberman, MD

Ventricular CSF Circulation

http://carecure.rutgers.edu/spinewire/Articles/SCIschemia/Sagittal_brain1.gif

Lisa McLeod HMS III

Gillian Lieberman, MD

17 weeks to 23 weeks GAIncrease sulcation (calcarine,parieto-occipital)

Cell migration creates Intermediate layer between germinal matrix and cortical ribbon

Reduced Ventricle size

Megendi & Lushka form allowing CSF flow to subarachnoid

Midline structures further reduce ventricle size (i.e. Corpus Call, Sept. Pallucidum)

Lower Brainstem MyelinationNL 17 Wk Fetus NL 23 Wk Fetus

Cortical Ribbon

Germinal matrix Atrium of Ventricle

Atrium of Ventricle

Septum Pallucidum

Subarachnoid CSF

Brainstem Myelination

Patent Aqueduct

Corpus callosum

BIDMC

Lower images from http://www.radnet.ucla.edu/residents/chief/residentrounds1.htm

BIDMC

Lisa McLeod HMS III

Gillian Lieberman, MD

28 Weeks to 33 Weeks GA28 Weeks to 33 Weeks GA

NL 28Wk Fetus NL 33Wk FetusIncreased Axonal Myelination of Basal Ganglia

Increased Sulcation (precentralgyrus, postcentral gyrus, Temporal Sulci)

Maturation of ArachnoidGranulations (less subarachnoid fluid)

Increased Contrast between white and grey matter

BIDMChttp://www.radnet.ucla.edu/residents/chief/residentrounds1.htm

BIDMC

BIDMC

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Lisa McLeod HMS III

Gillian Lieberman, MD

Patient K.A.:Patient K.A.: 33yo F at 18 weeks GA presents for high risk ultrasound 33yo F at 18 weeks GA presents for high risk ultrasound

of fetus with of fetus with h/oh/o

choroid plexus cysts at first trimester choroid plexus cysts at first trimester exam.exam.

Findings this examFindings this exam::PersistancePersistance of abnormal choroid plexusof abnormal choroid plexus

Mild Borderline Mild Borderline VentriculomegalyVentriculomegaly (9mm prominent lateral (9mm prominent lateral ventricles)ventricles)

7mm Cyst in the Posterior 7mm Cyst in the Posterior FossaFossa

Ventricular Ventricular SeptalSeptal DefectDefect

Lisa McLeod HMS III

Gillian Lieberman, MD

Patient K.A. 18 weeksNL Patient 18 weeksProminent ventricular atrium (cursor on medial reflection)

Dangling choroid plexus

(>3mm from medial reflection)

Cyst in posterior fossa

Images from BIDMCAbove from http://www.centrus.com.br

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Lisa McLeod HMS III

Gillian Lieberman, MD

VentriculomegalyVentriculomegaly::DefinedDefined as enlargement of the ventricles to greater than 10mm without as enlargement of the ventricles to greater than 10mm without an associated an associated macrocephalymacrocephaly

FrequencyFrequency 0.50.5--2/1000 live births2/1000 live births

Natural HistoryNatural History Reversible (29%), Stable (57%), or lead to Reversible (29%), Stable (57%), or lead to Hydrocephalus (14%)*Hydrocephalus (14%)*

PrognosisPrognosis –– Highly dependant on etiologyHighly dependant on etiologyGood when no associated malformations present. BUT Ultrasound haGood when no associated malformations present. BUT Ultrasound has a 20s a 20--60% false negative rate in diagnosis of associated 60% false negative rate in diagnosis of associated abnl’sabnl’s..Bad if associated malformations, male gender, severe enlargementBad if associated malformations, male gender, severe enlargement (>15mm), (>15mm), extension to 3extension to 3rdrd/4/4thth ventricles, or appears early in gestation.ventricles, or appears early in gestation.

* Values difficult to interpret given number of terminations for

this finding.

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Lisa McLeod HMS III

Gillian Lieberman, MD

Etiologies of Etiologies of VentriculomegalyVentriculomegalyPrimary causes:Primary causes:

20% 20% AqueductalAqueductal stenosisstenosis (isolated ~18%)(isolated ~18%)**MyelomeningoceleMyelomeningocele with with ChiariChiari malformationmalformationAgenesis of the Corpus Agenesis of the Corpus CallosumCallosum (10%)(10%)DandyDandy--Walker malformation Walker malformation (prognosis variant (prognosis variant dep.) dep.) * * HoloprosencephalyHoloprosencephaly**HydranencephalyHydranencephalyLissencephalyLissencephaly

Secondary causes:Secondary causes:IntraventricularIntraventricular hemorrhagehemorrhageCerebral ischemiaCerebral ischemiaInfections (CMV, HSV, Infections (CMV, HSV, ToxoToxo, , VaricellaVaricella))TumorsTumors

*often associated with chromosomal *often associated with chromosomal abnl’sabnl’s

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Lisa McLeod HMS III

Gillian Lieberman, MD

Patient workPatient work--up for up for VentriculomegalyVentriculomegaly

Maternal Blood Tests (Rubella, Maternal Blood Tests (Rubella, ParvoParvo, HIV, , HIV, Torch, antiTorch, anti--platelet abs)platelet abs)KaryotypeKaryotype of fetusof fetusFetal echocardiogramFetal echocardiogramFetal MRIFetal MRI

CNS: Symmetry & CNS: Symmetry & DistrubutionDistrubution, Cell layers, , Cell layers, Choroid, Posterior Choroid, Posterior FossaFossa, Aqueduct patency,, Aqueduct patency,ExtracranialExtracranial: Other signs of : Other signs of aneuploidyaneuploidy

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Lisa McLeod HMS III

Gillian Lieberman, MD

Isolated Isolated AqueductalAqueductal

StenosisStenosis

in 32 Week Fetusin 32 Week Fetus

Stenosed

Aqueduct

NL 4th

VentricleIntact Vermis

Ventriculomegaly

Images from BIDMC

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Lisa McLeod HMS III

Gillian Lieberman, MD

MyelomeningoceleMyelomeningocele

with with ChiariChiari

Malformation Malformation in 23 week Fetusin 23 week Fetus

Angular Ventricles

Herniated cerebellum & Brainstem

Lumbar Neural Tube Defect Causing Tethered Cord

Images from BIDMC

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Lisa McLeod HMS III

Gillian Lieberman, MD

Dandy Walker Variant Vs. Dandy Walker Variant Vs. ArachnoidArachnoid

Cyst in Cyst in 26 Week Fetuses26 Week Fetuses

Agenesis/Dysgenesis

of Cerebellar

Vermis

Bilateral Symmetry of Ventricles

Assymetry

Septation

and Mass effect on Adjacent tissues

Intact Cerebellum

Images from BIDMC

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Lisa McLeod HMS III

Gillian Lieberman, MD

Hemorrhage Vs. Agenesis of Corpus Hemorrhage Vs. Agenesis of Corpus CallosumCallosum in 26 Week Fetusesin 26 Week Fetuses

Colpocephaly: Prominent Occipital Horns

Absent Corpus Callosum

Hypointense

Parenchyma = Hemorrhage/clot blocking outflow tract

Images from BIDMC

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Lisa McLeod HMS III

Gillian Lieberman, MD

Posterior Posterior fossafossa difficult to conclusively assessdifficult to conclusively assess

What is the origin of the posterior cyst? What is the origin of the posterior cyst?

Why are the ventricles so prominent?Why are the ventricles so prominent?

What is this child’s prognosis?What is this child’s prognosis?

Since ultrasound could not conclusively Since ultrasound could not conclusively dxdx, same day , same day fetal MRI ordered.fetal MRI ordered.

Back to Patient K.A………………Back to Patient K.A………………

Lisa McLeod HMS III

Gillian Lieberman, MD

Dandy Walker Variant with Cortical AtrophyDandy Walker Variant with Cortical Atrophy

Mild Cerebellar

Hypoplasia

Thinned Cortex

Intact Corpus Callosum

Images from BIDMC

Fetal Findings Were:Fetal Findings Were:

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Lisa McLeod HMS III

Gillian Lieberman, MD

How Should K.A. Be Counseled?How Should K.A. Be Counseled?

Depending on mother’s wishes, amniocentesis should Depending on mother’s wishes, amniocentesis should be recommended be recommended

Dandy Walker variant can have mild prognosisDandy Walker variant can have mild prognosis

Cortical thinning implies perturbed brain developmentCortical thinning implies perturbed brain development

Given ventricular prominence plus associated Given ventricular prominence plus associated malformations (VSD) prognosis is poormalformations (VSD) prognosis is poor

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Lisa McLeod HMS III

Gillian Lieberman, MD

When to use MRI:When to use MRI:Obese mothersObese mothers

Low position of head Low position of head

Calcification of craniumCalcification of cranium

CNS anomalies not CNS anomalies not diagnosable by USdiagnosable by US

When HASTE ultra fast When HASTE ultra fast spin echo MRI availablespin echo MRI available

When NOT to use MRI:When NOT to use MRI:Too much fetal Too much fetal movementmovement

Suspected cardiac Suspected cardiac anomaliesanomalies

Early gestational age (too Early gestational age (too many incidental findings)many incidental findings)

Absolute Absolute contrindicationscontrindications(claustrophobia, metal)(claustrophobia, metal)

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Lisa McLeod HMS III

Gillian Lieberman, MD

Future Uses of Fetal CNS MRI:Future Uses of Fetal CNS MRI:

Help Guide Patient Counseling When Abnormalities are FoundHelp Guide Patient Counseling When Abnormalities are Found

New outlook into patient selection for in New outlook into patient selection for in uteroutero interventions:interventions:High probability of good outcome for cases of isolated High probability of good outcome for cases of isolated ventriculomegalyventriculomegaly/hydrocephalus/hydrocephalus

Useful correlations between Ventricle morphology and Useful correlations between Ventricle morphology and underlying soft tissue defects:underlying soft tissue defects:

ColpocephalusColpocephalus Agenesis of Corpus Call.Agenesis of Corpus Call.Angular Anterior Horns Angular Anterior Horns MeningomyeloceleMeningomyeloceleFused Anterior Horns Absence of Sept Fused Anterior Horns Absence of Sept

pallucidumpallucidum

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Lisa McLeod HMS III

Gillian Lieberman, MD

••

GarelGarel

C, C, ChantrelChantrel

E, E, BrisseBrisse

H, H, ElmalehElmaleh

M, M, LutonLuton

D, D, OuryOury

JF, JF, SebagSebag

G, Hassan M. Fetal G, Hassan M. Fetal CerbralCerbral

Cortex: Normal Gestational Landmarks Identified Using Prenatal MCortex: Normal Gestational Landmarks Identified Using Prenatal MR Imaging. AJNR 2001; 22: R Imaging. AJNR 2001; 22: 184184--189189

••

Girard N, Girard N, RaybaudRaybaud

C, C, PoncetPoncet

M In Vivo MR Study of Brain Maturation in Normal Fetuses. M In Vivo MR Study of Brain Maturation in Normal Fetuses. AJNR 1995; 16:407AJNR 1995; 16:407--413413

••

Levine D, Levine D, TropTrop

I, Mehta T, Barnes PD MR Appearance of Fetal Cerebral VentriclI, Mehta T, Barnes PD MR Appearance of Fetal Cerebral Ventricle Morphology. e Morphology. Radiology 2002; 223(3):652Radiology 2002; 223(3):652--660660

••

Simon EM, Goldstein RB, Simon EM, Goldstein RB, CoakleyCoakley

FV, Filly RA, Broderick KC, FV, Filly RA, Broderick KC, MusciMusci

TJ, TJ, BarkovichBarkovich

AJ Fast AJ Fast MR Imaging of Fetal CNS Anomalies In MR Imaging of Fetal CNS Anomalies In UteroUtero. Am J . Am J NeurorediolNeurorediol

2000; 21:16882000; 21:1688--16981698••

Levine D, Barnes PD Cortical Maturation in Normal and Abnormal FLevine D, Barnes PD Cortical Maturation in Normal and Abnormal Fetuses as Assessed with etuses as Assessed with Prenatal MR Imaging. Radiology 1999; 210:751Prenatal MR Imaging. Radiology 1999; 210:751--758758

••

Levine D, Barnes PD, Madsen JR, Li W, Edelman RR Fetal Central Levine D, Barnes PD, Madsen JR, Li W, Edelman RR Fetal Central Nervous System Anomalies: Nervous System Anomalies: MR Imaging Augments MR Imaging Augments SonographicSonographic

Diagnosis. Radiology 1997; 204:635Diagnosis. Radiology 1997; 204:635--642642••

OiOi

S Diagnosis, Outcome, and Management of Fetal S Diagnosis, Outcome, and Management of Fetal AbnomalitiesAbnomalities: Fetal Hydrocephalus Child’s : Fetal Hydrocephalus Child’s Neuro 19(7Neuro 19(7--8):5088):508--516516

••

GarelGarel

C, C, LutonLuton

D, D, OuryOury

J, et al Ventricular Dilatations. Child’s Neuro 19(7J, et al Ventricular Dilatations. Child’s Neuro 19(7--8): 5178): 517--523 523

References:References:

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Lisa McLeod HMS III

Gillian Lieberman, MD

Suggested ReadingSuggested Reading

SD Brown, Children’s Hospital and Massachusetts General Hospital, Boston, MA; JA Estroff

and CE Barnewalt, Children’s Hospital, Boston, MA. Fetal MRI. Applied Radiology 2004; 33(2) 9-25.

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Lisa McLeod HMS III

Gillian Lieberman, MD

Dr. Deborah LevineDr. Deborah LevineDr. Michelle SwireDr. Michelle SwireDr. Dr. IlseIlse CastroCastro--AragonAragonDr. Gillian LiebermanDr. Gillian LiebermanPamela Pamela LepkowskiLepkowskiWebmaster Larry BarbarasWebmaster Larry Barbaras

Acknowledgements:Acknowledgements: