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Use of MRI in Evaluating Fetal Ventriculomegaly

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Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

January 2004<br />

<strong>Use</strong> <strong>of</strong> <strong>MRI</strong> <strong>in</strong> Evaluat<strong>in</strong>g<br />

<strong>Fetal</strong> <strong>Ventriculomegaly</strong><br />

Lisa McLeod, Harvard Medical School Year III<br />

Gillian Lieberman, MD<br />

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


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Objectives:<br />

Review basic fetal CNS development and neuroanatomy<br />

Discuss DDx <strong>of</strong> ventriculomegaly documented on fetal<br />

ultrasound<br />

Illustrate the use <strong>of</strong> fetal <strong>MRI</strong> <strong>in</strong> differentiat<strong>in</strong>g these diagnoses diagnoses<br />

and its impact on management<br />

Identify pros and cons <strong>of</strong> Ultrasound and <strong>MRI</strong> for fetal survey<br />

Future directions <strong>of</strong> use <strong>of</strong> fetal <strong>MRI</strong> <strong>in</strong> diagnosis <strong>of</strong> etiology <strong>of</strong> <strong>of</strong><br />

ventriculomegaly<br />

2


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Landmarks <strong>of</strong> fetal bra<strong>in</strong><br />

development visible by <strong>MRI</strong><br />

Glial Cell Migration<br />

Visible @ 22 weeks GA<br />

Cells migrate from<br />

ventricular periphery<br />

toward cortical ribbon<br />

T2 Hypo<strong>in</strong>tense<br />

Sulcation/Ventricles<br />

Sulcation/Ventricles<br />

Axonal Maturation/Myel<strong>in</strong>ation<br />

Maturation/ Myel<strong>in</strong>ation<br />

Caudal-cephalic/Dorsal<br />

Caudal cephalic/Dorsal-ventral ventral<br />

T2 Hypo<strong>in</strong>tense<br />

Agyric (exc. Sylvian) Sylvian)<br />

until<br />

24 weeks<br />

Physio Hydrocephalus<br />

resolves from 14 weeks<br />

Both T2 Hyper<strong>in</strong>tense<br />

3


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Ventricular CSF Circulation<br />

http://carecure.rutgers.edu/sp<strong>in</strong>ewire/Articles/SCIschemia/Sagittal_bra<strong>in</strong>1.gif<br />

4


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Corpus callosum<br />

BIDMC<br />

17 weeks to 23 weeks GA<br />

Increase sulcation (calcar<strong>in</strong>e,parieto-occipital)<br />

Cell migration creates Intermediate layer between<br />

germ<strong>in</strong>al matrix and cortical ribbon<br />

Reduced Ventricle size<br />

Megendi & Lushka form allow<strong>in</strong>g CSF flow<br />

to subarachnoid<br />

Midl<strong>in</strong>e structures further reduce ventricle<br />

size (i.e. Corpus Call, Sept. Pallucidum)<br />

Lower Bra<strong>in</strong>stem Myel<strong>in</strong>ation<br />

NL 17 Wk Fetus NL 23 Wk Fetus<br />

Atrium <strong>of</strong> Ventricle<br />

Cortical<br />

Ribbon<br />

Subarachnoid<br />

CSF<br />

Septum Pallucidum<br />

Patent Aqueduct<br />

Bra<strong>in</strong>stem Myel<strong>in</strong>ation<br />

BIDMC<br />

Germ<strong>in</strong>al<br />

matrix Atrium <strong>of</strong> Ventricle<br />

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


BIDMC<br />

Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

28 Weeks to 33 Weeks GA<br />

NL 28Wk Fetus NL 33Wk Fetus<br />

Increased Axonal Myel<strong>in</strong>ation <strong>of</strong><br />

Basal Ganglia<br />

Increased Sulcation (precentral<br />

gyrus, postcentral gyrus, Temporal<br />

Sulci)<br />

Maturation <strong>of</strong> Arachnoid<br />

Granulations (less subarachnoid<br />

fluid)<br />

Increased Contrast between<br />

white and grey matter<br />

http://www.radnet.ucla.edu/residents/chief/residentrounds1.htm<br />

BIDMC<br />

BIDMC


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Patient K.A.:<br />

33yo F at 18 weeks GA presents for high risk ultrasound<br />

<strong>of</strong> fetus with h/o<br />

choroid plexus cysts at first trimester<br />

exam.<br />

F<strong>in</strong>d<strong>in</strong>gs this exam: exam<br />

Persistance <strong>of</strong> abnormal choroid plexus<br />

Mild Borderl<strong>in</strong>e <strong>Ventriculomegaly</strong> (9mm prom<strong>in</strong>ent lateral<br />

ventricles)<br />

7mm Cyst <strong>in</strong> the Posterior Fossa<br />

Ventricular Septal Defect<br />

7


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

NL Patient 18 weeks<br />

Above from http://www.centrus.com.br<br />

Patient K.A. 18 weeks<br />

Images from BIDMC<br />

Prom<strong>in</strong>ent<br />

ventricular<br />

atrium (cursor on<br />

medial reflection)<br />

Dangl<strong>in</strong>g<br />

choroid plexus<br />

(>3mm from<br />

medial reflection)<br />

Cyst <strong>in</strong> posterior<br />

fossa


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

<strong>Ventriculomegaly</strong>:<br />

<strong>Ventriculomegaly</strong><br />

Def<strong>in</strong>ed as enlargement <strong>of</strong> the ventricles to greater than 10mm without<br />

an associated macrocephaly<br />

Frequency 0.5-2/1000 0.5 2/1000 live births<br />

Natural History Reversible (29%), Stable (57%), or lead to<br />

Hydrocephalus (14%)*<br />

Prognosis – Highly dependant on etiology<br />

Good when no associated malformations present. BUT Ultrasound has has<br />

a 20- 20<br />

60% false negative rate <strong>in</strong> diagnosis <strong>of</strong> associated abnl’s. abnl’s<br />

Bad if associated malformations, male gender, severe enlargement (>15mm),<br />

extension to 3 rd /4 th ventricles, or appears early <strong>in</strong> gestation.<br />

* Values difficult to <strong>in</strong>terpret given number <strong>of</strong> term<strong>in</strong>ations for this f<strong>in</strong>d<strong>in</strong>g.<br />

9


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Etiologies <strong>of</strong> <strong>Ventriculomegaly</strong><br />

Primary causes:<br />

20% Aqueductal stenosis (isolated ~18%)* ~18%)<br />

Myelomen<strong>in</strong>gocele with Chiari malformation<br />

Agenesis <strong>of</strong> the Corpus Callosum (10%)<br />

Dandy-Walker Dandy Walker malformation (prognosis variant<br />

dep.) *<br />

Holoprosencephaly*<br />

Holoprosencephaly<br />

Hydranencephaly<br />

Lissencephaly<br />

Secondary causes:<br />

Intraventricular hemorrhage<br />

Cerebral ischemia<br />

Infections (CMV, HSV, Toxo, Toxo,<br />

Varicella) Varicella<br />

Tumors<br />

*<strong>of</strong>ten associated with chromosomal abnl’s<br />

10


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Patient work-up work up for<br />

<strong>Ventriculomegaly</strong><br />

Maternal Blood Tests (Rubella, Parvo, Parvo,<br />

HIV,<br />

Torch, anti-platelet anti platelet abs)<br />

Karyotype <strong>of</strong> fetus<br />

<strong>Fetal</strong> echocardiogram<br />

<strong>Fetal</strong> <strong>MRI</strong><br />

CNS: Symmetry & Distrubution, Distrubution,<br />

Cell layers,<br />

Choroid, Posterior Fossa, Fossa,<br />

Aqueduct patency,<br />

Extracranial: Extracranial:<br />

Other signs <strong>of</strong> aneuploidy<br />

11


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Isolated Aqueductal<br />

NL 4th Ventricle<br />

Stenosed<br />

Aqueduct<br />

Stenosis<br />

Intact Vermis<br />

<strong>in</strong> 32 Week Fetus<br />

<strong>Ventriculomegaly</strong><br />

Images from BIDMC<br />

12


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Myelomen<strong>in</strong>gocele<br />

with Chiari<br />

<strong>in</strong> 23 week Fetus<br />

Herniated cerebellum &<br />

Bra<strong>in</strong>stem<br />

Lumbar Neural Tube Defect<br />

Caus<strong>in</strong>g Tethered Cord<br />

Malformation<br />

Images from BIDMC<br />

Angular Ventricles<br />

13


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Dandy Walker Variant Vs. Arachnoid<br />

26 Week Fetuses<br />

Bilateral Symmetry <strong>of</strong><br />

Ventricles<br />

Agenesis/Dysgenesis <strong>of</strong><br />

Cerebellar Vermis<br />

Assymetry<br />

Images from BIDMC<br />

Cyst <strong>in</strong><br />

Intact Cerebellum<br />

Septation and Mass effect<br />

on Adjacent tissues<br />

14


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Hemorrhage Vs. Agenesis <strong>of</strong> Corpus Callosum<br />

<strong>in</strong> 26 Week Fetuses<br />

Hypo<strong>in</strong>tense Parenchyma =<br />

Hemorrhage/clot block<strong>in</strong>g outflow tract<br />

Absent Corpus Callosum<br />

Colpocephaly: Prom<strong>in</strong>ent Occipital<br />

Horns<br />

Images from BIDMC<br />

15


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Back to Patient K.A………………<br />

Posterior fossa difficult to conclusively assess<br />

What is the orig<strong>in</strong> <strong>of</strong> the posterior cyst?<br />

Why are the ventricles so prom<strong>in</strong>ent?<br />

What is this child’s prognosis?<br />

S<strong>in</strong>ce ultrasound could not conclusively dx, dx,<br />

same day<br />

fetal <strong>MRI</strong> ordered.<br />

16


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

<strong>Fetal</strong> F<strong>in</strong>d<strong>in</strong>gs Were:<br />

Dandy Walker Variant with Cortical Atrophy<br />

Mild Cerebellar<br />

Hypoplasia<br />

Images from BIDMC<br />

Th<strong>in</strong>ned Cortex<br />

Intact Corpus Callosum


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

How Should K.A. Be Counseled?<br />

Depend<strong>in</strong>g on mother’s wishes, amniocentesis should<br />

be recommended<br />

Dandy Walker variant can have mild prognosis<br />

Cortical th<strong>in</strong>n<strong>in</strong>g implies perturbed bra<strong>in</strong> development<br />

Given ventricular prom<strong>in</strong>ence plus associated<br />

malformations (VSD) prognosis is poor<br />

18


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

When to use <strong>MRI</strong>:<br />

Obese mothers<br />

When to use <strong>MRI</strong>:<br />

Low position <strong>of</strong> head<br />

Calcification <strong>of</strong> cranium<br />

CNS anomalies not<br />

diagnosable by US<br />

When HASTE ultra fast<br />

sp<strong>in</strong> echo <strong>MRI</strong> available<br />

When NOT to use <strong>MRI</strong>:<br />

Too much fetal<br />

movement<br />

When NOT to use <strong>MRI</strong>:<br />

Suspected cardiac<br />

anomalies<br />

Early gestational age (too<br />

many <strong>in</strong>cidental f<strong>in</strong>d<strong>in</strong>gs)<br />

Absolute contr<strong>in</strong>dications<br />

(claustrophobia, metal)<br />

19


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Future <strong>Use</strong>s <strong>of</strong> <strong>Fetal</strong> CNS <strong>MRI</strong>:<br />

Help Guide Patient Counsel<strong>in</strong>g When Abnormalities are Found<br />

New outlook <strong>in</strong>to patient selection for <strong>in</strong> utero <strong>in</strong>terventions:<br />

<strong>in</strong>terventions:<br />

High probability <strong>of</strong> good outcome for cases <strong>of</strong> isolated<br />

ventriculomegaly/hydrocephalus<br />

ventriculomegaly/hydrocephalus<br />

<strong>Use</strong>ful correlations between Ventricle morphology and<br />

underly<strong>in</strong>g s<strong>of</strong>t tissue defects:<br />

Colpocephalus Agenesis <strong>of</strong> Corpus Call.<br />

Angular Anterior Horns Men<strong>in</strong>gomyelocele<br />

Fused Anterior Horns Absence <strong>of</strong> Sept<br />

pallucidum<br />

20


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Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

References:<br />

Garel<br />

C, Chantrel<br />

E, Brisse<br />

H, Elmaleh<br />

M, Luton<br />

D, Oury<br />

JF, Sebag<br />

G, Hassan M. <strong>Fetal</strong> Cerbral<br />

Cortex: Normal Gestational Landmarks Identified Us<strong>in</strong>g Prenatal MR MR<br />

Imag<strong>in</strong>g. AJNR 2001; 22:<br />

184-189 184 189<br />

Girard N, Raybaud<br />

C, Poncet<br />

M In Vivo MR Study <strong>of</strong> Bra<strong>in</strong> Maturation <strong>in</strong> Normal Fetuses.<br />

AJNR 1995; 16:407-413 16:407 413<br />

Lev<strong>in</strong>e D, Trop<br />

I, Mehta T, Barnes PD MR Appearance <strong>of</strong> <strong>Fetal</strong> Cerebral Ventricle Ventricle<br />

Morphology.<br />

Radiology 2002; 223(3):652-660<br />

223(3):652 660<br />

Simon EM, Goldste<strong>in</strong> RB, Coakley<br />

FV, Filly RA, Broderick KC, Musci<br />

TJ, Barkovich<br />

AJ Fast<br />

MR Imag<strong>in</strong>g <strong>of</strong> <strong>Fetal</strong> CNS Anomalies In Utero. Utero.<br />

Am J Neurorediol<br />

2000; 21:1688-1698<br />

21:1688 1698<br />

Lev<strong>in</strong>e D, Barnes PD Cortical Maturation <strong>in</strong> Normal and Abnormal Fetuses Fetuses<br />

as Assessed with<br />

Prenatal MR Imag<strong>in</strong>g. Radiology 1999; 210:751-758 210:751 758<br />

Lev<strong>in</strong>e D, Barnes PD, Madsen JR, Li W, Edelman RR <strong>Fetal</strong> Central Nervous System Anomalies:<br />

MR Imag<strong>in</strong>g Augments Sonographic<br />

Diagnosis. Radiology 1997; 204:635-642 204:635 642<br />

Oi<br />

S Diagnosis, Outcome, and Management <strong>of</strong> <strong>Fetal</strong> Abnomalities: Abnomalities:<br />

<strong>Fetal</strong> Hydrocephalus Child’s<br />

Neuro 19(7-8):508 19(7 8):508-516 516<br />

Garel<br />

C, Luton<br />

D, Oury<br />

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

21


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Suggested Read<strong>in</strong>g<br />

SD Brown, Children’s Hospital and Massachusetts General Hospital,<br />

Boston, MA; JA Estr<strong>of</strong>f and CE Barnewalt, Children’s Hospital,<br />

Boston, MA. <strong>Fetal</strong> <strong>MRI</strong>. Applied Radiology 2004; 33(2) 9-25.<br />

22


Lisa McLeod HMS III<br />

Gillian Lieberman, MD<br />

Acknowledgements:<br />

Dr. Deborah Lev<strong>in</strong>e<br />

Dr. Michelle Swire<br />

Dr. Ilse Castro-Aragon<br />

Castro Aragon<br />

Dr. Gillian Lieberman<br />

Pamela Lepkowski<br />

Webmaster Larry Barbaras<br />

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