Spina Bifida

Question 1

Max acceptable bladder pressure

Answer 1

40 cm H20

Question 2

When to do surgery for scoli in spina bifida

Answer 2

50 degrees

Question 3

Meningomyelocele

Answer 3

herniation of meninges and neural elements – most common
Associated with hydrocephalus and Chiari type 2
75% lumbosacral

Question 4

Nerve we can feel related to bladder

Answer 4

Somatic nerves via pudendal, S2-S4 innervate the skeletal muscle component of the external urethral sphincter, contract during fill, relax during emptying.

Question 5

Worst outcome of Chiari type II

Answer 5

Respiratory compromise
Impaired hand function
Lower cranial nerve function

Question 6

Closed forms of Neural Tube Defects

Answer 6

encephalocele
meningocele
iniencephaly
lipomeningocele
sacral genesis
occult spinal dysraphism

Question 7

What level in spina bifida have most hip disloc

Answer 7

L3 based on muscle imbalance of hip flex and adduction present, other opposing do not

Question 8

Environmental occupational increase in NTD

Answer 8

painters, agriculture, industrial process workers, healthcare, transportation (solvents)

Question 9

How much does 4 mg/day help

Answer 9

Reduces risk by 70%

Question 10

Folic acid supplementation

Answer 10

All women childbearing: 400 micrograms/day
Women w spina bifida or prior NTD preg 4 mg (day 1 to 3 months)
High risk pregame’s (valproic acid or diabetes) 4 mg/day

Question 11

What is spina bifida occulta

Answer 11

bony defect with no herniation of meninges or nerves

CAN have tethered cord

Question 12

Effect of folic acid supplementation in grains in USA

Answer 12

4,000 to 3,000 NTD per year

Question 13

How many kids with Spina Bifida has tethered cord

Answer 13

Almost all (not all symptomatic)

Question 14

Calcium supplement Spina bifida

Answer 14

500 mg age 1-3
800 mg 3-8
1,300 mg after

Question 15

What does folate do

Answer 15

cofactor for the enzymatic process involved in purine and pyrimidine synthesis and facilitates the transfer of methyl groups during the metabolism of methionine and homocysteine

Question 16

Are L4 and L5 spina bifida levels ambulators

Answer 16

Yes, by 4 years

Question 17

CNS malformation associated with NTDs

Answer 17

Spinal Cord tethering
Arnold -Chiari malformations
Hydrocephalus
Forebrain abnormalities (that cause decreased cognition)

Question 18

MTHFR

Answer 18

increased risk level 7.2 times

Question 19

Med most associated with NTD

Answer 19

Valproic acid

Question 20

Signs of hydrocephalus

Answer 20

headache
vomiting
drowsiness
change in behavior
diplopia
papilledema

Question 21

Signs of tethered cord in Spina Bifida

Answer 21

Increased lower extremity spasticity
Decline in lower extremity strength
Worsening scoliosis
Back pain
Worsened urologic function

Question 22

Kind of foot joint to watch for in spina bifida

Answer 22

Charcot joints

Question 23

Elevated homocysteine levels

Answer 23

known risk factor

Question 24

Effect of personal intake of folic acid in preg

Answer 24

50% decrease NTD

Question 25

Spinal level of parasympathetic innervation of bladder

Answer 25

S2 to S4, innervates the funds of the bladder and releases acetylcholine which causes emptying

Question 26

Other test for spinabifida

Answer 26

Amnio | Fetal MRI

Question 27

How common is Syrinx in Spina Bifida

Answer 27

40%

Question 28

Meningocele

Answer 28

Bony defect with herniation of meninges

Question 29

Most important muscles in Spina bifida for gait

Answer 29

Hip abductors

Question 30

Goal of feet in spina bifida

Answer 30

Plantar-grade feet

Question 31

What is the classic lemon and banana sign on Ultrasound

Answer 31

How spina bifida looks spina bifida, splaying of pedicles plus shape of head and banana shaped foramen magnum

Question 32

Age of sacral level spina bifida ambulator

Answer 32

2 years

Question 33

Scoliosis risk in lumbosacral spinal bifida

Answer 33

5 to 10%

Question 34

Other things to watch out for with Chiari type II malformations

Answer 34

``` Worsened function to the point of tetraplegia Ocular motility (saccadic eye, fix, and pursuit) ```

Question 35

Nonverbal learning disorders NVLD are common in spina bifida

Answer 35

Difficulty with Motor Visual-spatial Social

Question 36

Signs of syrinx in spina bifida

Answer 36

Worsening function above level of lesion

Question 37

Scoliosis risk in Thoracic Spina Bifida

Answer 37

80 to 100%

Question 38

Prenatal screening

Answer 38

Quad screening: 1. Alpha feta-protein (AFP) 2. human chorionic gonadotropin (HCG) 3. estriol 4. inhibin A

Question 39

Genes involved in NTD

Answer 39

Sonic Hedgehog PTC gene SCRIB PRICKLE

Question 40

Open forms of Neural tube defects

Answer 40

anencephaly | myelomeningocele

Question 41

Bladder capacity

Answer 41

Sum of age + 2 in oz Babies is weight in kg x 7 Teans, 400-500

Question 42

Chiari Type II Malformation

Answer 42

``` Very common (80-90%) Medulla, pons, elongated 4th ventricle, cerebellar vermis into the cervical spine. Almost always hydrocephalus ```

Question 43

Spinal level of sympathetic innervation of bladder

Answer 43

T10-L1 The sympathetic control causes the detrusor to relax and bladder neck and posterior urethra to contract, effectively allowing storage of urine

Question 44

Which population has ongoing higher spina bifida

Answer 44

Hispanics