Neural Tube Defects/Spina Bifida Flashcards
(39 cards)
1
Q
Neural Tube Defects
A
Grouping of malformations of the…
- Spinal Cord
- Brain
- Vertebrae
2
Q
3 Major NTDs
A
- Encephalocele
- Ancephaly
- Spina Bifida
3
Q
Enecephalocele (3 Major NTDs)
A
- A malformation of the skull which allows a portion of the brain to protrude in a sac.
- Most encephaloceles occur in the occipital region of the brain; though sometimes in the frontal region.
- Typically results in intellectual disabilities, hydrocephalus, neuromuscular spasticity and seizure disorder.
4
Q
Anencephaly (3 Major NTDs)
A
- A most severe congenital malformation of both skull and brain in which there is no neural development above the brain stem.
- Half of fetuses with anencephaly do not survive birth.
- Those who survive rarely survive infancy.
5
Q
Spina Bifida (3 Major NTDs)
A
Considered to be the most common neural tube defect
- Identified as a split of the vertebral arches that typically result in a protruding meningeal sac that may contain a portion of the spinal cord.
- Greater than 10% of the general population have a benign separation of the vertebral arches without any visual abnormalities on their back, a sac at birth, or other neurologic symptoms.
- These children are not likely referred to OT services based on this diagnosis alone.
6
Q
Meningocele (Spina Bifida)
A
- Refers to an exposed membranous sac covering the spinal cord.
- In this form of spina bifida, the spinal cord is not entrapped; thus, children have no symptoms.
- This is referred to as spinal bifida occulta and is the most common and benign form of NTD.
7
Q
Meningomyelocele (Spina Bifida)
A
- Associated with a malformed spinal cord within the sac.
- This disorder is MOST associated with spina bifida and accounts for most likely OT referrals of all NTDs.
- Evidence-based support a strong link between NTDs and maternal folic acid deficiency.
8
Q
Meningomyelocele Symptoms (Spina Bifida)
A
- Complete or partial paralysis
- Sensory loss below the lesion of the spinal abnormality
- Chiari type II malformation with hydrocephalus
- Neurogenic (absence of innervation) to bowel and bladder
- Hydrocephalus
9
Q
Chiari type II
A
Malformation in which brainstem and part of the cerebellum are displaced downward toward the neck, rather than within the skull
10
Q
What is the Incidence of Chiari type II among children with Spina Bifida?
A
Nearly all children with a meningomyelocele above the pelvic, or sacral level will have this abnormality.
11
Q
What are the Signs and Symptoms of Chiari type II?
A
The child may have difficulty swallowing, apnea episodes, difficulty breathing during sleep, arching of the head backwards to relieve the pressure resulting from brainstem and spinal cord compression.
12
Q
What is the Medical Intervention for Chiari type II?
A
Decompression surgery
13
Q
Hydrocephalus Incidence and Diagnosis
A
- Occurs in 75-95% of children with meningomyelocele or spina bifida, especially in lower thoracolumbar spinal lesions.
- Results from an abnormal cerebral spinal fluid flow, resulting in enlargement on the ventricles of the brain.
- Diagnosed by neuroimaging studies: ultrasound, CT, or MRI
14
Q
What is the Medical Management of Hydrocephalus?
A
- Surgically implanted shunt that diverts the CSF from ventricles to abdominal cavity where it can be absorbed.
- Ongoing evaluation for shunt blockage or infection
15
Q
Diagnosis of NTDs
A
- In utero, or before the child is born: Malformation of NTDs occur during the first stages of CNS formation; usually occurs by 26 days post egg fertilization and before a woman knows she is pregnant.
- NTDs are diagnosed prenatally by measuring levels of alpha-fetoprotein (AFP) via maternal blood test at approx. 17 weeks of pregnancy. AFP is a chemical found in fetal spinal fluid, and in the presence of an NTD, AFP can leak from the open spine into the amniotic fluid, then into the maternal blood stream.
- If suspected, a follow up MRI or 3D ultrasound may be done. Early diagnosis helps parents and delivery team plan for a C-Section delivery in a center with a NICU and a pediatric neurosurgeon.
16
Q
Top two priorities after birth: (Neonatal Medical Intervention: Meningomyelocele)
A
- To prevent spinal cord infection such as meningitis
- To protect exposed spinal nerves and associated structures fro further injury.
17
Q
Surgical Interventions at Birth (Neonatal Medical Intervention: Meningomyelocele)
A
- Surgical closure of the spinal defect within first few days of life
- Ventricular shunt placement – to prevent cerebral spinal fluid (CSF) from building up causing progressive hydrocephalus and potential brain injury. *
- Hydrocephalus occurs in greater than 60% of children with spina bifida and requires a surgically implanted shunt.
- Shunting diverts the CSF from enlarged ventricles to other parts of the body, often the abdominal cavity where it can be absorbed.
18
Q
Hydrocephalus and Ventricular Shunts
A
Shunts can become blocked or infected, especially in the first year of life. Growth of the child is another factor that may warrant shunt revision.
- By age five, many children have had multiple shunt replacement surgeries
- Early detection of shunt failure or infection is critical
- Occupational therapists working with children with a shunt placement must be aware of onset of new neurological symptoms as these may be an indicate of shunt dysfunction requiring immediate medical attention
19
Q
Associated Impairments of NTDs
A
- Functional Mobility
- Extensive lower extremity bracing, splinting, mobility devices to include crutches, parapodiums, and wheelchair use is often indicated.
- Musculoskeletal and Movement-Related Functions:
20
Q
Functional Mobility (Associated Impairments of NTDs)
A
The higher the level of the meningomyelocele and the greater the muscle weakness, the more ambulation will be impaired.
21
Q
Musculoskeletal and Movement-Related Functions (Associated Impairments of NTDs)
A
Occurs in the presence of partial and total paralysis for children with spina bifida
- Calcaneus deformity, aka clubfoot
- Hip deformities
- Arthritis of the hips and knees
- Scoliosis and kyphosis
22
Q
Scoliosis and Kyphosis
A
- Greater than 90% of children with lesions above sacral level have a spinal curvature.
- Each may be a congenital anomaly (present at birth) or acquired (developed in childhood).
- Proper management includes implications for seating and positioning, self-care, respiration, and general quality of life.
23
Q
Medical Management of Scoliosis and Kyphosis
A
Critical in a growing child for development of sitting, functional mobility, and even lung capacity
- Orthopedic support jacket: referred to as a thoracocolumbosacral orthosis, or TLSO
- Surgical Intervention: a more severe and conservative intervention. Inserting metal rods (internal fixation) to stabilize the spine
- Spinal fusion surgery
24
Q
Mental Functions
A
- Greater than 75% of children with spina bifida have an IQ within typical range
- However, they often have greater impairments in perceptual skills, memory, and organizational abilities that may result in diagnosis of learning disabilities as they become school aged.
- Children without hydrocephalus have higher IQs, better memory and executive function skills.
25
Executive Function (mental functions)
- One’s ability to plan, initiate, sequence and sustain a task.
- School-age children with spina bifida may demonstrate deficits in academic (particularly math), social participation, and self-care occupations.
26
Impact of Hydrocephalus (mental functions)
Secondary brain injury from prenatal hydrocephalus or brain infections account for more severe intellectual disabilities
27
Urinary Function (urinary system functions)
- Dysfunction present in all children with meningomyleceles and referred to as a neurogenic bladder.
- Higher incidence of kidney malformation.
28
Function of the Bladder (urinary system functions)
29
Medical Management of Urinary System Functions
- Catheterization
- Artificial Urinary Sphincter
- Botox
30
Catheterization (Urinary System Functions)
Insertion of a clean catheter through the urethra and into the bladder to empty the urine.
- Carried out greater than 4X a day by caregiver, or by the child once old enough.
- Constant monitoring for bladder infections typical of children with neurogenic bladder.
- Long term prophylactic antibiotics, such as Bactrim or Keflex given to prevent infections.
31
Artificial Urinary Sphincter (Urinary System Functions)
Known as “volitional voiding”
32
Botox (Urinary System Functions)
Inserted into the bladder muscle to relax muscle and increase bladder capacity
33
Bowel System Dysfuction
Related to a) decreased motor propulsion on the intestines and b) decreased sensation of the rectum. Bowel management training begins at age 2.
-Issues include: a) constipation, and b) incontinence
34
Medical Intervention: Bowel System Dysfuction
Recent advances in surgical procedures to include the ACE procedure in which a surgical hole I place on the left side of the abdomen allowing for a direct connection between the abdominal wale and the colon, allowing for greater irrigation of the colon on a regular basis.
35
Biofeedback Training (Bowel System Dysfuction)
Can be used with older children with low-level (sacral) meningomyelocele.
36
Decubitus ulcers/Skin Sores (medical management issues)
- Specific to feet and buttocks due to weightbearing on areas with decreased sensation.
- Increased healing time due to poor vascular and sensory system function.
* Adaptive seating systems a priority for children who are wheelchair users. Prevention is key!
37
Latex Allergies (medical management issues)
- Increased incidence of latex allergies in children with spina bifida as they progress through childhood.
- Etiology remains unclear; however, these individuals have increased exposure to latex in surgical gloves due to daily catheterization and bowel management and frequent surgical procedures.
38
Intervention for Latex Allergies
- Provide a latex-free environment; to include gloves and non-latex catheters.
- Children’s play materials such as balloons and rubber balls may also be a concern for children with latex allergies.
39
What is the Impact of Spina Bifida on Occupational Performance?
- ADLs: Bathing, toileting and toilet hygiene, dressing, functional mobility, personal device care, personal hygiene and grooming, and sexual activity
- IADLs: Driving and community mobility, health management and maintenance
- Education: Participation in academic and non-academic educational activities
- Play and Leisure Participation: Access to play equipment and activities, community leisure activities
- Social Participation: Community, family, and peer
