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Fetal	
  Nuerosonogram	
  
Professor Hassan Nasrat FRCS, FRCOG
The Fetal Medicine Clinic
The First Clinic

JUCOG January 2013

Sunday, July 28, 13


Microcephaly

Holoprosencephaly

Head normal or small


Chiari	
  Malforma3on

ACC



Dia



Anencephaly

Occipital Encephalocele
Schizencephaly

Schizencephaly

Circle of Willis Mallformation
Vascular	
  
Malforma3ons

SOP

Pilu

Imaging Findings
 Herniated brain tissue
 „cyst within the cyst“
 Ventriculomegaly 7080%
 Microcephaly 25%
 Polyhydramnios
 Oligohydramnios



Encephalocele

PF-­‐Fluid-­‐Cyst

CAVE:
 Associated with multiple
syndroms ( Meckel- Gruber )

Hydrance

halus , T 21
Ventriculomegaly
Hemimegalencephaly

Arachnoid	
  cyst

Hydranecphaly

Yong seok et a

2

Sunday, July 28, 13
Congenital	
  CNS	
  Anomalies
o Incidence	
  in	
  longtem	
  studies	
  about	
  1	
  %
o 	
  Only	
  minimal	
  identified	
  at	
  birth	
  
o 	
  Screening	
  Increases	
  The	
  Number	
  Of	
  
Referred	
  Cases	
  For	
  Evaluation	
  Of	
  Suspected	
  
CNS	
  Anomalies.	
  
o The	
  CNS	
  sonographic	
  appearance	
  changes	
  
throughout	
  pregnancy	
  	
  
Sunday, July 28, 13
Learning	
  Objec3ves

✤ Embryonic	
  development	
  of	
  the	
  CNS	
  
in	
  relation	
  to	
  sonographic	
  findings

✤ Standard	
   Sonographic	
   Examination	
  
of	
  the	
  CNS	
  

✤ Fetal	
  

Neurosonography	
   and	
   the	
  
	
  
Role	
  of	
  3	
  D	
   	
   (systemic	
  approach	
  to	
  
examination	
  of	
  the	
  Posterior	
  Fossa)
4

Sunday, July 28, 13
Embryology of the
CNS

Sunday, July 28, 13
At	
   5th	
   Week	
   The	
   Cells	
  
Destined	
   To	
   Form	
   The	
  
Notochord	
   Infiltrate	
   Into	
  
The	
  Embryonic	
  Disc.	
  

I t	
   I n d u c e s	
   T h e	
  
Overlying	
   Embryonic	
  
Tissue	
   To	
  Thicken	
  And	
  
Ultimately	
   Fold	
   Over	
  
And	
   Fuse	
   As	
   The	
   The	
   Fusion	
   Starts	
   In	
  
Neural	
  Tube.	
  
The	
   Midtrunk	
   Of	
   The	
  
E m b r y o	
  
A n d	
  
Subsequently	
   Extends	
  
To	
   The	
   Cranial	
   And	
  
Caudal	
  Ends	
  
Neural	
  Crest

Neural	
  Groove

Neural	
  Plate
Sunday, July 28, 13

Ectoderm

Neural	
  Tube
Prosencephalon

Mesencephalon

Rhombencephalon

7
Sunday, July 28, 13
Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at
8
the end of 8 weeks of gestation. The development of premature ventricular system is seen.
Sunday, July 28, 13
Prosencephalon

Mesencephalon

Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at
8
the end of 8 weeks of gestation. The development of premature ventricular system is seen.
Sunday, July 28, 13
Normal brain development on the mid-sagittal section between 8 and 12 weeks of gestation). Note the remarkable changing of premature brain
9
appearance.

Sunday, July 28, 13
AJR:166,

AJR:166,

Changing	
  Ultrasound	
  appearance	
  of	
  the	
  
The	
  Posterior	
  Fossa	
  throughout	
  gesta3on	
  
SONOGRAPHIC

February 1996

February 1996
AJR:166,
February 1996

ANATOMY

SONOGRAPHIC
ANATOMY
SONOGRAPHIC

OF DEVELOPING

433

CEREBELLUM

OF DEVELOPING
CEREBELLUM
ANATOMY
OF DEVELOPING
CEREBELLUM

433

433

10

C
Sunday,
C
Fig.
C C July 28, 13

D
13.-Drawings

depicting

some

relevant

features

D

DD

of fetal cerebellar

development.
The vermis develops superiorly to inferiorly.
Hypoplasia or developmental arrest results in
varying size deficits of the inferior portion, leaving
a relatively square defect that communicates with
the fourth ventricle and separates the lower
cerebellar hemispheres.

11
Sunday, July 28, 13
C

D

Fig. 13.-Drawings
depicting
some relevant features
of fetal cerebellar
development.
A, Axial drawing
of developing
cerebellum
at 5 weeks’ gestational
age shows that developing cerebellar hemispheres have not yet grown
toward midline and thatfourth
ventricle is covered only byfourth ventricular roof,which
is onlytwo
cell layers thickatthis
stage of development.
B, Sagittal drawing of developing cerebellum at 10 weeks’ gestational age shows small cerebellum located rostrally over fourth ventricle,

with caudal fourth ventricle being covered only by thin fourth ventricular roof.
C, Sagittal
drawing at 16 weeks’ gestational age shows further caudal growth of cerebellum
ening
0,

of caudal

Sagfttal

fourth
drawing

ventricular
roof.
at 17 weeks’ gestational

age shows

cerebellum

We have shown that the sonognaphic
appearance
of normal cemebellar
development
can resemble
pathology
early in
the second
trimester.
Our findings
indicate
that the mature
relationships
of the posterior
fossa structures
are not established until at least 18 weeks’ gestational
age; therefore,
the
Sunday, July 28, 13 diagnosis
prenatal
sonographic
of Dandy-Walker
complex

and vermis

covering

4. Achinon

entire

R, Tadmor

ten of pregnancy:

and vermis over fourth
fourth

0. Screening

tnansvaginal

ventrIcle,

teno

thic
and

with thick-

ventricle.

for fetal anomalies

versus transabdominal
1991 1:186-191

during

the first tnimesUltra-

sonography.

sound Obstet Gynecol
5. Nicolaides
KH, Azan G, Byrne D, Mansur C, Marks K. Fetal nuchal translucency: ultrasound screening for chromosomal
defects in first trimester of
pregnancy.
BMJ 1992:304:867-869
6. Bronshtein
M, Blumenfeld
I, Kohn J, Blumenfeld Z. Detection ofcleft lip by early

12
of posteriorB, Next caudal image identifies fetus.
fossa in 13- to 14-week-old
called
in stea
fourth ventricular roof joining cerebellar hemispheres acquisition Vermis
fetus.
A, Vermis isand separating
identified
between
cerebellar
hemispheres
rostrally
(arrow).
age of posteriorfossacaudally
in
(arrow)
fourth ventricle
and cisterna
magna.
but not
B, Next caudal image identifies fourth ventricular roof joining cerebellar hemispheres
fetus. Vermis is identified
(arrow) and separating
fourth ventricle
and cisterna
magna.
but not caudally at this s

Effect	
  of	
  Gesta=onal	
  age	
  (Posterior	
  Fossa)

Fig. 7.-Axial

Fig. 7.-A
and

tenor fossa
tenor fossa in 16-weekA and
A and B, Caudally,
thickto enoug
thick enough
be v
and sagittal and sagittal
(B) planes

axial

sagittal

sonograms of posterior fossa in 16-week-old fetus
fourth ventricular roof is visualized in both planes (arrow)
13
Sunday, July 28, 13
gure 1 Transvaginal scan of a 14-week fetus. (a) Oblique-1 (sagittal) section: the fetus is facing left. The choroid plexus fills the antrum
ure 1 Transvaginal The of aa14-week appear Oblique-1 but are normal; (b)the fetus facing left. The choroid plexus fills the antrum
Transvaginal scan of 14-week fetus. (a) Oblique-1 (sagittal) section: a Frontal-2 (coronal) The choroid plexus fills the horns
ure 1lateral ventricle. scan anterior hornsfetus. (a)prominent, (sagittal) section: the fetus isis facing left. section through the anterior antrum
the
thelateral ventricle. The anterior horns appear normal for this are normal;age; ahowever, this same sonographic picture at anterior horn
he lateral ventricle. The anterior horns appear prominent, but are normal; (b) a Frontal-2 (coronal) section through the 20 weeks or
the lateral ventricles. The anterior horns are prominent, but gestational (b) Frontal-2 (coronal) section through the anterior horns
the is consistent with ventriculomegaly or hydrocephalus this gestational age; however, this same sonographic picture 20 weeks or
he lateral ventricles. The anterior horns are normal for
ore lateral ventricles. The anterior horns are normal for this gestational age; however, this same sonographic picture atat 20 weeks o
re is consistent with ventriculomegaly or hydrocephalus
re is consistent with ventriculomegaly or hydrocephalus

Effect	
  Of	
  Scanning	
  Level	
  (Posterior	
  Fossa)

Higher Still
gure 2Lower-most Section (horizontal) views through the posterior fossa. (a) This is the lower-most section (see insert). The
Three serial, almost axial
Somewhat Higher
ure
ermis2 Three serial, almost axial (horizontal) views through fourth ventriclefossa. (a) a widethe this gestational age, normal) median
gure2 appears to be open (arrow) and communicates with the the posterior fossa. (a) This isis (at lower-most section (see insert). The
Three serial, almost axial (horizontal) views through the posterior through This the lower-most section (see insert). Th
mis appears to be open (arrow)and communicates with rightfourth ventricle the cerebellar (at this gestational age, normal) median
perture (foramenbeopen (arrow) (b) somewhat higher,withthe fourth left sides ofthrough a widehemispheres appear closer to eachmedia
rmis appears to of Magendie); and communicates the the and ventricle through a wide (at this gestational age, normal) other
rture (foramen of Magendie); (b) somewhat higher,
rrow); (foramenof Magendie); (b) defect’ is seen and theright and left sides appears as a discrete No ‘vermiancloser to each othe
(c) higher still, Appears To
entity. C, appear
ertureThe Vermisno ‘vermian somewhat higher, the fourth and left sides of the cerebellar hemispherescerebellum Defect’other
right ventricle (4) of the cerebellar hemispheres appear closer to each
Thethefourth ventricle (4) appears asas a discrete entity. C, cerebellum
Right And Left Sides a discrete entity. C, cerebellum
row); (c) higher still, no ‘vermian defect’ is seen and the fourth ventricle (4) appears
row); (c) higher still, (arrow) And is seen and
Be Open no ‘vermian defect’

Is Seen And The
Of
The
Cerebellar
Communicates With The
eduncular cistern (cisterna magna) and theHemispheres its closest anatomic structures, namely the cavum septi
fourth ventriFourth Ventricle (4)
Appear
its closestand the pericallosal artery, follow acavum septi
anatomic structures, namely the well-known
duncular ucistern(cisterna magna) land week,fourthventricistern (cisterna n t r i c e the fourth‘normal’
pellucidi anatomic structures, namely As cavum sep
e. Later, r t h
its closest
the
duncularafter the 16th postmenstrual the this ventriFo
Ve magna) and Closer
Appears
A
pellucidi and the pericallosal artery, reach a developmenLater, after the 16th postmenstrualand developmentTo Each Other thepericallosal artery, follow a a well-know
week, this ‘normal’
developmental timetable. They do not follow well-known
pen space narrows as postmenstrual week, this ‘normal’
of
pellucidi and
. Later, after the 16th the growth
Through A giving
en vermis narrows Wide growth and development of
(arrow);
tal stage that allows for Discrete reach a developmene space narrows as the rise to and development of developmental timetable. They do notEntity. a developmen
developmental timetable. sonographic imaging until postThey do not reach
en space progress,as the growth the median aperture

oramen progress, giving rise to the median aperture
e vermis of Magendie) (Figureto the median aperture
vermis progress, giving rise 2). Again, this normal
ramen of Magendie) be interpretedAgain, this normal
onographic finding may (Figure 2). Again, this normal
ramen of Magendie) (Figure 2). by those unfamiliar
Sunday, finding may
nographic July 28, 13 be interpreted by those unfamiliar

tal stage that allows for To search for their presence before
14
menstrual that allows forsonographic imaging until posttal stage weeks 18–19. sonographic imaging until pos
menstrual weeks 18–19. To search for their presence before
they reach weeks 18–19. To search fordevelopment would
menstrual this critical stage in their their presence befor
they reach this critical stage in their development would
Effect	
  of	
  Gesta=onal	
  age	
  (Lateral	
  Ventricles)

15
Sunday, July 28, 13
The fetal cerebellum
Pitfalls in diagnosis
Hypoplasia Or Dysplasia Should Not Be
Diagnosed Prior To 18 Weeks, Before Vermian
Development Is Complete.
An Abnormally Steep Scanning Angle May
Mimic A Prominent Cleft Between The
Lower
Portions
Of
The
Cerebellar
Hemispheres.
16
Sunday, July 28, 13
Conclusion

•TheCNS	
  

displays	
   remarkable	
  
embryological	
   and	
   developmental	
  
changes	
  throughout	
  gestation.

•Standard	
  Approach	
  of	
  examination	
  and	
  

evaluation	
   of	
   the	
   CNS	
   Should	
   Be	
  
Followed
17

Sunday, July 28, 13
Standard Sonographic
Examination of the
FEtal CNS

18
Sunday, July 28, 13
Sunday, July 28, 13
Sonography	
  of	
  the	
  CNS

Basic	
  Examination “Neurosongram”

Sunday, July 28, 13
Planes	
  of	
  Basic	
  Examina3on
Axial	
  Planes

Sunday, July 28, 13

Sagibal	
  Planes
Axial	
  Planes

Sunday, July 28, 13
Axial	
  Planes
a:	
  Transventricular

Sunday, July 28, 13
Axial	
  Planes
a:	
  Transventricular
b:	
  Transthalamic	
  

Sunday, July 28, 13
Axial	
  Planes
a:	
  Transventricular
b:	
  Transthalamic	
  

C:	
  Transcerebeller	
  

Sunday, July 28, 13
The	
  Transventricular	
  plane

Sunday, July 28, 13
The	
  Transventricular	
  plane

Frontal	
  hones

Sunday, July 28, 13
The	
  Transventricular	
  plane

Frontal	
  hones
Atrium

Sunday, July 28, 13
The	
  Transventricular	
  plane

Frontal	
  hones
Atrium

Sunday, July 28, 13

Choroid	
  
Plexus
The	
  Transventricular	
  plane
Cavum	
  Sep3	
  Pellucidi

Frontal	
  hones
Atrium

Sunday, July 28, 13

Choroid	
  
Plexus
The	
  Transthalamic	
  	
  Plane

Sunday, July 28, 13
The	
  Transthalamic	
  	
  Plane

Thalami

Sunday, July 28, 13
The	
  Transthalamic	
  	
  Plane

Thalami

Hyppocamas	
  
Gyrus

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane

T
T

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane

T
T

Cavum	
  Sep3	
  Pellucidi

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane

Frontal	
  hones

T
T

Cavum	
  Sep3	
  Pellucidi

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane
Cerebellum
Frontal	
  hones

T
T

Cavum	
  Sep3	
  Pellucidi

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane
Cerebellar	
  vermis
Cerebellum
Frontal	
  hones

T
T

Cavum	
  Sep3	
  Pellucidi

Sunday, July 28, 13
The	
  Transcerebeller	
  	
  plane
Cerebellar	
  vermis
Cerebellum
Frontal	
  hones

T
T

Cavum	
  Sep3	
  Pellucidi

Sunday, July 28, 13

Cistrerna	
  Magna
2-­‐10	
  mm
Sagibal	
  Planes	
  	
  

Sunday, July 28, 13
Sagibal	
  Planes	
  	
  

A:	
  The	
  Midsagittal	
  
Plan

Sunday, July 28, 13
Sagibal	
  Planes	
  	
  
b:	
  Parasgittal	
  plane
A:	
  The	
  Midsagittal	
  
Plan

Sunday, July 28, 13
Mid	
  SagiGal	
  Plane	
  
Corpus	
  Callosum

Cavum	
  Sep3	
  Pellucidi

Cerebellum
4th	
  V
27
Sunday, July 28, 13
Mid	
  SagiGal	
  Plane	
  

27
Sunday, July 28, 13
The	
  Corpus	
  Callosum

28
Sunday, July 28, 13
The	
  Corpus	
  Callosum
Lateral	
  Ventricles
Splenium

Corpus	
  Callosum

Thalamus
hypothalamus
Third	
  Ventricle
Pituitary

Fourth	
  ventricle
midbrain

28
Sunday, July 28, 13
Para-­‐SagiGal	
  Plane	
  

29
Sunday, July 28, 13
Basic Examniation Checklist
Head + Neck
Midline & Falx
Cavum septi pellucidi
Lateral cerebral ventricls
Choroid Plexus
Cerebellum
Cisterna magna

30
Sunday, July 28, 13
Main	
  Abnormali3es	
  can	
  be	
  
Suspected	
  on	
  Basic	
  Planes

31
Sunday, July 28, 13


Holoprosencephaly

Microcephaly

Head normal or small


Chiari	
  Malforma3on

ACC



Dia



Anencephaly

Occipital Encephalocele
Schizencephaly

Schizencephaly

Circle of Willis Mallformation
Vascular	
  
Malforma3ons

SOP

Pilu

Imaging Findings
 Herniated brain tissue
 „cyst within the cyst“
 Ventriculomegaly 7080%
 Microcephaly 25%
 Polyhydramnios
 Oligohydramnios



Encephalocele

PF-­‐Fluid-­‐Cyst

CAVE:
 Associated with multiple
syndroms ( Meckel- Gruber )

Hydrance

halus , T 21
Ventriculomegaly
Hemimegalencephaly

Arachnoid	
  cyst

Hydranecphaly

Yong seok et a

32

Sunday, July 28, 13


Holoprosencephaly

Microcephaly

Head normal or small


Chiari	
  Malforma3on

ACC



Dia



Anencephaly

Occipital Encephalocele
Schizencephaly

Schizencephaly

Circle of Willis Mallformation
Vascular	
  
Malforma3ons

SOP

Pilu

Imaging Findings
 Herniated brain tissue
 „cyst within the cyst“
 Ventriculomegaly 7080%
 Microcephaly 25%
 Polyhydramnios
 Oligohydramnios



Encephalocele

PF-­‐Fluid-­‐Cyst

CAVE:
 Associated with multiple
syndroms ( Meckel- Gruber )

Hydrance

halus , T 21
Ventriculomegaly
Hemimegalencephaly

Arachnoid	
  cyst

Hydranecphaly

Yong seok et a

32

Sunday, July 28, 13


Holoprosencephaly

Microcephaly

Head normal or small


Chiari	
  Malforma3on

ACC



Dia



Anencephaly

Occipital Encephalocele
Schizencephaly

Schizencephaly

Circle of Willis Mallformation
Vascular	
  
Malforma3ons

SOP

Pilu

Imaging Findings
 Herniated brain tissue
 „cyst within the cyst“
 Ventriculomegaly 7080%
 Microcephaly 25%
 Polyhydramnios
 Oligohydramnios



Encephalocele

PF-­‐Fluid-­‐Cyst

CAVE:
 Associated with multiple
syndroms ( Meckel- Gruber )

Hydrance

halus , T 21
Ventriculomegaly
Hemimegalencephaly

Arachnoid	
  cyst

Hydranecphaly

Yong seok et a

32

Sunday, July 28, 13


Holoprosencephaly

Microcephaly

Head normal or small


Chiari	
  Malforma3on

ACC



Dia



Anencephaly

Occipital Encephalocele
Schizencephaly

Schizencephaly

Circle of Willis Mallformation
Vascular	
  
Malforma3ons

SOP

Pilu

Imaging Findings
 Herniated brain tissue
 „cyst within the cyst“
 Ventriculomegaly 7080%
 Microcephaly 25%
 Polyhydramnios
 Oligohydramnios



Encephalocele

PF-­‐Fluid-­‐Cyst

CAVE:
 Associated with multiple
syndroms ( Meckel- Gruber )

Hydrance

halus , T 21
Ventriculomegaly
Hemimegalencephaly

Arachnoid	
  cyst

Hydranecphaly

Yong seok et a

32

Sunday, July 28, 13
•Ventriculomegaly	
  (hydrocephalus)
•Absent	
  Cavum	
  Septum	
  Pellucidum
•Agenesis	
  of	
  the	
  Corpus	
  Callosum
•Fluid	
  Collection	
  in	
  the	
  posterior	
  fossa
33
Sunday, July 28, 13
Ventriculomegaly	
  (hydrocephalus)

Mild	
  10	
  –	
  15	
  mm
Low	
  Risk	
  

mean	
  =	
  6-­‐8	
  
mm

Severe	
  >	
  15	
  mm	
  
High	
  Risk	
  

(<	
  10	
  mm	
  is	
  normal).	
  	
  Independent	
  
of	
  gesta7onal	
  age	
  
Sunday, July 28, 13
Pathogenesis:	
  Ventriculomegaly
Lee	
  Lateral	
  Ventricle

Right	
  Lateral	
  Ventricle

Foramen	
  of	
  Monro	
  

Aqueduct	
  of	
  Sylvius

4th	
  	
  Ventricle

3rd	
  	
  Ventricle

Cisterna	
  Magna
35

Sunday, July 28, 13
Absent	
  CSP

•Square	
  Shaped,	
  Interrupts	
  and	
  Fills	
  The	
  Space	
  Between	
  The	
  Frontal	
  Horns
•The	
  CSP:	
  Becomes	
  Visible	
  At	
  16	
  	
  Weeks,	
  Obliterate	
  Near	
  Term
Sunday, July 28, 13
Absent	
  CSP
Cavum	
  Sep3	
  Pellucidi

•Square	
  Shaped,	
  Interrupts	
  and	
  Fills	
  The	
  Space	
  Between	
  The	
  Frontal	
  Horns
•The	
  CSP:	
  Becomes	
  Visible	
  At	
  16	
  	
  Weeks,	
  Obliterate	
  Near	
  Term
Sunday, July 28, 13
Absent	
  CSP
A	
   rare	
   finding	
   usually	
   discovered	
   Postnatally	
   in	
  
children	
  evaluated	
  for	
  developmental	
  delay.
Associated	
  with	
  various	
  brain	
  malformations:
agenesis	
  of	
  the	
  corpus	
  callosum
Holoprosencephaly.
Setpo-­‐optic	
  dysplasia.

Secondary	
  to	
  disruptive	
  process:	
  Hydrocephalus,	
  
Chiari	
  II	
  malformation,	
  hydranecephaly.	
  

Sunday, July 28, 13
Agenesis	
  of	
  the	
  Corpus	
  Callosum	
  	
  

38
Sunday, July 28, 13
21-­‐week	
  Fetus	
  With	
  Par=al	
  Agenesis	
  Of	
  The	
  Corpus	
  Callosum

Only	
   The	
   Rostrum	
   (1),	
   Genu	
   (2)	
   And	
   Body	
   (3)	
   Are	
   Visible;	
   The	
   Splenium	
   Is	
  
Missing.	
  The	
  Corpus	
  Callosum	
  Is	
  Short	
  Posteriorly	
  And	
  Does	
  Not	
   Seem	
   To	
   Overlay	
  
The	
  Quadrigeminal	
  Plate
Sunday, July 28, 13
Outcome	
  of	
  fetal	
  ACC
 Va r i e s	
   b e t we e n	
   co m p l e te l y	
   a sy m p to m a 3 c	
  
appearance	
  and	
  severe	
  neurologic	
  problems
 50	
   –	
   100	
   %	
   of	
   isolated	
   cases	
   will	
   have	
   normal	
  
neurological	
   development	
   at	
   3-­‐11	
   years	
   but	
   Poor	
  
prognosis	
  with	
  associated	
  anomalies
 	
  Progressive	
  decline	
  in	
  intellect	
  over	
  the	
  years
 	
  Most	
  need	
  special	
  educa3on	
  
Long-­‐term	
  follow-­‐up	
  of	
  children	
  with	
  prenatally	
  diagnosed	
  agenesis	
  of	
  corpus	
  callosum	
  (ACC)	
  
J.	
  H.	
  Stupin	
  et	
  al,	
  USOG,	
  32,	
  2008
Sunday, July 28, 13
Fluid	
  Collec3on	
  in	
  the	
  Posterior	
  Fossa

41
Sunday, July 28, 13
Fluid	
  Collec3on	
  in	
  the	
  Posterior	
  Fossa
•Megacisterna Magna

41
Sunday, July 28, 13
Fluid	
  Collec3on	
  in	
  the	
  Posterior	
  Fossa
•Megacisterna Magna

•Blak’s Pouch Cyst

41
Sunday, July 28, 13
Fluid	
  Collec3on	
  in	
  the	
  Posterior	
  Fossa
•Megacisterna Magna

•D-W Malformation &DW- Variant

•Blak’s Pouch Cyst

41
Sunday, July 28, 13
Fluid	
  Collec3on	
  in	
  the	
  Posterior	
  Fossa
•Megacisterna Magna

•Blak’s Pouch Cyst

•D-W Malformation &DW- Variant

•Arachnoid Cyst

41
Sunday, July 28, 13
Anomalies Of The
Meninges
•Megacisterna Magna

•Blak’s Pouch Cyst

Anomalies
Cerebellum

•D-W Malformation &DW- Variant

•Arachnoid Cyst

42
Sunday, July 28, 13
Mega–Cisterna Magna
An Enlargement Of The Cisterna Magna Beyond 10
Mm With Intact Vermis

43
Sunday, July 28, 13
Pathogenesis: Mega Cisterna Magna
Lateral	
  Ventricle

Third	
  
Ventricle
Cerebral	
  Aqueduct

Choriod	
  Plexus

Fourth	
  Ventricle

44
Sunday, July 28, 13
Pathogenesis: Mega Cisterna Magna
The Foramina Of

Lateral	
  Ventricle

Luschka And Magendie
Fenestrate Delayed
Third	
  
Ventricle
Cerebral	
  Aqueduct

Choriod	
  Plexus

Fourth	
  Ventricle

44
Sunday, July 28, 13
Prognosis:

• Isolated Cases: (97%-100%) Are Normal.
• If Not Isolated: Only 11% Have Normal Outcome.
Nonisolated Cases Have VM, Congenital Infection, Or
Karyotype Abnormalities.
A Large Cisterna Magna Require Careful Search For
Other Abnormalities.
45
Sunday, July 28, 13
Blake’s Pouch Cyst

46
Sunday, July 28, 13
Pathogenesis: Blake’s Pouch Cyst
Nonfenestration of the
foramina of Luschka and

Lateral	
  Ventricle

Magendie leads to dilatation
of the fourth ventricle and
and elevation of the vermis
away from the brain stem.

Third	
  
Ventricle
Cerebral	
  Aqueduct

Choriod	
  Plexus

Fourth	
  Ventricle

There is no communication between the
cyst and the subarachnoid space
47
Sunday, July 28, 13
Pathogenesis: Blake’s Pouch Cyst
Nonfenestration of the
foramina of Luschka and

Lateral	
  Ventricle

Magendie leads to dilatation
of the fourth ventricle and
and elevation of the vermis
away from the brain stem.

Third	
  
Ventricle
Cerebral	
  Aqueduct

Choriod	
  Plexus

Fourth	
  Ventricle

There is no communication between the
cyst and the subarachnoid space
47
Sunday, July 28, 13
Dandy-Walker Malformation
A Spectrum Of Anomalies Of The Posterior Fossa.

• Dandy-Walker Malformation:
✦Increase Of The Posterior Fossa,
✦Complete Or Partially Agenesis Of The Cerebellar
Vermis,
✦A Tentorium Elevation

• Variant Of Dandy-Walker:
✦Hypoplasia Of The Cerebellar

Vermis In Different
Degrees With Or Without Increase Of The Posterior
Fossa.
48

Sunday, July 28, 13
ctions

Dandy-Walker Malformation

Elevated tentorium and
high position of the
torcula
Small, rotated, raised,
or absent vermis

Cystic dilation of the
fourth ventricle
communicating with a
posterior fossa fluid
space
49
Sunday, July 28, 13
The Prognosis :
Better In Isolated DWS.
Karyotype Abnormalities In About 15%.
Neonatal Mortality:
12% To 55%.
Neonatal Morbidity:
•Intelligence Is Normal In About 40%
•Borderline In 20%
•Subnormal In 40%.
50
Sunday, July 28, 13
igure 2 2 Blake’s Pouch Cyst the torcular Herophili (arrows) inferred
the
Dandy–Walker Malformation
Figure The position ofof torcular Herophili (arrows) is is inferre
The position Normal
The Torcular Is Found In A
The Torcular Is
n ultrasound by the direction ofof the tentorium DisplacedIn In (a) th
tentorium cerebelli. Higher
Position, At About The Same Level
on ultrasound by the direction the Than Usual, Indicating That Thisthe
cerebelli. (a)
As The found
Insertion Of The
orcular isSitefound in normal position, A at about the same level as
torcular is OfOnin a Posterior position, about the same level as
a normal
Is at
Neck Muscles
The
he site ofof insertion of the neck muscles on the posterior skull; thi
insertion of the neck muscles on the posterior skull; this
Skull
the site
51
a Blake’s13pouch cyst. In (b) the torcular is displaced higher than
is a Blake’s pouch cyst. In (b) the torcular is displaced higher than
Sunday, July 28,
Arachnoid Cysts
•

Are Benign, Noncommunicating Fluid
Collections
Within
Arachnoid
Membranes.

•

Location: Intracranially And In The
Spinal Canal.

•

Order Of Frequency Are The Sylvian
Fissure Or Temporal Fossa, Posterior
Fossa, Over The Cerebral Convexity,
And Midline Supratentorial,

•

Most Appear Stable And Require No
Surgical Treatment. Occasionally They
Interfere With CSF Circulation And
Require Decompression.

Sunday, July 28, 13
The Differential Diagnosis
Depends On The Location.
In The Posterior Fossa:
DandyWalker Malformation, Inferior Vermian
Hypoplasia, Mega–cisterna Magna, And Blake’s Pouch
Cysts.
Supratentorial Cysts:
Cavum Veli Interpositi, Aneurysm Of Vein Of Galen,
Hemorrhage, And Cystic Tumors.

53
Sunday, July 28, 13
Prenatal diagnosis and outcome of fetal posterior
fossa fluid collections
G. GANDOLFI COLLEONI et al,
Ultrasound Obstet Gynecol 2012; 39: 625–631

54
Sunday, July 28, 13
105
Fetuses

Blake’s Pouch Cyst
N = 32
Arachnoid Cyst

N=1

Megacisterna Magna
N = 27
Cerebellar Hypoplasia
N=2
Sonographic
diagnoses
were accurate
in 88%
Sunday, July 28, 13

Dandy – Walker Malformation
N=26
Vermian Hypoplasia
N=17

55
✦ Isolated

Cases Of Blake’s Pouch Cyst And
Megacisterna Magna Have An Excellent Prognosis,
With A High Probability Of Intrauterine Resolution
And Normal Intellectual Development In Almost All
Cases.

✦ Dandy

– Walker Malformation And Vermian
Hypoplasia, Even When They Appear Isolated
Antenatally, Are Associated With An Abnormal
Outcome In Half Of Cases.
56

Sunday, July 28, 13
Conclusion
•Black’s	
   Pouch	
   Cyst,	
   DW	
   Malformation,	
   and	
  

Mega-­‐Cisterna	
   Magna	
   Can	
   give	
   Similar	
  
Sonographic	
  features.	
  	
  

•However	
  the	
  prognosis	
  is	
  greatly	
  varialbe.
•Careful	
  Neurosonographic	
  assessment	
  using	
  3	
  
D	
  or	
  Fetal	
  MRI	
  is	
  often	
  Needed

57
Sunday, July 28, 13
Technical Guideline
How do we do it? Practical advice on imaging-based
techniques and investigations

Three dimensional ultrasound
examination of the fetal central
nervous system
Gianluigi Pilu, Tullio Ghi, Angela Carletti,
Maria Segata, Antonella Perolo, Nicola Rizzo
From the Department of Obstetrics and Gynecology
University of Bologna, Italy
Address for correspondence: gianluigi.pilu@unibo.it

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
Sunday, July 28, 13
3D	
   ultrasound	
   is	
   a	
   data	
   set	
  
that	
   contains	
   a	
   large	
   number	
  
o f	
   2 D	
   p l a n e s	
   ( B -­‐ m o d e	
  
images).	
  
e.g.	
   If	
   the	
   page	
   of	
   a	
   book	
   is	
  
one	
   2D	
   plane,	
   then	
   the	
   book	
  
itself	
  is	
  the	
  en3re	
  data	
  set.	
  
The	
   3	
   D	
   probe	
   acquire	
   the	
   data	
   by	
  
moving	
   a	
   B	
   mode	
   transducer	
   within	
   a	
  
housing	
  like	
  a	
  hand	
  held	
  Japanese	
  fan	
  .

Sunday, July 28, 13
Pyramid	
  Of	
  Volume	
  Informa=on

✴ “Walking”	
  through	
  the	
  
volume	
   is	
   similar	
   to	
  
leafing	
   through	
   the	
  
pages	
   of	
   a	
   book	
   i.e.	
  
walking	
   through	
   the	
  
various	
  2D	
  planes	
  that	
  
make	
   up	
   the	
   entire	
  
volume.	
  	
  

✴ The	
  

Volume	
   can	
   be	
  
dissected	
  in	
  any	
  plane,	
  
to	
   get	
   “Multiplanar	
  
Imaging”	
  

Sunday, July 28, 13

the	
   acquired	
   volume	
   unlike	
   the	
  
defined	
  rectangle	
   shape	
   of	
  a	
   book	
  
looks	
  like	
  a	
  pyramid	
  or	
  triangle	
   of	
  
volume	
   informa3on	
   with	
   a	
   broad	
  
base	
  
3D volumes of the fetal brain obtained from
an axial approach: the ‘start’ scan
Cavum septi pellucidi

midline

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
61
Sunday, July 28, 13
midline

A

C
Sunday, July 28, 13

B

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
midline

A

C
Sunday, July 28, 13

B

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
midline

A

B
A and B rotated on Z
plane until midline is
aligned with C plane

C
Sunday, July 28, 13

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
A

B

Corpus callosum + cavum septi pellucidi

Cerebellar vermis

C
Sunday, July 28, 13

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245

Acoustic shadow
midline

Corpus	
  callosum
Cavum	
  sep*	
  pellucidi
midline

Corpus	
  callosum	
  +	
  cavum	
  sep*	
  pellucidi
Originally	
  published	
  in	
  Ultrasound Obstet Gynecol 2007; 30: 233–245
Sunday, July 28, 13

64
Angled	
  Insona3on	
  of	
  Posterior	
  Fossa	
  to	
  
Visualize	
  brain	
  Stem

4v

Brain	
  stem

Cerebellar	
  vermis

Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
Sunday, July 28, 13

65
hemisphere

hemisphere

tentorium

4v
vermis

hemisphere

hemisphere

tentorium

vermis

vermian fissures

4v
Sunday, July 28, 13

Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
body

atrium
Occipital	
  
horn

Temporal	
  horn

Sylvian	
  fissure
Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
Sunday, July 28, 13

67
68
Sunday, July 28, 13
69
Sunday, July 28, 13
70
Sunday, July 28, 13
71
Sunday, July 28, 13
72
Sunday, July 28, 13
73
Sunday, July 28, 13
Agenesis	
  of	
  the	
  
corpus	
  callosum

Normal	
  corpus	
  callosum

3v

Absent	
  corpus	
  callosum

3v

Par3al	
  agenesis

3v

Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
Sunday, July 28, 13

74
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on

Axial view

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on

Cavum	
  Sep3	
  
Pellucidi

Axial view

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on

Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Axial view

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
Cisterna	
  Magna
Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Axial view

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
Cisterna	
  Magna
Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Tentorium

Axial view

Cisterna	
  Magna

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
Cisterna	
  Magna
Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Tentorium

Axial view

Cisterna	
  Magna

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
Cisterna	
  Magna
Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Tentorium

Axial view

Cisterna	
  Magna

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
Cisterna	
  Magna
Cavum	
  Sep3	
  
Pellucidi

Cerebellar	
  vermis

Tentorium

Axial view

Cisterna	
  Magna

SagiGal	
  view

Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012
Sunday, July 28, 13
Applica=on	
  of	
  3	
  D	
  Imaging	
  in	
  
Prenatal	
  diagnosis	
  of	
  Fetal	
  
Posterior	
  Fossa	
  Fluid	
  Collec=on

76
Sunday, July 28, 13
Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  
Collec=ons
Gandolfi	
  Colleoni	
  et	
  al.,	
  UOG	
  2012

Brainstem–vermis and brainstem–tentorium angles allow accurate
categorization of fetal upward rotation of cerebellar vermis
P. VOLPE*, et al

Ultrasound Obstet Gynecol 2012; 39: 632–635

77
Sunday, July 28, 13
Categoriza3on	
  of	
  posterior	
  fossa	
  fluid	
  collec3ons	
  (1)

Sunday, July 28, 13
Categoriza3on	
  of	
  posterior	
  fossa	
  fluid	
  collec3ons	
  (1)
Blake’s	
  pouch	
  cyst
Upward	
  rotation	
  of	
  an	
  intact	
  vermis	
  
with	
  normal	
  torcular

Findings

Megacisterna	
  magna
Cisterna	
  magna	
  >10mm	
  with	
  intact	
  
and	
  normally	
  positioned	
  cerebellum

SagiGal

	
  

Axial

Sunday, July 28, 13

D-­‐W	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  
Upward	
  rotation	
  of	
  the	
  vermis	
  
(normal	
  or	
  hypoplastic)	
  with
elevated	
  torcular
Axial	
  View

• Transverse

Diameter Of
The Cerebellum.
• The Intactness And Size
Of The Vermis.
• The Depth Of The
Cisterna Magna (10 Mm)
Sunday, July 28, 13

79
Cavum	
  Sep3	
  
Pellucidi
The	
  Tentorium:	
  Level	
  	
  

The	
  Vermis:	
  Shape,	
  Size,	
  Fissures

Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento
80
this case
Sunday, July 28, 13 after acquisition of an ultrasound volume starting from an axi
Cavum	
  Sep3	
  
Pellucidi

Brainstem-tentorium
(BT) angle

Brainstem-vermis
Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento
(BV) angle
81

this case after acquisition of an ultrasound volume starting from an axi

Sunday, July 28, 13
1 Measurement of brainstem–vermis and and brainstem–tentorium (BT) angles.median view of the of the fetal obtained (in
(a)
Measurement of brainstem–vermis (BV)(BV) brainstem–tentorium (BT) angles. (a) A(a) A A median viewfetal brain is brain is obta
Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tentorium (BT) angles.
median view of the fetal brain is obtained
e after acquisitionan ultrasound volume starting from from an axial view) and theanatomic landmarks are identified. (b) A (b)
ter acquisition of of an ultrasound volume starting an axial axial and the main main anatomic landmarks are identified. lin
this case after acquisition of an ultrasound volume starting from anview) view) and the main anatomic landmarks are identified. (b) line i
A
angentially to dorsal aspect of the brain stem stema and a line is line is drawn tangentially to the contour of thethe cerebellar
dorsal aspect of of brain and and a second drawn tangentially to the the ventral contour of of the cerebe
gentially to the theto the dorsal aspectthe the brain stemsecondsecond line is drawn tangentially toventralventral contourcerebellar
drawn tangentially
the interposed angle is the BV BV BV angle; the BT angle (2) is measured between the first line andthird line tangential thethe tentoriu
interposed angle (1) (1) is the the angle; the BT (2) is measured between the first first line and a line tangential to to tentorium
vermis; the interposed angle (1) isangle; the BT angleangle (2) is measured between theline and a third a third line tangential to the te

Measurement Of Brainstem–vermis (BV) Angle (1) And
Brainstem–tentorium (BT) In Three Conditions

Blake’s Pouch
Cyst

Cerebellar Vermis
Hypoplasi

Dandy–Walker
Malformation.

The Angles Has The Widest Measurement In DA
Malformation
82

Figure 2 Measurement of brainstem–vermis (BV) angle (1) and brainstem–tentorium (BT) angle (2) in fetuses with: (a) Blake’s pouch cys
Sunday, July 28, 13
Blake’s pouch cyst
Dandy–Walker malformation

Vermian hypoplasia
Dandy–Walker malformation

12
12

7
12

23.0
63.5
34.9
63.5

Brainstem–vermis Angle

2.8
17.6
5.4
17.6

60

40

20

0

0

7.0
15.1

32–52
15.1

51–1

45–66
51–112

Brainstem– Tentorium Angle

Normal

Normal

Blake’s pouch Vermian Dandy–Walker
cyst
hypoplasia malformation

Blake’s pouch Vermian Dandy–Walker
cyst
hypoplasia malformation
Figure 3 Box-and-whisker plot of distribution of brainstem–vermis
angle in controls and in fetuses with upward rotation of the
cerebellar vermis. Medians of distribution line inside each box,
Figure 3 Box-and-whisker plot are indicated by a of brainstem–vermis
th
th
th
th
angle in25 and 75and in fetuses withlimits and 5 and 95 ofpercentiles
controls percentiles by box upward rotation the
by lower and upper bars, respectively.

Brainstem–tentorium angle (°)

Brainstem–tentorium angle (°)

Brainstem–vermis angle (°)

Brainstem–vermis angle (°)

20

67.2 7.1

80

80

40

42.2
52.1
67.2

80

80

60

19–26
45–112
24–40
45–112

60 60

40

40

20

20 Normal

Blake’s pouch
cyst

Normal

Vermian Dandy–Walker
hypoplasia malformation

Blake’s pouch

Vermian

Dandy–Walk

cyst
hypoplasia malformatio
Figure 4 Box-and-whisker plot of distribution of brainstem–
tentorium angle in controls and in fetuses with upward rotation of
the cerebellar vermis. Medians are indicated by a line inside each
Figure 4 Box-and-whisker plot of distribution of brainstem–
box, 25th and 75th percentiles by box limits and 5th and 95th
tentorium angle in controls respectively.
percentiles by lower and upper bars,and in fetuses with upward rotation o

Box-and-whisker plot of distribution of

Box-and-whisker indicated by line inside each of
cerebellar vermis. Medians are plot of a distribution box,
th and 75th percentiles by box limits and 5th and 95th percentiles
25
cerebellar vermis. Medians are in controls and in
brainstem–vermis angle in controls and in thebrainstem– tentorium angleindicated by a line inside each
by lower and upper bars, < 18◦ and a BT angle < 45◦ . The BV
th
had a BV angle respectively.
box, 25th and 75th upward rotation limits andcerebellarth
fetuses with percentiles by box of (BV) and and 95
the 5
fetuses with upward increased inof theofcerebellar Table 2 Statistical comparison ofupper bars, respectively.
angle was significantly rotation each
the three
percentiles by lower and brainstem–vermis
brainstem–tentorium (BT) angles in controls and in fetuses with
subgroups of anomalies
3, Table 2),
angle
vermis. Medians are indicated by a line inside
vermis. Medians and (Figure angle <a45◦theThe BV upward rotation of the cerebellar vermis
had a increasing with18◦ are indicated by condition. The
BV angle < increasing a BT of the line inside
.
severity
each box, 25th and 75th of brainstem–vermis (BV) and
Table 2 Statistical comparison percentiles by box limits
BT angle 25th and similar pattern, of by box
angle was significantly increased percentilesthere was
each box, demonstrated a75th in each but the three
P (Mann–Whitney U-test)
brainstem–tentorium (BT) angles in controls and in fetuses with
more of anomalies (Figure (Figure 4, Table 2).
subgroups overlapping among groups3, Table 2), the angle
th and of the percentiles by lower and upper
th cerebellar vermis
and
upward5
limits and 5th and 95th percentiles by lower Comparison*rotation 95
BV angle
BT angle
increasing with increasing severity of the condition. The
BT and upper S I O N respectively.
angle Sdemonstrated a similar pattern, but there was
D I C U S bars,
more overlapping among groups (Figure 4, Table 2).
Our results suggest that measurement of the BV angle
discriminates
Sunday, July 28, 13 accurately posterior fossa fluid collections

bars, respectively.

Controls vs Blake’s pouch cyst
fetuses
Controls vs Dandy–Walker
Comparison*
fetuses

< 0.00000005 (Mann–Whitney U-test)
P < 0.000005
< 0.00000005 < 0.00000005
BV angle

BT angle
Conclusion
Fetal posterior fossa fluid collections associated
with upward rotation of the cerebellar vermis range
from benign asymptomatic conditions to severe
abnormalities associated with neurological
impairment.
The most frequent of these anomalies, Blake’s
pouch cyst, vermian hypoplasia and Dandy–
Walker malformation, have a similar sonographic
appearance but a very different prognosis
84
Sunday, July 28, 13
In	
  Summary

85
Sunday, July 28, 13
Examination Of The Posterior Fossa And
The Cerebellum
Axial View
Midsagittal Views

86
Sunday, July 28, 13
Prac3cal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  Cys3c	
  like	
  Lesions

Sunday, July 28, 13
Prac3cal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  Cys3c	
  like	
  Lesions
1. Is	
  the	
  Vermis	
  Present?Is	
  the	
  Vermis	
  intact?

Sunday, July 28, 13
Prac3cal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  Cys3c	
  like	
  Lesions
1. Is	
  the	
  Vermis	
  Present?Is	
  the	
  Vermis	
  intact?
2. Is	
  the	
  Toruclar	
  in	
  a	
  normal	
  posi3on	
  (tentorial	
  
Cerebelli)?

Sunday, July 28, 13
Prac3cal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  Cys3c	
  like	
  Lesions
1. Is	
  the	
  Vermis	
  Present?Is	
  the	
  Vermis	
  intact?
2. Is	
  the	
  Toruclar	
  in	
  a	
  normal	
  posi3on	
  (tentorial	
  
Cerebelli)?
3. What	
  is	
  the	
  shape	
  of	
  the	
  cerebellar	
  clee?

Sunday, July 28, 13
Prac3cal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  Cys3c	
  like	
  Lesions
1. Is	
  the	
  Vermis	
  Present?Is	
  the	
  Vermis	
  intact?
2. Is	
  the	
  Toruclar	
  in	
  a	
  normal	
  posi3on	
  (tentorial	
  
Cerebelli)?
3. What	
  is	
  the	
  shape	
  of	
  the	
  cerebellar	
  clee?
4. Brainstem–vermis (BV) Angle And Brainstem–
tentorium (BT) Angle

Sunday, July 28, 13
Blacke’s	
  Pouch	
  Cyst

Cystegacisterna	
  Magna

Ultrasound Obstet Gynecol 2012; 39: 625–631
Published online 14 May 2012 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/uog.11071

Prenatal diagnosis and outcome of fetal posterior fossa fluid
collections
G. GANDOLFI COLLEONI*, E. CONTRO*, A. CARLETTI*, T. GHI*, G. CAMPOBASSO†,
G. REMBOUSKOS†, G. VOLPE‡, G. PILU* and P. VOLPE†

Vermian	
  Hypoplasia

D-­‐W	
  Malforma3on

*Department of Obstetrics and Gynecology, University of Bologna, Bologna, Italy; †Fetal Medicine Unit, Di Venere and Sarcone Hospitals,
ASL Bari, Bari, Italy; ‡Department of Obstetrics and Gynecology, University of Bari, Bari, Italy

K E Y W O R D S: cerebellar anomalies; Dandy–Walker malformation; fetus; megacisterna magna; prenatal diagnosis; ultrasound

ABSTRACT

Cerebellar	
  Hypoplasia
Objective To evaluate the accuracy

of fetal imaging
in differentiating between diagnoses involving posterior
fossa fluid collections and to investigate the postnatal
outcome of affected infants.
Methods This was a retrospective study of fetuses with
posterior fossa fluid collections, carried out between 2001
and 2010 in two referral centers for prenatal diagnosis. All
fetuses underwent multiplanar neurosonography. Parents
were also offered fetal magnetic resonance imaging (MRI)
and karyotyping. Prenatal diagnosis was compared with
autopsy or postnatal MRI findings and detailed follow-up
was attempted by consultation of medical records and
interview with parents and pediatricians.

fluid collections from mid gestation. Blake’s pouch cyst
and megacisterna magna are risk factors for associated
anomalies but when isolated have an excellent prognosis,
with a high probability of intrauterine resolution and
normal intellectual development in almost all cases.
Conversely, Dandy–Walker malformation and vermian
hypoplasia, even when they appear isolated antenatally,
are associated with an abnormal outcome in half of cases.
Copyright © 2012 ISUOG. Published by John Wiley &
Sons, Ltd.

Arachinoid	
  Cyst-­‐Pos	
  Fossa	
  

INTRODUCTION

88
Fluid collections in the fetal posterior fossa encompass
a wide spectrum of different megacisterna magna;
Figure 1 Categorization of posterior fossa fluid collections on ultrasound: (a,b) Blake’s pouch cyst; (c,d) entities, ranging from(e,f) vermian
Sunday, July 28, 13 During the study period, 105 fetuses were examnormal variants to severe anomalies1 . fossa.
hypoplasia; (g,h) Dandy–Walker malformation; (i,j) cerebellar hypoplasia; (k,l) arachnoid cyst of the posterior They may have
Results
Normal

Megacisterna	
  magna

Blake’s	
  pouch	
  cyst
tentorium

Vermian	
  hypoplasia

Dandy-­‐Walker	
  malforma3on

Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
Sunday, July 28, 13

89
Take	
  Home	
  Message
Standard	
  and	
  Fetal	
  
Neurosonography

90
Sunday, July 28, 13
91
Sunday, July 28, 13
✦ examina3on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol

91
Sunday, July 28, 13
✦ examina3on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol

✦ Examina3on	
   should	
   include	
   at	
   least	
   three	
   axial	
  
planes.

91
Sunday, July 28, 13
✦ examina3on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol

✦ Examina3on	
   should	
   include	
   at	
   least	
   three	
   axial	
  
planes.

✦ In	
   Each	
   plane	
  

the	
   defined	
   landmarks	
   should	
  
should	
  be	
  reported	
  as	
  normal	
  or	
  suspicious

91
Sunday, July 28, 13
✦ examina3on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol

✦ Examina3on	
   should	
   include	
   at	
   least	
   three	
   axial	
  
planes.

✦ In	
   Each	
   plane	
  

the	
   defined	
   landmarks	
   should	
  
should	
  be	
  reported	
  as	
  normal	
  or	
  suspicious

✦ In	
  the	
  presence	
  of	
  possible	
  abnormali3es	
  pa3ent	
  
should	
   be	
   referred	
   for	
   detailed	
   neuorsonogram	
  
which	
  include	
  mutli-­‐planner	
  3	
  D	
  Sanning.

91
Sunday, July 28, 13
✦ examina3on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol

✦ Examina3on	
   should	
   include	
   at	
   least	
   three	
   axial	
  
planes.

✦ In	
   Each	
   plane	
  

the	
   defined	
   landmarks	
   should	
  
should	
  be	
  reported	
  as	
  normal	
  or	
  suspicious

✦ In	
  the	
  presence	
  of	
  possible	
  abnormali3es	
  pa3ent	
  
should	
   be	
   referred	
   for	
   detailed	
   neuorsonogram	
  
which	
  include	
  mutli-­‐planner	
  3	
  D	
  Sanning.

✦ 3	
   D	
   scanning	
   with	
   mul3planner	
   analysis	
   offers	
  
comparable	
  analysis	
  to	
  fetal	
  MRI

91
Sunday, July 28, 13
Thanks	
  
92
Sunday, July 28, 13

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Fetal Neurosonogram

  • 1. Fetal  Nuerosonogram   Professor Hassan Nasrat FRCS, FRCOG The Fetal Medicine Clinic The First Clinic JUCOG January 2013 Sunday, July 28, 13
  • 2.  Microcephaly Holoprosencephaly Head normal or small  Chiari  Malforma3on ACC  Dia   Anencephaly Occipital Encephalocele Schizencephaly Schizencephaly Circle of Willis Mallformation Vascular   Malforma3ons SOP Pilu Imaging Findings  Herniated brain tissue  „cyst within the cyst“  Ventriculomegaly 7080%  Microcephaly 25%  Polyhydramnios  Oligohydramnios  Encephalocele PF-­‐Fluid-­‐Cyst CAVE:  Associated with multiple syndroms ( Meckel- Gruber ) Hydrance halus , T 21 Ventriculomegaly Hemimegalencephaly Arachnoid  cyst Hydranecphaly Yong seok et a 2 Sunday, July 28, 13
  • 3. Congenital  CNS  Anomalies o Incidence  in  longtem  studies  about  1  % o  Only  minimal  identified  at  birth   o  Screening  Increases  The  Number  Of   Referred  Cases  For  Evaluation  Of  Suspected   CNS  Anomalies.   o The  CNS  sonographic  appearance  changes   throughout  pregnancy     Sunday, July 28, 13
  • 4. Learning  Objec3ves ✤ Embryonic  development  of  the  CNS   in  relation  to  sonographic  findings ✤ Standard   Sonographic   Examination   of  the  CNS   ✤ Fetal   Neurosonography   and   the     Role  of  3  D     (systemic  approach  to   examination  of  the  Posterior  Fossa) 4 Sunday, July 28, 13
  • 6. At   5th   Week   The   Cells   Destined   To   Form   The   Notochord   Infiltrate   Into   The  Embryonic  Disc.   I t   I n d u c e s   T h e   Overlying   Embryonic   Tissue   To  Thicken  And   Ultimately   Fold   Over   And   Fuse   As   The   The   Fusion   Starts   In   Neural  Tube.   The   Midtrunk   Of   The   E m b r y o   A n d   Subsequently   Extends   To   The   Cranial   And   Caudal  Ends   Neural  Crest Neural  Groove Neural  Plate Sunday, July 28, 13 Ectoderm Neural  Tube
  • 8. Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at 8 the end of 8 weeks of gestation. The development of premature ventricular system is seen. Sunday, July 28, 13
  • 9. Prosencephalon Mesencephalon Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at 8 the end of 8 weeks of gestation. The development of premature ventricular system is seen. Sunday, July 28, 13
  • 10. Normal brain development on the mid-sagittal section between 8 and 12 weeks of gestation). Note the remarkable changing of premature brain 9 appearance. Sunday, July 28, 13
  • 11. AJR:166, AJR:166, Changing  Ultrasound  appearance  of  the   The  Posterior  Fossa  throughout  gesta3on   SONOGRAPHIC February 1996 February 1996 AJR:166, February 1996 ANATOMY SONOGRAPHIC ANATOMY SONOGRAPHIC OF DEVELOPING 433 CEREBELLUM OF DEVELOPING CEREBELLUM ANATOMY OF DEVELOPING CEREBELLUM 433 433 10 C Sunday, C Fig. C C July 28, 13 D 13.-Drawings depicting some relevant features D DD of fetal cerebellar development.
  • 12. The vermis develops superiorly to inferiorly. Hypoplasia or developmental arrest results in varying size deficits of the inferior portion, leaving a relatively square defect that communicates with the fourth ventricle and separates the lower cerebellar hemispheres. 11 Sunday, July 28, 13
  • 13. C D Fig. 13.-Drawings depicting some relevant features of fetal cerebellar development. A, Axial drawing of developing cerebellum at 5 weeks’ gestational age shows that developing cerebellar hemispheres have not yet grown toward midline and thatfourth ventricle is covered only byfourth ventricular roof,which is onlytwo cell layers thickatthis stage of development. B, Sagittal drawing of developing cerebellum at 10 weeks’ gestational age shows small cerebellum located rostrally over fourth ventricle, with caudal fourth ventricle being covered only by thin fourth ventricular roof. C, Sagittal drawing at 16 weeks’ gestational age shows further caudal growth of cerebellum ening 0, of caudal Sagfttal fourth drawing ventricular roof. at 17 weeks’ gestational age shows cerebellum We have shown that the sonognaphic appearance of normal cemebellar development can resemble pathology early in the second trimester. Our findings indicate that the mature relationships of the posterior fossa structures are not established until at least 18 weeks’ gestational age; therefore, the Sunday, July 28, 13 diagnosis prenatal sonographic of Dandy-Walker complex and vermis covering 4. Achinon entire R, Tadmor ten of pregnancy: and vermis over fourth fourth 0. Screening tnansvaginal ventrIcle, teno thic and with thick- ventricle. for fetal anomalies versus transabdominal 1991 1:186-191 during the first tnimesUltra- sonography. sound Obstet Gynecol 5. Nicolaides KH, Azan G, Byrne D, Mansur C, Marks K. Fetal nuchal translucency: ultrasound screening for chromosomal defects in first trimester of pregnancy. BMJ 1992:304:867-869 6. Bronshtein M, Blumenfeld I, Kohn J, Blumenfeld Z. Detection ofcleft lip by early 12
  • 14. of posteriorB, Next caudal image identifies fetus. fossa in 13- to 14-week-old called in stea fourth ventricular roof joining cerebellar hemispheres acquisition Vermis fetus. A, Vermis isand separating identified between cerebellar hemispheres rostrally (arrow). age of posteriorfossacaudally in (arrow) fourth ventricle and cisterna magna. but not B, Next caudal image identifies fourth ventricular roof joining cerebellar hemispheres fetus. Vermis is identified (arrow) and separating fourth ventricle and cisterna magna. but not caudally at this s Effect  of  Gesta=onal  age  (Posterior  Fossa) Fig. 7.-Axial Fig. 7.-A and tenor fossa tenor fossa in 16-weekA and A and B, Caudally, thickto enoug thick enough be v and sagittal and sagittal (B) planes axial sagittal sonograms of posterior fossa in 16-week-old fetus fourth ventricular roof is visualized in both planes (arrow) 13 Sunday, July 28, 13
  • 15. gure 1 Transvaginal scan of a 14-week fetus. (a) Oblique-1 (sagittal) section: the fetus is facing left. The choroid plexus fills the antrum ure 1 Transvaginal The of aa14-week appear Oblique-1 but are normal; (b)the fetus facing left. The choroid plexus fills the antrum Transvaginal scan of 14-week fetus. (a) Oblique-1 (sagittal) section: a Frontal-2 (coronal) The choroid plexus fills the horns ure 1lateral ventricle. scan anterior hornsfetus. (a)prominent, (sagittal) section: the fetus isis facing left. section through the anterior antrum the thelateral ventricle. The anterior horns appear normal for this are normal;age; ahowever, this same sonographic picture at anterior horn he lateral ventricle. The anterior horns appear prominent, but are normal; (b) a Frontal-2 (coronal) section through the 20 weeks or the lateral ventricles. The anterior horns are prominent, but gestational (b) Frontal-2 (coronal) section through the anterior horns the is consistent with ventriculomegaly or hydrocephalus this gestational age; however, this same sonographic picture 20 weeks or he lateral ventricles. The anterior horns are normal for ore lateral ventricles. The anterior horns are normal for this gestational age; however, this same sonographic picture atat 20 weeks o re is consistent with ventriculomegaly or hydrocephalus re is consistent with ventriculomegaly or hydrocephalus Effect  Of  Scanning  Level  (Posterior  Fossa) Higher Still gure 2Lower-most Section (horizontal) views through the posterior fossa. (a) This is the lower-most section (see insert). The Three serial, almost axial Somewhat Higher ure ermis2 Three serial, almost axial (horizontal) views through fourth ventriclefossa. (a) a widethe this gestational age, normal) median gure2 appears to be open (arrow) and communicates with the the posterior fossa. (a) This isis (at lower-most section (see insert). The Three serial, almost axial (horizontal) views through the posterior through This the lower-most section (see insert). Th mis appears to be open (arrow)and communicates with rightfourth ventricle the cerebellar (at this gestational age, normal) median perture (foramenbeopen (arrow) (b) somewhat higher,withthe fourth left sides ofthrough a widehemispheres appear closer to eachmedia rmis appears to of Magendie); and communicates the the and ventricle through a wide (at this gestational age, normal) other rture (foramen of Magendie); (b) somewhat higher, rrow); (foramenof Magendie); (b) defect’ is seen and theright and left sides appears as a discrete No ‘vermiancloser to each othe (c) higher still, Appears To entity. C, appear ertureThe Vermisno ‘vermian somewhat higher, the fourth and left sides of the cerebellar hemispherescerebellum Defect’other right ventricle (4) of the cerebellar hemispheres appear closer to each Thethefourth ventricle (4) appears asas a discrete entity. C, cerebellum Right And Left Sides a discrete entity. C, cerebellum row); (c) higher still, no ‘vermian defect’ is seen and the fourth ventricle (4) appears row); (c) higher still, (arrow) And is seen and Be Open no ‘vermian defect’ Is Seen And The Of The Cerebellar Communicates With The eduncular cistern (cisterna magna) and theHemispheres its closest anatomic structures, namely the cavum septi fourth ventriFourth Ventricle (4) Appear its closestand the pericallosal artery, follow acavum septi anatomic structures, namely the well-known duncular ucistern(cisterna magna) land week,fourthventricistern (cisterna n t r i c e the fourth‘normal’ pellucidi anatomic structures, namely As cavum sep e. Later, r t h its closest the duncularafter the 16th postmenstrual the this ventriFo Ve magna) and Closer Appears A pellucidi and the pericallosal artery, reach a developmenLater, after the 16th postmenstrualand developmentTo Each Other thepericallosal artery, follow a a well-know week, this ‘normal’ developmental timetable. They do not follow well-known pen space narrows as postmenstrual week, this ‘normal’ of pellucidi and . Later, after the 16th the growth Through A giving en vermis narrows Wide growth and development of (arrow); tal stage that allows for Discrete reach a developmene space narrows as the rise to and development of developmental timetable. They do notEntity. a developmen developmental timetable. sonographic imaging until postThey do not reach en space progress,as the growth the median aperture oramen progress, giving rise to the median aperture e vermis of Magendie) (Figureto the median aperture vermis progress, giving rise 2). Again, this normal ramen of Magendie) be interpretedAgain, this normal onographic finding may (Figure 2). Again, this normal ramen of Magendie) (Figure 2). by those unfamiliar Sunday, finding may nographic July 28, 13 be interpreted by those unfamiliar tal stage that allows for To search for their presence before 14 menstrual that allows forsonographic imaging until posttal stage weeks 18–19. sonographic imaging until pos menstrual weeks 18–19. To search for their presence before they reach weeks 18–19. To search fordevelopment would menstrual this critical stage in their their presence befor they reach this critical stage in their development would
  • 16. Effect  of  Gesta=onal  age  (Lateral  Ventricles) 15 Sunday, July 28, 13
  • 17. The fetal cerebellum Pitfalls in diagnosis Hypoplasia Or Dysplasia Should Not Be Diagnosed Prior To 18 Weeks, Before Vermian Development Is Complete. An Abnormally Steep Scanning Angle May Mimic A Prominent Cleft Between The Lower Portions Of The Cerebellar Hemispheres. 16 Sunday, July 28, 13
  • 18. Conclusion •TheCNS   displays   remarkable   embryological   and   developmental   changes  throughout  gestation. •Standard  Approach  of  examination  and   evaluation   of   the   CNS   Should   Be   Followed 17 Sunday, July 28, 13
  • 19. Standard Sonographic Examination of the FEtal CNS 18 Sunday, July 28, 13
  • 21. Sonography  of  the  CNS Basic  Examination “Neurosongram” Sunday, July 28, 13
  • 22. Planes  of  Basic  Examina3on Axial  Planes Sunday, July 28, 13 Sagibal  Planes
  • 25. Axial  Planes a:  Transventricular b:  Transthalamic   Sunday, July 28, 13
  • 26. Axial  Planes a:  Transventricular b:  Transthalamic   C:  Transcerebeller   Sunday, July 28, 13
  • 28. The  Transventricular  plane Frontal  hones Sunday, July 28, 13
  • 29. The  Transventricular  plane Frontal  hones Atrium Sunday, July 28, 13
  • 30. The  Transventricular  plane Frontal  hones Atrium Sunday, July 28, 13 Choroid   Plexus
  • 31. The  Transventricular  plane Cavum  Sep3  Pellucidi Frontal  hones Atrium Sunday, July 28, 13 Choroid   Plexus
  • 32. The  Transthalamic    Plane Sunday, July 28, 13
  • 33. The  Transthalamic    Plane Thalami Sunday, July 28, 13
  • 34. The  Transthalamic    Plane Thalami Hyppocamas   Gyrus Sunday, July 28, 13
  • 35. The  Transcerebeller    plane T T Sunday, July 28, 13
  • 36. The  Transcerebeller    plane T T Cavum  Sep3  Pellucidi Sunday, July 28, 13
  • 37. The  Transcerebeller    plane Frontal  hones T T Cavum  Sep3  Pellucidi Sunday, July 28, 13
  • 38. The  Transcerebeller    plane Cerebellum Frontal  hones T T Cavum  Sep3  Pellucidi Sunday, July 28, 13
  • 39. The  Transcerebeller    plane Cerebellar  vermis Cerebellum Frontal  hones T T Cavum  Sep3  Pellucidi Sunday, July 28, 13
  • 40. The  Transcerebeller    plane Cerebellar  vermis Cerebellum Frontal  hones T T Cavum  Sep3  Pellucidi Sunday, July 28, 13 Cistrerna  Magna 2-­‐10  mm
  • 41. Sagibal  Planes     Sunday, July 28, 13
  • 42. Sagibal  Planes     A:  The  Midsagittal   Plan Sunday, July 28, 13
  • 43. Sagibal  Planes     b:  Parasgittal  plane A:  The  Midsagittal   Plan Sunday, July 28, 13
  • 44. Mid  SagiGal  Plane   Corpus  Callosum Cavum  Sep3  Pellucidi Cerebellum 4th  V 27 Sunday, July 28, 13
  • 45. Mid  SagiGal  Plane   27 Sunday, July 28, 13
  • 47. The  Corpus  Callosum Lateral  Ventricles Splenium Corpus  Callosum Thalamus hypothalamus Third  Ventricle Pituitary Fourth  ventricle midbrain 28 Sunday, July 28, 13
  • 49. Basic Examniation Checklist Head + Neck Midline & Falx Cavum septi pellucidi Lateral cerebral ventricls Choroid Plexus Cerebellum Cisterna magna 30 Sunday, July 28, 13
  • 50. Main  Abnormali3es  can  be   Suspected  on  Basic  Planes 31 Sunday, July 28, 13
  • 51.  Holoprosencephaly Microcephaly Head normal or small  Chiari  Malforma3on ACC  Dia   Anencephaly Occipital Encephalocele Schizencephaly Schizencephaly Circle of Willis Mallformation Vascular   Malforma3ons SOP Pilu Imaging Findings  Herniated brain tissue  „cyst within the cyst“  Ventriculomegaly 7080%  Microcephaly 25%  Polyhydramnios  Oligohydramnios  Encephalocele PF-­‐Fluid-­‐Cyst CAVE:  Associated with multiple syndroms ( Meckel- Gruber ) Hydrance halus , T 21 Ventriculomegaly Hemimegalencephaly Arachnoid  cyst Hydranecphaly Yong seok et a 32 Sunday, July 28, 13
  • 52.  Holoprosencephaly Microcephaly Head normal or small  Chiari  Malforma3on ACC  Dia   Anencephaly Occipital Encephalocele Schizencephaly Schizencephaly Circle of Willis Mallformation Vascular   Malforma3ons SOP Pilu Imaging Findings  Herniated brain tissue  „cyst within the cyst“  Ventriculomegaly 7080%  Microcephaly 25%  Polyhydramnios  Oligohydramnios  Encephalocele PF-­‐Fluid-­‐Cyst CAVE:  Associated with multiple syndroms ( Meckel- Gruber ) Hydrance halus , T 21 Ventriculomegaly Hemimegalencephaly Arachnoid  cyst Hydranecphaly Yong seok et a 32 Sunday, July 28, 13
  • 53.  Holoprosencephaly Microcephaly Head normal or small  Chiari  Malforma3on ACC  Dia   Anencephaly Occipital Encephalocele Schizencephaly Schizencephaly Circle of Willis Mallformation Vascular   Malforma3ons SOP Pilu Imaging Findings  Herniated brain tissue  „cyst within the cyst“  Ventriculomegaly 7080%  Microcephaly 25%  Polyhydramnios  Oligohydramnios  Encephalocele PF-­‐Fluid-­‐Cyst CAVE:  Associated with multiple syndroms ( Meckel- Gruber ) Hydrance halus , T 21 Ventriculomegaly Hemimegalencephaly Arachnoid  cyst Hydranecphaly Yong seok et a 32 Sunday, July 28, 13
  • 54.  Holoprosencephaly Microcephaly Head normal or small  Chiari  Malforma3on ACC  Dia   Anencephaly Occipital Encephalocele Schizencephaly Schizencephaly Circle of Willis Mallformation Vascular   Malforma3ons SOP Pilu Imaging Findings  Herniated brain tissue  „cyst within the cyst“  Ventriculomegaly 7080%  Microcephaly 25%  Polyhydramnios  Oligohydramnios  Encephalocele PF-­‐Fluid-­‐Cyst CAVE:  Associated with multiple syndroms ( Meckel- Gruber ) Hydrance halus , T 21 Ventriculomegaly Hemimegalencephaly Arachnoid  cyst Hydranecphaly Yong seok et a 32 Sunday, July 28, 13
  • 55. •Ventriculomegaly  (hydrocephalus) •Absent  Cavum  Septum  Pellucidum •Agenesis  of  the  Corpus  Callosum •Fluid  Collection  in  the  posterior  fossa 33 Sunday, July 28, 13
  • 56. Ventriculomegaly  (hydrocephalus) Mild  10  –  15  mm Low  Risk   mean  =  6-­‐8   mm Severe  >  15  mm   High  Risk   (<  10  mm  is  normal).    Independent   of  gesta7onal  age   Sunday, July 28, 13
  • 57. Pathogenesis:  Ventriculomegaly Lee  Lateral  Ventricle Right  Lateral  Ventricle Foramen  of  Monro   Aqueduct  of  Sylvius 4th    Ventricle 3rd    Ventricle Cisterna  Magna 35 Sunday, July 28, 13
  • 58. Absent  CSP •Square  Shaped,  Interrupts  and  Fills  The  Space  Between  The  Frontal  Horns •The  CSP:  Becomes  Visible  At  16    Weeks,  Obliterate  Near  Term Sunday, July 28, 13
  • 59. Absent  CSP Cavum  Sep3  Pellucidi •Square  Shaped,  Interrupts  and  Fills  The  Space  Between  The  Frontal  Horns •The  CSP:  Becomes  Visible  At  16    Weeks,  Obliterate  Near  Term Sunday, July 28, 13
  • 60. Absent  CSP A   rare   finding   usually   discovered   Postnatally   in   children  evaluated  for  developmental  delay. Associated  with  various  brain  malformations: agenesis  of  the  corpus  callosum Holoprosencephaly. Setpo-­‐optic  dysplasia. Secondary  to  disruptive  process:  Hydrocephalus,   Chiari  II  malformation,  hydranecephaly.   Sunday, July 28, 13
  • 61. Agenesis  of  the  Corpus  Callosum     38 Sunday, July 28, 13
  • 62. 21-­‐week  Fetus  With  Par=al  Agenesis  Of  The  Corpus  Callosum Only   The   Rostrum   (1),   Genu   (2)   And   Body   (3)   Are   Visible;   The   Splenium   Is   Missing.  The  Corpus  Callosum  Is  Short  Posteriorly  And  Does  Not   Seem   To   Overlay   The  Quadrigeminal  Plate Sunday, July 28, 13
  • 63. Outcome  of  fetal  ACC  Va r i e s   b e t we e n   co m p l e te l y   a sy m p to m a 3 c   appearance  and  severe  neurologic  problems  50   –   100   %   of   isolated   cases   will   have   normal   neurological   development   at   3-­‐11   years   but   Poor   prognosis  with  associated  anomalies   Progressive  decline  in  intellect  over  the  years   Most  need  special  educa3on   Long-­‐term  follow-­‐up  of  children  with  prenatally  diagnosed  agenesis  of  corpus  callosum  (ACC)   J.  H.  Stupin  et  al,  USOG,  32,  2008 Sunday, July 28, 13
  • 64. Fluid  Collec3on  in  the  Posterior  Fossa 41 Sunday, July 28, 13
  • 65. Fluid  Collec3on  in  the  Posterior  Fossa •Megacisterna Magna 41 Sunday, July 28, 13
  • 66. Fluid  Collec3on  in  the  Posterior  Fossa •Megacisterna Magna •Blak’s Pouch Cyst 41 Sunday, July 28, 13
  • 67. Fluid  Collec3on  in  the  Posterior  Fossa •Megacisterna Magna •D-W Malformation &DW- Variant •Blak’s Pouch Cyst 41 Sunday, July 28, 13
  • 68. Fluid  Collec3on  in  the  Posterior  Fossa •Megacisterna Magna •Blak’s Pouch Cyst •D-W Malformation &DW- Variant •Arachnoid Cyst 41 Sunday, July 28, 13
  • 69. Anomalies Of The Meninges •Megacisterna Magna •Blak’s Pouch Cyst Anomalies Cerebellum •D-W Malformation &DW- Variant •Arachnoid Cyst 42 Sunday, July 28, 13
  • 70. Mega–Cisterna Magna An Enlargement Of The Cisterna Magna Beyond 10 Mm With Intact Vermis 43 Sunday, July 28, 13
  • 71. Pathogenesis: Mega Cisterna Magna Lateral  Ventricle Third   Ventricle Cerebral  Aqueduct Choriod  Plexus Fourth  Ventricle 44 Sunday, July 28, 13
  • 72. Pathogenesis: Mega Cisterna Magna The Foramina Of Lateral  Ventricle Luschka And Magendie Fenestrate Delayed Third   Ventricle Cerebral  Aqueduct Choriod  Plexus Fourth  Ventricle 44 Sunday, July 28, 13
  • 73. Prognosis: • Isolated Cases: (97%-100%) Are Normal. • If Not Isolated: Only 11% Have Normal Outcome. Nonisolated Cases Have VM, Congenital Infection, Or Karyotype Abnormalities. A Large Cisterna Magna Require Careful Search For Other Abnormalities. 45 Sunday, July 28, 13
  • 75. Pathogenesis: Blake’s Pouch Cyst Nonfenestration of the foramina of Luschka and Lateral  Ventricle Magendie leads to dilatation of the fourth ventricle and and elevation of the vermis away from the brain stem. Third   Ventricle Cerebral  Aqueduct Choriod  Plexus Fourth  Ventricle There is no communication between the cyst and the subarachnoid space 47 Sunday, July 28, 13
  • 76. Pathogenesis: Blake’s Pouch Cyst Nonfenestration of the foramina of Luschka and Lateral  Ventricle Magendie leads to dilatation of the fourth ventricle and and elevation of the vermis away from the brain stem. Third   Ventricle Cerebral  Aqueduct Choriod  Plexus Fourth  Ventricle There is no communication between the cyst and the subarachnoid space 47 Sunday, July 28, 13
  • 77. Dandy-Walker Malformation A Spectrum Of Anomalies Of The Posterior Fossa. • Dandy-Walker Malformation: ✦Increase Of The Posterior Fossa, ✦Complete Or Partially Agenesis Of The Cerebellar Vermis, ✦A Tentorium Elevation • Variant Of Dandy-Walker: ✦Hypoplasia Of The Cerebellar Vermis In Different Degrees With Or Without Increase Of The Posterior Fossa. 48 Sunday, July 28, 13
  • 78. ctions Dandy-Walker Malformation Elevated tentorium and high position of the torcula Small, rotated, raised, or absent vermis Cystic dilation of the fourth ventricle communicating with a posterior fossa fluid space 49 Sunday, July 28, 13
  • 79. The Prognosis : Better In Isolated DWS. Karyotype Abnormalities In About 15%. Neonatal Mortality: 12% To 55%. Neonatal Morbidity: •Intelligence Is Normal In About 40% •Borderline In 20% •Subnormal In 40%. 50 Sunday, July 28, 13
  • 80. igure 2 2 Blake’s Pouch Cyst the torcular Herophili (arrows) inferred the Dandy–Walker Malformation Figure The position ofof torcular Herophili (arrows) is is inferre The position Normal The Torcular Is Found In A The Torcular Is n ultrasound by the direction ofof the tentorium DisplacedIn In (a) th tentorium cerebelli. Higher Position, At About The Same Level on ultrasound by the direction the Than Usual, Indicating That Thisthe cerebelli. (a) As The found Insertion Of The orcular isSitefound in normal position, A at about the same level as torcular is OfOnin a Posterior position, about the same level as a normal Is at Neck Muscles The he site ofof insertion of the neck muscles on the posterior skull; thi insertion of the neck muscles on the posterior skull; this Skull the site 51 a Blake’s13pouch cyst. In (b) the torcular is displaced higher than is a Blake’s pouch cyst. In (b) the torcular is displaced higher than Sunday, July 28,
  • 81. Arachnoid Cysts • Are Benign, Noncommunicating Fluid Collections Within Arachnoid Membranes. • Location: Intracranially And In The Spinal Canal. • Order Of Frequency Are The Sylvian Fissure Or Temporal Fossa, Posterior Fossa, Over The Cerebral Convexity, And Midline Supratentorial, • Most Appear Stable And Require No Surgical Treatment. Occasionally They Interfere With CSF Circulation And Require Decompression. Sunday, July 28, 13
  • 82. The Differential Diagnosis Depends On The Location. In The Posterior Fossa: DandyWalker Malformation, Inferior Vermian Hypoplasia, Mega–cisterna Magna, And Blake’s Pouch Cysts. Supratentorial Cysts: Cavum Veli Interpositi, Aneurysm Of Vein Of Galen, Hemorrhage, And Cystic Tumors. 53 Sunday, July 28, 13
  • 83. Prenatal diagnosis and outcome of fetal posterior fossa fluid collections G. GANDOLFI COLLEONI et al, Ultrasound Obstet Gynecol 2012; 39: 625–631 54 Sunday, July 28, 13
  • 84. 105 Fetuses Blake’s Pouch Cyst N = 32 Arachnoid Cyst N=1 Megacisterna Magna N = 27 Cerebellar Hypoplasia N=2 Sonographic diagnoses were accurate in 88% Sunday, July 28, 13 Dandy – Walker Malformation N=26 Vermian Hypoplasia N=17 55
  • 85. ✦ Isolated Cases Of Blake’s Pouch Cyst And Megacisterna Magna Have An Excellent Prognosis, With A High Probability Of Intrauterine Resolution And Normal Intellectual Development In Almost All Cases. ✦ Dandy – Walker Malformation And Vermian Hypoplasia, Even When They Appear Isolated Antenatally, Are Associated With An Abnormal Outcome In Half Of Cases. 56 Sunday, July 28, 13
  • 86. Conclusion •Black’s   Pouch   Cyst,   DW   Malformation,   and   Mega-­‐Cisterna   Magna   Can   give   Similar   Sonographic  features.     •However  the  prognosis  is  greatly  varialbe. •Careful  Neurosonographic  assessment  using  3   D  or  Fetal  MRI  is  often  Needed 57 Sunday, July 28, 13
  • 87. Technical Guideline How do we do it? Practical advice on imaging-based techniques and investigations Three dimensional ultrasound examination of the fetal central nervous system Gianluigi Pilu, Tullio Ghi, Angela Carletti, Maria Segata, Antonella Perolo, Nicola Rizzo From the Department of Obstetrics and Gynecology University of Bologna, Italy Address for correspondence: gianluigi.pilu@unibo.it Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 Sunday, July 28, 13
  • 88. 3D   ultrasound   is   a   data   set   that   contains   a   large   number   o f   2 D   p l a n e s   ( B -­‐ m o d e   images).   e.g.   If   the   page   of   a   book   is   one   2D   plane,   then   the   book   itself  is  the  en3re  data  set.   The   3   D   probe   acquire   the   data   by   moving   a   B   mode   transducer   within   a   housing  like  a  hand  held  Japanese  fan  . Sunday, July 28, 13
  • 89. Pyramid  Of  Volume  Informa=on ✴ “Walking”  through  the   volume   is   similar   to   leafing   through   the   pages   of   a   book   i.e.   walking   through   the   various  2D  planes  that   make   up   the   entire   volume.     ✴ The   Volume   can   be   dissected  in  any  plane,   to   get   “Multiplanar   Imaging”   Sunday, July 28, 13 the   acquired   volume   unlike   the   defined  rectangle   shape   of  a   book   looks  like  a  pyramid  or  triangle   of   volume   informa3on   with   a   broad   base  
  • 90. 3D volumes of the fetal brain obtained from an axial approach: the ‘start’ scan Cavum septi pellucidi midline Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 61 Sunday, July 28, 13
  • 91. midline A C Sunday, July 28, 13 B Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
  • 92. midline A C Sunday, July 28, 13 B Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
  • 93. midline A B A and B rotated on Z plane until midline is aligned with C plane C Sunday, July 28, 13 Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
  • 94. A B Corpus callosum + cavum septi pellucidi Cerebellar vermis C Sunday, July 28, 13 Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 Acoustic shadow
  • 95. midline Corpus  callosum Cavum  sep*  pellucidi midline Corpus  callosum  +  cavum  sep*  pellucidi Originally  published  in  Ultrasound Obstet Gynecol 2007; 30: 233–245 Sunday, July 28, 13 64
  • 96. Angled  Insona3on  of  Posterior  Fossa  to   Visualize  brain  Stem 4v Brain  stem Cerebellar  vermis Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 Sunday, July 28, 13 65
  • 97. hemisphere hemisphere tentorium 4v vermis hemisphere hemisphere tentorium vermis vermian fissures 4v Sunday, July 28, 13 Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
  • 98. body atrium Occipital   horn Temporal  horn Sylvian  fissure Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 Sunday, July 28, 13 67
  • 105. Agenesis  of  the   corpus  callosum Normal  corpus  callosum 3v Absent  corpus  callosum 3v Par3al  agenesis 3v Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 Sunday, July 28, 13 74
  • 106. Normal  Posterior  Fossa  At  Midgesta=on Axial view SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 107. Normal  Posterior  Fossa  At  Midgesta=on Cavum  Sep3   Pellucidi Axial view SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 108. Normal  Posterior  Fossa  At  Midgesta=on Cavum  Sep3   Pellucidi Cerebellar  vermis Axial view SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 109. Normal  Posterior  Fossa  At  Midgesta=on Cisterna  Magna Cavum  Sep3   Pellucidi Cerebellar  vermis Axial view SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 110. Normal  Posterior  Fossa  At  Midgesta=on Cisterna  Magna Cavum  Sep3   Pellucidi Cerebellar  vermis Tentorium Axial view Cisterna  Magna SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 111. Normal  Posterior  Fossa  At  Midgesta=on Cisterna  Magna Cavum  Sep3   Pellucidi Cerebellar  vermis Tentorium Axial view Cisterna  Magna SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 112. Normal  Posterior  Fossa  At  Midgesta=on Cisterna  Magna Cavum  Sep3   Pellucidi Cerebellar  vermis Tentorium Axial view Cisterna  Magna SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 113. Normal  Posterior  Fossa  At  Midgesta=on Cisterna  Magna Cavum  Sep3   Pellucidi Cerebellar  vermis Tentorium Axial view Cisterna  Magna SagiGal  view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  • 114. Applica=on  of  3  D  Imaging  in   Prenatal  diagnosis  of  Fetal   Posterior  Fossa  Fluid  Collec=on 76 Sunday, July 28, 13
  • 115. Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid   Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Brainstem–vermis and brainstem–tentorium angles allow accurate categorization of fetal upward rotation of cerebellar vermis P. VOLPE*, et al Ultrasound Obstet Gynecol 2012; 39: 632–635 77 Sunday, July 28, 13
  • 116. Categoriza3on  of  posterior  fossa  fluid  collec3ons  (1) Sunday, July 28, 13
  • 117. Categoriza3on  of  posterior  fossa  fluid  collec3ons  (1) Blake’s  pouch  cyst Upward  rotation  of  an  intact  vermis   with  normal  torcular Findings Megacisterna  magna Cisterna  magna  >10mm  with  intact   and  normally  positioned  cerebellum SagiGal   Axial Sunday, July 28, 13 D-­‐W                                         Upward  rotation  of  the  vermis   (normal  or  hypoplastic)  with elevated  torcular
  • 118. Axial  View • Transverse Diameter Of The Cerebellum. • The Intactness And Size Of The Vermis. • The Depth Of The Cisterna Magna (10 Mm) Sunday, July 28, 13 79
  • 119. Cavum  Sep3   Pellucidi The  Tentorium:  Level     The  Vermis:  Shape,  Size,  Fissures Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento 80 this case Sunday, July 28, 13 after acquisition of an ultrasound volume starting from an axi
  • 120. Cavum  Sep3   Pellucidi Brainstem-tentorium (BT) angle Brainstem-vermis Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento (BV) angle 81 this case after acquisition of an ultrasound volume starting from an axi Sunday, July 28, 13
  • 121. 1 Measurement of brainstem–vermis and and brainstem–tentorium (BT) angles.median view of the of the fetal obtained (in (a) Measurement of brainstem–vermis (BV)(BV) brainstem–tentorium (BT) angles. (a) A(a) A A median viewfetal brain is brain is obta Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tentorium (BT) angles. median view of the fetal brain is obtained e after acquisitionan ultrasound volume starting from from an axial view) and theanatomic landmarks are identified. (b) A (b) ter acquisition of of an ultrasound volume starting an axial axial and the main main anatomic landmarks are identified. lin this case after acquisition of an ultrasound volume starting from anview) view) and the main anatomic landmarks are identified. (b) line i A angentially to dorsal aspect of the brain stem stema and a line is line is drawn tangentially to the contour of thethe cerebellar dorsal aspect of of brain and and a second drawn tangentially to the the ventral contour of of the cerebe gentially to the theto the dorsal aspectthe the brain stemsecondsecond line is drawn tangentially toventralventral contourcerebellar drawn tangentially the interposed angle is the BV BV BV angle; the BT angle (2) is measured between the first line andthird line tangential thethe tentoriu interposed angle (1) (1) is the the angle; the BT (2) is measured between the first first line and a line tangential to to tentorium vermis; the interposed angle (1) isangle; the BT angleangle (2) is measured between theline and a third a third line tangential to the te Measurement Of Brainstem–vermis (BV) Angle (1) And Brainstem–tentorium (BT) In Three Conditions Blake’s Pouch Cyst Cerebellar Vermis Hypoplasi Dandy–Walker Malformation. The Angles Has The Widest Measurement In DA Malformation 82 Figure 2 Measurement of brainstem–vermis (BV) angle (1) and brainstem–tentorium (BT) angle (2) in fetuses with: (a) Blake’s pouch cys Sunday, July 28, 13
  • 122. Blake’s pouch cyst Dandy–Walker malformation Vermian hypoplasia Dandy–Walker malformation 12 12 7 12 23.0 63.5 34.9 63.5 Brainstem–vermis Angle 2.8 17.6 5.4 17.6 60 40 20 0 0 7.0 15.1 32–52 15.1 51–1 45–66 51–112 Brainstem– Tentorium Angle Normal Normal Blake’s pouch Vermian Dandy–Walker cyst hypoplasia malformation Blake’s pouch Vermian Dandy–Walker cyst hypoplasia malformation Figure 3 Box-and-whisker plot of distribution of brainstem–vermis angle in controls and in fetuses with upward rotation of the cerebellar vermis. Medians of distribution line inside each box, Figure 3 Box-and-whisker plot are indicated by a of brainstem–vermis th th th th angle in25 and 75and in fetuses withlimits and 5 and 95 ofpercentiles controls percentiles by box upward rotation the by lower and upper bars, respectively. Brainstem–tentorium angle (°) Brainstem–tentorium angle (°) Brainstem–vermis angle (°) Brainstem–vermis angle (°) 20 67.2 7.1 80 80 40 42.2 52.1 67.2 80 80 60 19–26 45–112 24–40 45–112 60 60 40 40 20 20 Normal Blake’s pouch cyst Normal Vermian Dandy–Walker hypoplasia malformation Blake’s pouch Vermian Dandy–Walk cyst hypoplasia malformatio Figure 4 Box-and-whisker plot of distribution of brainstem– tentorium angle in controls and in fetuses with upward rotation of the cerebellar vermis. Medians are indicated by a line inside each Figure 4 Box-and-whisker plot of distribution of brainstem– box, 25th and 75th percentiles by box limits and 5th and 95th tentorium angle in controls respectively. percentiles by lower and upper bars,and in fetuses with upward rotation o Box-and-whisker plot of distribution of Box-and-whisker indicated by line inside each of cerebellar vermis. Medians are plot of a distribution box, th and 75th percentiles by box limits and 5th and 95th percentiles 25 cerebellar vermis. Medians are in controls and in brainstem–vermis angle in controls and in thebrainstem– tentorium angleindicated by a line inside each by lower and upper bars, < 18◦ and a BT angle < 45◦ . The BV th had a BV angle respectively. box, 25th and 75th upward rotation limits andcerebellarth fetuses with percentiles by box of (BV) and and 95 the 5 fetuses with upward increased inof theofcerebellar Table 2 Statistical comparison ofupper bars, respectively. angle was significantly rotation each the three percentiles by lower and brainstem–vermis brainstem–tentorium (BT) angles in controls and in fetuses with subgroups of anomalies 3, Table 2), angle vermis. Medians are indicated by a line inside vermis. Medians and (Figure angle <a45◦theThe BV upward rotation of the cerebellar vermis had a increasing with18◦ are indicated by condition. The BV angle < increasing a BT of the line inside . severity each box, 25th and 75th of brainstem–vermis (BV) and Table 2 Statistical comparison percentiles by box limits BT angle 25th and similar pattern, of by box angle was significantly increased percentilesthere was each box, demonstrated a75th in each but the three P (Mann–Whitney U-test) brainstem–tentorium (BT) angles in controls and in fetuses with more of anomalies (Figure (Figure 4, Table 2). subgroups overlapping among groups3, Table 2), the angle th and of the percentiles by lower and upper th cerebellar vermis and upward5 limits and 5th and 95th percentiles by lower Comparison*rotation 95 BV angle BT angle increasing with increasing severity of the condition. The BT and upper S I O N respectively. angle Sdemonstrated a similar pattern, but there was D I C U S bars, more overlapping among groups (Figure 4, Table 2). Our results suggest that measurement of the BV angle discriminates Sunday, July 28, 13 accurately posterior fossa fluid collections bars, respectively. Controls vs Blake’s pouch cyst fetuses Controls vs Dandy–Walker Comparison* fetuses < 0.00000005 (Mann–Whitney U-test) P < 0.000005 < 0.00000005 < 0.00000005 BV angle BT angle
  • 123. Conclusion Fetal posterior fossa fluid collections associated with upward rotation of the cerebellar vermis range from benign asymptomatic conditions to severe abnormalities associated with neurological impairment. The most frequent of these anomalies, Blake’s pouch cyst, vermian hypoplasia and Dandy– Walker malformation, have a similar sonographic appearance but a very different prognosis 84 Sunday, July 28, 13
  • 125. Examination Of The Posterior Fossa And The Cerebellum Axial View Midsagittal Views 86 Sunday, July 28, 13
  • 126. Prac3cal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  Cys3c  like  Lesions Sunday, July 28, 13
  • 127. Prac3cal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  Cys3c  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? Sunday, July 28, 13
  • 128. Prac3cal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  Cys3c  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posi3on  (tentorial   Cerebelli)? Sunday, July 28, 13
  • 129. Prac3cal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  Cys3c  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posi3on  (tentorial   Cerebelli)? 3. What  is  the  shape  of  the  cerebellar  clee? Sunday, July 28, 13
  • 130. Prac3cal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  Cys3c  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posi3on  (tentorial   Cerebelli)? 3. What  is  the  shape  of  the  cerebellar  clee? 4. Brainstem–vermis (BV) Angle And Brainstem– tentorium (BT) Angle Sunday, July 28, 13
  • 131. Blacke’s  Pouch  Cyst Cystegacisterna  Magna Ultrasound Obstet Gynecol 2012; 39: 625–631 Published online 14 May 2012 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/uog.11071 Prenatal diagnosis and outcome of fetal posterior fossa fluid collections G. GANDOLFI COLLEONI*, E. CONTRO*, A. CARLETTI*, T. GHI*, G. CAMPOBASSO†, G. REMBOUSKOS†, G. VOLPE‡, G. PILU* and P. VOLPE† Vermian  Hypoplasia D-­‐W  Malforma3on *Department of Obstetrics and Gynecology, University of Bologna, Bologna, Italy; †Fetal Medicine Unit, Di Venere and Sarcone Hospitals, ASL Bari, Bari, Italy; ‡Department of Obstetrics and Gynecology, University of Bari, Bari, Italy K E Y W O R D S: cerebellar anomalies; Dandy–Walker malformation; fetus; megacisterna magna; prenatal diagnosis; ultrasound ABSTRACT Cerebellar  Hypoplasia Objective To evaluate the accuracy of fetal imaging in differentiating between diagnoses involving posterior fossa fluid collections and to investigate the postnatal outcome of affected infants. Methods This was a retrospective study of fetuses with posterior fossa fluid collections, carried out between 2001 and 2010 in two referral centers for prenatal diagnosis. All fetuses underwent multiplanar neurosonography. Parents were also offered fetal magnetic resonance imaging (MRI) and karyotyping. Prenatal diagnosis was compared with autopsy or postnatal MRI findings and detailed follow-up was attempted by consultation of medical records and interview with parents and pediatricians. fluid collections from mid gestation. Blake’s pouch cyst and megacisterna magna are risk factors for associated anomalies but when isolated have an excellent prognosis, with a high probability of intrauterine resolution and normal intellectual development in almost all cases. Conversely, Dandy–Walker malformation and vermian hypoplasia, even when they appear isolated antenatally, are associated with an abnormal outcome in half of cases. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd. Arachinoid  Cyst-­‐Pos  Fossa   INTRODUCTION 88 Fluid collections in the fetal posterior fossa encompass a wide spectrum of different megacisterna magna; Figure 1 Categorization of posterior fossa fluid collections on ultrasound: (a,b) Blake’s pouch cyst; (c,d) entities, ranging from(e,f) vermian Sunday, July 28, 13 During the study period, 105 fetuses were examnormal variants to severe anomalies1 . fossa. hypoplasia; (g,h) Dandy–Walker malformation; (i,j) cerebellar hypoplasia; (k,l) arachnoid cyst of the posterior They may have Results
  • 132. Normal Megacisterna  magna Blake’s  pouch  cyst tentorium Vermian  hypoplasia Dandy-­‐Walker  malforma3on Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 Sunday, July 28, 13 89
  • 133. Take  Home  Message Standard  and  Fetal   Neurosonography 90 Sunday, July 28, 13
  • 135. ✦ examina3on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol 91 Sunday, July 28, 13
  • 136. ✦ examina3on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦ Examina3on   should   include   at   least   three   axial   planes. 91 Sunday, July 28, 13
  • 137. ✦ examina3on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦ Examina3on   should   include   at   least   three   axial   planes. ✦ In   Each   plane   the   defined   landmarks   should   should  be  reported  as  normal  or  suspicious 91 Sunday, July 28, 13
  • 138. ✦ examina3on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦ Examina3on   should   include   at   least   three   axial   planes. ✦ In   Each   plane   the   defined   landmarks   should   should  be  reported  as  normal  or  suspicious ✦ In  the  presence  of  possible  abnormali3es  pa3ent   should   be   referred   for   detailed   neuorsonogram   which  include  mutli-­‐planner  3  D  Sanning. 91 Sunday, July 28, 13
  • 139. ✦ examina3on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦ Examina3on   should   include   at   least   three   axial   planes. ✦ In   Each   plane   the   defined   landmarks   should   should  be  reported  as  normal  or  suspicious ✦ In  the  presence  of  possible  abnormali3es  pa3ent   should   be   referred   for   detailed   neuorsonogram   which  include  mutli-­‐planner  3  D  Sanning. ✦ 3   D   scanning   with   mul3planner   analysis   offers   comparable  analysis  to  fetal  MRI 91 Sunday, July 28, 13