Module 5.1 : Fetal Spinal Pathology Flashcards

1
Q

Development of spine

A
  • each vertebrae develop from 3 ossification centers
    • centrum
    • neural processes
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2
Q

What does the centrum form

A
  • central portion of vertebral body
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3
Q

What does the neural processes form

A
  • pedicles
  • transverse processes
  • laminae
  • spinous process
  • posterolateral part of vertebral body
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4
Q

when is Ossification complete

A
  • ossification is not complete in than laminae until 18 weeks
  • can potentially miss a small spina bifida before the
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5
Q

Risk of NTD increase with

A
  • valproic acid (meds for seizures)
  • maternal diabetes
    + type 1 or 2
  • folic acid deficiency
  • history of spinal defects
    + previous pregnancy
    + family history
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6
Q

what is Spina bifida

A
  • defect in bones structure of spinal canal that may result in Bernita ion of its content
  • a neural tube defect
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7
Q

what are the contents of the spine that might herniate in a spina bifida

A
  • meninges
  • CSF
  • neural tissue
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8
Q

2 Other NTD

A
  • encephalocele
    + brain herniation
  • anencephaly
    + missing all = brain and and skull
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9
Q

2 Variations of spina bifida

A

Spina bifida occulta

Spina bifida Alperta

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10
Q

Spina bifida Occulta

A
  • involves only the deeper layers
  • may only see a skin dimple or patch of hair on the skin
  • closed defect
  • less common
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11
Q

Spina bifida aperta

A
  • involves all layers from dura to skin
  • open defect
  • more often
  • two types
    + meningocele
    + myelomeningocele
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12
Q

Spina bifida aperta - meningocele

A
  • contains meninges and CSF
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13
Q

Spina bifida aperta - myelomeningocele

A
  • contains meninges, CSF, and neural tissue

- more common

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14
Q

Screening for NTD - MS AFP

A
  • quad screen at 16 weeks
  • maternal serum alpha fetal protein
  • AFP produced by liver
  • crosses placenta and varies with gestational age
  • AFP is elevated with NTD not covered by skin
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15
Q

6 Other causes of elevated AFP

A
- abdominal wall defects
  \+ omphalocele
  \+ gastrodchisis (bowel outside)
- twins
- fetal death
- urinary obstruction
- cystic hygroma
- incorrect dates
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16
Q

where do NTDs usually occur

A
  • in the lumbosacral spine
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17
Q

NTD in lumbar spine is associated with

A
- Meckel Gruber syndrome
   \+ encephalocele
   \+ polycystic kidneys
   \+ polydactyly
- T18
- triploidy
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18
Q

what is a NTD caused by

A
  • spinal dysraphism
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19
Q

what is spinal dysphism

A
  • abnormal closure of the spine
  • aka rachischisis
  • failure of fusion of the vertebral arches
20
Q

NTD landmarks correspond to what

A
  • level of defect
21
Q

three NTD landmarks

A
  • T-12 most distal rib
  • L-5 superior margin of iliac wing
  • S-4 most caudal ossification center int eh 2nd trimester
22
Q

Other system affected by NTD

A
  • legs
    + club foot
    + hip dislocation (not seen on US)
  • causes by imbalance of muscular activity due to nerve involvement of NTD
  • nerves exposed to amniotic fluid get damages
23
Q

does a higher a lower NTD have a worse prognosis

A
  • the higher the NTD the worse off the baby is
24
Q

Spinal NTD on US

A
  • splayed laminae
  • protruding mass or cyst
  • look for associated Arnold Chiari II and ventriculomegaly
25
Q

What plane is assessment on spine done best in

A

Transverse

26
Q

Prognosis of NTD

A
  • depends on location and extent of neural tissue involved

- more superior the worse off

27
Q

characteristics of Ineincephaly

A
  • special case of dyraphism involving back of cranium and c spine
  • Inein refers to name of neck
  • segmentation errors of upper spine shortens neck and head becomes drosifelxed
  • known as star gazing position
28
Q

ineincephaly is associated with what other NTD

A
  • anencephaly
29
Q

what would you expect to see in the brain with ineincephaly

A
  • hydrocephaly from ventricles not being able to drain
30
Q

what is Scoliosis

A
  • lateral curvature of spine
31
Q

Kyphosis

A
  • exaggerates curvature of spine in sagittal Plane
32
Q

Scoliosis and kyphosis associated with

A
  • amniotic band syndrome
  • skeletal dysplasia
  • VACTERL
  • caudal regression syndrome
33
Q

what is Caudal regression

A
  • range of abnormalities of the spine

+ absence of part of sacrum up to absence of lumbar spine

34
Q

what maternal disease increases risk of caudal regression

A
  • increased risk with

+ type 1 maternal diabetes and insulin dependant diabetes (IDDM)

35
Q

characteristics of Sirenomelia (mermaid syndrome)

A
  • absence of sacrum
  • fusion of legs
  • re tail atresia
  • renal dysgenisis or agenisis
  • oligohydroamnious (no kidneys no urine)
36
Q

prognosis of sirenomelia

A
  • very poor all die in infancy
37
Q

what is the most sever form of caudal regression

A
  • sirenomelia
38
Q

VACTERL sequence

A
  • Vertebral (dysraphism)
  • Anal (atresia)
  • Cardiovascular
  • Tacheo-Esophageal (fistula or atresia)
  • renal
  • Limb (radial Ray)
39
Q

VACTERL is often associated with what 4 other anomalies

A
- caudal regression
   \+ sirenomelia 
- spina bifida
- congenital scoliosis 
- hemi vertebrae
40
Q

prognosis of VACTERL

A
  • depends on severity and extent of involvement
41
Q

what is a Sacroccygeal teratoma

A
  • contains elements of many tissue

- 4 types

42
Q

what three structures can teratomas can arise form

A
  • sacrum or coccyx
  • brain
  • gonads
43
Q

the four types of teratomas

A
  • I external mass predominant (most common)
  • II external mass with internal components
  • III internal mass predominant with smaller external component (least common)
  • IV presacral mass (internal only) (second least common) MOST MISSED
44
Q

Teratomas on US

A
  • mass on rum area adjacent to spine
  • most are solid or mixed = malignant
  • cystic = benign
  • May see calcification
  • displaces pelvic structures may compress ureters = hydronephrosis
  • if AV shunting May see heart failure
45
Q

Teratoma prognosis

A
  • depends on size of mass and severity of structure displacement
  • solid mass more malignant
  • if > 4.5 cm cesarean done
46
Q

DDX of teratoma

A
  • rectal duplication
  • anterior myelomeningocele
  • sarcoma