Spina Bifida

Question 1

What is Spina Bifida?

Answer 1

Defective development of any part of the spinal cord

commonly lower cord

Question 2

What are the different names for spina bifida?

Answer 2

● Neural tube defect
● Meningomyelocele
● Myelomenigecele
● Myelodysplasia
● Spina Bifida

Question 3

What is the etiology of spina bifida?

Answer 3

● No definitive cause defined

● Genetics

• associated with chromosomal aberrations
• recurrent risk for siblings is 2-3%
• Irish/Celtic origin

● Teratogens (including prescription meds)

• excess maternal alcohol intake
• use of valproic acid (anticonvulsant)

● Nutritional Deficits
-Folic acid deficiency - supplementation has resulted in a decreased

● Environmental
-Increased internal body temperature

Question 4

What occurs during embryology 17-19 days?

Answer 4

neural plate formation
● brain and spinal cord are exposed
● occurs between 17-23 days

Question 5

What occurs during embryology 19-21 days post conception?

Answer 5

Neural fold formation

Forms CNS

Question 6

What occurs during embryology 22-23 days post conception?

Answer 6

Fusion of the neural folds

Question 7

What occurs during embryology 23-26 days post conception?

Answer 7

Rosteral (toward the head) closure occurs

Anencephaly

Question 8

What occurs during embryology 26-30 days post conception?

Answer 8

Caudal closure occurs

Myelomeningocele

Question 9

What is the difference between a normal fetus and one with spina bifida? When does the change occur?

Answer 9

Normally, neural tube closes at 28 days after
fertilization.

Spina Bifida: failure of neural tube to close properly

Question 10

How is spina bifida diagnosed?

Answer 10

• Ultrasound
• Protein screening (blood test-acetylcholine esterase)
• Amniotic fluid analysis/amniocentesis (high AFP)

Question 11

What is the incidence of spina bifida?

Answer 11

1/1000 births in the US

***unless you’re celtic or irish, then it could be 1/80

Question 12

What is spina bifida aperta?

Answer 12

One or many malformed vertebrae may allow

tissue to protrude from spinal canal and form a sac (may also be referred to as Spina Bifida Cystica)

Question 13

Where does spina bifida aperta most commonly occur?

Answer 13

Lumbar

Question 14

What are the types of spina bifida aperta?

Answer 14

Meningocele and Myelomeningocele

Question 15

What is spina bifida occulta?

Answer 15

non-fusion of the vertebral arches, no

disturbance of underlying neural tissue

Question 16

What are the characteristics of spina bifida occulta?

Answer 16

● Lipoma of the spinal cord, most are visible

● May be large/small, subcutaneous masses of fat

● Most commonly in lumbar or sacral spine

● Usually no neurologic or orthopedic problems

● High association with mild urinary tract disorders

Question 17

What is spina bifida aperta Meningocele?

Answer 17

● skin covered
● contains only meninges, does not contain functional nerve tissue
● Not as serious
● Not as common as
myelomeningocele
● Often seen with teratomas, tumors of the sacrococcyx and presacral space, and Currarino Syndrome

Question 18

What is spina bifida aperta Myelomeningocele?

Answer 18

● open spinal cord defect, with meninges and spinal cord protruding through vertebral defect
● not skin covered
(membrane covered)
● usually associated with spinal nerve paralysis (below the level of the
defect)

Question 19

What is hydrocephalus?

Answer 19

● Due to ventricular obstruction or overproduction of CSF

● 25% or more with
myelomeningocele are born
with hydrocephalus

● An additional 60% develop
hydrocephalus after surgery

● Hydrocephalus impacting on
motor cortex or pyramidal tracts

● Diminished upper limb
coordination is common

● 80-90% with hydrocephalus will require a VP shunt

● Arnold-Chiari type II malformation (extension of both cerebellar and brain stem tissue into the foramen magnum) may result in caudal displacement of the hindbrain through the foramen
magnum (obstructive)
–The cerebellum is now placing pressure on the brainstem and
spinal cord

Question 20

Arnold-Chiari type II malformation (extension of both cerebellar and brain stem tissue into the foramen magnum) may result in:

Answer 20

caudal displacement of the hindbrain through the foramen magnum (obstructive)
—The cerebellum is now placing pressure on the brainstem and
spinal cord

Question 21

What are the associated conditions with spina bifida?

Answer 21

Hydrocephalus

Meningitis

UTI and kidney infections

Ischemia and cord tethering

Sensory deficits

Decubiti

Cognitive dysfunction

Progressive neurological dysfunction

Seizures

Question 22

_______% of children with myelomeningocele
develop voluntary control of urinary or anal sphincter
(______ innervation)

Answer 22

Fewer than 5%

S2-S4 innervation

Question 23

What is sun setting of the eyes?

Answer 23

See more sclera of the eye

Question 24

Which musculoskeletal deformities are associated with spina bifida?

Answer 24

Congenital scoliosis

Club foot deformity

Question 25

What causes musculoskeletal deformities in children with spina bifida?

Answer 25

● neurologic dysfunction ● intrauterine positioning ● coexisting congenital malformations ● arthrogryposis ● habitually assumed postures after birth ● decreased AROM

Question 26

What are the typical postural problems in children with spina bifida?

Answer 26

forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, anterior pelvic tilt, rotational deformities of the hip or tibia, flexed hips, knees, pronated feet

Question 27

What is the association between scoliosis and spina bifida?

Answer 27

● more frequently observed in higher level lesions, increases in severity with age ● orthotic treatment of scoliosis common, to prevent further progression ● possible surgical fusion ● restrictive lung disease frequent infections

Question 28

True or false: Be aggressive in stretching children with spina bifida

Answer 28

True

Question 29

_______ level lesions (___-___) have unopposed flex + add which often leads to subluxation/dislocation

Answer 29

High level (L1-L2)

Question 30

True or false: There is no change in the knees of children with spina bifida

Answer 30

False. Contractures decrease in severity then increase

Question 31

Ankle and foot pathologies are most common with spina bifida to levels ________.

Answer 31

L5 and above

Question 32

What are the foot and ankle pathologies associated with spina bifida?

Answer 32

``` ● equinovarus deformity (talipes equinus) ● forefoot varus or valgus ● forefoot supination or pronation ● calcaneal varus or valgus ● pes cavus and planus ● claw toe deformity ```

Question 33

What is used to assess functional motor levels in spina bifida?

Answer 33

IMSG (International Myelodysplasia Study Group)

Question 34

With spina bifida, the motor level is defined as ...

Answer 34

the lowest intact functional neuromuscular segment.

Question 35

What are the types of lesions of spina bifida?

Answer 35

● complete transection- flaccid, absent reflexes ● incomplete lesion-spasticity, hyper-reflexia, possibly altered voluntary control ● skip lesions- isolate function below apparent level of the lesion.