Spina Bifida Flashcards by Colleen Caribardi

What is myelodysplasia?

An abnormality in development of the spinal cord

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what is anencephaly?

Lack of closure in region of head

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What is spina bifida?

Lack of closure below the head

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(true/false) Most infants with anencephaly live after birth

false (die within a few days of birth)

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definition: Congenital malformation of spinal cord

neural tube defect

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(true/false) spina bifida differs by racial/ethnic group

true –> more prevalent in hispanics

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The prevalence of spina bifida has (decreased/increased)

decreased

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What is spina bifida aperta?

spina bifida with visible or open lesions

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What is spina bifida occulta?

Spina bifida with hidden lesions

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What is a Myelomeningocele?

A form of spina bifida aperta that is characterized by a Sac containing the spina cord that protrudes dorsally and is not covered by skin.

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What form of spina bifida aperta is associated with spinal nerve paralysis?

Myelomeningocele

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What is meningocele?

A form of spina bifida aperta that is characterized by a sac with few to no nerves and is covered by skin.

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What form of spina bifida aperta does not have paralysis?

meningocele

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What is found in the sac when meningocele is present?

membranes and CSF –> minimal/no nerves

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(true/false) Spina bifida occulta has fusion of the vertebral arch.

FALSE

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(true/false) Spina bifida occulta has no tissue disturbance

true

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What is spina bifida occulta normally associated with?

mild urinary tract disorders

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Where is spina bifida occulta commonly found?

lumbosacral region

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What characteristic of spina bifida occulta is indicative of suspicion for it?

tuft of hair or dimple present

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What is a lipoma?

A visible form of spina bifida occulta that is a subcutaneous fat mass that varies in size.

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(true/false) Lipoma may/may not involve paralysis

true

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definition: Band separates spinal cord into hemicords

Diastematomyelia

–> Cord tethering may result in progressive weakness

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(true/false) Diastematomyelia may be associated with paralysis

true

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What is a Myelocystocele?

Cysts of the spinal cord that occurs in the lumbosacral region and is skin covered.

–> may or may not be associated with nerve impairment

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definition: Neural tube fails to close between 3rd and 4th weeks gestation

Abnormal neurulation

definition: Cells distal to S2 typically adhere together to form canals which attach to the spinal cord, this process occurs abnormally

Abnormal canalization

Who has a higher risk of having a baby with NTDs? Why?

a. Significantly higher risk in women who did not graduate from high school and lived in low-SES neighborhoods b. Women in higher social groups are more likely to use folic acid

NTDs are common in mothers < ___ y/o and > ___y/o

< 19 > 40

(true/false) There is an increased risk of NTDs with increased total caffeine consumption during the year before pregnancy

true

Antibacterial medication (preconceptional and first trimester) and use of SSRI’s increased risk of ______.

anencephaly

What is the cause of abnormal canalization?

UNKNOWN

What is the cause of abnormal neurulation?

- genetics - teratogens - nutritional deficiencies

When should mothers begin folic acid?

at least 3 months before conception

Folic acid use before conception decreases risk of an open defect by ___%.

70%

When is a Maternal serum alpha-fetoprotein screening positive?

Positive only with OPEN neural lesion

What is ultrasound more accurate in detecting?

More accurate in detecting cranial malformations associated with spina bifida, than in detecting abnormalities in the spine (lemon and banana sign)

(true/false) Those with NTDs have an associated chromosome error (Trisomy 13 or 18)

true (10%)

definition: the outline of the cerebellum looks like a banana, probably because of the herniation at the spinal defect - the outline of the skull resembles a lemon

banana sign of spina bifida

Why is a C-section commonly used with NTDs?

Cesarean section will spare exposed nerves and prevent infection by passing through the vaginal canal Back closure can be done more rapidly

What is chiari malformation the deformity of?

cerebellum, medulla, cervical spine

What is chiari malformation?

Posterior cerebellum herniates downward through foramen magnum --> May be due to small posterior fossae

What is the cause of hydrocephalus in most children?

chiari malformation

(true/false) paralysis may occur later in life

true

What can cause abnormal tissue growth?

pressure on the nerves

What causes the lack of tissue growth around the spinal cord?

ischemia and tethering of the cord

What is the test described below? Abduction of flexed hips (may feel clunk of relocation)

ortolani's test

What test is described below? Pressure directed posterior on a flexed, adducted hip

barlow's test --> Feel femoral head dislocated over posterior rim of acetabulum

What is osteoporosis caused by? What is the result of this?

a. hypotonia/paralysis and decreased WB b. osteoporotic Fx

(true/false) Often DO NOT correlate with motor levels

true

What is hydrocephalus?

Excessive accumulation of CSF in the ventricles of the brain

___% of children with spina bifida are born with hydrocephalus

25%

An additional ___% of spina bifida cases develop hydrocephalus after surgical closure of back lesion.

60%

(true/false) 80-90% of hydrocephalus cases will require a shunt

true

What can prolonged shunt dysfunction lead to?

Functional and Cognitive decline

definition: appear articulate and verbose although use a lot of jargon and cliché’s

cocktail party personality

What are the 3 possible causes of UE dyscoordination? --> Use UE’s for support rather than for exploration of objects

1. Cerebellar ataxia related to Arnold-Chiari Type II malformation 2. Motor cortex or pyramidal tract damage secondary to hydrocephalus 3. Motor learning deficits due to delayed development of hand

Cranial nerve palsies are a result of what?

1. Arnold-Chiari malformation 2. Hydrocephalus or dysplasia of the brainstem

The majority of spina bifida cases have (UMN/LMN) presentation

LMN --> 2/3 of cases had SOME UMN signs

what are causes of progressive neuro dysfunction in those with spina bifida?

- Shunt obstruction - Hydromyelia - Growth of a lipoma at the site of repair - Subarachnoid cysts of the cord - Spinal cord tethering

(true/false) 1/3 of kids with myelomeningocele will require surgery for tethered cord

True

definition: refers to an abnormal widening of the central canal of the spinal cord that creates a cavity in which cerebrospinal fluid (commonly known as spinal fluid) can accumulate and increase pressure on the spinal cord resulting in damage to the nerve cells and their connections

hydromyelia

Hydromyelia occurs in __-___% of children and adolescents with spina bifida

10-30%

___ medications can accentuate cognitive deficits or dyscoordination

anticonvulsant

(true/false) more than 5% of spina bifida cases develop voluntary control of b/b.

FALSE (less than 5%)

definition: . procedure that allows the patient to do a total washout of their colon by flushing out stool starting in the colon downward. This produces a bowel movement under the patient's control!

ACE procedure (antegrade colonic enema)

_____ ulcers occur in 85-95% of children by time reach young adulthood

decubitus

(true/false) obesity is common in children with spina bifida

true

Self-catheterization can begin at age ___.

age 5

How can you promote self catheterization?

1. Introduce bead stringing earlier to practice 2. Sitting balance 3. Practice fine motor skills

What do growth spurts increase the risk of in those with spina bifida?

risk of cord tethering

When should a patient with spina bifida be independent with ADLs while using an ADL (as needed)?

school age

(true/false) You may see progression in neurologic deficit when a spina bifida patient transitions into adulthood

true

What is the most important factor of spina bifida prognosis?

motor function

(true/false) FES may increase gait velocity and distance

true

Evidence supports that ____ min/day of stepping, bouncing and treadmill training starting at 6 months can help with gait

10 minutes