Health alterations class 2- Development

Important terms and concepts

Development (3 aspects)

1. Growth

2. Differentiation

3. Maturation

What are neural tube defects

1. Abnormalities that derive from the embryonic neural tube

2. Failure of the neural tube to close

When do neural tube defects occur

Around 4-6 weeks of pregnancy

Why does NL have the highest rate of neural tube defects in Canada?

Lack of folate in food and medicine

Pathophysiology of neural tube defects

Genetic mutation in the folate pathways (failure of the neural tube to close)

Additional factors that contribute to neural tube defects

1. Maternal obesity

2. Maternal diabetes mellitus

3. Low B12

4. Maternal hyperthermia

5. Use of anti epileptic drugs in pregnancy

Types of neural tube defects that commonly end in miscarriage or death of the baby

1. Cranioschisis

2. Exencephaly

3. Anencephaly

4. Encephalocele

Cranioschisis

Skull/cranium is not closes completely

Exencephaly

Brain is outside of the skull

Anencephaly

Absence of large portion of the brain or skull

Encephalocele

A sack containing part of the brain and nerves is on the skull

Most common neural tube defects

1. Rachischitis (spina bifida)

2. Meningocele (myelomeninhocele)

Prevention of neural tube defects

Folic acid (0.4mg/day or 4mg/day if previous ntd)

What has helped decrease the incidence of neural tube defects

Folic acid fortification in foods

When should you start taking folic acid?

Preconception

How do they check for neural tube defects?

Ultrasound at 18-19 weeks (continue to monitor throughout pregnancy)

Spina bifida

1. Congenital neural tube defect that affects the head and spinal column

2. Malformation of the neural tube can occur anywhere along the spine

Most common developmental disorder of the CNS

Spina bifida

2 types of spina bifida

1. Occulta

2. Cystica

Spina bifida that is visible

Spina bifida cystica

Spina bifida that isn’t visible

Spina bifida occulta

Skin indicators of spina bifida occulta

1. Skin depression or dimple

2. Port wine stain

3. Tufts of dark hair

4. Soft subcutaneous lipomas

What issues might a baby with spina bifida have

1. Problems with potty training

2. Trouble walking (gait issues or weak feet)

Two types of spina bifida cystica

1. Meningocele

2. Myelomeningocele

Spina bifida cystica: Meningocele

1. Sac containing meninges and spinal fluid but no neural elements (nerves)

2. No neurological deficits

Spina Bifida cystica: Myelomeningocele

1. Sac contains meninges, spinal fluid and nerves

2. Varying degrees of neurological deficit (location and magnitude of defect determines)

3. Defect not necessarily uniform on both sides

Most common spot for spina bifida to occur

Lower Sacral/lumbar region

What is the priority in a baby with spina bifida cystica (myomeningocile)

The sac rupturing

What dressings go over a spina bifida sac

Moist dressings with a plastic wrap over it

Why do you not use a rectal thermometer with spina bifida

Risk of rectal prolapse or the sac bursting

Things to monitor/assess with spina bifida

1. ICP

2. Drop foot

3. Neurological reflexes

4. Bladder or bowel incontinence

What allergy can people with spina bifida have

Latex

What foods should be avoided with a latex allergy

1. Banana

2. Avocado

3. Kiwis

4. Chestnuts

Why do people with spina bifida develop a latex allergy

Increased exposure (medical procedures, catheterization etc)

Cerebral palsy

1. A group of permanent and non progressive disorders of the development of movement and posture

2. Often associated with perceptual, language and intellectual deficits

What is cerebral palsy characterized by

Abnormal muscle tone and coordination

What do children with cerebral palsy also commonly have?

Epilepsy (15-60%)

Ethology of cerebral palsy

1. Prenatal brain abnormalities

2. Most causes are unknown

3. Intrauterine exposure to chorioamnionitis

Brain injury only in preterm babies

Periventricular leukomalacia

Diagnostic evaluation of CP

1. Infants at risk get a careful and early assessment

2. Neurological examination and history

3. Neuroimaging

4. Metabolic and genetic testing

Early warning signs of CP

1. Poor head control and clenched fists after 3 months

2. Stiff or rigid limbs

3. Arching back and pushing away

4. Floppy tone

5. Unable to sit without support at 8 months

6. Failure to smile by 3 months

7. Lack of interest in surroundings

8. Primitive reflexes

9. Excessive irritability, crying and sleeping

10. Feeding difficulties (gagging or choking)

Types of cerebral palsy

1. Spastic

2. Dyskinetic

3. Ataxic

4. Mixed

Most common type of cerebral palsy

Spastic

Characteristics of spastic cerebral palsy

1. Hypertonicity

2. Persistent primitive reflexes Excessive irritability

3. Inadequate protective reflexes

4. Altered speech quality

5. Poor coordination

6. Leg scissoring

7. Persistent muscle contraction

Characteristics of dyskinetic/athetoid cerebral palsy

1. Abnormal, constant and involuntary slow worm like movements

2. Decreased fine motor skills

3. No contractures

characteristics of ataxic cerebral palsy

1. Poor equilibrium and muscle coordination

2. Unsteady, wide based gait

Characteristics of dystonic cerebral palsy

1. Slow, twisting movements of the trunk or extremities

2. Drooling

3. Abnormal posture

Characteristics of dystonic/mixed cerebral palsy

a combination of spastic and athetonic cerebral palsy

What injections may be helpful for cerebral palsy patients

Botulinum toxin A (botox)