Fetal Neural Tube Pathology Flashcards

(152 cards)

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
The ventricular diameter doesnt change much from ___ to ___ weeks
15 - 35
26
What is the normal measurement for the ventricles
7m
27
What is the upper limit of normal for the ventricles
10mm
28
From the medial ventricle wall to choroid what should the measurement be less than
3mm
29
Choroid plexus should not do what in the atria of the ventricle
Dangle
30
When there is a dangling choroid is will always do what
Rest in a gravitationally dependant position
31
When there is Dangling choroids, how much brain tissue is present
Hardly any
32
What is the % of additional anomalies occurring in cases with hydrocephalus
70-80
33
With an obliterated cisterns magna where will the cerebellum be ___ and what will it demonstrate
Right up against the posterior skull, demonstrating a banana sign
34
In cases of hydrocephalus what is the normal appearance for the skull to have
Lemon sign
35
In Arnold Chiari II malformation, the cranial contents are pulled toward the spine which will do what to the frontal bones
Cause them to cave in, causing the lemon sign
36
When is the lemon sign seen and does it disappear
2nd trimester | Goes away in the 3rd trimester due to hydrocephalus from enlarging ventricles
37
DWM
Dandy walker malformation
38
What is DWM
An enlarged cisterns magna and a defect in the cerebellar vermis
39
In DWM what is the cisterna magna communicating with and through what
4th ventricle, through a defect in the cerebellum
40
In DWM what can possibly be enlarged and why
Ventricles because of the pressure in the posterior fossa
41
In DWM what is the defect in the cerebellar vermis
It is absent
42
In DWM what will eventually occur
Hydrocephalus
43
What is the dandy walker variant
Partial agenesis of the vermis, with a smaller cisterna magna and minimal dilation of the ventricles
44
What is the dandy walker variant associated with
Many syndromes
45
DWM is also associated with
``` Intellectual impairment Fetal death Agenesis of the corpus callosum Heart defect Genitourinary Polydactyl ```
46
What are the increased risk associated for a fetus developing DWM
Maternal viral infection Alcohol consumption Diabetes (type 1 or 2, that occurred prior to pregnancy)
47
What must the cerebellar view include when assessing DWM
Cavum. Septi pellucidi Peduncles Cerebellum
48
Is it normal or abnormal for a cisterna magna to measure >1cm
Abnormal
49
What is trying to be proved in a posterior fossa veiw for DWM
The cisterna magna is communicating with the 4th ventricle Is the cerebellar vermis absent or partially absent Enlarged ventricles
50
What is a DDX for DWM
Arachnoid cyst in the posterior fossa
51
What is arachnoid cysts
Fluid collection in the layers of the arachnoid membrane
52
Can arachnoid cysts occur anywhere in the brain
Yes
53
When does the corpus callosum start and end development
Starts @ 12 wks | Ends @ 20 wks
54
How does the corpus callosum develop
Anterior to posterior
55
What are the different types of agenesis of the corpus callosum
Complete Partial Developmental Acquired
56
What is developmental agenesis of the corpus callosum
Interruption in the formation
57
What is acquired agenesis of the corpus callosum
Insults caused secondary atrophy of previously developed parts
58
Agenesis of the corpus callosum appearance on US
Absence of CSP | Enlargement of the occipital horns of the ventricle only, will have pointed anterior horns (like a tear drop)
59
Agenesis of the corpus callosum is associated with
Can be an isolated finding other CNS abnormalities - Gyral dysplasia - DWM Abnormalities of the face, limbs and GU system
60
ACC
Agenesis of the corpus callosum
61
If ACC is suspected what will be the most important views needed
Profile Image the top of the head in the midline Sagittal plane -corpus callosum can be seen in this plane
62
What is the appearance of the corpus callosum on US
Hypoechoic structure that sits superior to the CSP
63
If the CSP is absent what will the appearance of the 3rd ventricular be on US
Will be elevated or high riding in the head
64
If the fetal head is low in the pelvis what can be done to get visualization for the brain
EV
65
If the ACC is isolated or partial what will be the prognosis be for function
Could be good as it may not affect function at all
66
Severity of decreased intellect is dependant on
Associated CNS abnormalities
67
CPC’s
Choroid plexus cysts
68
What are CPS’s
Cysts in the choroid plexus
69
Do CPC’c disappear and if so when
Yes, in the 2nd trimester
70
What are CPC’s associated with
Trisomy 18
71
What is the appearance of CPC’s on US
Hypoechoic or anechoic structure within the choroid plexus
72
CPC’s are deemed significant when they measure >/ to what?
3mm
73
If CPC’s are found how must they be demonstrated and measured
In 2 planes
74
Acrania
Absent calvaria
75
Calvaria
Skull
76
Anencephaly
No cerebral cortex
77
Exencephaly
Some cerebral cortex, but is abnormal | May be an early stage of anencephaly
78
What happens when brain tissue is exposed to amniotic fluid
It becomes damaged
79
What is the US appearance of Acrania-anencephaly-exencephaly
``` 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
US for Acrania-anencephaly-exencephaly can not be reliably diagnosed before _______
12/13 weeks
81
Why will Acrania-anencephaly-exencephaly cause polyhydramnios
Because the swallowing mechanism would be impaired but the kidneys would still be functions and outputting urine leading to lots of fluid
82
What is the prognosis for Acrania-anencephaly-exencephaly
Fatal
83
DDX for anencephaly
Amniotic band syndrome | Large encephalocele
84
What do you look for in amniotic band syndrome
Amputations Waving membrane Large encephalocele
85
What is it called when a fetus has a stroke in utero
Hydranencephaly
86
In hydranencephaly there will be
Variable absence of the cerebrum | Intact cranial vault and meninges
87
Hydranencephaly is the most severe form of
Porencephaly
88
What is a porencephaly
Brain bleed, due to low/lack of oxygen
89
Hydranencephaly is due to
Occlusion of ICA’s Infection Hemorrhage Destruction and resorption of brain matter
90
Is hydranencephaly common or rare
Rare
91
What is the appearance of hydranencephaly on US
Intact thalami- never fused Intact brain stem May or may not have intact falx cerebri Irregular brain matter @ periphery
92
What is the DDX of hydranencephaly
Severe hydrocephalus | Alobar holoprosencephaly
93
In the DDX of severe hydrocephalus for hydranencephaly what is the US appearance
Smooth brain matter along the periphery of cranium
94
In the DDX of alobar holoprosencephaly for hydranencephaly what is the US appearance
Fused thalami
95
What is the prognosis of hydranencephaly
Fatal
96
Porencephalic cysts occur when
Brain tissue is destroyed by hemorrhage or an infarct
97
What is the appearance of porencephalic cysts on US
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
What is are the DDX for porencephalic cysts
DWM Arachnoid cyst Hydrocephaly if it communicates
99
What is the prognosis for porencephalic cysts
Dependant on the degree of brain destruction
100
Are arachnoid cysts associated with anything
No
101
What is the US appearance of arachnoid cysts
Well defined cyst in the brain
102
What can arachnoid cysts demonstrate
Mass affect
103
What is the DDX for arachnoid cysts
dandy walker variant if it occurs in the posterior fossa | Porencephalic cyst
104
What is the prognosis for an arachnoid cyst
Good as long as the cyst does not compromise too much brain tissue or block the ventricles
105
What is an Encephalocele
Herniation of intracranial structures through a defect in the cranium
106
Most encephalocele occur where
In the midline of the occiput
107
Encephalocele may only contain ___ or can also be with _____
Meninges | Or with brain tissue also
108
Can Encephalocele occur anywhere
Yes
109
What lab value will be increased with Encephalocele
AFP
110
Encephalocele can be associated with
Nothing, can be an isolated finding | Often seen with Meckel-Gruber syndrome
111
What is Meckel-Gruber syndrome
An autosomal recessive lethal condition
112
Fetuses with Meckel_Gruber syndrome will have what 3 findings on US
Encephalocele Cystic renal dysplasia Polydactyly
113
In an Encephalocele with only the meninges protruding what is the appearance on US
Cystic structure with thin membrane | Cranial defect present
114
Is the brain is protruding in a encephalocele what is the appearance on US
Encapsulated echogenic mass, with cystic areas | Cranial defect present
115
What is the DDX of an Encephalocele
Cystic hydroma | Teratoma
116
What is the appearance of a cystic hygroma on US
Should see septations with the hygroma
117
What is the prognosis of Encephalocele
Dependant on: Size of defect Amount of brain tissue involved Whether or not it is associated with meckel-gruber syndrome
118
What is Schizencenphlay
Symmetrical clefts in the parietal or temporal lobes that extend from the ventricles to the cortical surface
119
How is the brain split in schizencephaly
Anterior and posterior segments
120
What is lissencephaly
Smooth brain, has no Salic or gyri due to abnormal migration of neutrons from the germinal matrix
121
When is the Dx for lissencephaly given
3rd trimester, not possible before then
122
What is microcephaly
Disproportionately small head compared to gestational age and abdominal circumference
123
What would plot the head well below the 5th%
>3 standard deviations below the mean
124
When is micrcephaly seen
Late in pregnancy
125
What does the Dx of mircocephaly imply
Failure of brain development
126
What would the profile view demonstrate in cases of microcephaly
A sloping forehead
127
What occurs in microcephaly due to less brain tissue
Ventriculomegaly
128
What is mircocephaly associated with
Other developmental abnormalities
129
What are the causes of microcephaly
``` Asphyxia Infections (CMV) Drugs Alcohol Irradiation ```
130
CMV
Cytomegalovirus
131
What is a Vein of Galen Aneurysm
A variety of AV malformations drain into this vein and it results in its distension as a single dilated midline fluid collection
132
When would the dilation of the vein of Galen occur
3rd trimester
133
What is the sonographic appearance of the vein of Galen
Dilated midline collection posterior to the thalamus and mid brain, between the posterior horns of the lateral ventricles
134
How is the vein of Galen easily Dx
With colour Doppler
135
What may appear prominent if there is a vein of Galen
Neck vessels
136
Vein of Galen can be a sign of what
Heart failure | -cardiomyopathy due to AV shunting
137
What is the DDX for a vein of Galen
Arachnoid cyst | Porencephalic cyst
138
What is the prognosis for a vein of Galen
Poor unless all anastomoses can be cauterized
139
Congenital brain tumors are
Rare
140
What is the most common lesion in the fetal brain
Teratoma
141
What are teratomas
Large echogenic masses with cystic spaces and occasional calcifications
142
What is the prognosis for fetal tumors
Dismal
143
What is craniosynstoses
Bizarre fusion of the cranial sutures
144
What is the cloverleaf cranial shape
All cranial sutures fuse before the brain is finished growing
145
What is one cause for dolichocephaly
Breech baby
146
If the view of the brain is especially clear what should be considered
Conditions that have poor cranial mineralization
147
Cranial mineralization can cause the brain to be compressed with what
Slight pressure
148
In osteogenesis imperfecta and hypophosphatasia what happens to the cranial bones and the brain
They are not ossified and the brain can easily be compressed
149
What are the 3 different types of holoprosencephaly
Alobar Semilobar Lobar
150
What is alobar holoprosencephaly
Little or no cortical mantle, single horseshoe shaped ventricle. Fused thalami and absent 3rd ventricle and falx
151
What is semilobar holoprosencephaly
Single horseshoe shaped ventricle with brain mantle. Incomplete fusion of the thalami. Absent falx and 3rd ventricle
152
What is lobar holoprosencephaly
Fused anterior horns, that are squared off. Incomplete falx and 3rd ventricle may be seen