Use of MRI in Evaluating Fetal Ventriculomegaly
Use of MRI in Evaluating Fetal Ventriculomegaly
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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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 />
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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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