Neural Tube Defects Questions

What are 5 common signs of shunt malfunction in infants:

1. Bulging fontanelle: soft spot located on top of head 2. Vomiting 3. Appetite change 4. "Sunset" eyes: eyes driven downward & lower part of pupil may be covered by lower eyelid 5. Edema, redness along shunt tract

Common signs of spina bifida occulta include (4) ____

1. Hairy patch over distal spine defect 2. Birthmark over distal spine (ex: hemangioma: rubbery, bright red nodule of extra blood vessels) 3. Lipoma: usually harmless deposit of fat under skin 4. Cavus feet (rare) (high arch)

What are 4 common signs of shunt malformation in toddlers?

1. Vomiting 2. Irritability 3. Headaches 4. Edema, redness along shunt tract

How early can myelodysplasia be identified by a skilled ultrasonagrapher?

16-18 weeks (or earlier)

What muscles are present (3/5 MMT) in a high lumbar level of malformation? (Select all that apply) A. Hip flexor & sartorius B. Hip ABDuctors C. Hip ADDuctor D. Knee extensors E. Ankle DF

3/5: hip flexors, hip ADDuctors & knee extensors

If prenatal surgery cannot be done, surgical repair & closure of a myelomeningocele usually occurs within ____

72 hours of birth

Motor classification thoracic level is ___ A. L1 & above B. L2-L3 C. L4-L5 D. S1-S2

A. L1 & above

Which of the following is true? A. Neural tube fuses first in middle and later at the 2 ends B. Neural tube fuses first at the 2 ends and later in the middle C. No one knows yet

A. Neural tube fuses first in middle and later at the 2 ends

What level often has hip flexion, hip ABDuction & hip ER contractions? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

A. Thoracic

What level often requires supervised living through lifespan & ambulation is therapeutic at best with parapodium? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

A. Thoracic

What are providers looking for with AFP testing?

AFP levels will be high (sample taken from material serum or amniotic fluid assay)

What muscles are absent in a high lumbar level of malformation? (Select all that apply) A. Hip flexor B. Hip extensors C. Hip ABDuctors D. Knee extensors E. Knee flexors F. All ankle movements

Absence of hip extensors, hip ABDuctors, knee flexors & all ankle movements

What are 2 ways for prenatal diagnosis of spina bifida?

Alpha-fetoprotein protein (AFP) testing or skilled ultrasonagraphers

How early prior to conception should a women start taking folic acid supplements?

At least 1 month prior

Why are women supposed to avoid hot tubs or saunas when pregnant?

Avoid overheating your body due to the associated risk of spina bifida (treat any fever ASAP too)

Development of the NS begins early in the ____ gestational week A. 2nd B. 3rd C. 4th D. 5th

B. 3rd

What level may achieve independence with increased modifications (walker/crutches early on, WC later on) & therapeutic ambulation with AD & HKAFO/RFO? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

B. High lumbar

Motor classification high lumbar is ___ A. L1 & above B. L2-L3 C. L4-L5 D. S1-S2

B. L2-L3

Strength of what muscles are absent in a low lumbar level of malformation? (Select all that apply) A. Hip extension B. Ankle DF C. Ankle PF D. All of the above

C. Ankle PF

Motor classification low lumbar is ___ A. L1 & above B. L2-L3 C. L4-L5 D. S1-S2

C. L4-L5

What level will have improved functional ID due to hip flexor/ADDuctor, medial hami & quad strength so they likely only required an AD with KAFO/solid AFO for ambulation - likely need wheeled mobility for longer community distances? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

C. Low lumbar

Failure of the neural tube to close at any point along the ____ initiates the defect of spina bifida

Caudal border

Hydrocephaly can cause caudal displacement of ____ into the ___

Caudal displacement of cerebellar tonsils & 4th ventricle into foremen magnum or upper cervical canal

If a women has a prenatal diagnosis of spina bifida, what does the birth plan usually consist of?

Cesarean section

Tethering can develop after SCI where scar tissue can block flow of fluids around spinal cord -> fluid pressure may cause ____ to form in the SC, a condition called ____

Cysts, syringomyelia

What muscle group has variable strength in a high sacral level of malformation? (Select all that apply) A. Hip ABDuctors B. Knee extensors C. Ankle DF D. Ankle PF

D. Ankle PF

What level likely only has contractures at the foot level? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

D. High sacral

What level may or may not require an AD, typically requires some type of orthosis (AFO/SMO/shoe insert), may not need support at home but still may use an AD for longer community distances? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

D. High sacral

Motor classification high sacral is ___ A. L1 & above B. L2-L3 C. L4-L5 D. S1-S2 E. S3-S5

D. S1-S2

List some goals/interventions for spina bifida?

Determine AD/orthosis, prevent contractures, facilitate head/trunk control, monitor motor/sensory function at baseline & reassessments, caregiver education et.c.

What level will be functionally independent and an independent community ambulator & may only require shoe inserts? A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

E. Low sacral

Motor classification low sacral is ___ A. L1 & above B. L2-L3 C. L4-L5 D. S1-S2 E. S3-S5

E. S3-S5

T/F: myelomeningoceles are just covered with skin

False, covered with skin, thin membrane, lipoma or they may be ruptured

T/F: even though the SC & nerves are usually normal in spina bifida occulta, this type usually causes disability or symptoms

False, rarely causes disability or symptoms

T/F: neural tube defects can only be repaired via postnatal surgery

False, with new technology many can be repaired via prenatal surgery

A lack of this nutrient ____ has been linked to an increased risk of spina bifida & what is the daily recommended amount?

Folic acid, 400 micrograms every day

What are other common orthopedic issues?

Foot deformities (club foot), hip dysplasia, severe contractures, spinal deformities (coronal/transverse plane deformities)

What are common signs of shunt malformation in school-aged kids?

Headaches, vomiting, edema/redness along shunt tract, lethargy irritability, seizures, handwriting changes, rapid growth of head circumference, new signs of nystagmus/eye squint, memory changes & personality changes

What are common foot contractures seen in spina bifida?

Heelcord, clubfoot & calcaneovalgus

Low lumbar level of malformation has weakness in ____ (3)

Hip extension, knee flexion & ankle/foot movements

What muscle strength is present in a low lumbar level of malformation? (Select all that apply) A. Quads B. Hip extension C. Hip flexor & ADDuctor D. Ankle PF E. Medial hamstring

Hip flexor, hip ADDuctor, medial hamstring & quads

What population of women has the highest rate of having a child affected by spina bifida?

Hispanic women

A ventriculoperitonal shunt (VP shunt) is used to treat ____ and is placed where?

Hydrocephaly - placed in the ventricles & travels down to the peritoneal cavity

What are 2 common urological problems and a treatment option?

Incontinence & increased bladder infections = self-catheterization

Women with a Hx of NTD need to ____ their daily folic acid intact

Increase (4000 micrograms)

There's an increased incidence of this allergy ___ in spina bifida kids (reported up to 73%)

Latex allergy

If someone has all muscle activity + bowel/bladder function may be normal, this person likely has what level of malformation?

Low sacral level (S3-S5)

80% of neural tube defects occurs in ____ region

Lumbar

What is the difference between meningocele and myelomeningocele?

Meningocele: only meninges & spinal fluid in sac, little or no nerve damage vs myelomeningocele: spinal cord/nerves are also in the sac, nerve damage present

Do people with thoracic level malformation has volitional lower limb movements?

No

Are motor & sensory deficit often clear cut?

No - sensory often doesn't correlate with motor levels & there's often 'skip' areas

Will kids with spina bifida effecting lumbar levels likely be community ambulation?

No - standing requires ankle control from gastroc. & glutes which are at the sacral levels

Does a low sacral level of malformation have any contractures?

None

What is affected in spina bifida occulta?

Only the osseous structure of the vertebral arch is affected = 1 or more vertebrae fail to fuse properly/malformed (hidden form of neural tube defect)

The course of tethered cord disorder is ____

Progressive

List some symptoms of tethered cord

Rapidly progressing scoliosis, hypertonus at 1 or several sites in LE, change in gait pattern, change in urologic function, increased tone on PROM, asymmetric changes in MMT, areas of decreasing strength or back discomfort

Which of the following are fully innervated in thoracic level? (Select all that apply) A. Shoulder girdle B. Abs & extensors C. Upper limb D. Neck musculature E. Intercostals & QL

Shoulder girdle, upper limb, neck musculature fully (variable function of trunk musculature)

___ is a commonly used term referring to various forms of Myelodysplasia: defective development of any part of the SC

Spina bifida

The more severe type of spina bifida is called ___

Spina bifida aperta

Which of the following is true about the types of spina bifida? (Select all the apply) A. Spina bifida occulta includes meningocele & myelomeningocele B. The neural tube & spinal cord are not intact in spina bifida occulta C. Spina bifida aperta includes meningocele & myelomeningocele D. The neural tube & spinal cord are intact in spina bifida occulta

Spina bifida aperta includes meningocele & myelomeningocele & the neural tube & spinal cord are intact in spina bifida occulta

The milder type of spina bifida is called ___

Spina bifida occulta

Tethered cord requires what treatment?

Surgical repair

___ is caused by tissue attachments (usually gets caught on scar tissue) that limits the movement of the SC which can cause abnormal stretching of the SC

Tethered cord

Decreased bone density with risk of Fx is seen at which level of malformation? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

Thoracic & high lumbar

What level of malformation will need a minimum of 1 hr/day in a stander? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

Thoracic & high lumbar

Which of the following are true regarding contractures? (Select all that apply) A. Thoracic & high lumbar often have knee flexion & knee extension B. Low lumbar often has just knee flexion C. High lumbar & low lumbar often have knee flexion & knee extension D. High sacral often has knee flexion & hip flexion

Thoracic & high lumbar often have knee flexion & knee extension while low lumbar often has just knee flexion

What level of malformation tend to have more involvement of the CNS & associated cognitive deficits?

Thoracic level

What level often has hip flexion contractures? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

Thoracic, high lumbar & low lumbar

lumbar hyperlordosis is seen at which level of malformation? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

Thoracic, high lumbar & low lumbar

Coxa valga with high risk of hip subluxation/dislocation is seen at which level of malformation? (Select all that apply) A. Thoracic B. High lumbar C. Low lumbar D. High sacral E. Low sacral

Thoracic, high lumbar & low lumbar (coxa valga only here)

T/F: if medical conditions such as diabetes or obesity are not under control before getting pregnant, then this may increase your risk of NTD

True

T/F: tethered spinal cord can cause sensory/motor changes and/or changes in bowel/bladder function

True

T/F: the inherently obvious presentation of myelodysplasia is paraplegia resulting from SC malformation

True

Prenatal surgery to repair NTD are significantly correlated with reductions of ____

VP shunt placement and hindbrain herniation (12 months)

By the end of week ___ of gestation, the neural tube should be closed

Week 4

Can kids with spina bifida have osteoporosis?

Yes

In a high sacral level of malformation, is there strength present in hip, knee & ankle groups?

Yes

Can infants with spina bifida develop hydrocephaly?

Yes - 25% born with HC & 70-90% will develop it following closure of their back lesion

Will kids with spina bifida & shunted hydrocephaly need a VP shunt for lifetime?

Yes, they will need lifetime fluid management & likely the shunt will require fixing/replacing at some point

What is the most common form of spina bifida?

spina bifida occulta