C11.1 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 boney 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

What is encephalocele

A

Brain herniation

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7
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
  • 15-20% of cases

Occult means hidden or concealed

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8
Q

Describe spina bifida aperta

A
  • involves all layers from dura skin
  • open defect
  • 80-85% of cases

Aperta means open

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9
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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10
Q

When is the quad screen done

A

16 wks

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11
Q

When would AFP be elevated w/ an NTD?

A

When the defect is not covered by skin

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12
Q

What are the other causes of elevated MSAFP

A

Abdo wall defects:

Omphalocele
Gastroschisis

Twins
Fetal death
Urinary obstruction
Cystic hygroma
Incorrect dates
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13
Q

NTD are associated w/ what syndromes

A

Meckel gruber syndrome which includes:
Encephalocele
Polycystic kindeys
Polydactyly

T21
Triploidy

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14
Q

What is spinal dysraphism

A

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

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15
Q

What is rachischisis

A

Another name for spinal dysraphism… failure of fusion of the vertebral arches

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16
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

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17
Q

Which limb or structures of the body are effected by NTD and how

A

Legs:
Club foot
Hip dislocation (not seen on US)

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

18
Q

How does a spinal NTD appear on US

A
  • splayed laminae
  • protruding mass or cyst
  • also look for Arnold chiari
19
Q

Is it normal to have some widening in the thoracic and lumbar areas of the spinal

A

Yes, b/c there are a lot of nerves that come off this area going to the limbs

20
Q

What is the prognosis of meningocele

A

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

21
Q

What is a hemivertebra and what does it lead to

A

Half a vertebra… leads to scoliosis

22
Q

What is iniencephaly

A

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

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

23
Q

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

A

Hydrocephalus

24
Q

What is scoliosis

A

Lateral curvature of the spine

25
Q

What is kyphosis

A

Exaggerated curve of the spine in the sag

26
Q

Scoliosis and kyphosis are associated w/ which conditions

A

Amniotic band syndrome
Skeletal dysphasia
VACTERL
Caudal regression syndrome

VASC

27
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

28
Q

What conditions are the VACTERL sequence associated w/

A

Caudal regression
spinal bifida
congenital scoliosis
hemi vertebrae

29
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

30
Q

When do you have an increase risk of caudal regression

A

w/ Type 1 diabetes or IDDM

31
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 (not fetal bladder)
32
Q

what is the prognosis for Sirenomelia/Mermaid syndrome?

A

fatal, all will die in infancy

33
Q

what is the most severe form of caudal regression

A

Sirenomelia/Mermaid syndrome

34
Q

what is a sacrococcygeal teratoma

where can they arise in the body?

A
  • mass that contains elements of many tissues

- sacrum, coccyx, brain or gonads

35
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)
36
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
37
Q

describe the echo texture of a sacrococcygeal teratoma

A
  • most are solid or mixed (85%) which have malignant tendencies
  • cystic (15%) benign tendencies
  • may show calcifications
38
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

39
Q

do solid sacrococcygeal teratomas have malignant tendencies

40
Q

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

41
Q

It’s best the assess a meningocele in which plane