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NM III > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

What is spina bifida?

A

Spina Bifida is a type of neural tube defect that occurs when one or more vertebrae fail to fuse at approximately 28 days of gestation, leaving the spinal cord unprotected in utero.

PREVALENCE:
-3 in 10,000 or 1 in 1,400 in the US
-Higher prevalence in Hispanics and Caucasians compared to African Americans and Asians
-90-95% have no family history of SB

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2
Q

Common co-morbidities

A

seizure

latex allergy

sensation decreased–> skin breakdown

hip subluxation, clubfoot, scoliosis

UE weakness

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3
Q

Most common level of spinal defect in spina bifida

A

L5-S1

** spina bifida can present with UMN and LMN signs –> higher lesions may have more UMN signs

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4
Q

When does development of the neural tube usually occur?

A

3-4 weeks gestation

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5
Q

Open spinal dysraphism

A

Meninges and/or nerve tissue are exposed to the external environment.

Most prevalent.

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6
Q

Closed spinal dysraphism

A

Meninges and nerve tissue are covered by skin.

Mildest form of the condition.

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7
Q

Myelomeningocele

A

The most common form of Spina Bifida but others forms of open and close lesions exist

-most severe form of the condition

Myelomeningocele is a severe form of spina bifida in which the spinal cord and nerves develop outside of the body and are contained in a fluid-filled sac that is visible outside of the back area.

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8
Q

Occulta meaning

A

hidden

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9
Q

Spina bifida occulta:

A

mildest and most common
one or more vertebrae is malformed
often no symptoms
common to have hair tuft growth over affected vertebrae

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10
Q

Meningocele

A

meninges protrude outside of spinal canal and are filled with spinal fluid

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11
Q

Lipomyelomeningocele

A

closed neural tube disorder
often requires surgery
fatty tissue protrudes into the spinal canal through an opening in the vertebrae
causes leg weakness and B/B issues

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12
Q

Myelomeningocele

A

most severe form of SB
portion of SC exposed through an opening in the spine
SC may or may not be covered by the meninges

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13
Q

Anencephaly

A

baby is born without parts of the brain and skull

type of neural tube defect

occurs when upper part of neural tube does not close properly during gestation
baby is born without forebrain and cerebrum
parts of the brain that the baby is born with are commonly not covered by skull or skin

prevalence: 1 in 4,600 infants born

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14
Q

Encephalocele

A

Neural tube defect→ lack of complete closure during pregnancy
Sac-like protrusion of brain matter and meninges through an opening in the skull

The opening may occur anywhere on skull from the nose to the back of the neck
—> most often on the back of the head, top of the head, or between the forehead and nose

Prevalence: 1 in 10,500 live births in the US

Etiology: the cause is generally unknown

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15
Q

Diagnosis prenatally:

A

13-15 weeks:
-blood lab test - AFP - 80% reliable
-The alpha fetoprotein test (AFP) is a blood test performed to measure, diagnose, or monitor fetal distress or fetal abnormalities. It can also detect some liver disorders and some cancers in adults.

16-24 weeks:
-fetal ultrasound, >90% reliable

16-18 weeks:
-amniocentesis: takes amniotic fluid from around your baby in the uterus
-100% accurate but more risky

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16
Q

Complexity of spina bifida:

A

-from minor to severe physical and mental disabilities

SEVERITY BASED ON:
-size/location of malformation
-open vs closed
-which spinal nerves are involved

**The higher the malformation occurs on the back, the greater the amount of nerve damage and loss of muscle function and sensation.

17
Q

CAUSES AND RISK FACTORS OF SB

A

CAUSE: unknown but likely multiple factors
-genetic
-nutritional - maternal nutrition
-environmental -teratogens

-could be related to Folic acid–> reduces risk of neural tube defects by up to 70%

FOLIC ACID REC:
-0.4 mg of folic acid should be taken by women of childbearing age
-dark leafy greens, whole grains, legumes

18
Q

MSK IMPAIRMENTS

A

foot abnormalities –> ex: clubfoot
shorter legs
decreased bone density
scoliosis
hip subluxation and dislocation

19
Q

NEURO IMPAIRMENTS

A

loss of sensation
loss of muscle strength and control
loss of B/B control
muscle contractures

20
Q

OTHER IMPAIRMENTS

A

hydrocephalus
Arnold Chiari Malformation
seizures
cognitive impairments
tethered cord syndrome
skin care - pressure sores, latex allergy

21
Q

What is hydrocephalus?

A

Hydrocephalus
Cause: disturbance of formation, flow, or absorption of CSF
fluid accumulation in ventricles
common among children born with myelomeningooele

Prevalence: 15-25% at birth, increases to 80-90% that will require a ventriculoperitoneal shunt

S/S:
headaches
nausea
lethargy
vision problems
hoarse cry
swallowing issues

cocktail party personality - articulate and verbose but contents include jargon and cliches

Synringomyelia- a fluid filled central cavity (spinal cord) often associated with hydrocephalus
(5-40% incidence) –> change in motor and sensory function, increased reflexes, pain
–> may require localized shut in the spinal cord

22
Q

Arnold Chiari Malformation Type II

A

leading cause of mortality in children with spina bifida

portion of the brainstem descends into the cervical spinal canal due to increased CSF pressure

S/S:
changes in breathing pattern
swallowing problems
ocular muscle palsies
lateral rectus weakness (CN VI)
weakness/spasticity in arms
bradycardia- can be severe

23
Q

Club foot and SB

A

in 30-50% infants with spina bifida
more common with T spine and lumbar lesions
Associated with:
-muscle imbalance
-contractures
-intrauterine positioning
-spasticity

24
Q

Tethered cord syndrome and SB

A

20-50% of children with myelomeningocele

caused by spinal cord becoming tethered to part of vertebral column → abnormal stretch of the spinal cord with the child’s growth

SIGNS/SYMPTOMS
weakness
scoliosis
pain –with vertebrae palpation
orthopedic deformity
abnormal gait and balance
–gait analysis indicated
urologic dysfunction

TREATMENT
surgical- to detach SC from vertebrae

Re-tethering occurs 10-15%

25
Q

Activity and Participation outcome measures

A

Alberta Infant Motor Scale- Motor function
-0-18 months

PEDI- goal setting –> parent/caregiver report
demonstrate change in function over time
–>higher scores means greater functionality

WeeFIM
-validated for use with children with spina bifida
-18 items, 7 levels
-6 months to 7 years
–> higher scores=more indepedence
-assessing self care, mobility, and cognition

School Function Assessment- Participation in school

SB Helath Related QOL-
–impact of impairment on life satisfication

26
Q

BFS outcome measures

A

Goniometry

American Spinal Injury Association impairment scale (ASIA) - ISNCSCI
–sensory and motor function

Hand held dynamometry or MMT

Gait and movement analysis

27
Q

Common stages of motor development

A

-in first 6 months - close to typical

-rolling: thoracic SB usually roll by 18 months with compensatory strategies –> many will crawl

-sitting: children with mid-lumbar SB can sit with support with delay and increased lordosis, typical with L4-L5 lesion

-pull to stand/cruise: can be done among children with low lumbar lesions, may progress to ambulation with assistive devices around age 2

28
Q

positive predictors of ambulation

A

low motor level

no history of shunt

no history of hip or knee contracture surgery

29
Q

What other muscles other than hip abductors are commonly weak in those that walk with a Tredelenburg pattern?

A

-hip extensors
-plantar flexors

30
Q

Medical management of SB

A

FIRST
-fetal or post-natal surgery
—fetal is higher risk
-can lead to improved mobility and fewer surgeries needed for shunt placement

SECOND
surgery to place shunt → to help drain CSF from brain
–may need additional surgery based on growth, clogged shunt, or infection

31
Q

Orthopedic considerations

A

Scoliosis or kyphosis
-common with lesions above L2
-may need surgery with Cobb Angle >40 degrees
—->fusion, growing rods

Hip subluxation and dislocation
-fractures 11-30% most common in the distal femur and femoral neck related to osteopenia
-watch out for warmth of skin or low-grade fever
-often present subacutely

32
Q

Early Intervention

A

Monitor joint alignment, muscle imbalance, and development of contractures
-stretching, orthoses, splints
-serial casting
-surgery

Tummy time
-to strengthen neck and mm. for crawling,
-improve sensory processing and motor planning

Bracing for ambulation
-soon after pull to tall kneel or pull to stand
-protects the lower limbs from torque and shear forces

Power mobility
-can start at 24 months of age
ensure proper fit, posture, technique

PBWSTT
-high repetition
-functionally relevant
-strength and control of muscles is improved
—motor development
-increases bone density
-increased amount of sensory input → more steps

33
Q

Motor function across the lifespan

A

-average age of onset for those that walk: age 3
–> most likely with a mobility support device

-many children will lose capacity later in life (around 11)

Independent living: 30-60%
Employment rate: 25-50%

34
Q

Interventions 1-5 years old

A

monitor any changes in neuro level, strength, gait, sensation, B/B, MSK changes
consider using standing frame or mobility device to encourage upright standing and WB
discuss/emphasize mobility options
use appropriate bracing to facilitate weak muscles and protect limbs from shear or torque forces
ensure proper wheelchair fit, posture, and technique

35
Q

Interventions 6-12 years old

A

focus on strength of the upper extremities to improve wheelchair mobility, ambulation, and functional activities
educate patient on how to don and doff orthoses independently
promote physical activity and weight-bearing through conversations with family and patient about adapted PE or adaptive sports

36
Q

Concerns across life span

A

ADULTHOOD CONCERNS:
-increased body size
-joint degradation
-decreased bone density
-poor control and painful movement
-motivation

*consider low impact activity

NM III (19 decks)

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