Fetal Neural Tube Pathology Flashcards

1
Q

Where is is CSF produced

A

Choroid plexus

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2
Q

Where are the choroid plexus located

A

Floor of the lateral ventricles
Roof of the third ventricle
Posterior wall of the 4th ventricle

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3
Q

What is the path of CSF flow

A
Produced in the choroid plexus 
Lateral ventricle 
Interventricular foramen
3rd ventricle 
4th ventricle 
Magendie 
Luchka
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4
Q

What are the landmarks for the lateral ventricle measurement

A

Csp
Antrum of the ventricle
V of the ambient cistern
Parieto-occipital fissure

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5
Q

Hydrocephalus is an

A

Increase in CSF that results in the enlargement of the ventricular system

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6
Q

Hydrocephalus is usually due to

A

An obstruction along the pathway of GSF

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7
Q

What is the most common cranial anomaly

A

Hydrocephalus

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8
Q

What are the 2 different types of hydrocephalus

A

True

Ventriculomegaly

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9
Q

What is true hydrocephalus

A

CSF obstruction

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10
Q

What is ventriculomegaly the result from

A

Brain atrophy

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11
Q

What can a brain atrophy be caused by

A

IGUR or stroke in utero

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12
Q

What does ventriculomegaly cause

A

The ventricles enlarge because of a small brain

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13
Q

What are the causes of true hydrocephalus

A

NTD
Aqueduct stenosis
Dandy Walker malformation

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14
Q

What is an aqueduct stenosis

A

CSF can not flow from 3rd ventricle to 4th ventricle

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15
Q

In a aqueduct stenosis which ventricles become enlarged

A

Lateral

3rd

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16
Q

In an aqueduct stenosis which ventricle remains normal

A

4th

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17
Q

What are the 2 different types of Hydrocephalus

A

Extraventicular

Intraventicular

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18
Q

What is spina bifida

A

An extraventricular obstruction

CSF cannot flow normally through spina canal and back up in the ventricles of the brain

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19
Q

In spina bifida what happens to the ventricles

A

They all become affected

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20
Q

What is excess CSF caused by

A

Excess secretion from a choirs plexus papilloma

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21
Q

Is Excess CSF an intra or extra ventricular obstruction

A

Extraventicular

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22
Q

What are the different types of extraventicular obstruction

A

Spina bifida
Excess CSF
Dandy walker malformation

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23
Q

What is a intraventricular obstruction

A

Aqueduct stenosis

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24
Q

What do you do when Evaluating ventricular size

A

Measure the atrial Diameter

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25
Q

The ventricular diameter doesnt change much from ___ to ___ weeks

A

15 - 35

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26
Q

What is the normal measurement for the ventricles

A

7m

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27
Q

What is the upper limit of normal for the ventricles

A

10mm

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28
Q

From the medial ventricle wall to choroid what should the measurement be less than

A

3mm

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29
Q

Choroid plexus should not do what in the atria of the ventricle

A

Dangle

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30
Q

When there is a dangling choroid is will always do what

A

Rest in a gravitationally dependant position

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31
Q

When there is Dangling choroids, how much brain tissue is present

A

Hardly any

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32
Q

What is the % of additional anomalies occurring in cases with hydrocephalus

A

70-80

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33
Q

With an obliterated cisterns magna where will the cerebellum be ___ and what will it demonstrate

A

Right up against the posterior skull, demonstrating a banana sign

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34
Q

In cases of hydrocephalus what is the normal appearance for the skull to have

A

Lemon sign

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35
Q

In Arnold Chiari II malformation, the cranial contents are pulled toward the spine which will do what to the frontal bones

A

Cause them to cave in, causing the lemon sign

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36
Q

When is the lemon sign seen and does it disappear

A

2nd trimester

Goes away in the 3rd trimester due to hydrocephalus from enlarging ventricles

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37
Q

DWM

A

Dandy walker malformation

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38
Q

What is DWM

A

An enlarged cisterns magna and a defect in the cerebellar vermis

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39
Q

In DWM what is the cisterna magna communicating with and through what

A

4th ventricle, through a defect in the cerebellum

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40
Q

In DWM what can possibly be enlarged and why

A

Ventricles because of the pressure in the posterior fossa

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41
Q

In DWM what is the defect in the cerebellar vermis

A

It is absent

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42
Q

In DWM what will eventually occur

A

Hydrocephalus

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43
Q

What is the dandy walker variant

A

Partial agenesis of the vermis, with a smaller cisterna magna and minimal dilation of the ventricles

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44
Q

What is the dandy walker variant associated with

A

Many syndromes

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45
Q

DWM is also associated with

A
Intellectual impairment 
Fetal death 
Agenesis of the corpus callosum
Heart defect 
Genitourinary
Polydactyl
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46
Q

What are the increased risk associated for a fetus developing DWM

A

Maternal viral infection
Alcohol consumption
Diabetes (type 1 or 2, that occurred prior to pregnancy)

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47
Q

What must the cerebellar view include when assessing DWM

A

Cavum. Septi pellucidi
Peduncles
Cerebellum

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48
Q

Is it normal or abnormal for a cisterna magna to measure >1cm

A

Abnormal

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49
Q

What is trying to be proved in a posterior fossa veiw for DWM

A

The cisterna magna is communicating with the 4th ventricle
Is the cerebellar vermis absent or partially absent
Enlarged ventricles

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50
Q

What is a DDX for DWM

A

Arachnoid cyst in the posterior fossa

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51
Q

What is arachnoid cysts

A

Fluid collection in the layers of the arachnoid membrane

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52
Q

Can arachnoid cysts occur anywhere in the brain

A

Yes

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53
Q

When does the corpus callosum start and end development

A

Starts @ 12 wks

Ends @ 20 wks

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54
Q

How does the corpus callosum develop

A

Anterior to posterior

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55
Q

What are the different types of agenesis of the corpus callosum

A

Complete
Partial
Developmental
Acquired

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56
Q

What is developmental agenesis of the corpus callosum

A

Interruption in the formation

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57
Q

What is acquired agenesis of the corpus callosum

A

Insults caused secondary atrophy of previously developed parts

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58
Q

Agenesis of the corpus callosum appearance on US

A

Absence of CSP

Enlargement of the occipital horns of the ventricle only, will have pointed anterior horns (like a tear drop)

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59
Q

Agenesis of the corpus callosum is associated with

A

Can be an isolated finding

other CNS abnormalities

  • Gyral dysplasia
  • DWM

Abnormalities of the face, limbs and GU system

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60
Q

ACC

A

Agenesis of the corpus callosum

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61
Q

If ACC is suspected what will be the most important views needed

A

Profile
Image the top of the head in the midline Sagittal plane
-corpus callosum can be seen in this plane

62
Q

What is the appearance of the corpus callosum on US

A

Hypoechoic structure that sits superior to the CSP

63
Q

If the CSP is absent what will the appearance of the 3rd ventricular be on US

A

Will be elevated or high riding in the head

64
Q

If the fetal head is low in the pelvis what can be done to get visualization for the brain

A

EV

65
Q

If the ACC is isolated or partial what will be the prognosis be for function

A

Could be good as it may not affect function at all

66
Q

Severity of decreased intellect is dependant on

A

Associated CNS abnormalities

67
Q

CPC’s

A

Choroid plexus cysts

68
Q

What are CPS’s

A

Cysts in the choroid plexus

69
Q

Do CPC’c disappear and if so when

A

Yes, in the 2nd trimester

70
Q

What are CPC’s associated with

A

Trisomy 18

71
Q

What is the appearance of CPC’s on US

A

Hypoechoic or anechoic structure within the choroid plexus

72
Q

CPC’s are deemed significant when they measure >/ to what?

A

3mm

73
Q

If CPC’s are found how must they be demonstrated and measured

A

In 2 planes

74
Q

Acrania

A

Absent calvaria

75
Q

Calvaria

A

Skull

76
Q

Anencephaly

A

No cerebral cortex

77
Q

Exencephaly

A

Some cerebral cortex, but is abnormal

May be an early stage of anencephaly

78
Q

What happens when brain tissue is exposed to amniotic fluid

A

It becomes damaged

79
Q

What is the US appearance of Acrania-anencephaly-exencephaly

A
Facial structure and orbits are present 
No calvaria (skull) above the orbits 
-image through face would resemble a frog 
Usually polyhydramnios
Very active fetus
80
Q

US for Acrania-anencephaly-exencephaly can not be reliably diagnosed before _______

A

12/13 weeks

81
Q

Why will Acrania-anencephaly-exencephaly cause polyhydramnios

A

Because the swallowing mechanism would be impaired but the kidneys would still be functions and outputting urine leading to lots of fluid

82
Q

What is the prognosis for Acrania-anencephaly-exencephaly

A

Fatal

83
Q

DDX for anencephaly

A

Amniotic band syndrome

Large encephalocele

84
Q

What do you look for in amniotic band syndrome

A

Amputations
Waving membrane
Large encephalocele

85
Q

What is it called when a fetus has a stroke in utero

A

Hydranencephaly

86
Q

In hydranencephaly there will be

A

Variable absence of the cerebrum

Intact cranial vault and meninges

87
Q

Hydranencephaly is the most severe form of

A

Porencephaly

88
Q

What is a porencephaly

A

Brain bleed, due to low/lack of oxygen

89
Q

Hydranencephaly is due to

A

Occlusion of ICA’s
Infection
Hemorrhage
Destruction and resorption of brain matter

90
Q

Is hydranencephaly common or rare

A

Rare

91
Q

What is the appearance of hydranencephaly on US

A

Intact thalami- never fused
Intact brain stem
May or may not have intact falx cerebri
Irregular brain matter @ periphery

92
Q

What is the DDX of hydranencephaly

A

Severe hydrocephalus

Alobar holoprosencephaly

93
Q

In the DDX of severe hydrocephalus for hydranencephaly what is the US appearance

A

Smooth brain matter along the periphery of cranium

94
Q

In the DDX of alobar holoprosencephaly for hydranencephaly what is the US appearance

A

Fused thalami

95
Q

What is the prognosis of hydranencephaly

A

Fatal

96
Q

Porencephalic cysts occur when

A

Brain tissue is destroyed by hemorrhage or an infarct

97
Q

What is the appearance of porencephalic cysts on US

A

Destroyed brain tissue, that is becoming cystic
-usually along the periventicular halo
- close to lateral ventricles
May or may not communicate with the ventricles

98
Q

What is are the DDX for porencephalic cysts

A

DWM
Arachnoid cyst
Hydrocephaly if it communicates

99
Q

What is the prognosis for porencephalic cysts

A

Dependant on the degree of brain destruction

100
Q

Are arachnoid cysts associated with anything

A

No

101
Q

What is the US appearance of arachnoid cysts

A

Well defined cyst in the brain

102
Q

What can arachnoid cysts demonstrate

A

Mass affect

103
Q

What is the DDX for arachnoid cysts

A

dandy walker variant if it occurs in the posterior fossa

Porencephalic cyst

104
Q

What is the prognosis for an arachnoid cyst

A

Good as long as the cyst does not compromise too much brain tissue or block the ventricles

105
Q

What is an Encephalocele

A

Herniation of intracranial structures through a defect in the cranium

106
Q

Most encephalocele occur where

A

In the midline of the occiput

107
Q

Encephalocele may only contain ___ or can also be with _____

A

Meninges

Or with brain tissue also

108
Q

Can Encephalocele occur anywhere

A

Yes

109
Q

What lab value will be increased with Encephalocele

A

AFP

110
Q

Encephalocele can be associated with

A

Nothing, can be an isolated finding

Often seen with Meckel-Gruber syndrome

111
Q

What is Meckel-Gruber syndrome

A

An autosomal recessive lethal condition

112
Q

Fetuses with Meckel_Gruber syndrome will have what 3 findings on US

A

Encephalocele
Cystic renal dysplasia
Polydactyly

113
Q

In an Encephalocele with only the meninges protruding what is the appearance on US

A

Cystic structure with thin membrane

Cranial defect present

114
Q

Is the brain is protruding in a encephalocele what is the appearance on US

A

Encapsulated echogenic mass, with cystic areas

Cranial defect present

115
Q

What is the DDX of an Encephalocele

A

Cystic hydroma

Teratoma

116
Q

What is the appearance of a cystic hygroma on US

A

Should see septations with the hygroma

117
Q

What is the prognosis of Encephalocele

A

Dependant on:
Size of defect
Amount of brain tissue involved
Whether or not it is associated with meckel-gruber syndrome

118
Q

What is Schizencenphlay

A

Symmetrical clefts in the parietal or temporal lobes that extend from the ventricles to the cortical surface

119
Q

How is the brain split in schizencephaly

A

Anterior and posterior segments

120
Q

What is lissencephaly

A

Smooth brain, has no Salic or gyri due to abnormal migration of neutrons from the germinal matrix

121
Q

When is the Dx for lissencephaly given

A

3rd trimester, not possible before then

122
Q

What is microcephaly

A

Disproportionately small head compared to gestational age and abdominal circumference

123
Q

What would plot the head well below the 5th%

A

> 3 standard deviations below the mean

124
Q

When is micrcephaly seen

A

Late in pregnancy

125
Q

What does the Dx of mircocephaly imply

A

Failure of brain development

126
Q

What would the profile view demonstrate in cases of microcephaly

A

A sloping forehead

127
Q

What occurs in microcephaly due to less brain tissue

A

Ventriculomegaly

128
Q

What is mircocephaly associated with

A

Other developmental abnormalities

129
Q

What are the causes of microcephaly

A
Asphyxia 
Infections (CMV)
Drugs
Alcohol 
Irradiation
130
Q

CMV

A

Cytomegalovirus

131
Q

What is a Vein of Galen Aneurysm

A

A variety of AV malformations drain into this vein and it results in its distension as a single dilated midline fluid collection

132
Q

When would the dilation of the vein of Galen occur

A

3rd trimester

133
Q

What is the sonographic appearance of the vein of Galen

A

Dilated midline collection posterior to the thalamus and mid brain, between the posterior horns of the lateral ventricles

134
Q

How is the vein of Galen easily Dx

A

With colour Doppler

135
Q

What may appear prominent if there is a vein of Galen

A

Neck vessels

136
Q

Vein of Galen can be a sign of what

A

Heart failure

-cardiomyopathy due to AV shunting

137
Q

What is the DDX for a vein of Galen

A

Arachnoid cyst

Porencephalic cyst

138
Q

What is the prognosis for a vein of Galen

A

Poor unless all anastomoses can be cauterized

139
Q

Congenital brain tumors are

A

Rare

140
Q

What is the most common lesion in the fetal brain

A

Teratoma

141
Q

What are teratomas

A

Large echogenic masses with cystic spaces and occasional calcifications

142
Q

What is the prognosis for fetal tumors

A

Dismal

143
Q

What is craniosynstoses

A

Bizarre fusion of the cranial sutures

144
Q

What is the cloverleaf cranial shape

A

All cranial sutures fuse before the brain is finished growing

145
Q

What is one cause for dolichocephaly

A

Breech baby

146
Q

If the view of the brain is especially clear what should be considered

A

Conditions that have poor cranial mineralization

147
Q

Cranial mineralization can cause the brain to be compressed with what

A

Slight pressure

148
Q

In osteogenesis imperfecta and hypophosphatasia what happens to the cranial bones and the brain

A

They are not ossified and the brain can easily be compressed

149
Q

What are the 3 different types of holoprosencephaly

A

Alobar
Semilobar
Lobar

150
Q

What is alobar holoprosencephaly

A

Little or no cortical mantle, single horseshoe shaped ventricle. Fused thalami and absent 3rd ventricle and falx

151
Q

What is semilobar holoprosencephaly

A

Single horseshoe shaped ventricle with brain mantle. Incomplete fusion of the thalami. Absent falx and 3rd ventricle

152
Q

What is lobar holoprosencephaly

A

Fused anterior horns, that are squared off. Incomplete falx and 3rd ventricle may be seen