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Professor Hassan Nasrat FRCS, FRCOG
The Fetal Medicine Clinic
The First Clinic
JUCOG January 2013
Fetal	
  Nuerosonogram	
...
2
Microcephaly
Anencephaly
Chiari	
  Malforma,on


 Head normal or smallHead normal or small
DiaDia



halus ,...
Congenital	
  CNS	
  Anomalies
o Incidence	
  in	
  longtem	
  studies	
  about	
  1	
  %
o 	
  Only	
  minimal	
  identif...
4
✤Embryonic	
  development	
  of	
  the	
  CNS	
  
in	
  relation	
  to	
  sonographic	
  findings
✤Standard	
   Sonograp...
Embryology of the
CNS
Sunday, July 28, 13
At	
   5th	
   Week	
   The	
   Cells	
  
Destined	
   To	
   Form	
   The	
  
Notochord	
  Infiltrate	
  Into	
  
The	
  ...
7
Prosencephalon Mesencephalon
Rhombencephalon
Sunday, July 28, 13
Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at
the end ...
Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at
the end ...
Normal brain development on the mid-sagittal section between 8 and 12 weeks of gestation). Note the remarkable changing of...
10
Changing	
  Ultrasound	
  appearance	
  of	
  the	
  
The	
  Posterior	
  Fossa	
  throughout	
  gesta,on	
  
C D
AJR:1...
11
The vermis develops superiorly to inferiorly.
Hypoplasia or developmental arrest results in
varying size deficits of the...
12
C D
Fig. 13.-Drawings depicting some relevant features of fetal cerebellar development.
A, Axial drawing of developing ...
sagittalaxial
sonograms of posterior fossa in 16-week-old fetus
13
of posterior fossa in 13- to 14-week-old fetus. called ...
Lower-most Section
The Vermis Appears To
Be Open (arrow) And
Communicates With The
Fourth Ventricle
Through A Wide
Somewha...
15
Effect	
  of	
  Gesta=onal	
  age	
  (Lateral	
  Ventricles)
Sunday, July 28, 13
16
Hypoplasia Or Dysplasia Should Not Be
Diagnosed Prior To 18 Weeks, Before Vermian
Development Is Complete.
An Abnormall...
17
Conclusion
•TheCNS	
   displays	
   remarkable	
  
embryological	
   and	
   developmental	
  
changes	
  throughout	
 ...
18
Standard Sonographic
Examination of the
FEtal CNS
Sunday, July 28, 13
Sunday, July 28, 13
Sonography	
  of	
  the	
  CNS
Basic	
  Examination “Neurosongram”
Sunday, July 28, 13
Planes	
  of	
  Basic	
  ExaminaEon
Axial	
  Planes SagiZal	
  Planes
Sunday, July 28, 13
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
C:	
  Transcerebeller	
  
b:	
  Transthalamic	
  
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
Choroid	
  
Plexus
Atrium
Sunday, July 28, 13
The	
  Transventricular	
  plane
Cavum	
  SepE	
  Pellucidi
Frontal	
  hones
Choroid	
  
Plexus
Atrium
Sunday, July 28, 13
The	
  Transthalamic	
  	
  Plane
Sunday, July 28, 13
Thalami
The	
  Transthalamic	
  	
  Plane
Sunday, July 28, 13
Thalami
Hyppocamas	
  
Gyrus
The	
  Transthalamic	
  	
  Plane
Sunday, July 28, 13
T
T
The	
  Transcerebeller	
  	
  plane
Sunday, July 28, 13
Cavum	
  SepE	
  Pellucidi
T
T
The	
  Transcerebeller	
  	
  plane
Sunday, July 28, 13
Cavum	
  SepE	
  Pellucidi
Frontal	
  hones
T
T
The	
  Transcerebeller	
  	
  plane
Sunday, July 28, 13
Cerebellum
Cavum	
  SepE	
  Pellucidi
Frontal	
  hones
T
T
The	
  Transcerebeller	
  	
  plane
Sunday, July 28, 13
Cerebellar	
  vermis
Cerebellum
Cavum	
  SepE	
  Pellucidi
Frontal	
  hones
T
T
The	
  Transcerebeller	
  	
  plane
Sunday...
Cerebellar	
  vermis
Cistrerna	
  Magna
2-­‐10	
  mm
Cerebellum
Cavum	
  SepE	
  Pellucidi
Frontal	
  hones
T
T
The	
  Tra...
SagiZal	
  Planes	
  	
  
Sunday, July 28, 13
SagiZal	
  Planes	
  	
  
A:	
  The	
  Midsagittal	
  
Plan
Sunday, July 28, 13
SagiZal	
  Planes	
  	
  
b:	
  Parasgittal	
  plane
A:	
  The	
  Midsagittal	
  
Plan
Sunday, July 28, 13
27
Mid	
  SagiGal	
  Plane	
  
Corpus	
  Callosum Cavum	
  Sep,	
  Pellucidi
Cerebellum
4th	
  V
Sunday, July 28, 13
27
Mid	
  SagiGal	
  Plane	
  
Sunday, July 28, 13
28
The	
  Corpus	
  Callosum
Sunday, July 28, 13
28
Corpus	
  Callosum
Lateral	
  Ventricles
Third	
  Ventricle
midbrain
Pituitary
Splenium
Thalamus
hypothalamus
Fourth	
 ...
29
Para-­‐SagiGal	
  Plane	
  
Sunday, July 28, 13
30
Basic Examniation ChecklistBasic Examniation Checklist
Head + NeckHead + Neck
Midline & FalxMidline & Falx
Cavum septi ...
Main	
  AbnormaliEes	
  can	
  be	
  
Suspected	
  on	
  Basic	
  Planes
31
Sunday, July 28, 13
32
Microcephaly
Anencephaly
Chiari	
  Malforma,on


 Head normal or smallHead normal or small
DiaDia



halus ...
32
Microcephaly
Anencephaly
Chiari	
  Malforma,on


 Head normal or smallHead normal or small
DiaDia



halus ...
32
Microcephaly
Anencephaly
Chiari	
  Malforma,on


 Head normal or smallHead normal or small
DiaDia



halus ...
32
Microcephaly
Anencephaly
Chiari	
  Malforma,on


 Head normal or smallHead normal or small
DiaDia



halus ...
33
•Ventriculomegaly	
  (hydrocephalus)
•Absent	
  Cavum	
  Septum	
  Pellucidum
•Agenesis	
  of	
  the	
  Corpus	
  Callo...
(<	
  10	
  mm	
  is	
  normal).	
  	
  Independent	
  
of	
  gesta7onal	
  age	
  
Mild	
  10	
  –	
  15	
  mm
Low	
  Ris...
Le]	
  Lateral	
  Ventricle
Right	
  Lateral	
  Ventricle
3rd	
  	
  Ventricle
4th	
  	
  Ventricle
35
Aqueduct	
  of	
  S...
•Square	
  Shaped,	
  Interrupts	
  and	
  Fills	
  The	
  Space	
  Between	
  The	
  Frontal	
  Horns
•The	
  CSP:	
  Bec...
•Square	
  Shaped,	
  Interrupts	
  and	
  Fills	
  The	
  Space	
  Between	
  The	
  Frontal	
  Horns
•The	
  CSP:	
  Bec...
A	
   rare	
   finding	
   usually	
   discovered	
   Postnatally	
   in	
  
children	
  evaluated	
  for	
  developmental...
38
Agenesis	
  of	
  the	
  Corpus	
  Callosum	
  	
  
Sunday, July 28, 13
Only	
   The	
   Rostrum	
   (1),	
   Genu	
   (2)	
   And	
   Body	
   (3)	
   Are	
   Visible;	
   The	
   Splenium	
   ...
Outcome	
  of	
  fetal	
  ACC
 Varies	
   between	
   completely	
   asymptomaEc	
  
appearance	
  and	
  severe	
  neuro...
41
Fluid	
  Collec,on	
  in	
  the	
  Posterior	
  Fossa
Sunday, July 28, 13
41
Fluid	
  Collec,on	
  in	
  the	
  Posterior	
  Fossa
•Megacisterna Magna
Sunday, July 28, 13
41
Fluid	
  Collec,on	
  in	
  the	
  Posterior	
  Fossa
•Blak’s Pouch Cyst
•Megacisterna Magna
Sunday, July 28, 13
41
Fluid	
  Collec,on	
  in	
  the	
  Posterior	
  Fossa
•Blak’s Pouch Cyst
•Megacisterna Magna •D-W Malformation &DW- Var...
41
Fluid	
  Collec,on	
  in	
  the	
  Posterior	
  Fossa
•Blak’s Pouch Cyst
•Megacisterna Magna
•Arachnoid Cyst
•D-W Malfo...
42
•Blak’s Pouch Cyst
•Megacisterna Magna
•Arachnoid Cyst
•D-W Malformation &DW- Variant
Anomalies Of The
Meninges
Anomali...
Mega–Cisterna Magna
43
An Enlargement Of The Cisterna Magna Beyond 10
Mm With Intact Vermis
Sunday, July 28, 13
44
Lateral	
  Ventricle
Pathogenesis: Mega Cisterna Magna
Cerebral	
  Aqueduct
Choriod	
  Plexus
Third	
  
Ventricle
Fourt...
44
Lateral	
  Ventricle
Pathogenesis: Mega Cisterna Magna
Cerebral	
  Aqueduct
Choriod	
  Plexus
Third	
  
Ventricle
TheFo...
45
Prognosis:
•Isolated Cases: (97%-100%)Are Normal.
•If Not Isolated:Only 11% Have Normal Outcome.
Nonisolated Cases Have...
46
Blake’s Pouch Cyst
Sunday, July 28, 13
47
Lateral	
  Ventricle
Cerebral	
  Aqueduct
Choriod	
  Plexus
Third	
  
Ventricle
Nonfenestration of the
foramina of Lusc...
47
Lateral	
  Ventricle
Cerebral	
  Aqueduct
Choriod	
  Plexus
Third	
  
Ventricle
Nonfenestration of the
foramina of Lusc...
Dandy-Walker Malformation
48
ASpectrum OfAnomalies Of The Posterior Fossa.
• Dandy-Walker Malformation:
✦Increase Of The P...
49
ctions
Cystic dilation of the
f o u r t h v e n t r i c l e
communicating with a
posterior fossa fluid
space
Small, rot...
50
ThePrognosis :
BetterInIsolatedDWS.
KaryotypeAbnormalities InAbout 15%.
Neonatal Mortality:
12% To 55%.
Neonatal Morbid...
51
Dandy–Walker Malformation
The Torcular Is Displaced Higher
Than Usual, Indicating That This
Is A
Figure 2 The position ...
• Are Benign, Noncommunicating Fluid
Collections Within Arachnoid
Membranes.
• Location: Intracranially And In The
Spinal ...
The Differential Diagnosis
53
Depends On The Location.
In The Posterior Fossa:
DandyWalker Malformation, Inferior Vermian
...
54
Prenatal diagnosis and outcome of fetal posterior
fossa fluid collections
G. GANDOLFI COLLEONI et al,
Ultrasound Obstet ...
Blake’s Pouch Cyst
N = 32
Megacisterna Magna
N = 27
Dandy – Walker Malformation
N=26
Vermian Hypoplasia
N=17
Cerebellar Hy...
56
✦Isolated Cases Of Blake’s Pouch Cyst And
Megacisterna Magna Have An Excellent Prognosis,
With A High Probability Of In...
57
•Black’s	
   Pouch	
   Cyst,	
   DW	
   Malformation,	
   and	
  
Mega-­‐Cisterna	
   Magna	
   Can	
   give	
   Simila...
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
Technical Guideline
How do we do it? Practical advice ...
3D	
   ultrasound	
   is	
   a	
   data	
   set	
  
that	
   contains	
   a	
   large	
   number	
  
of	
   2D	
   planes	...
Pyramid	
  Of	
  Volume	
  Informa=on
✴ “Walking”	
  through	
  the	
  
volume	
   is	
   similar	
   to	
  
leafing	
   t...
61
3D volumes of the fetal brain obtained from
an axial approach: the ‘start’ scan
3D volumes of the fetal brain obtained ...
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
midline
A B
C
Sunday, July 28, 13
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
midline
A B
C
Sunday, July 28, 13
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
midline
A B
C
A and B rotated on Z
plane until midline...
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
A B
C
Corpus callosum + cavum septi pellucidi
Cerebell...
Originally	
  published	
  in	
  Ultrasound Obstet Gynecol 2007; 30: 233–245
midline
midline
Corpus	
  callosum
Cavum	
  s...
Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
4v
Brain	
  stem Cerebellar	
 ...
Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245
4v
hemisphere
hemisphere
hemisphere
hemisphere
vermis
...
Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
body atrium
Occipital	
  
horn...
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
Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
3v
Normal	
  corpus	
  callosu...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Prenatal	
  diagnosis	
  and	
  outcome	
  of	...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Prenatal	
  diagno...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cerebellar	
  verm...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cisterna	
  Magna
...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cisterna	
  Magna
...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cisterna	
  Magna
...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cisterna	
  Magna
...
Normal	
  Posterior	
  Fossa	
  At	
  Midgesta=on
SagiGal	
  viewAxial view
Cavum	
  Sep,	
  
Pellucidi
Cisterna	
  Magna
...
76
Applica=on	
  of	
  3	
  D	
  Imaging	
  in	
  
Prenatal	
  diagnosis	
  of	
  Fetal	
  
Posterior	
  Fossa	
  Fluid	
 ...
77
Prenatal	
  diagnosis	
  and	
  outcome	
  of	
  fetal	
  posterior	
  fossa	
  fluid	
  
Collec=ons
Gandolfi	
  Colleoni...
Categoriza,on	
  of	
  posterior	
  fossa	
  fluid	
  collec,ons	
  (1)
Sunday, July 28, 13
Blake’s	
  pouch	
  cyst Megacisterna	
  magna D-­‐W	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
  	
...
79
•Transverse Diameter Of
The Cerebellum.
•The Intactness And Size
Of The Vermis.
•The Depth Of The
Cisterna Magna (10 Mm...
80Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento
this case after acquisition of an ultrasound volume st...
81Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento
this case after acquisition of an ultrasound volume st...
Blake’s Pouch
Cyst
Measurement Of Brainstem–vermis (BV) Angle (1) And
Brainstem–tentorium (BT) In Three Conditions
Cerebel...
Dandy–Walker malformation 12 63.5 17.6 45–112 67.2 15.1 51–1
80
60
40
20
0
Brainstem–vermisangle(°)
Normal Blake’s pouch
c...
84
Fetal posterior fossa fluid collections associated
with upward rotation of the cerebellar vermis range
from benign asym...
85
In	
  Summary
Sunday, July 28, 13
Examination Of The Posterior Fossa And
The Cerebellum
Midsagittal ViewsAxial View
86
Sunday, July 28, 13
PracEcal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  CysEc	
  like	
  Lesions
Sunday, Ju...
PracEcal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  CysEc	
  like	
  Lesions
1. Is	
  t...
PracEcal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  CysEc	
  like	
  Lesions
1. Is	
  t...
PracEcal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  CysEc	
  like	
  Lesions
1. Is	
  t...
PracEcal	
  Approach	
  to	
  the	
  DD	
  of	
  Posterior	
  Fossa	
  
Cyst	
  and	
  CysEc	
  like	
  Lesions
1. Is	
  t...
88
Ultrasound Obstet Gynecol 2012; 39: 625–631
Published online 14 May 2012 in Wiley Online Library (wileyonlinelibrary.co...
Originally	
  published	
  in	
  Ultrasound	
  Obstet	
  Gynecol	
  2007;	
  30:	
  233–245
Normal Megacisterna	
  magna B...
Standard	
  and	
  Fetal	
  
Neurosonography
90
Take	
  Home	
  Message
Sunday, July 28, 13
91
Sunday, July 28, 13
91
✦examina,on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol
Sunday, July 28, 13
91
✦examina,on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol
✦Examina,on	
   should...
91
✦examina,on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol
✦Examina,on	
   should...
91
✦examina,on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol
✦Examina,on	
   should...
91
✦examina,on	
  of	
  the	
  Fetal	
  CNS	
  should	
  be	
  follow	
  a	
  
Standard	
  Protocol
✦Examina,on	
   should...
92
Thanks	
  
Sunday, July 28, 13
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Fetal neurosonogram jucog feb 2013

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  1. 1. Professor Hassan Nasrat FRCS, FRCOG The Fetal Medicine Clinic The First Clinic JUCOG January 2013 Fetal  Nuerosonogram   Sunday, July 28, 13
  2. 2. 2 Microcephaly Anencephaly Chiari  Malforma,on    Head normal or smallHead normal or small DiaDia    halus , T 21halus , T 21 Ventriculomegaly HydranceHydrance Hydranecphaly Encephalocele Occipital EncephaloceleOccipital Encephalocele Imaging FindingsImaging Findings  Herniated brain tissueHerniated brain tissue  „„cyst within the cystcyst within the cyst““  Ventriculomegaly 70Ventriculomegaly 70-- 80%80%  Microcephaly 25%Microcephaly 25%  PolyhydramniosPolyhydramnios  OligohydramniosOligohydramnios CAVE:CAVE:  Associated with multipleAssociated with multiple syndroms ( Meckelsyndroms ( Meckel-- Gruber )Gruber ) Pilu Holoprosencephaly Hemimegalencephaly Arachnoid  cyst ACC SOP Schizencephaly SchizencephalySchizencephaly PF-­‐Fluid-­‐Cyst Yong seok et a Vascular   Malforma,ons Circle of Willis MallformationCircle of Willis Mallformation Sunday, July 28, 13
  3. 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. 4. 4 ✤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) Learning  Objec,ves Sunday, July 28, 13
  5. 5. Embryology of the CNS Sunday, July 28, 13
  6. 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   Neural  Tube.   The   Fusion   Starts   In   The   Midtrunk   Of   The   E m b r y o   A n d   Subsequently   Extends   To   The   Cranial   And   Caudal  Ends   Neural  Crest Neural  TubeNeural  Groove Neural  Plate Ectoderm Sunday, July 28, 13
  7. 7. 7 Prosencephalon Mesencephalon Rhombencephalon Sunday, July 28, 13
  8. 8. Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at the end of 8 weeks of gestation. The development of premature ventricular system is seen. 8 Sunday, July 28, 13
  9. 9. Three orthogonal images and thick slice of three-dimensional reconstructed image (lower right) of normal brain at the end of 8 weeks of gestation. The development of premature ventricular system is seen. 8 Prosencephalon Mesencephalon Sunday, July 28, 13
  10. 10. Normal brain development on the mid-sagittal section between 8 and 12 weeks of gestation). Note the remarkable changing of premature brain appearance. 9 Sunday, July 28, 13
  11. 11. 10 Changing  Ultrasound  appearance  of  the   The  Posterior  Fossa  throughout  gesta,on   C D AJR:166, February 1996 SONOGRAPHIC ANATOMY OF DEVELOPING CEREBELLUM 433 C D AJR:166, February 1996 SONOGRAPHIC ANATOMY OF DEVELOPING CEREBELLUM 433 Fig. 13.-Drawings depicting some relevant features of fetal cerebellar development.C D AJR:166, February 1996 SONOGRAPHIC ANATOMY OF DEVELOPING CEREBELLUM 433 C DSunday, July 28, 13
  12. 12. 11 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. Sunday, July 28, 13
  13. 13. 12 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 and vermis over fourth ventrIcle, with thick- ening of caudal fourth ventricular roof. 0, Sagfttal drawing at 17 weeks’ gestational age shows cerebellum and vermis covering entire fourth ventricle. We have shown that the sonognaphic appearance of nor- mal cemebellar development can resemble pathology early in the second trimester. Our findings indicate that the mature relationships of the posterior fossa structures are not estab- lished until at least 18 weeks’ gestational age; therefore, the prenatal sonographic diagnosis of Dandy-Walker complex 4. Achinon R, Tadmor 0. Screening for fetal anomalies during the first tnimes- ten of pregnancy: tnansvaginal versus transabdominal sonography. Ultra- sound Obstet Gynecol 1991 1:186-191 5. Nicolaides KH, Azan G, Byrne D, Mansur C, Marks K. Fetal nuchal translu- cency: 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 teno thic and Sunday, July 28, 13
  14. 14. sagittalaxial sonograms of posterior fossa in 16-week-old fetus 13 of posterior fossa in 13- to 14-week-old fetus. called acquisition in stea A, Vermis is identified between cerebellar hemispheres rostrally (arrow). age of posteriorfossa in 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 Fig. 7.-Axial and tenor fossa in 16-week- A and B, Caudally, thick enough to be v and sagittal (B) planes B, Next caudal image identifies fourth ventricular roof joining cerebellar hemispheres fetus. Vermis (arrow) and separating fourth ventricle and cisterna magna. but not caudally Fig. 7.-A tenor fossa A and thick enoug and sagittal fourth ventricular roof is visualized in both planes (arrow) Effect  of  Gesta=onal  age  (Posterior  Fossa) Sunday, July 28, 13
  15. 15. Lower-most Section The Vermis Appears To Be Open (arrow) And Communicates With The Fourth Ventricle Through A Wide Somewhat Higher Higher Still No ‘vermian Defect’ Is Seen And The Fourth Ventricle (4) Appears As A Discrete Entity. 14 duncular cistern (cisterna magna) and the fourth ventri- . Later, after the 16th postmenstrual week, this ‘normal’ en space narrows as the growth and development of e vermis progress, giving rise to the median aperture ramen of Magendie) (Figure 2). Again, this normal its closest anatomic structures, namely the cavum sep pellucidi and the pericallosal artery, follow a well-know developmental timetable. They do not reach a developmen tal stage that allows for sonographic imaging until pos menstrual weeks 18–19. To search for their presence befor ure 1 Transvaginal scan of a 14-week fetus. (a) Oblique-1 (sagittal) section: the fetus is facing left. The choroid plexus fills the antrum the lateral ventricle. The anterior horns appear prominent, but are normal; (b) a Frontal-2 (coronal) section through the anterior horn the lateral ventricles. The anterior horns are normal for this gestational age; however, this same sonographic picture at 20 weeks o re is consistent with ventriculomegaly or hydrocephalus gure 2 Three serial, almost axial (horizontal) views through the posterior fossa. (a) This is the lower-most section (see insert). Th rmis appears to be open (arrow) and communicates with the fourth ventricle through a wide (at this gestational age, normal) media erture (foramen of Magendie); (b) somewhat higher, the right and left sides of the cerebellar hemispheres appear closer to each othe row); (c) higher still, no ‘vermian defect’ is seen and the fourth ventricle (4) appears as a discrete entity. C, cerebellum duncular cistern (cisterna magna) and the fourth ventri- Later, after the 16th postmenstrual week, this ‘normal’ en space narrows as the growth and development of vermis progress, giving rise to the median aperture ramen of Magendie) (Figure 2). Again, this normal nographic finding may be interpreted by those unfamiliar its closest anatomic structures, namely the cavum septi pellucidi and the pericallosal artery, follow a well-known developmental timetable. They do not reach a developmen- tal stage that allows for sonographic imaging until post- menstrual weeks 18–19. To search for their presence before they reach this critical stage in their development would ure 1 Transvaginal scan of a 14-week fetus. (a) Oblique-1 (sagittal) section: the fetus is facing left. The choroid plexus fills the antrum he lateral ventricle. The anterior horns appear prominent, but are normal; (b) a Frontal-2 (coronal) section through the anterior horns he lateral ventricles. The anterior horns are normal for this gestational age; however, this same sonographic picture at 20 weeks or re is consistent with ventriculomegaly or hydrocephalus ure 2 Three serial, almost axial (horizontal) views through the posterior fossa. (a) This is the lower-most section (see insert). The mis appears to be open (arrow) and communicates with the fourth ventricle through a wide (at this gestational age, normal) median rture (foramen of Magendie); (b) somewhat higher, the right and left sides of the cerebellar hemispheres appear closer to each other row); (c) higher still, no ‘vermian defect’ is seen and the fourth ventricle (4) appears as a discrete entity. C, cerebellum eduncular cistern (cisterna magna) and the fourth ventri- e. Later, after the 16th postmenstrual week, this ‘normal’ pen space narrows as the growth and development of e vermis progress, giving rise to the median aperture oramen of Magendie) (Figure 2). Again, this normal onographic finding may be interpreted by those unfamiliar its closest anatomic structures, namely the cavum septi pellucidi and the pericallosal artery, follow a well-known developmental timetable. They do not reach a developmen- tal stage that allows for sonographic imaging until post- menstrual weeks 18–19. To search for their presence before they reach this critical stage in their development would 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 the lateral ventricle. The anterior horns appear prominent, but are normal; (b) a Frontal-2 (coronal) section through the anterior horns the lateral ventricles. The anterior horns are normal for this gestational age; however, this same sonographic picture at 20 weeks or ore is consistent with ventriculomegaly or hydrocephalus gure 2 Three serial, almost axial (horizontal) views through the posterior fossa. (a) This is the lower-most section (see insert). The ermis appears to be open (arrow) and communicates with the fourth ventricle through a wide (at this gestational age, normal) median perture (foramen of Magendie); (b) somewhat higher, the right and left sides of the cerebellar hemispheres appear closer to each other rrow); (c) higher still, no ‘vermian defect’ is seen and the fourth ventricle (4) appears as a discrete entity. C, cerebellum The Right And Left Sides Of The Cerebellar Hemispheres Appear Closer To Each Other (arrow); Effect  Of  Scanning  Level  (Posterior  Fossa) Sunday, July 28, 13
  16. 16. 15 Effect  of  Gesta=onal  age  (Lateral  Ventricles) Sunday, July 28, 13
  17. 17. 16 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. The fetal cerebellum Pitfalls in diagnosis Sunday, July 28, 13
  18. 18. 17 Conclusion •TheCNS   displays   remarkable   embryological   and   developmental   changes  throughout  gestation. •Standard  Approach  of  examination  and   evaluation   of   the   CNS   Should   Be   Followed Sunday, July 28, 13
  19. 19. 18 Standard Sonographic Examination of the FEtal CNS Sunday, July 28, 13
  20. 20. Sunday, July 28, 13
  21. 21. Sonography  of  the  CNS Basic  Examination “Neurosongram” Sunday, July 28, 13
  22. 22. Planes  of  Basic  ExaminaEon Axial  Planes SagiZal  Planes Sunday, July 28, 13
  23. 23. Axial  Planes Sunday, July 28, 13
  24. 24. Axial  Planes a:  Transventricular Sunday, July 28, 13
  25. 25. Axial  Planes a:  Transventricular b:  Transthalamic   Sunday, July 28, 13
  26. 26. Axial  Planes a:  Transventricular C:  Transcerebeller   b:  Transthalamic   Sunday, July 28, 13
  27. 27. The  Transventricular  plane Sunday, July 28, 13
  28. 28. The  Transventricular  plane Frontal  hones Sunday, July 28, 13
  29. 29. The  Transventricular  plane Frontal  hones Atrium Sunday, July 28, 13
  30. 30. The  Transventricular  plane Frontal  hones Choroid   Plexus Atrium Sunday, July 28, 13
  31. 31. The  Transventricular  plane Cavum  SepE  Pellucidi Frontal  hones Choroid   Plexus Atrium Sunday, July 28, 13
  32. 32. The  Transthalamic    Plane Sunday, July 28, 13
  33. 33. Thalami The  Transthalamic    Plane Sunday, July 28, 13
  34. 34. Thalami Hyppocamas   Gyrus The  Transthalamic    Plane Sunday, July 28, 13
  35. 35. T T The  Transcerebeller    plane Sunday, July 28, 13
  36. 36. Cavum  SepE  Pellucidi T T The  Transcerebeller    plane Sunday, July 28, 13
  37. 37. Cavum  SepE  Pellucidi Frontal  hones T T The  Transcerebeller    plane Sunday, July 28, 13
  38. 38. Cerebellum Cavum  SepE  Pellucidi Frontal  hones T T The  Transcerebeller    plane Sunday, July 28, 13
  39. 39. Cerebellar  vermis Cerebellum Cavum  SepE  Pellucidi Frontal  hones T T The  Transcerebeller    plane Sunday, July 28, 13
  40. 40. Cerebellar  vermis Cistrerna  Magna 2-­‐10  mm Cerebellum Cavum  SepE  Pellucidi Frontal  hones T T The  Transcerebeller    plane Sunday, July 28, 13
  41. 41. SagiZal  Planes     Sunday, July 28, 13
  42. 42. SagiZal  Planes     A:  The  Midsagittal   Plan Sunday, July 28, 13
  43. 43. SagiZal  Planes     b:  Parasgittal  plane A:  The  Midsagittal   Plan Sunday, July 28, 13
  44. 44. 27 Mid  SagiGal  Plane   Corpus  Callosum Cavum  Sep,  Pellucidi Cerebellum 4th  V Sunday, July 28, 13
  45. 45. 27 Mid  SagiGal  Plane   Sunday, July 28, 13
  46. 46. 28 The  Corpus  Callosum Sunday, July 28, 13
  47. 47. 28 Corpus  Callosum Lateral  Ventricles Third  Ventricle midbrain Pituitary Splenium Thalamus hypothalamus Fourth  ventricle The  Corpus  Callosum Sunday, July 28, 13
  48. 48. 29 Para-­‐SagiGal  Plane   Sunday, July 28, 13
  49. 49. 30 Basic Examniation ChecklistBasic Examniation Checklist Head + NeckHead + Neck Midline & FalxMidline & Falx Cavum septi pellucidiCavum septi pellucidi Lateral cerebral ventriclsLateral cerebral ventricls Choroid PlexusChoroid Plexus CerebellumCerebellum Cisterna magnaCisterna magna Sunday, July 28, 13
  50. 50. Main  AbnormaliEes  can  be   Suspected  on  Basic  Planes 31 Sunday, July 28, 13
  51. 51. 32 Microcephaly Anencephaly Chiari  Malforma,on    Head normal or smallHead normal or small DiaDia    halus , T 21halus , T 21 Ventriculomegaly HydranceHydrance Hydranecphaly Encephalocele Occipital EncephaloceleOccipital Encephalocele Imaging FindingsImaging Findings  Herniated brain tissueHerniated brain tissue  „„cyst within the cystcyst within the cyst““  Ventriculomegaly 70Ventriculomegaly 70-- 80%80%  Microcephaly 25%Microcephaly 25%  PolyhydramniosPolyhydramnios  OligohydramniosOligohydramnios CAVE:CAVE:  Associated with multipleAssociated with multiple syndroms ( Meckelsyndroms ( Meckel-- Gruber )Gruber ) Pilu Holoprosencephaly Hemimegalencephaly Arachnoid  cyst ACC SOP Schizencephaly SchizencephalySchizencephaly PF-­‐Fluid-­‐Cyst Yong seok et a Vascular   Malforma,ons Circle of Willis MallformationCircle of Willis Mallformation Sunday, July 28, 13
  52. 52. 32 Microcephaly Anencephaly Chiari  Malforma,on    Head normal or smallHead normal or small DiaDia    halus , T 21halus , T 21 Ventriculomegaly HydranceHydrance Hydranecphaly Encephalocele Occipital EncephaloceleOccipital Encephalocele Imaging FindingsImaging Findings  Herniated brain tissueHerniated brain tissue  „„cyst within the cystcyst within the cyst““  Ventriculomegaly 70Ventriculomegaly 70-- 80%80%  Microcephaly 25%Microcephaly 25%  PolyhydramniosPolyhydramnios  OligohydramniosOligohydramnios CAVE:CAVE:  Associated with multipleAssociated with multiple syndroms ( Meckelsyndroms ( Meckel-- Gruber )Gruber ) Pilu Holoprosencephaly Hemimegalencephaly Arachnoid  cyst ACC SOP Schizencephaly SchizencephalySchizencephaly PF-­‐Fluid-­‐Cyst Yong seok et a Vascular   Malforma,ons Circle of Willis MallformationCircle of Willis Mallformation Ventriculomegaly Sunday, July 28, 13
  53. 53. 32 Microcephaly Anencephaly Chiari  Malforma,on    Head normal or smallHead normal or small DiaDia    halus , T 21halus , T 21 Ventriculomegaly HydranceHydrance Hydranecphaly Encephalocele Occipital EncephaloceleOccipital Encephalocele Imaging FindingsImaging Findings  Herniated brain tissueHerniated brain tissue  „„cyst within the cystcyst within the cyst““  Ventriculomegaly 70Ventriculomegaly 70-- 80%80%  Microcephaly 25%Microcephaly 25%  PolyhydramniosPolyhydramnios  OligohydramniosOligohydramnios CAVE:CAVE:  Associated with multipleAssociated with multiple syndroms ( Meckelsyndroms ( Meckel-- Gruber )Gruber ) Pilu Holoprosencephaly Hemimegalencephaly Arachnoid  cyst ACC SOP Schizencephaly SchizencephalySchizencephaly PF-­‐Fluid-­‐Cyst Yong seok et a Vascular   Malforma,ons Circle of Willis MallformationCircle of Willis Mallformation ACC Ventriculomegaly Sunday, July 28, 13
  54. 54. 32 Microcephaly Anencephaly Chiari  Malforma,on    Head normal or smallHead normal or small DiaDia    halus , T 21halus , T 21 Ventriculomegaly HydranceHydrance Hydranecphaly Encephalocele Occipital EncephaloceleOccipital Encephalocele Imaging FindingsImaging Findings  Herniated brain tissueHerniated brain tissue  „„cyst within the cystcyst within the cyst““  Ventriculomegaly 70Ventriculomegaly 70-- 80%80%  Microcephaly 25%Microcephaly 25%  PolyhydramniosPolyhydramnios  OligohydramniosOligohydramnios CAVE:CAVE:  Associated with multipleAssociated with multiple syndroms ( Meckelsyndroms ( Meckel-- Gruber )Gruber ) Pilu Holoprosencephaly Hemimegalencephaly Arachnoid  cyst ACC SOP Schizencephaly SchizencephalySchizencephaly PF-­‐Fluid-­‐Cyst Yong seok et a Vascular   Malforma,ons Circle of Willis MallformationCircle of Willis Mallformation ACC PF-­‐Fluid-­‐Cyst Ventriculomegaly Sunday, July 28, 13
  55. 55. 33 •Ventriculomegaly  (hydrocephalus) •Absent  Cavum  Septum  Pellucidum •Agenesis  of  the  Corpus  Callosum •Fluid  Collection  in  the  posterior  fossa Sunday, July 28, 13
  56. 56. (<  10  mm  is  normal).    Independent   of  gesta7onal  age   Mild  10  –  15  mm Low  Risk   Severe  >  15  mm   High  Risk   mean  =  6-­‐8   mm Ventriculomegaly  (hydrocephalus) Sunday, July 28, 13
  57. 57. Le]  Lateral  Ventricle Right  Lateral  Ventricle 3rd    Ventricle 4th    Ventricle 35 Aqueduct  of  Sylvius Foramen  of  Monro   Cisterna  Magna Pathogenesis:  Ventriculomegaly Sunday, July 28, 13
  58. 58. •Square  Shaped,  Interrupts  and  Fills  The  Space  Between  The  Frontal  Horns •The  CSP:  Becomes  Visible  At  16    Weeks,  Obliterate  Near  Term Absent  CSP Sunday, July 28, 13
  59. 59. •Square  Shaped,  Interrupts  and  Fills  The  Space  Between  The  Frontal  Horns •The  CSP:  Becomes  Visible  At  16    Weeks,  Obliterate  Near  Term Cavum  SepE  Pellucidi Absent  CSP Sunday, July 28, 13
  60. 60. 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.   Absent  CSP Sunday, July 28, 13
  61. 61. 38 Agenesis  of  the  Corpus  Callosum     Sunday, July 28, 13
  62. 62. 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 21-­‐week  Fetus  With  Par=al  Agenesis  Of  The  Corpus  Callosum Sunday, July 28, 13
  63. 63. Outcome  of  fetal  ACC  Varies   between   completely   asymptomaEc   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  educaEon   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. 64. 41 Fluid  Collec,on  in  the  Posterior  Fossa Sunday, July 28, 13
  65. 65. 41 Fluid  Collec,on  in  the  Posterior  Fossa •Megacisterna Magna Sunday, July 28, 13
  66. 66. 41 Fluid  Collec,on  in  the  Posterior  Fossa •Blak’s Pouch Cyst •Megacisterna Magna Sunday, July 28, 13
  67. 67. 41 Fluid  Collec,on  in  the  Posterior  Fossa •Blak’s Pouch Cyst •Megacisterna Magna •D-W Malformation &DW- Variant Sunday, July 28, 13
  68. 68. 41 Fluid  Collec,on  in  the  Posterior  Fossa •Blak’s Pouch Cyst •Megacisterna Magna •Arachnoid Cyst •D-W Malformation &DW- Variant Sunday, July 28, 13
  69. 69. 42 •Blak’s Pouch Cyst •Megacisterna Magna •Arachnoid Cyst •D-W Malformation &DW- Variant Anomalies Of The Meninges Anomalies Cerebellum Sunday, July 28, 13
  70. 70. Mega–Cisterna Magna 43 An Enlargement Of The Cisterna Magna Beyond 10 Mm With Intact Vermis Sunday, July 28, 13
  71. 71. 44 Lateral  Ventricle Pathogenesis: Mega Cisterna Magna Cerebral  Aqueduct Choriod  Plexus Third   Ventricle Fourth  Ventricle Sunday, July 28, 13
  72. 72. 44 Lateral  Ventricle Pathogenesis: Mega Cisterna Magna Cerebral  Aqueduct Choriod  Plexus Third   Ventricle TheForaminaOf LuschkaAndMagendie FenestrateDelayed Fourth  Ventricle Sunday, July 28, 13
  73. 73. 45 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. Sunday, July 28, 13
  74. 74. 46 Blake’s Pouch Cyst Sunday, July 28, 13
  75. 75. 47 Lateral  Ventricle Cerebral  Aqueduct Choriod  Plexus Third   Ventricle Nonfenestration of the foramina of Luschka and Magendie leads to dilatation of the fourth ventricle and and elevation of the vermis away from the brain stem. Fourth  Ventricle Pathogenesis: Blake’s Pouch Cyst There is no communication between the cyst and the subarachnoid space Sunday, July 28, 13
  76. 76. 47 Lateral  Ventricle Cerebral  Aqueduct Choriod  Plexus Third   Ventricle Nonfenestration of the foramina of Luschka and Magendie leads to dilatation of the fourth ventricle and and elevation of the vermis away from the brain stem. Fourth  Ventricle Pathogenesis: Blake’s Pouch Cyst There is no communication between the cyst and the subarachnoid space Sunday, July 28, 13
  77. 77. Dandy-Walker Malformation 48 ASpectrum OfAnomalies Of The Posterior Fossa. • Dandy-Walker Malformation: ✦Increase Of The Posterior Fossa, ✦Complete Or Partially Agenesis Of The CerebellarVermis, ✦ATentorium Elevation • Variant Of Dandy-Walker: ✦Hypoplasia Of The Cerebellar Vermis In Different Degrees With Or Without Increase Of The Posterior Fossa. Sunday, July 28, 13
  78. 78. 49 ctions Cystic dilation of the f o u r t h v e n t r i c l e communicating with a posterior fossa fluid space Small, rotated, raised, or absent vermis Elevated tentorium and high position of the torcula Dandy-Walker Malformation Sunday, July 28, 13
  79. 79. 50 ThePrognosis : BetterInIsolatedDWS. KaryotypeAbnormalities InAbout 15%. Neonatal Mortality: 12% To 55%. Neonatal Morbidity: •Intelligence Is NormalInAbout40% •Borderline In 20% •Subnormal In 40%. Sunday, July 28, 13
  80. 80. 51 Dandy–Walker Malformation The Torcular Is Displaced Higher Than Usual, Indicating That This Is A Figure 2 The position of the torcular Herophili (arrows) is inferre on ultrasound by the direction of the tentorium cerebelli. In (a) th torcular is found in a normal position, at about the same level as the site of insertion of the neck muscles on the posterior skull; thi is a Blake’s pouch cyst. In (b) the torcular is displaced higher than igure 2 The position of the torcular Herophili (arrows) is inferred n ultrasound by the direction of the tentorium cerebelli. In (a) the orcular is found in a normal position, at about the same level as he site of insertion of the neck muscles on the posterior skull; this a Blake’s pouch cyst. In (b) the torcular is displaced higher than Blake’s Pouch Cyst The Torcular Is Found In A Normal Position, At About The Same Level As The Site Of Insertion Of The Neck Muscles On The Posterior Skull Sunday, July 28, 13
  81. 81. • 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 RequireDecompression. Arachnoid Cysts Sunday, July 28, 13
  82. 82. The Differential Diagnosis 53 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. Sunday, July 28, 13
  83. 83. 54 Prenatal diagnosis and outcome of fetal posterior fossa fluid collections G. GANDOLFI COLLEONI et al, Ultrasound Obstet Gynecol 2012; 39: 625–631 Sunday, July 28, 13
  84. 84. Blake’s Pouch Cyst N = 32 Megacisterna Magna N = 27 Dandy – Walker Malformation N=26 Vermian Hypoplasia N=17 Cerebellar Hypoplasia N=2 55 105 Fetuses Arachnoid Cyst N=1 Sonographic d i a g n o s e s were accurate in 88% Sunday, July 28, 13
  85. 85. 56 ✦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. Sunday, July 28, 13
  86. 86. 57 •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 Conclusion Sunday, July 28, 13
  87. 87. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 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 Sunday, July 28, 13
  88. 88. 3D   ultrasound   is   a   data   set   that   contains   a   large   number   of   2D   planes   (B-­‐mode   images).   e.g.   If   the   page   of   a   book   is   one   2D   plane,   then  the   book   itself  is  the  enEre  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. 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”   the   acquired   volume   unlike   the   defined  rectangle  shape  of  a  book   looks  like  a  pyramid  or  triangle  of   volume  informaEon   with  a  broad   base   Sunday, July 28, 13
  90. 90. 61 3D volumes of the fetal brain obtained from an axial approach: the ‘start’ scan 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 Sunday, July 28, 13
  91. 91. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 midline A B C Sunday, July 28, 13
  92. 92. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 midline A B C Sunday, July 28, 13
  93. 93. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 midline A B C A and B rotated on Z plane until midline is aligned with C plane Sunday, July 28, 13
  94. 94. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 A B C Corpus callosum + cavum septi pellucidi Cerebellar vermis Acoustic shadow Sunday, July 28, 13
  95. 95. Originally  published  in  Ultrasound Obstet Gynecol 2007; 30: 233–245 midline midline Corpus  callosum Cavum  sep*  pellucidi Corpus  callosum  +  cavum  sep*  pellucidi 64 Sunday, July 28, 13
  96. 96. Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 4v Brain  stem Cerebellar  vermis Angled  Insona,on  of  Posterior  Fossa  to   Visualize  brain  Stem 65 Sunday, July 28, 13
  97. 97. Originally published in Ultrasound Obstet Gynecol 2007; 30: 233–245 4v hemisphere hemisphere hemisphere hemisphere vermis tentorium tentorium 4v vermis vermian fissures Sunday, July 28, 13
  98. 98. Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 body atrium Occipital   horn Temporal  horn Sylvian  fissure 67 Sunday, July 28, 13
  99. 99. 68 Sunday, July 28, 13
  100. 100. 69 Sunday, July 28, 13
  101. 101. 70 Sunday, July 28, 13
  102. 102. 71 Sunday, July 28, 13
  103. 103. 72 Sunday, July 28, 13
  104. 104. 73 Sunday, July 28, 13
  105. 105. Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 3v Normal  corpus  callosum Absent  corpus  callosum 3v 3v Par,al  agenesis 74 Agenesis  of  the   corpus  callosum Sunday, July 28, 13
  106. 106. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  107. 107. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  108. 108. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  109. 109. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cisterna  Magna Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Sunday, July 28, 13
  110. 110. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cisterna  Magna Tentorium Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Cisterna  Magna Sunday, July 28, 13
  111. 111. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cisterna  Magna Tentorium Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Cisterna  Magna Sunday, July 28, 13
  112. 112. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cisterna  Magna Tentorium Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Cisterna  Magna Sunday, July 28, 13
  113. 113. Normal  Posterior  Fossa  At  Midgesta=on SagiGal  viewAxial view Cavum  Sep,   Pellucidi Cisterna  Magna Tentorium Cerebellar  vermis Prenatal  diagnosis  and  outcome  of  fetal  posterior  fossa  fluid  Collec=ons Gandolfi  Colleoni  et  al.,  UOG  2012 Cisterna  Magna Sunday, July 28, 13
  114. 114. 76 Applica=on  of  3  D  Imaging  in   Prenatal  diagnosis  of  Fetal   Posterior  Fossa  Fluid  Collec=on Sunday, July 28, 13
  115. 115. 77 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 categorizationoffetalupwardrotationofcerebellarvermis P. VOLPE*, et al Ultrasound Obstet Gynecol 2012; 39: 632–635 Sunday, July 28, 13
  116. 116. Categoriza,on  of  posterior  fossa  fluid  collec,ons  (1) Sunday, July 28, 13
  117. 117. Blake’s  pouch  cyst Megacisterna  magna D-­‐W                                                           Findings Upward  rotation  of  an  intact  vermis   with  normal  torcular Cisterna  magna  >10mm  with  intact   and  normally  positioned  cerebellum Upward  rotation  of  the  vermis   (normal  or  hypoplastic)  with elevated  torcular SagiGal   Axial Categoriza,on  of  posterior  fossa  fluid  collec,ons  (1) Sunday, July 28, 13
  118. 118. 79 •Transverse Diameter Of The Cerebellum. •The Intactness And Size Of The Vermis. •The Depth Of The Cisterna Magna (10 Mm) Axial  View Sunday, July 28, 13
  119. 119. 80Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento this case after acquisition of an ultrasound volume starting from an axi Cavum  Sep,   Pellucidi The  Vermis:  Shape,  Size,  Fissures The  Tentorium:  Level     Sunday, July 28, 13
  120. 120. 81Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tento this case after acquisition of an ultrasound volume starting from an axi Brainstem-vermis (BV) angle Brainstem-tentorium (BT)angle Cavum  Sep,   Pellucidi Sunday, July 28, 13
  121. 121. Blake’s Pouch Cyst Measurement Of Brainstem–vermis (BV) Angle (1) And Brainstem–tentorium (BT) In Three Conditions Cerebellar Vermis Hypoplasi Dandy–Walker Malformation. The Angles Has The Widest Measurement In DA Malformation 82 Figure 1 Measurement of brainstem–vermis (BV) and brainstem–tentorium (BT) angles. (a) A median view of the fetal brain is obtained this case after acquisition of an ultrasound volume starting from an axial view) and the main anatomic landmarks are identified. (b) A lin drawn tangentially to the dorsal aspect of the brain stem and a second line is drawn tangentially to the ventral contour of the cerebellar vermis; the interposed angle (1) is the BV angle; the BT angle (2) is measured between the first line and a third line tangential to the tentoriu Figure 2 Measurement of brainstem–vermis (BV) angle (1) and brainstem–tentorium (BT) angle (2) in fetuses with: (a) Blake’s pouch cys Measurement of brainstem–vermis (BV) and brainstem–tentorium (BT) angles. (a) A median view of the fetal brain is obtained (in ter acquisition of an ultrasound volume starting from an axial view) and the main anatomic landmarks are identified. (b) A line i gentially to the dorsal aspect of the brain stem and a second line is drawn tangentially to the ventral contour of the cerebellar interposed angle (1) is the BV angle; the BT angle (2) is measured between the first line and a third line tangential to the tentorium 1 Measurement of brainstem–vermis (BV) and brainstem–tentorium (BT) angles. (a) A median view of the fetal brain is obta e after acquisition of an ultrasound volume starting from an axial view) and the main anatomic landmarks are identified. (b) angentially to the dorsal aspect of the brain stem and a second line is drawn tangentially to the ventral contour of the cerebe the interposed angle (1) is the BV angle; the BT angle (2) is measured between the first line and a third line tangential to the te Sunday, July 28, 13
  122. 122. Dandy–Walker malformation 12 63.5 17.6 45–112 67.2 15.1 51–1 80 60 40 20 0 Brainstem–vermisangle(°) Normal Blake’s pouch cyst Vermian hypoplasia Dandy–Walker 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 are indicated by a line inside each box, 25th and 75th percentiles by box limits and 5th and 95th percentiles by lower and upper bars, respectively. had a BV angle < 18◦ and a BT angle < 45◦ . The BV angle was significantly increased in each of the three subgroups of anomalies (Figure 3, Table 2), the angle increasing with increasing severity of the condition. The BT angle demonstrated a similar pattern, but there was more overlapping among groups (Figure 4, Table 2). 80 60 40 20 Brainstem–tentoriumangle(°) Normal Blake’s pouch cyst Vermian hypoplasia Dandy–Walk malformatio Figure 4 Box-and-whisker plot of distribution of brainstem– tentorium angle in controls and in fetuses with upward rotation o the cerebellar vermis. Medians are indicated by a line inside each box, 25th and 75th percentiles by box limits and 5th and 95th percentiles by lower and upper bars, respectively. Table 2 Statistical comparison of brainstem–vermis (BV) and brainstem–tentorium (BT) angles in controls and in fetuses with upward rotation of the cerebellar vermis P (Mann–Whitney U-test) Comparison* BV angle BT angle Blake’s pouch cyst 12 23.0 2.8 19–26 42.2 7.1 32–52 Vermian hypoplasia 7 34.9 5.4 24–40 52.1 7.0 45–66 Dandy–Walker malformation 12 63.5 17.6 45–112 67.2 15.1 51–112 80 60 40 20 0 Brainstem–vermisangle(°) Normal Blake’s pouch cyst Vermian hypoplasia Dandy–Walker 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 are indicated by a line inside each box, 25th and 75th percentiles by box limits and 5th and 95th percentiles by lower and upper bars, respectively. had a BV angle < 18◦ and a BT angle < 45◦ . The BV angle was significantly increased in each of the three subgroups of anomalies (Figure 3, Table 2), the angle increasing with increasing severity of the condition. The BT angle demonstrated a similar pattern, but there was more overlapping among groups (Figure 4, Table 2). DISCUSSION Our results suggest that measurement of the BV angle discriminates accurately posterior fossa fluid collections 80 60 40 20 Brainstem–tentoriumangle(°) Normal Blake’s pouch cyst Vermian hypoplasia Dandy–Walker malformation 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 box, 25th and 75th percentiles by box limits and 5th and 95th percentiles by lower and upper bars, respectively. Table 2 Statistical comparison of brainstem–vermis (BV) and brainstem–tentorium (BT) angles in controls and in fetuses with upward rotation of the cerebellar vermis P (Mann–Whitney U-test) Comparison* BV angle BT angle Controls vs Blake’s pouch cyst fetuses < 0.00000005 < 0.000005 Controls vs Dandy–Walker fetuses < 0.00000005 < 0.00000005 Box-and-whisker plot of distribution of brainstem–vermis angle in controls and in fetuses with upward rotation of the cerebellar vermis. Medians are indicated by a line inside each box, 25th and 75th percentiles by box limits and 5th and 95th percentiles by lower and upper bars, respectively. 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 box,25th and 75th percentiles byboxlimits and 5th and 95th percentiles by lower and upper bars,respectively. Brainstem–vermis Angle Brainstem–TentoriumAngle Sunday, July 28, 13
  123. 123. 84 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 appearancebutaverydifferentprognosis Conclusion Sunday, July 28, 13
  124. 124. 85 In  Summary Sunday, July 28, 13
  125. 125. Examination Of The Posterior Fossa And The Cerebellum Midsagittal ViewsAxial View 86 Sunday, July 28, 13
  126. 126. PracEcal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  CysEc  like  Lesions Sunday, July 28, 13
  127. 127. PracEcal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  CysEc  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? Sunday, July 28, 13
  128. 128. PracEcal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  CysEc  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posiEon  (tentorial   Cerebelli)? Sunday, July 28, 13
  129. 129. PracEcal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  CysEc  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posiEon  (tentorial   Cerebelli)? 3. What  is  the  shape  of  the  cerebellar  cled? Sunday, July 28, 13
  130. 130. PracEcal  Approach  to  the  DD  of  Posterior  Fossa   Cyst  and  CysEc  like  Lesions 1. Is  the  Vermis  Present?Is  the  Vermis  intact? 2. Is  the  Toruclar  in  a  normal  posiEon  (tentorial   Cerebelli)? 3. What  is  the  shape  of  the  cerebellar  cled? 4. Brainstem–vermis (BV) Angle And Brainstem– tentorium (BT) Angle Sunday, July 28, 13
  131. 131. 88 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† *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 KEYWORDS: cerebellar anomalies; Dandy–Walker malformation; fetus; megacisterna magna; prenatal diagnosis; ultrasound ABSTRACT 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. Results During the study period, 105 fetuses were exam- 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. INTRODUCTION Fluid collections in the fetal posterior fossa encompass a wide spectrum of different entities, ranging from normal variants to severe anomalies1 . They may have Figure 1 Categorization of posterior fossa fluid collections on ultrasound: (a,b) Blake’s pouch cyst; (c,d) megacisterna magna; (e,f) vermian hypoplasia; (g,h) Dandy–Walker malformation; (i,j) cerebellar hypoplasia; (k,l) arachnoid cyst of the posterior fossa. Blacke’s  Pouch  Cyst Cystegacisterna  Magna Vermian  Hypoplasia D-­‐W  Malforma,on Cerebellar  Hypoplasia Arachinoid  Cyst-­‐Pos  Fossa   Sunday, July 28, 13
  132. 132. Originally  published  in  Ultrasound  Obstet  Gynecol  2007;  30:  233–245 Normal Megacisterna  magna Blake’s  pouch  cyst Vermian  hypoplasia Dandy-­‐Walker  malforma,on tentorium 89 Sunday, July 28, 13
  133. 133. Standard  and  Fetal   Neurosonography 90 Take  Home  Message Sunday, July 28, 13
  134. 134. 91 Sunday, July 28, 13
  135. 135. 91 ✦examina,on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol Sunday, July 28, 13
  136. 136. 91 ✦examina,on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦Examina,on   should   include   at   least   three   axial   planes. Sunday, July 28, 13
  137. 137. 91 ✦examina,on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦Examina,on   should   include   at   least   three   axial   planes. ✦In   Each   plane   the   defined   landmarks   should   should  be  reported  as  normal  or  suspicious Sunday, July 28, 13
  138. 138. 91 ✦examina,on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦Examina,on   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  abnormali,es  pa,ent   should   be   referred   for   detailed   neuorsonogram   which  include  mutli-­‐planner  3  D  Sanning. Sunday, July 28, 13
  139. 139. 91 ✦examina,on  of  the  Fetal  CNS  should  be  follow  a   Standard  Protocol ✦Examina,on   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  abnormali,es  pa,ent   should   be   referred   for   detailed   neuorsonogram   which  include  mutli-­‐planner  3  D  Sanning. ✦3   D   scanning   with   mul,planner   analysis   offers   comparable  analysis  to  fetal  MRI Sunday, July 28, 13
  140. 140. 92 Thanks   Sunday, July 28, 13
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