Module 5 : Fetal Neuro Pathology Flashcards

1
Q

What produces CSF

A

Choroid plexus

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2
Q

Where are the choroid plexus located

A
  • body of lat ventricle
  • roof of third vent
  • superior lateral walls of fourth vent
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3
Q

Flow of CSF through the brain

A

Choroid plexus&raquo_space; lat vents&raquo_space; interventricular foramen&raquo_space; 3rd vent&raquo_space; cerebral aqueduct&raquo_space; fourth vent&raquo_space; megendie ( spinal cord) and Lushka ( brain)

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4
Q

landmarks for lateral ventricle measurement

A
  • CSP
  • antrum of ventricle
  • V of the ambient cistern
  • parietal-occiptal fissure
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5
Q

Normal fourth vent location

A
  • anterior and inferior edge of cerebellum
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6
Q

Hydrocephalus

A
  • increase in CSF that results in enlargement of ventricular system
  • usually due to obstruction along the pathway to CSF
  • MOST COMMON CRANIAL ANOMALY
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7
Q

two causes of hydrocephalus

A
- true hydrocephalus 
   \+ CSF obstruction 
- ventriculomegaly 
   \+ resulting from brain atrophy 
   \+ small brain, ventricles have space to enlarge into
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8
Q

three causes of true hydrocephalus

A
  • neural tube defect NTD
  • aquaductal stenosis
  • dandy walker malformation
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9
Q

Intraventricular obstruction hydrocephalus - aqueduct stenosis

A
  • csf can’t flow from 3rd vent to 4th vent

- lat and 3rd bent enlarged but 4th vent normal

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10
Q

Extraventricular obstruction hydrocephalus - spine bifida

A
  • CSF can’t flow normally through the spinal canal and back up in ventricle of brain
  • ALL ventricles effects s
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11
Q

Extraventricukar obstruction hydrocephalus - excess CSF

A
  • less common

- excess secretion from a choroid plexus papilloma (tumor)

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12
Q

Evaluating ventricular size

A
- measure ATRIAL diameter
  \+ size doesn’t change much 15 - 35
   \+ normal size 7mm
   \+ 10 mm upper limit of normal
- < 3mm from medial vent wall to choroid
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13
Q

Dangling choroid

A
  • always rests in a gravitationally dependent position

- sign of hydrocephalus

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14
Q

Where to look when hydrocephalus is suspected

A
  • additional anomalies tend to occur with hydrocephalus
  • posterior fossa views
    + cisterna magna and cerebellum
    = obliterated cisterna magna
    = deformed cerebellum (banana)
    = lemon sign
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15
Q

Banana sign

A
  • indicates obliterated cisterna magna

- often ARNOLD CHIARI

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16
Q

Lemon sign

A
  • resulting when cranial contents are pulled toward spine with Arnold Chiari II malformation
    + associated with spina bifida
  • frontal bones caved in
  • seen in second trimester
  • lemon sign disappears in 3rd trimester due to resulting in hydrocephalus from enlarging ventricles of blocked CSF
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17
Q

Dandy walker malformation DWM

A
  • enlarged cisterna magna and defect in the cerebellar VERMIS
  • the cisterna magna communicates with the 4th vent through a defect in cerebellum
  • ventricles can be enlarged due to pressure in the post fossa
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18
Q

Dandy walker variant

A
  • partial agenisis of the vermis with a smaller cisterna magna and minimal dilation of the ventricles
  • associated with many syndromes
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19
Q

DWM associated with

A
  • intellectual impairment and fetal steam
  • AGENISIS OF CORPUS CALLOSUM
  • heart defects
  • genitourinary
  • polydactyly
  • increase risk
    + maternal viral infection
    + alcohol consumption
    + maternal diabetes
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20
Q

Assessing DWM

A
  • cisterna magna > 1cm abnormal
  • cerebellar view must include
    + caveman septi pellucidi
    + peduncles
    + cerebellum
  • measure
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21
Q

What to prove for DWM

A
  • cisterna magna communicates with 4th vent
  • cerebbellaR VERMIS absent or partially absent
  • enlarged ventricles
  • DDx = arachnoid cyst in posterior fossa
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22
Q

when does the corpus callous begin and finish developing

A
  • starts 12 weeks ends at 20 weeks
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23
Q

development of corpus callous

A
  • anterior to posterior
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24
Q

absence of corpus callosum extent and cause

A
  • complete or partial

- developmental or acquired

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25
Q

developmental absence of corpus callosum cause

A
  • interruption in the formation
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26
Q

acquired absence of corpus callosum cause

A
  • insults cause secondary atrophy of previous developed parts
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27
Q

absence of corpus callosum associated with what

A
  • may be isolated
  • CNS abnormalites
    + gyro dysplasia (smooth brain)
    + DWM
  • also associated with anomalies of the face limbs and genitourinary system
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28
Q

sonography of agenisis of corpus callosum ACC

A
  • ABSENCE OF CSP

- enlargement of occipital horns only

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29
Q

6 steps to take if ACC suspected

A
  • profile image
  • image top of head in sagitall plane
  • CC should be visualized in this plane
  • hypo echoic structure that sits superior to CSP
  • if CSP is absent the 3rd ventricle will be elevated or high riding in the head
  • try EV if fetal head is low in pelvis
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30
Q

prognosis of ACC

A
  • if isolated and partial may not effect function at all

- severity of deceased intellect depends on associated CNS abnormalities

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31
Q

Choroid plexus cysts CPC

A
  • cysts in choroid plexus
  • usually disappear in 2nd trimester
  • associated with trisomy 18
    + 1 / 200 are associate with T18
  • HAVE TO BE > 3MM
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32
Q

Acrania

A
  • absent skull / cal aria
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33
Q

Anencephaly

A
  • no or distorted cerebral cortex
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34
Q

Exenceohaly

A
  • some cerebral cortex but abnormal
  • early stage of anencephaly
  • brain tissue exposed to amniotic fluid gets damages
  • brain tissue in beginning (excencephaly) then by time fetus is imaged there is minimal tissue left so diagnosed anencephaly
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35
Q

Sonographic features of anencephaly

A
  • facial structures present and orbits
  • no calvaria (skull) above orbits
  • image through face would resemble a frog
  • polyhydroamnios (no swallowing)
  • very active fetus
  • can not be reliable diagnoses before 12-13 weeks
36
Q

ddx for anencephaly

A
- amniotic band syndrome 
   \+ look for other amputation 
   \+ waving membrane
   \+ fetus stuck to side wall 
- large encephalocele
  \+ look for any sign of cranial bones
37
Q

hydranencephaly

A
  • variable absence of cerebrum
    + destruction of brain tissue
  • intact cranial vault and meninges
  • most sever form of porencephaly
38
Q

what causes hydranenchephaly

A
  • occlusion of ICA
  • infection r hemorrhage
  • destruction and resorption of brain matter
39
Q

sonography of hydranenchaply

A
  • intact thalami with are never fused and brain stem
  • may or may not have intact falx cerebri
  • irregular brain matter at periphery
40
Q

DDX of hydranencephaly

A
  • sever hydrocephalus
    + smooth along periphery of cranium
  • alobar holoprosencephaly
    + would have fused thalami
41
Q

prognosis of hydranencephaly

A
  • fatal
42
Q

Arachnoid cysts in post fossa

A
  • fluid collections in the layers of teh arachnoid membrane

- cysts can occur anywhere in the brain

43
Q

when do porencephalic cysts occur

A
  • occurs when brain tissue is destroyed by hemorrhage or an infarct
44
Q

what does porencephalic look like on ultrasound

A
  • destroyed brain tissue becomes cystic
  • usually along the periventricular halo
    + close to lateral ventricles
  • may or may not communicate with the ventricles
45
Q

DDX of porenchepalic cysts

A
  • dandy walker malformation
  • arachnoid cyst
  • hydrocephaly if it communicates
46
Q

prognosis of porenchepalic cysts

A
  • depends on degree of brain destruction
47
Q

DDX of arachnoid cysts

A
  • dandy walker variant if in posterior fossa

- porenchephalic cysts

48
Q

prognosis of arachnoid cysts

A
  • is good as long as cyst does not destroy too much brain tissue or block ventricles
49
Q

what is encephalocele

A
  • herniation of inter cranial structures through a defect in the cranium
50
Q

what is contained in an encephalocele

A
  • may contain only meninges

- or with brain tissue also

51
Q

where do most encephaloceles occur

A
  • midline occiput

- but can be anywhere if in nasal area difficult to see

52
Q

what lab value will be increased with an encephalocele

A
  • AFP
53
Q

common syndromes associated with encephalocele

A
  • isolated

- meckel-gruber syndrome

54
Q

what is meckel - Gruber syndrome and what are 3 signs of it

A
  • autosomal recessive lethal condition
  • encephalocele
  • cystic renal dysplasia (large echogenic kidneys)
  • polydactyly
55
Q

sonography of encephalocele - if only meninges protruding

A
  • cystic structures within membrane

- cranial defect present

56
Q

sonography of encephalocele - if brain is protruding

A
  • encapsulated echogenic mass with cystic areas

- cranial defect present

57
Q

what other brain anomaly will occur with encephalocele

A
  • hydrocephalus due to CSF blockage
58
Q

DDX of encephalocele

A
  • cystic hygroma
    + should see septations in cystic area with hygromas
  • teratoma
59
Q

prognosis of encephalocele

A
  • depends
    + size of defect
    + amount of brain tissue involved
    + whether or not it is associated with meckel-gruber syndrome
60
Q

what is schizencepahly

A
  • symmetrical clefts in the parietal and temporal lobes that extend from the ventricles to the cortical surface
  • brain is split into anterior and posterior segments of the brain
61
Q

what is lissencephaly

A
  • smooth brain
  • ## no sulci or gyri due to abnormal migration of neutrons from the germinal matrix
62
Q

in what trimester is diagnosis of lissencephaly possible

A
  • third trimester
63
Q

what is microcephaly and when in the pregnancy is it seen

A
  • disproportionally small head compared to gestational age and abdominal circumference
  • late pregnancy
64
Q

how many standard deviations below average is considered microcephaly and what does this diagnosis imply about future brain development

A
  • > 3

- implies failure of brain development

65
Q

sonography of microcephaly

A
  • profile would demonstrate a sloping forehead

- ventriculomegaly occurs due to less brain tissue

66
Q

what other abnormalities are associated with microcephaly

A
  • many other developmental abnormalities
67
Q

causes of microcephaly

A
  • asphyxia
  • infections (cytomegalovirus CMV)
  • drugs
  • alcohol
  • radiation
68
Q

what is a vein of Galen aneurysm

A
  • a variety of AV malformations drain into the vein of Galen and result in tis distention as a single dilated midline fluid collection
69
Q

when does dilation of the vein of Galen occur

A
  • third trimester
70
Q

sonography of vein of Galen

A
  • dilated midline collection posterior to the thalamus and midbrain between the posterior horns of the lateral ventricles
  • easily diagnosed with color doppler
  • neck vessels appear prominent
  • signs of heart failure
    + cardiomyopathy due to AV shunting
71
Q

DDX of vein of Galen

A
  • arachnoid cyst

- porencephalic cyst

72
Q

prognosis for vein of Galen

A
  • poor unless all anastomosis can be cauterized
73
Q

are congenital tumors of the brain common or rare

A
  • rare
74
Q

what is the most common tumor of the brain

A
  • teratoma

+ large echogenic masses with cystic space and occasional calcifications

75
Q

what is the prognosis of tumors in the brain

A
  • dismal
76
Q

3 different cranial shapes

A
  • craniosynostoses
  • dolichocephalic
  • brachiocephalic
77
Q

what does a craniosynostoses head look like

A
  • bizarre fusion of the cranial sutures

- clover leaf appearance= all fuse before brain is finished growing

78
Q

what does a dolichocephalic head look like

A
  • BPD/OFD = CI (cephalic index)
  • <75
  • narrow head
  • one cause is a breech baby
79
Q

what does a brachiocephalic head look like

A
  • BPD/OFD = CI
  • > 85
  • wide head
80
Q

what is cranial mineralization

A
  • if view of the brain is especially clear you should consider conditions which have poor mineralization
  • also if brain can be compressed with slight pressure
81
Q

in what 2 syndromes are the cranial bones not ossified and what can you do the the skull as a result

A
  • osteogenesis imperfecta
  • hypophosphatasia
  • can compress the brain
82
Q

three types of holoprosencepahly

A
  • alobar
  • semilobar
  • lobar
83
Q

characteristics of alobar holoprosencephaly

A
  • little to no cortical mantle
  • single HORSESHOE shaped ventricle
  • fused thalami and absent 3rd ventricle and falx
84
Q

characteristics of semilobar holoprocencephaly

A
  • single horseshoe shaped ventricle with brain mantle
  • incomplete fusion of thalami
  • absent falx and third ventricle
85
Q

characteristics of lobar holoprosencephaly

A
  • fused anterior horns that are squared off

- incomplete falx and 3rd ventricle may be seen