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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

When does ossification of the laminae occur

A

18 wks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When in wks could you possibly miss a small spinal bifida

A

Before 18 wks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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’

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Describe spina bifida aperta

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

Aperta means open

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

which type of spinal bifida aperta is more severe

A

Myelomeningocele

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

When is the quad screen done

A

16 wks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When would AFP be elevated w/ an NTD?

A

When the defect is not covered by skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

NTD are associated w/ what syndromes

A

Meckel gruber syndrome
T18
Triploidy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is spinal dysraphism

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is rachischisis

A

Another name for spinal dysraphism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

A

TRX

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
Q

What would you expect to see in the brain w/ iniencephaly

A

Hydrocephalus

26
Q

iniencephaly is asociated w/ what other condition

A

anencephaly

27
Q

What is scoliosis

A

Lateral curvature of the spine

28
Q

What is kyphosis

A

Exaggerated curve of the spine in the sag

29
Q

Scoliosis and kyphosis are associated w/ which conditions

A

Amniotic band syndrome
Skeletal dysphasia
VACTERL
Caudal regression syndrome

30
Q

What is caudal regression

A

Range of anomalies of the spine…. includes absence of part of the sacrum, up to the absence of the lumbar spine

31
Q

When do you have an increase risk of caudal regression

A

w/ Type 1 diabetes

32
Q

what is Sirenomelia/Mermaid syndrome? what abonormailites does it cause

A

fetus lacks a sacrum and legs are fused

  • rectal atresia
  • renal agenesis
  • oligohydramnios (no fetal bladder)
33
Q

what is the prognosis for Sirenomelia/Mermaid syndrome?

A

fatal

34
Q

what is the most severe form of caudal regression

A

Sirenomelia/Mermaid syndrome

35
Q

What is the VACTERL sequence

A
Vertebral (dysraphism)
Anal (atresia)
Cardiovascular 
Tracheo-esophageal (fistula)
Renal
Limb (radial Ray)

You must have 3+ of these conditions to be VACTERL

36
Q

What conditions are the VACTERL sequence associated w/

A

Caudal regression
spinal bifida
congenital scoliosis
hemi vertebrae

37
Q

prognosis for VACTERL

A

depends on severity and extent of involvement

38
Q

what is a sacrococcygeal teratoma

where can they arise in the body?

A
  • rare mass that contains elements of many tissues

- sacrum, coccyx, brain or gonads

39
Q

what are the 4 types of sacrococcygeal teratoma

A
  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
Q

how does a sacrococcygeal teratoma appear on US

A
  • 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
Q

describe the variable echo textures of a sacrococcygeal teratoma

how are they linked to bengin vs malignancy

A
  • most are solid or mixed which have malignant tendencies
  • cystic -benign tendencies
  • may show calcifications
42
Q

whats the prognosis of a sacrococcygeal teratoma

whats the DDX

A

depends on size and severity of structural displacements

rectal duplication, anterior myelomeningocele, sarcoma

43
Q

if a sacrococcygeal teratoma is > what value will the baby have to be delivered by C section

A

> 4.5cm