M5.1: Fetal Spinal Pathology Flashcards

1
Q

Describe the 3 ossification centres of the spine

What do the 2 neural processes form

A

The central part is formed by the centrum

The 2 lateral neural processes form:

Peduncles
Transverse processes
Laminae
Spinous process
Posterolateral part of the vertebral body
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2
Q

When does ossification of the laminae occur

A

18 wks

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3
Q

When in wks could you possibly miss a small spinal bifida

A

Before 18 wks

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4
Q

Risks of and NTD increases with which factors

A
Valproic acid (medication for seizures)
Maternal diabetes (type 1)
Folic acid deficiency
History of spinal defects
 -previous preg
 -family history
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5
Q

What is spinal bifida

A

NTD in the structure of the spinal canal that can lead to the herniation of its contents including possibly meninges, CSF and neural tissue

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6
Q

Describe spina bifida occulta

A
  • involves only the deeper layers
  • May only see a skin dimple or a path of hair on the back
  • closed defect, not easily seen

think of a ‘cult’

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7
Q

Describe spina bifida aperta

A
  • involves all layers from dura to skin
  • open defect
  • accounts for majority of cases

Aperta means open

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8
Q

What are the 2 types of spina bifida aperta

A
  1. meningocele- includes meninges and CSF
    - completely cystic
  2. Myelomeningocele- includes meninges, CSF and neural tissue/nerves
    - not completely cystic
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9
Q

which type of spinal bifida aperta is more severe

A

Myelomeningocele

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10
Q

fetuses w/ a meningocele display what US appearances when scanning the head

A

Arnold Chiari type 2- lemon sign, banana sign, no cistena magna

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11
Q

When is the quad screen done

A

16 wks

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12
Q

When would AFP be elevated w/ an NTD?

A

When the defect is not covered by skin

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13
Q

where is AFP produced by the fetus

does it cross the placenta

A

liver

yes…. its important to consider gestational age and values will vary

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14
Q

What are the other causes of elevated MSAFP

A
Abdo wall defects (Omphalocele or Gastroschisis)
multiple preg
Fetal death
Urinary obstruction
Cystic hygroma
Incorrect dates
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15
Q

NTD are associated w/ what syndromes

A

Meckel gruber syndrome
T18
Triploidy

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16
Q

What is spinal dysraphism

A

Abnormal closure of the spine/failure of fusion of vertebral arches

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17
Q

What is rachischisis

A

Another name for spinal dysraphism

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18
Q

What are the radiological landmarks for the spine

A

T-12; most distal rib
L5: superior margin of the iliac wing/crest
S4: most caudal ossification center in the 2nd trim

19
Q

how are the legs effected by NTDs and why

A

Legs - Club foot and Hip dislocation (not seen on US)

Caused by an imbalance of muscular activity due to nerve involvement of the neural tube defect

20
Q

How does a spinal NTD appear on US

A
  • splayed laminae
  • protruding mass or cyst
  • also look for Arnold chiari and ventriculomegally
21
Q

how do NTDs usually effect amniotic fluid levels

A

polyhydramnios

22
Q

assessment of the spine is best done in which plane

23
Q

What is the prognosis of spinal dysraphism

A

Depends on the location and extent of the neural tissue involved … more superior in the spine the greater the disability

24
Q

What is iniencephaly

A

case of dysraphism involving the back of the cranium and c-spine…

… there are segmentation errors of the upper spine which shorten the neck and head into a dorsiflexed position (called the star gazing position)

25
What would you expect to see in the brain w/ iniencephaly
Hydrocephalus
26
iniencephaly is asociated w/ what other condition
anencephaly
27
What is scoliosis
Lateral curvature of the spine
28
What is kyphosis
Exaggerated curve of the spine in the sag
29
Scoliosis and kyphosis are associated w/ which conditions
Amniotic band syndrome Skeletal dysphasia VACTERL Caudal regression syndrome
30
What is caudal regression
Range of anomalies of the spine.... includes absence of part of the sacrum, up to the absence of the lumbar spine
31
When do you have an increase risk of caudal regression
w/ Type 1 diabetes
32
what is Sirenomelia/Mermaid syndrome? what abonormailites does it cause
fetus lacks a sacrum and legs are fused - rectal atresia - renal agenesis - oligohydramnios (no fetal bladder)
33
what is the prognosis for Sirenomelia/Mermaid syndrome?
fatal
34
what is the most severe form of caudal regression
Sirenomelia/Mermaid syndrome
35
What is the VACTERL sequence
``` Vertebral (dysraphism) Anal (atresia) Cardiovascular Tracheo-esophageal (fistula) Renal Limb (radial Ray) ``` You must have 3+ of these conditions to be VACTERL
36
What conditions are the VACTERL sequence associated w/
Caudal regression spinal bifida congenital scoliosis hemi vertebrae
37
prognosis for VACTERL
depends on severity and extent of involvement
38
what is a sacrococcygeal teratoma | where can they arise in the body?
- rare mass that contains elements of many tissues | - sacrum, coccyx, brain or gonads
39
what are the 4 types of sacrococcygeal teratoma
1. external mass predominant (most common) 2. external mass w/ internal compounds 3. internal mass predominant w/ smaller external components 4. pre sacral mass (internal only)
40
how does a sacrococcygeal teratoma appear on US
- mass in rump area adjacent to the spine - will displace pelvic structures and may compress ureters causing hydro - can show signs of heart failure of theres Av shunting
41
describe the variable echo textures of a sacrococcygeal teratoma how are they linked to bengin vs malignancy
- most are solid or mixed which have malignant tendencies - cystic -benign tendencies - may show calcifications
42
whats the prognosis of a sacrococcygeal teratoma whats the DDX
depends on size and severity of structural displacements rectal duplication, anterior myelomeningocele, sarcoma
43
if a sacrococcygeal teratoma is > what value will the baby have to be delivered by C section
>4.5cm