Congenital Spinal Cord Injury

Question 1

2 main categories of spinal cord injury:

Answer 1

1. Congenital SCI
2. Traumatic SCI

Question 2

Where does congenital SCI occurs?

Answer 2

in utero

Question 3

Defect in neural tube closure & overlying posterior vertebral arches

Answer 3

Spina Bifida

Question 4

Two types of spina bifida:

Answer 4

1. Spina bifida occulta
2. Spina bifida cystica

Question 5

In what type of congenital SCI there is a failure of one or more of the vertebral arches to fuse in the 3rd month of gestation, there no SC and meninges involved, and is covered by skin with a patch of hair or dimple ?

Answer 5

Spina Bifida Oculta

Question 6

Are there usually any resultant neurological or musculoskeletal impairments seen in pt with spina bifida oculta?

Answer 6

no

Question 7

Cystic protrusions of meninges alone or both meninges & spinal cord through defective vertebral arches

Answer 7

Spina Bifida Cystica

Question 8

What are the types of spina bifida cystica?

Which is the most common type?

Answer 8

1. Meningocele
2. Myelocele (myelocystocele)
3. Lipomeningocele
4. Myelomeningocele (most common)

Question 9

Answer 9

Meningocele

Question 10

Answer 10

myelomeningocele

Question 12

Answer 12

spina bifida oculta

Question 13

Which is the milder form of spina bifida?

Answer 13

meningocele

Question 14

Neural tube closes normally, but central canal of spinal cord distends & produces large, skin covered cyst:

Answer 14

myelocele (myelocystocele)

Question 15

Protrusion of meninges & CSF into cystic sac, SC remains within vertebral canal, and is relatively uncommon form of spina bifida

Answer 15

meningocele

Question 16

• Rare form of spina bifida
• Prompt medical intervention is required
• Neural tube closes normally, but is distended due to cystic swelling

Answer 16

Myelocele (myelocystocele)

Question 17

Due to vertebral defect associated with superficial fatty mass (lipoma) that merges with lower level of spinal cord

Answer 17

lipomeningocele

Question 18

Most common & severe form of spina bifida cystica where both spinal cord & meninges protrude into cystic sac, and results in abnormal transmission of neural impulses

Answer 18

myelomeningocele (spina bifida)

Question 19

Incidence of spina bifida has decreased worldwide since implementation of ____ ______ fortification programs

Answer 19

folic acid

Question 20

Detection of spina bifida via _______
has decreased the incidence of spina bifida

Answer 20

amniocentesis:

• which a small amount of amniotic fluid, which contains fetal tissues, is sampled from the amniotic sac
• Significant levels of alpha-fetoprotein in
  amniotic fluid aids detection

Question 21

etiology of spina bifida

Answer 21

• cause is unknown
• Enviromental and genetic factors
• Folic acid and vitamin A deficiencies

Question 22

80% of neural tube defects can be detected
via:

Answer 22

maternal serum alpha-fetoprotein levels

Question 23

Clinical manifestation of spina bifida (mayelomeningocele):

Answer 23

• Sensory: all types of sensory loss
• MSK: weakness/paralysis, deformities
• Neurological: hydrocephalus
• Integumentary

Question 24

Early detection of spina bifida (mayelomeningocele) can afford possibility for:

Answer 24

• newer in utero treatments of hydrocephalus
• in utero surgical repair of myelomeningocele

Question 25

In spina bifida, how is the weakness/paralysis presented if SC is involved **below** level of lesion?

Answer 25

* flaccid paralysis * absent reflexes

Question 26

In spina bifida, how is the weakness/paralysis presented if SC **remains intact** below level of lesion?

Answer 26

* Flaccid paralysis immediately below lesion * Hyperactive reflexes more distally

Question 27

In spina bifida, **sensory loss** below level of lesion includes:

Answer 27

**all types** * Kinesthetic * Proprioceptive * Somatosensory

Question 28

Osteoporosis fx may occur in pt with spina bifida due to:

Answer 28

no stress on the bones

Question 29

Spina bifida orthopedic deformities

Answer 29

* lumbar khyphosis * hip flexion, knee extension & ankle DF contractures * equinovarus (clubfoot) * hip flex/add/IR = hip dislocation

Question 30

What is **hydrocephalus**?

Answer 30

blockage of normal flow of CSF between ventricles & spinal cord

Question 31

Hydrocephalus occurs occurs in approx. in how many cases of spina bifida?

Answer 31

**80 – 90% of cases**

Question 32

If hydrocephalus not treated:

Answer 32

brain damage and death

Question 33

What is **Chiari II Malformation?**

Answer 33

* Congenital anomaly of hindbrain involving herniation of medulla into upper Cx canal * Children with myelomeningocele have a 99% chance of having this malformation * Occurs usually between C1 to C4

Question 34

What is **Hydromyelia**:

Answer 34

* Dilation of center canal of spinal cord due to accumulation of CSF

Question 35

What is Tethered Cord?

Answer 35

* Closely associated with spina bifida oculta * Pathological fixation of the spinal cord in an abnormal caudal location

Question 36

Presenting symptoms in **tethered cord:**

Answer 36

* Decreased strength * LE spasticity * Back pain at site of sac closure * Scoliosis development

Question 37

Common symptoms of **hydromyelia:**

Answer 37

* Rapidly progressive scoliosis (from mm imbalance) * UE weakness * Spasticity * Ascending motor loss in LE’s

Question 38

Symptoms of Chiari II Malformation:

Answer 38

* May be symptomatic or asymptomatic * Symptomatic: see respiratory & bulbar function impairments, sometimes UE weakness

Question 39

how is tethered cord treated?

Answer 39

cord release

Question 40

**Neurological impairments** in myelomeningocele (spina bifida)

Answer 40

* Bowel & bladder dysfunction * Cognitive impairment & Learning issues: * impaired intellectual function * Moderate – severe visual-motor perceptual deficits

Question 41

Integumentary impairments in myelomeningocele:

Answer 41

* Latex allergy * Risks of pressure sores

Question 42

Medical mgmt of myelomeningocele (spina bifida):

Answer 42

* surgical sac closure 24-48 h after birth * in utero surgery * hydrocephalus mgmt: * ventricular shunt placed

Question 43

what is the main casue of death for myelomeningocele

Answer 43

* Increased intracranial pressure * infections of CNS

Question 44

Survival rate for myelomeningocele is

Answer 44

85%

Question 45

Multiple episodes of P.T needed throughout the lifespan in pt living with spina bifida. What are the **pediatric** and **adult** goals?

Answer 45

* **Pediatric Goals:** * Achievement of developmental motor milestones * Enhance independent mobility and transfers * Maximize stability to facilitate play, social development, learning, language * **Adolescent/Adult Goals:** * Address changing needs as growth continues/aging occurs * Post-surgical rehab needs