Introduction


There are a number of conditions and diagnoses that are neurological in nature that may impact normal development in children. Two common neurological diagnoses include cerebral palsy and myelodysplasia, or spina bifida.

Cerebral Palsy (CP)


CP refers to a heterogeneous group of neurological disorders of the development of movement and posture that are permanent and attributable to nonprogressive disturbances that occurred in the developing fetal or infant brain (16). CP is often seen in children immediately after birth if there is a severe impairment or it can also be seen within the first years of life if the impairment is mild. CP can cause the child to have developmental delays later in life. In addition to impaired motor and postural control, CP may involve varying amounts of other impairments in cognition, motor, sensation and communication. There are three distinct types of CP which will be discussed further in detail: ataxic, dyskinetic and spastic.

Causes


A variety of factors have been linked to the development of CP and the cause of this condition is still being researched (2). Congenital CP is typically caused by a brain injury that occurs in the womb while the child is developing, during the birthing process or shortly after. Though the injury may occur immediately at birth it may not be detected until years later. The brain injury is sometimes unknown but is typically related to one of the following events (17):
  • Hypoxic or ischemic event
  • trauma to the brain during birthing process (typically in an infant with already compromised health)
  • Infections during pregnancy
  • Genetics
  • Multiple births
  • Premature birth
  • Severe, untreated jaundice in the infant
  • Rh incompatibility
  • CVA within the first month of life can also result in the development of CP (2).

Diagnosis (2)

There is not a single test that can be done to either confirm or rule out CP. In severe cases diagnosis may be done shortly after birth but if a child has mild CP then diagnosis may not happen until 3-5 years of age when the brain is more fully developed (18). Often parents are the first to notice that their child is missing important milestones. Once contacting the doctor the following are all taken into consideration to give the diagnosis of CP (18):
  • Delayed milestones
  • Growth chart standards for height and weight
  • Abnormal movement patterns (increased or decreased muscle tone)
  • Abnormal primitive reflexes and postural responses
  • Genetic specialist may be consulted about hereditary components
  • Assessment of vision, speech, cognition
  • MRI, cranial ultrasound of CT scan to determine brain damage
  • Infant neuromotor testing (PT may play a role here)
    • Alberta Infant Motor Scales (AIMS)
    • Test of Infant Motor Performance (TIMP)
    • NeuroSensory Motor Development Assessment (NSMDA)

Classification


Types based on movement patterns (1, 2)
  • 1. Spastic- Children experience ‍spasticity‍ (increased muscle tone) and hyper-reflexes which can negatively impact posture and movement. The motor cortex and sensorimotor cortex are damaged.
  • 2. Dyskinetic- damage to the basal ganglia which is involved in movement control can result in two different movement patterns (dystonic and athetoid subtypes of CP)
    • Dystonic- Movements are involuntary sustained contractions.
    • ‍Athetotic‍- Movements are a continual, slow writhing usually of peripheral limbs which leads to decreased postural stability

  • 3. Ataxic- Caused by damage to the cerebellum. This kids have difficulty with coordinated and controlled voluntary movements.

Based on Limb Involvement:

external image CP_type.gif
(http://www.ofcp.ca/images/CP_type.gif)

Based on Function:

Gross Motor Function Classification System (GMFCS) (2,3)‍
  • Primary system used to classify disability in gross motor function
  • Five levels based on age (2-4 years, 4-6 years, 6-12 years and 12-18 years)
  • Children should not be classified before the age of 2
  • Prognostic
  • Stable over time
  • Gives physicians and other medical professionals a way to plan interventions
  • For more information:

gfmcs.jpg gfm.jpg
Images above taken from Campbell text

GMFM-66

GMFM-66 is different than the GMFCS. The GMFCS is a way of classifying children with CP while the GMFM-66 is a criterion-referenced assessment which helps to determine function within a GMFCS level by using a series of 66 observations in dimensions of lying and rolling, sitting, crawling and kneeling, standing, walking, running and jumping.

Online scoring sheet:
http://bluewirecs.tzo.com/canchild/patches/GMFMScoresheet.pdf

Reference percentiles:
http://motorgrowth.canchild.ca/en/MotorGrowthCurves/percentiles.asp

As explained in an article by ‍‍Narayanan (16) ‍‍all children with CP will improve in motor function up to the age of about 7. After age 7 a decline in improvement will be seen in children in stages 3-5 of the GMFCS while children in stage 1-2 will remain relatively stable.

g.jpg

Common Impairments in body structure/function (2,3)
  • Decreased strength
  • Increased tone (spasticity)
  • Decreased muscle flexibility
    • Muscle growth may not keep up to bone growth during development (2).
  • Incoordination
  • Loss of selective motor control
  • Torsion of long bones
  • Early degeneration of weight bearing joints
  • Impaired balance
  • Secondary impairments
    • Contractures
      • Most at risk are shoulder adductors; the elbow, wrist, and finger flexors; the hip flexors and adductors; the knee flexors; and the ankle plantar flexors (2).
    • Joint instabilities
    • Bony deformities
    • Hip subluxation and dislocation
      • Often due to hip flexion and adduction contractures

INFANT


Evaluation/Examination:
Baseline developed for:
  • Growth: often affected in children with CP, measurements should be taken a regular intervals to be compared to age matched norms
    • Take anthropometric measurements of the head, length, and weight
  • Improvements: Once you have the baseline at infancy the child can be tracked throughout their lifetime and course of treatment to determine if there status is worsening, improving, or remaining constant.
  • Deterioration: Deterioration is easier to detect if the baseline evaluation is comprehensive and thorough
  • Maturation: The child will still mature but the rate at which the child matures may not be typical. Note maturational changes that affect function. Maturational changes don’t always have a negative effect but they still need to be documented
  • Effects of treatment: The effects of your treatment need to be tracked to determine if the child and family are receiving the best intervention for the particular impairment.

Look at the proficiency and efficiency of motor control
  • Is the child able to:
    • Align body parts
    • Bear weight
    • Shift weight
    • Resist gravity
    • Maintain stability
    • Isolate movement
    • Integrate movement
  • Does the child use an appropriate amount of energy for these tasks
  • If intervention is needed take into account the effects of infant position as it relates to postural reactions and reflexes, when prescribing seating and positioning assistive devices

Note the baby’s temperament, behavioral state regulation, and tolerance of handling

Goals & Interventions (2):**
  • Parent Education (2)
    • Consider pictures in HEP
    • Video with instruction can be helpful in coaching parent
    • Support family in coping with CP
    • Handling Techniques
      • Make sure that no 1 position dominates
      • Encourage active movement of the infant
      • Positioning that will allow voluntary movement
  • Use of equipment to facilitate participation
    • Sitting supports at 6 months to promote upper extremity use
    • Standing supports at 12 months
    • Postural stability
  • Sensorimotor awareness
    • Sucking on fingers
    • Mouth therapy, moving of tongue for infants that cannot move toy/object to mouth
    • Facilitate play and environmental exploration
  • Intervention ideas from an article by Cameron, et al. (4)
    • Note: infants in this study whose parents had better compliance performed better overall on scores of motor development using the AIMS test.

Neonatal Intervention.png

PRESCHOOL AGED


Examination/Evaluation: (Preschool/School)
Primary focus is on assessing activity
  • Determine level of impairment and how it affects participation

Regular and routinely scheduled tests should be administered to continuing the monitoring process
Assess:

  • Transfers
  • Communication
  • Self-care
  • Sleep patterns
  • Amount of dependence on caregivers
  • Adaptive equipment
    • Appropriateness/Effectiveness
  • ADL modifications
**Note personal and environmental factors present in the child such as attention, cooperation, location, and the child’s reaction.
  • Does the child perform daily tasks to his/her full ability?

Goals and Interventions:
Focus on the prevention of secondary impairments and optimizing gross motor skills
  • Easier to predict the child’s probable level of motor skill acquisition
Target activities that promote concentric/eccentric muscle force production
  • Transitional movements
  • Ball gymnastics
  • Treadmill use
  • Tricycle riding
  • Stairs
Spasticity management
  • Due to short term affects of PT intervention should be an adjunct to treatment
    • Education about pharmaceutical/surgical options and referral if necessary
Orthopedic
  • The hip development at this age is an important aspect requiring assessment and monitoring
Activity Promotion/Mobility
  • Determine if the child will gain the skills to complete the activity or if modification is necessary
  • Encourage exploration for the development of motor problem solving
    • This is an age where children gather feedback based on trial and error allow
  • Playing at this age is when the child is the most productive
    • Encourage typical play whenever possible
    • Recommend adaptive toys/solutions when needed
As always develop your treatment from a family based perspective and continue to educate the family (2)

SCHOOL AGED


Goals & Interventions
  • Maintain gross motor skills
    • reach a plateau further gross motor skill development- focus on maintaining these skills (2).
  • Managing primary impairments and prevent secondary impairments
    • Management of spasticity and contractures
    • Postural deformities can get worse during growth spurts
    • May undergo orthopedic surgeries for a variety of reasons to address
      • contractures
      • bony deformities
      • posture
      • biomechanics for gait
      • pain
    • These procedures may include tendon lengthening or transfers, osteotomies, or fusions.
    • Treatment to prevent secondary impairments may include aerobic training, strengthening, and flexibility (2).
  • Maximize participation in age-appropriate activities- figuring out how to use the gross motor skills the child has to maximize participation in age-appropriate activities (2).
  • Encourage child to take an active role in PT
    • The child can take a more active role in the decision-making process.
    • They can begin to problem-solve issues that arise that may impact their independence or involvement at school or in the community is essential (2).
  • Continue family education- Continuing family education is still important. Although these
    • Families have lived with the diagnosis of CP for several years - still need information that is up-to-date and age-appropriate.
    • Parents of children with CP may have physical and psychological health issues as well.
      • Encouraged to remember to take care of their own health.
      • Continued education about lifting and transfer training should be included as the parents and child age (2).

Palisano, R., Begnoche, D., Chiarello, L., Bartlett, D., McCoy, S., Chang, H. Amount of Focus of Physical Therapy and Occupational Therapy and Occupational Therapy for Young Children with Cerebral Palsy

Other Interventions


While traditional physical therapy is still appropriate for these kids, here are some ideas about other interventions that could be incorporated at this age as well.

Exercise Program (5)

Due to decreased mobility most children with CP have significantly lower aerobic and anaerobic capacities which can negatively impact daily functional activities. This population also may have decreased physical activity compared to other children due to their disabilities which may result in a sedentary lifestyle and further health issues as adults. This study found significant improvements in fitness levels and quality of life/participation in children who participated in this exercise program in addition to usual care. Below is a link to the article as well as a link to the specific exercise program (over 200 pages long) with detailed lessons and activities appropriate for exercise training for kids with CP.
CP exercise program (at bottom of page)
Article on CP exercise program

Wii Training (6)

This article was an early case study about using the Wii as a treatment intervention with a 13 year old with CP. This treatment kept coming to PT new and interesting which is important in this population and this age range since they have already had a lot of PT. It was also very age-appropriate since normal teenagers play video games. It also allowed an opportunity for interaction with peers since a fellow student came to a couple of sessions and the two students play against one another. All of the games in this study could be done sitting or standing so it could be used for different levels of severity. Additionally the appendix at the end has some useful information for using the Wii in any type of treatment discussing the games and the different PT goals and rationales such as improved trunk control, endurance, etc.


Hippotherapy (8)

Another treatment option that is used with school aged children with CP is hippotherapy. Sessions include horseback riding with the goal of improving trunk and head stability. With each step the child is challenged to maintain a stable trunk position. With improvements in trunk stability additional gains in mobility are often seen because proximal stability leads to distal mobility. The following video also mentioned that throughout a 45 minute session the child may have 3000+ chances to practice adjusting to the movements of the horse, it works different muscles than other treatments, and helps with endurance.



Aquatic Therapy (9)
Aquatic therapy is also a common treatment for children with CP. Like using the Wii in therapy, it is age appropriate since most kids enjoy swimming and can be done in groups with other children. Due to the principles of water, balance and mobility are easier in the water than on land because gravity is reduced. It creates a supportive environment with less concerns about falls and reduces the force on their joints. Water increases the resistance against movement which can help with strengthening. With kids who are hypotonic movements are easier because of the buoyancy of water and water can help relax kids who have hypertonia.


Dance Therapy: uses skilled and complex movements to work on range of motion and strengthening. And, it's fun!
http://www.ncpad.org/895/5018/Cerebral~Palsy~and~Ballet

Yoga: can help with increasing core strength as well as strength, flexibility and body awareness

Sensory Integration:

Medical Interventions

Since CP presents in so many ways there are numerous medical interventions. These include surgical and pharmaceutical interventions.
  • Selective dorsal rhizotomy- Surgical procedure to treat spasticity by cutting the dorsal sensory rootlets to the spinal cord. This removes the sensory stimulation that triggers spasticity but maintains motor function to the lower extremity. Electromyographic testing is used to select which rootlets are linked to spasticity while preserving the others. Physical therapy is needed afterwards to address new motor patterns and correct postures. Studies have shown the best results with patients who don't have contractures and exhibit good selective motor control as well as have good motivation, cognition, and support (1,2).
  • Orthopedic surgery- In cases involving muscle contractures or posture and joint deformities, orthopedic surgery is beneficial. Surgeries may include tendon transfers or lengthening, osteotomies, or fusions. The goal of these procedures is to improve biomechanical alignment (2).
  • Botox- Another treatment for spasticity is botox injections into the involved muscles. Botox works by blocking the release of acetylcholine into the neuromuscular junction so an action potential and contraction is not produced in the muscle, temporarily paralyzing it. The effects of an injection may last up to 4 months. Muscles that are commonly treated include the hip flexors and adductors, hamstrings, and gastroc. Muscles that easily develop contractures or exhibit increased spasticity are selected (2).
  • ‍‍Baclofen‍‍- This medication is also used to treat spasticity. It can be given by mouth but large doses are usually needed to produce a response so in children with CP it is usually administered intrathecally into the subarachnoid space using an implanted baclofen pump (2).

Myelodysplasia

Dorland's Medical Dictionary defines as "defective development of any part (especially the lower segments) of the spinal cord". Spina bifida is the commonly used term for different types of myelodysplasias. Spina bifida is divided into two types: aperta/cystica which are visible/open lesions and occulta which are hidden/non visible lesions. These lesions occur when the neural tube does not completely close during formation. The tube starts closing at 4 weeks gestation and is completely closed by 6 weeks as shown in the figure below. (2, 21)
external image spina-bifida-lg-enlg+-+embryo.jpg
These children and adolescents challenge pediatric physical therapists with the vast amount of body systems involved and the varying degrees of disability seen. It is important to have the knowledge of the manifestations of this‍‍ condition‍‍ as well as be able to anticipate future needs and potential problems. (2)

Types


external image ch10t10-29.jpg
external image spina-bifida.jpg

‍‍http://www.ncbi.nlm.nih.gov/books/NBK27180/‍‍ (21)

Causes:

Although there is no true "cause" of SB these factors have been associated with increased birth incidence of SB
Genetics
SB can be associated with genetic abnormalities including chromosomal aberrations and other genetic syndromes. The recurrence rate has been documented from 2.5-5% after the birth of one child with SB and doubles after the birth of two children with SB.

Environmental
  • Teratogens such as alcohol and anti-convulsant drugs (valproic acid, carbamazepine)
  • Low socioeconomic class
  • Maternal Obesity

Nutritional Deficiences
FOLIC ACID! FOLIC ACID! FOLIC ACID!
external image folic.jpg
It is very important for women of child bearing age to be taking in enough folic acid. Research recommends taking a multivitamin and/or a folic acid supplement. Women with a first-degree relative with SB or have had a previous SB pregnancy should take 4 mg per day and those women without a positive history should take 0.4 mg per day. ‍‍The issue lies in that the neural tube will start closing at 4 weeks and will be completely closed around 6 weeks.‍‍ Many women do not even know that they are pregnant at this point which means that damage will be done before they have a chance to take any precautions. Most multivitamins have the recommended daily amount of folic acid but you can also get folic acid from the foods in the chart.
(2, 21)






Impairments

Musculoskeletal Deformities
‍‍‍‍
Static and dynamic balance should be observed in: sitting, quadriped, kneeling, half kneeling, and standing as well as transitions to and from these positions. Check symmetry and weight bearing.
Sleeping and sitting patterns should be documented to see if any are contributing to the postural/joint deformities. These include, but are not limited to:
frog leg position in prone or supine, W sitting, ring sitting, heel sitting, cross legged sitting, and crouch sitting).

Thoracic to L2 Lesion
Mid to Low Lumbar Lesion
Sacral Lesion
Spine
L spine lordotic
Increased lumbar lordosis
Increased lumbar lordosis
Hips
Flexion, abduction, ER
Flexion contracture
Mild flexion contracture
Knees
Flexion contractures
Flexion contracture, genu valgus
Mild flexion contracture
Ankles
Plantar flexion contractures

Varus or valgus
Feet

calcaneal valgus, pronated foot with WBing, WB on calcaneus
Pronated or supinated foot, WB on calcaneus
Crouched standing/walking is a normal postural deviation that is seen across all lesion levels. As seen in the picture below it is characterized by persistant hip and knee flexion and an increased lumbar lordosis. This posture occurs because of muscle weakness. Contractures occur over time because the muscles shorten from standing in this crouched position.
external image crouch_gait_470.jpg

Spine**
  • Scoliosis can be congenital usually related to underlying vertebral anomalies and this curve is usually inflexible. Acquired scoliosis is generally caused by a muscle imbalance and the curve is flexible until maturity has been reached. Seen more frequently in higher lesion levels. Severe scoliosis (and kyphosis) can limit chest wall expansion and interfere with ventilation. Can also cause frequent respiratory infections.
  • Paralytic kyphosis occurs in approximately 1/3 of children by adolescents, progressing at a rate of 7-8% per year.
  • Hyperlordosis
  • Spine deformities can also lead to poor sitting posture, muscle imbalance, and recurrent skin ulcerations.
  • Goal is to create/maintain a neutral/balanced trunk and pelvis. Generally an orthotic (TLSO) is used until the child has reached an age when they can surgically fuse the spine--age 10-11 in girls; age 12-13 in boys. (2)

Osteoporosis:
Hypotonic and/or flaccid musculature can lead to decreased bone density. The bone density in ambulators is reported to be 38-44% higher than nonfunctional ambulators (2).

Motor Paralysis:
The motor level is defined as the lowest intact, functional neuromuscular segment. Spinal lesions can be asymmetrical therefore motor function should be classified for both the right and left sides. Involvement can manifest in three different ways 1.) lesions resembling complete cord transection 2.) incomplete lesions 3.) skip lesions. Skip lesions occur when more inferior segments are functioning despite the presence of one or more nonfunctional segments between the intact more superior spinal segments. Important to evaluate levels below the level of functioning to determine if there is a skip lesion. McDonald, et al concluded from their study that it is more useful to group individuals for SB by the strength of specific muscle groups rather than traditional neurosegmental levels as shown in the International Myelodysplasia Study Group Criteria for Assigning Motor Levels (2).

Untitled.png


Sensory Deficits:
Sensation is often times not clear cut. These levels do not necessarily correlate with the motor levels and there may be skips areas that lack sensation. For this reason it is important to test not only all dermatomes, but also multiple sites in each dermatome to have an accurate representation of what the child is lacking. Vibration sensation and either light touch or pinprick sensation should be tested. We need to educate the child and their family on their lack of sensation and safety should be emphasized especially with temperature and when barefoot. It is also important to educate the families on skin inspection and pressure relief techniques so they can become part of their daily routine. (2)

Hydrocephalus:
Approximately 25% of children with SB are born with hydrocephalus and an additional 60% will develop it after surgical closure of their back lesion. Of the children that develop hydrocephalus 80-90% will require a CSF shunt, which shunts CSF from the
lateral ventricles to the peritoneal space where the CSF can be resorbed. The figure to the right shows the placement of a shunt.
external image shunt.gif
.
Since the shunt is a foreign body it is important to remember that it can become infected or obstructed. Repeated or prolonged shunt dysfunction or infections can cause a decline in function and cognition in the child. Below is a table of signs and symptoms of shunt dysfunction in different age groups. (2, 20)
Infants
Toddlers
Children & Adults
Enlargement of head
Full or tense fontanelle
Swelling or redness on shunt tract
Fever
Irritability
Seizures
Sunset eyes (eyes looking downward)
Enlargement of head
Fever
Swelling or redness on shunt tract
Vomiting
Headache
Sleepiness
Seizures
Irritability
Headaches
Vomiting
Fever
Swelling or redness on shunt tract
Irritability
Ataxia
Personality changes
Visual disturbances
Decline in academic performance
Decline in motivation
Confusion
Seizures
Sleepiness
Incontinence
http://www.spinabifidamoms.com/english/about.html
(20)
Arnold Chiari Malformation: part of the cerebellum (cerebellar tonsils) may protrude through the foramen magnum. The tonsils interfere with the flow of CSF causing it to back up into the subarachnoid spaces thus producing hydrocephalus.
http://www.webmd.com/migraines-headaches/arnold-chiari-malformation-10486

Cognitive/Language Dysfunction:
Positive Factors:
Negative Factors:
  • Early closure of spinal lesion
  • Antibiotic intervention to prevent meningitis
  • Absence of hydrocephalus
  • No infections or cerebral hemorrhage
  • Intelligence scores tend to be higher in lumbar and sacral lesions
  • (2)
  • Having significant CNS infections
  • Intelligence scores tend to be lower in thoracic lesions
  • Hydrocephalus
  • Presence of infection or cerebral hemorrhage
  • (2)
  • There is a 3x higher incidence of low IQ scores in children with spina bifida
  • Intellectual function correlates inversely with the level of spinal cord dysfunction. The higher the lesion, the lower the IQ score.
  • Hydrocephalus alone does not exclude normal cognitive function, however, its complications do, i.e. repeated CNS complications with meningitis can lead to significant cognitive deficits. Concentration and attention deficits are related to hydrocephalus. (7)
  • The term "cocktail party personality" represents children with good verbal ability that create the impression of higher intellectual functioning than is found on formal testing.
    • These kiddo's will tell you that they are capable of performing a task however in reality they are not able to complete said task.
    • High verbal skills
    • Lack social skills
    • Despite the initial appearance of being capable, they are often impaired (their performance in daily life is below what they superficially appear capable of).
    • Is important for PT's to directly observe skills that these children report that they can perform to determine if info provided by the patient is accurate. (2)(7)

Latex Allergy:
  • Up to 73% of children have been reported to have latex allergies.
  • Latex may be present in a number of items so we need to be careful
    • W/C seats and liners
    • Foam rubber lining on splints and braces
    • Elastic on diapers and clothes
    • Pacifiers
    • Balls
    • Examination gloves
    • Theraband(7)(2)

Upper Limb Dyscoordination:
The dysocoordination can possibly be explained by 3 causes: 1) cerebellar ataxia most likely related to Arnold Chiari type II malformation; 2) motor cortex or pyramidal tract damage secondary to hydrocephalus; or 3) motor learning deficits resulting from the use of upper limbs for balance and support rather than manipulation and exploration. Their movements can be described as halting and deliberate, rather than the expected smooth, continuous motion of able-bodied children. There is a heavy reliance on visual feedback instead of kinesthetic sense.(2)

Visuoperceptual Deficits:
May be present in kiddo's that have upper limb dyscordination. (2)

Cranial Nerve Palsies:
May occur if the Arnold-Chiari malformation is present along with hydrocephalus or dysplasia of the brain stem.
  • Ocular muscle palsies can occur (abducens, optic, oculomotor). Only ~27% of the participants in a study of 322 children had normal vision.
  • Glossopharyngeal
  • Vagus nerve issues can cause pharyngeal and laryngeal dysfunction (croupy, hoarse cry) and swallowing difficulties. Bradycardia may also occur in severe Arnold-Chiari type II malformations.(2)

Spasticity:
May range from flaccid to normal to spastic. Depends whether they have CNS abnormalities, or paralysis below the segmental level of their spinal lesion, or if they have lower motor neuron symptoms vs upper motor neuron symptoms.(2)

Progressive Neurologic Dysfunction:
  • Loss of sensation
  • Loss of strength
  • Pain at the site of the sac repair
  • Pain radiating along a dermatome
  • Initial onset or worsening of spasticity
  • Development or rapid progression of scoliosis
  • Development of a lower limb deformity
  • Change in bower or bladder sphincter control
  • Compression of spinal curd due to severe herniation of intervertebral disks
  • These changes may be due to CSF shunt obstruction, hydromyelia, growth of a dermoid or lipoma at the site of repair, subarachnoid cysts of the cord, or spinal cord tethering (cord tethering occurs from scarring of the neural placode or spinal cord to the overlying dura or skin with resultant traction on neural structures).(2)

Seizures:
Occur in 10%-30% of children/adolescents with spina bifida. May be due to brain malformation, CSF shunt malfunction or infection, and residual brain damage from shunt infection or malfunction.(2)

Neurogenic Bowel/Bladder:
<5% develop voluntary control of their urinary or anal sphincter. (This is due to the abnormal/absent function of spinal segments S2-S4)
  • Bladder: This typically occurs due to the dyssynergy between the detrusor muscle of the bladder and the external urethral sphincter (i.e., the bladder contracts but the sphincter does not relax to allow the flow of urine out of the urethra). It is now standard practice for newborns with spina bifida to undergo a urologic work-up 7 days post partum. Early implementation of intermittent catheterization in infancy helps to prevent future problems. Effective bladder intervention is achieved w/clean (not sterile) intermittent catheterization on a regularly timed schedule for voiding. The risk of injury to the urethra or bladder from clean intermittent catheterization is sufficiently low to allow young children to be taught to catheterize themselves. Mastery of the technique is usually achieved by age 6 to 8 years depending on the severity of involvement. The physical therapist must be aware of the method used for urine drainage as it relates to w/c positioning, transfer techniques, and orthoses so that assistive devices do not interfere w/effective performance of urine drainage techniques.
  • Bowel: The anal sphincter can be flaccid, hypotonic, or spastic. Treatment is with an individually designed bowel program . Also, instituting bowel training before age 7 years correlates w/improved outcomes by means of better compliance. (2)
‍‍
Obesity:
Is a common problem that may further complicate orthotic fitting, w/c fitting, and can also affect independence with functional mobility. For children who are ambulatory, a greater expenditure of energy is required to participate in physical play activities therefore it is less common for these kiddo's to participate in those activities therefore will be more sedentary. Research studies have shown that the caloric intake should be lower in individuals with spina bifida (that use a w/c as their primary means of mobility) than able-bodied peers (<1500 calories/day). This amount is chosen because individuals with spina bifida that use a w/c for their primary mode of mobility have decreased muscle mass coupled with lower extremity inactivity. When assessing the child for being overweight/obese, the height to weight ratio should not be used because of their short stature, decreased linear length secondary to spine or lower limb deformities, and decreased growth of paretic limbs. Rather an arm span-weight ratio is more appropriate (0.9 arm span for children w/no leg muscle mass usually seen in thoracic and high-lumbar levels; 0.95 arm span for those w/partial loss of muscle mass seen in mid- and low-lumbar lesions; or 1.0 arm span for children with no muscle mass loss). A regular exercise program is essential to promote cardiovascular heath and to maintain a healthy body weight (as cardiovascular issues are more common in obese individuals). Try to make age-appropriate physical activities that are fun and at which they can succeed; in this way, physical activity is positively reinforced and a lifelong pattern of engaging in such activities is developed.(2)

Skin Breakdown:
  • ‍Occurs in 85-95% of all children with spina bifida by the time they reach young adulthood.
  • Perineal decubiti and breakdown over the apex of the spinal kyphotic curve occurred in 82% of thoracic level lesions, 62% of high lumbar level lesions, 50-53% of those with lower level lesions.
  • Predictors of skin breakdown:
    • Excessive pressure during ambulation or while resting feet on w/c footrests
    • Foot rigidity
    • Non-plantigrade position (toe-walking, excessive eversion or inversion, or not having a normal heel-toe gt pattern)
    • Surgical arthrodesis
    • Clawing of the toes
  • Etiology:
    • Tissue ischemia from excessive pressure
    • Casts or orthotic devices producing a breakdown
    • Urine and stool soiling producing skin maceration
    • Friction and shear forces
    • Burns
    • Excessive weight bearing over bony prominences of the pelvis as a result of spinal deformity, obesity, lower limb autonomic dysfunction w/vascular insufficiency or venous stasis, and tenuous tissue postoperatively over bony prominences.
    • AGE!!!
      • Young children who are not toilet trained have breakdown from skin soiling (ammonia burns)
      • Young active children have the greatest frequency of friction burns on knees and feet from scooting along rugs, hot water scalds, and pressure ulcers from orthoses or casts.
      • Older children, adolescents, and young adults develop skin breakdown over lower limb bony prominences from the increased pressure of a larger body habitus, asymmetric weight bearing resulting from deformities, abrasions of the buttocks or lower limbs resulting from poor transfer skills, improperly fitted orthoses, and lower limb vascular problems.(2)

‍‍Age-Specific Examination and Physical Therapy Intervention:‍‍ (2)


Typical Participation Restrictions: Causes and Implications
Examination of Impairments
Ongoing Monitoring:
PT Goals and Strategies:
Prevention of Secondary Impairments and Activity Limitations
Infancy
-Parents fearful to handle their infant
-Medical complications
-Decreased stimulation due to fear from other family members
-Infant may be impeded by the additional parental duties (e.g., bowel/bladder intervention, frequent medical visits, and hospitalizations).
-Joint contractures and deformities
-Motor and sensory deficits
-Hypotonia
-Upper limb dyscoordination
-CNS dysfunction
-Visual and perceptual disorders
-Cognitive deficits
-Lack of normal infant movements, combined w/impaired sensation, decreases kinesthetic awareness and inhibited perceptuomotor development.
-Hip/knee contractures might be present (normal infant might be stuck at 35degrees)
-Manage dislocated hips and foot deformities
-Orthopedic intervention for deformities
-Achieving a plantigrade foot position is important (for shoe fit, positioning and weight distribution in sitting, and stability when bearing weight for standing pivot transfers or ambulation).
-Goal is to establish a reliable baseline of muscle function and sensation.
-Therapists often do not record specific strength grades but use a "present or absent" scale, or a 3-point ordinal scale (apparently normal, weak, or absent).
-Monitor joint alignment, muscle imbalance, muscle tone, and the development of contractures.
-Look for signs of shunt malformation
-Look for behavioral changes or seizure disorders.
-Written instructions w/verbal explanations
-Positioning techniques
-Stretching ex's
-Soft tissue mob's in conjunction w/diaper changes
-Adaptive equipment
-Skin inspection of insensate area
-Education if hips are dislocated (proper positiong, double diapering, night-positioning orthosis)
Toddler and Preschool age
-Mobility device might be indicated if not independent by the end of the 1st year
-Limited early mobility may result in a lack of curiosity and initiative. The child may learn to be passively dependent.
-Play opportunities might be limited if the child does not get around like other children.
-Self-esteem may be low
-May not be independent with ADL's and miss out on play time due to waiting for caregivers
-Poor hand-eye coordination which may impact handwriting, feeding skills and dressing.
-ROM is expected to be WNL's by the end of the 1st year.
-Functional muscle testing techniques should be used (gait observations, heel- and toe-walking, climbing up/down steps, SLS, toe touching, squat to stand, toe touching, bridging, prone kicking, sit-ups, etc.)
-Children think the muscle names are humorous (which maintains their attention).
-Sensation can be tested by having the child identify between light touch and pinprick or by having the child point to the spot that was touched.
-ADL's - what can the child do? what are they having trouble with? are there adaptations that could be made?
-Monitor joint alignment, mm imbalance, contractures, posture, and signs of progressive neurologic dysfunction.
-Strengthening act's
-Family education/involvement
-Posture exercises (therapy ball)
-FES, biofeedback, e-stim
-Goal is to improve independence, efficiency, and effectiveness of ADL's and mobility.
-Teach the child and have them actively participate in skin inspection, bowel and bladder intervention, donning/doffing orthoses, w/c intervention, and ADL's.
-It's important to help the parents understand how incontinence retards their child's normal sexual exploration, learning, and social inhibitions that normal preschool children learn.
-Showing the parents normative values for eating, dressing, and grooming may give parents guidance as to where their child should be developmentally.
-Assistive devices: electric w/c (as young as 24months), castor cart, loftstrand crutches
-Orthotics
-Use positive reinforcement
-Same as above
-Habitual postural positions that contribute to deforming forces should be discouraged.
-It is essential to emphasize an upright posture when a child is learning to walk
-Postural deviations that look insignificant when a child is young are often magnified once body proportions increase so it's important to capture these issues.
School age
-Decreased self-esteem
-Increased dependence on caregivers (if they are not independent w/ADL's)
-Mobility limitations can reinforce dependent behaviors if other children carry his/her books or lunch tray or push the w/c
-Decreased socialization
-Inability to play if they have physical disabilities.
-May be excluded from recess or PE.
-Visuoperceptual, sensory, or perceptuomotor issues might impact writing, speech, problem solving, and cognitive abilities.
-Multiple hospitalizations may affect school ability.
-Objective MMT or myometry should be used to show strength.
-Independence w/ADL items and assist with basic household chores
-Assess child's ability to carry items
-Assess child's ability to perform w/c push-up for adequate pressure relief
-NSG should evaluate bowel and bladder function
-The home, school, and community environment should be accessible so that these individuals can fully participate in all activities.
-Both physical and social barriers to participation must be addressed
-Monitor joint alignment, muscle imbalance, muscle tone, and the development of contractures.
-Make sure the child is independent with daily skin inspection.
-Should be observed closely during periods of rapid growth b/c they are at risk for loss of function as a result of cord tethering.
-Same as above
-Improve flexibility of low back extensors, hip flexors, hamstrings, and shoulder girdle musculature
-Encourage child's participation in PE or physical activities in school
-Proper positioning while sleeping/sitting.
-W/c positioning (and cushion)
-Look at efficiency with ADL's
-Assistive devices for ambulation
-Adaptive equipment
-Recreational and physical activities should be emphasized including: swimming, low-impact aerobics (there are a lot of videos), cycling, rowing, cross-country skiing, roller and ice skating, and aerobic dance (www.cureourchildren.org/sports.htm).
Continue per above.
  • In all age groups, PTs should look at joint alignment, strength, muscle imbalance, contractures, muscle extensibility, and posture. As the child ages, PT's should also look at gait, sensation, ADL's, motor skills, functional skills, and body awareness.
  • Common goals across the life span are to prevent joint contractures, correct existing deformities, prevent or minimize the effects of sensory and motor deficiency, and optimize mobility within natural environments. (2)
  • Here is a 2 page pdf that describes the segments of the spine, muscle functions that are associated with each segment, possible orthopedic concerns, possible orthotics needed, possible equipment for functional mobility, and possible cognition/executive functioning issues that might persist.

Adolescence and adulthood

It’s all about independence.

As of course was the case with younger people with spina bifida, the functional limitations of adolescents and adults with spina bifida are highly variable and can span from mild to severe. In adolescence individuals with spina bifida may begin participating in the development of their IEP, if they have one, and like other teens their age, work to assert greater independence in their daily lives. Most individuals are able to be independent with clothing, bathing, toileting and most other ADL’s. Most with SB are able to grow up to live independently, hold a job, have successful relationships, marriages, children, and interact positively with their community. Many with SB go about their lives leaving little or no outward sign of disability. Still others have more severe limitations of function and may require assistive devices, special adaptations or assistance with some tasks, but it is not an unreasonable expectation for any individual with SB to lead a productive, fulfilling life. (14)

Physical health
It has been found that participation in physical activities by individuals with SB decreases from childhood into adolescence.1 Thus it is all the more important to stress exercise and physical activity among adolescents with movement-related conditions like spina bifida. Physical therapy often serves a role in teaching exercises for strengthening, mobility and endurance for people with this condition. It is important to consider participation-related goals for these individuals, particularly as they enter into a period in life typically marked by decreased dependence on parental caregivers and increased social interaction and influence with peers. We can help encourage physically engaging activities that a patient can participate in with friends such as going on walks, lifting weights, and sports. Summer camps and recreational facilities exist to engage individuals with disabilities and these may serve as a resource for teens with spina bifida, but it may be equally beneficial for these teens to participate with their typically developing peers as well, as they are able. (11)

Team Spina Bifida athletic programs – Spina Bifida Association http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8275991/k.96F3/Team_Spina_Bifida.htm

Disabled Sports USA http://dsusa.org/index.html

Psychological impact of disability in adolescence
Adolescence can be a hard time for anyone, regardless of disability, and thus it is important to monitor the psychological and emotional health of those with a disability during the teen years. The need for an assistive device may limit a young persons’ participation with their peers in sports and extracurricular activities, which may have a profound impact on self-esteem and life satisfaction. (11)

As teens enter their dating years, it is important for caregivers have an open dialogue with their children about sex and sexuality. It has been found that young adults with SB often experience a delay in receiving sexual education due to relatively low levels of sexual experience compared to their peers.2

Sex and Spina Bifida
Spina bifida does not prevent most people from having healthy, fulfilling sexual experiences. However some adaptations are necessary for some individuals. Sexual functioning can be affected in spina bifida as with other injuries of the spinal cord, though these deficits do not typically affect fertility and many can be overcome with simple adapations. Due to the influence of genetics on spina bifida incidence, parents who were born with spina bifida have a higher likelihood of having a child with the condition. (12)

The sexual deficits caused by spina bifida typically affect men more than women. Sexual function considerations include continence issues, decreased sensation, erectile dysfunction, inadequate vaginal lubrication and difficulty in positioning for sex. Women may have difficulty having sex due to limitations of the joints and musculature of the hip and pelvis as well as bone density issues. (12)

It would be remiss not to add that the experience of sexuality and fatherhood are enhanced, and in most cases, preceded by a loving, caring relationship which includes acceptance and understanding by both partners.” – Spina Bifida Association, “Men’s Health - Urologic and Sexual Function” (13)

Continence issues with regards to sex can typically be avoided by voiding the bowel and bladder before and after sex, positioning for sex can usually be achieved through simple adaptations, and other issues relating to sexual function such as erectile dysfunction and lubrication can typically be treated the same way as when they occur in the general population. It is worth noting that a high percentage of individuals with SB also have an allergy to latex. To prevent transmission of STDs and STIs, it is important to consider use of latex-free condoms for sexually active people with SB.(13)

Here is a link to an 88 page powerpoint by a physician that discusses sexual dysfunction/issues that may occur with spina bifida. The physician does a very good job at describing issues that may occur and how to address those issues.
http://www.google.com/url?sa=t&rct=j&q=&esrc=s&frm=1&source=web&cd=2&ved=0CDoQFjAB&url=http%3A%2F%2Fconference.spinabifidaassociation.org%2Fatf%2Fcf%2F%257B0c3388cf-9fd0-48f2-ba64-e32db2dbf36c%257D%2FWIENER%2C%2520JOHN-SEXUAL%2520FUNCTIONING.PPT&ei=xTqEUKL7M8i6yAGzs4GIDw&usg=AFQjCNH4NU1-scXbabJ3CBtDcdm_tkpS0Q&sig2=9ONPGPo8C6btwgcjJsgcKA (10)

Tethered Spinal Cord
Physical maturation presents a problem for individuals with SB and a tethered spinal cord due to stretching of the nerve fibers as the person grows. In this condition, the caudal end of the spinal cord has a fibrous attachment to the surrounding tissue instead of moving freely in the spinal canal. This can lead to scoliosis and other deformities. A tethered spinal cord can be a primary problem, independent of SB, but it occurs with greater frequency in SB than the general population. Diagnosis is made through MRI. A tethered spinal cord may be released with surgery, but requires close followup to ensure there is not recurrence. (15)

Patients with SB and a tethered spinal cord may present with progressive symptomology or a sudden regression in functional skills coinciding with a growth spurt. Weakness in the legs, loss of lower extremity sensation, back pain, and changes in bowel and bladder function may be indicative of a tethered spinal cord. Refer out! (15)
external image lolo-jones-flag.jpg
US Olympic hurdler, Lolo Jones, was discovered to have a tethered spinal cord after complaining of somatosensory and proprioceptive deficits that affected her ability to compete in her sport. Surgery to release the spinal cord was performed successfully in 2011 and in 2012, she finished fourth in the 100 meter hurdles at the London Olympics.

Equipment for CP and Spina Bifida:

Due to the wide range of needs children with neurological conditions have many different types of assistive equipment may be utilized to help address the posture and mobility problems that negatively impact the participation.
  • Seating- Supportive posture and positioning are important in this patient population due to decreased postural control, spasticity, and the possible development of contractures. In children with poor postural stabilization supportive seating can provide the trunk control they might need to better interact with their environment whether this be playing, feeding, communication, social interaction, etc. Seating should be age appropriate, meet the needs functional needs of the child, and realistic for the home environment and family lifestyle (1, 2).
  • Standers- Weight bearing through standing is important to increase bone density, improve muscle extensibility especially in the lower extremity, decrease spasticity, prevent contractures, and encourage correct posture. Standing with CP kids begins around the age of 1 and will continue with those with more severe impairments to allow for standing transfers as the child grows up to reduce the need for lifting (2).
  • Braces or orthotics- To help with joint alignment, prevent contractures, improve postural control and gait biomechanics, and to address lower extremity deformities, a child may have braces or orthotics. The device will depend on the needs of the child. AFOs are commonly used by children in this population (2).
  • Assistive devices- Mobility is another key factor for this group. Assistive devices for mobility may include power wheelchairs, manual wheelchair, walkers, crutches, or canes. Again this depends on the functional needs and impairments of the child. The mobility device may be different depending on the setting-- i.e. one mode of mobility at home or in the classroom while another may be used in the community. Posterior walkers are commonly used to encourage a more upright posture instead of encouraging a flexed posture with a front walker (2).
  • external image nimbo-grl.gifexternal image images?q=tbn:ANd9GcQsdriBE5PLO-Lh7avsVZipm1e3zlR3gbFzAgJJcQlsEPQRy3pBexternal image images?q=tbn:ANd9GcR6MyT_GUpQ4NYzml0CgQl0NQg4HQLptk53J0HjaxXhZzZPMV1Dexternal image images?q=tbn:ANd9GcTBz0Ll4sPwHr5FqljGAkcUKe0Yh2nBlwPvypL0sfo6Wpl-TylWQ62agIg
  • http://www.youtube.com/watch?v=ds-kUvYkkss

Resources for Parents for CP and Spina Bifida:


Case summary:




References

1. Umphred, D. (2001). Neurological Rehabilitation (4th ed.). p. 259-283. St. Louis, MO: Mosby, Inc.
2. Campbell, S., Orlin, M., & Palisano, R. (2012). Physical Therapy for Children (4th Ed.). p. 577-627. St. Louis: Saunders Elsevier.
3. Narayanan, U. (2012). Management of children with ambulatory cerebral palsy: an evidence-based review. Journal of Pediatric Orthopaedics, 32(2), 172-181.
4. Cameron, E.C., Maehle, V., Reid, J.: The effects of an early physical therapy intervention for very preterm, very low birth weight infants: A randomized controlled clinical trial. Pediatric Physical Therapy. 17, 2005, 107–119.
5. Verschuren, O. et al. (2009). Functionally based exercise program for children and adolescents with cerebral palsy. Accessed on 10-16-12 from http://www.netchild.nl/research-fitness.html.
6. Deutsch, J. et al. (2008). Use of a low-cost, commercially available gaming console (Wii) for rehabilitation of an adolescent with cerebral palsy. Journal of Physical Therapy, (88)10, 1196- 1207.
7. Cuccurullo, S. (2004). Physical Medicine and Rehabilitation Board Review. Chapter 10. New York, NY: Demos Medical Publishing.
8. Pasternak, N. Riding for Life posted on YouTube as Ride On St Louis Participates in Hippotherapy & Cerebral Palsy Study at Washington University by RideOnSTL. Accessed 10-20-12 from http://www.youtube.com/watch?v=UHUIZGF4o3U&feature=youtu.be.
9.Kelly, M. & Darrah, J. (2007). Aquatic exercise for children with cerebral palsy. Developmental Medicine & Child Neurology, (47)12, 838-842.
10. Wiener, J.S, Sexual Function in Men with Spina Bifida Putting the FUN in Function. Spina Bifida Association. Division of Urologic Surgery. Duke University Medical Center, Durham, NC. http://www.google.com/url?sa=t&rct=j&q=&esrc=s&frm=1&source=web&cd=2&ved=0CDoQFjAB&url=http%3A%2F%2Fconference.spinabifidaassociation.org%2Fatf%2Fcf%2F%257B0c3388cf-9fd0-48f2-ba64-e32db2dbf36c%257D%2FWIENER%2C%2520JOHN-SEXUAL%2520FUNCTIONING.PPT&ei=xTqEUKL7M8i6yAGzs4GIDw&usg=AFQjCNH4NU1-scXbabJ3CBtDcdm_tkpS0Q&sig2=9ONPGPo8C6btwgcjJsgcKA
11. Kelly EH, Altiok H, Gorzkowski JA, Abrams JR, Vogel LC. How does participation of youth with spina bifida vary by age? Clin Orthop Relat Res. May 2011;469(5):1236-1245.
12. Shoshan L, Ben-Zvi D, Meyer S, Katz-Leurer M. Sexuality in relation to independence in daily functions among young people with spina bifida living in Israel. Rehabil Nurs. Jan-Feb 2012;37(1):11-17; quiz 17-18.
13. "Men's Health - Urologic and Sexual Function." Spina Bifida Association. Ed. Spina Bifida Association. 1 Jan. 2012. Web. 21 Oct. 2012. <http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8031517/apps/s/content.asp?ct=12073157>.
14. "Spina Bifida." Centers for Disease Control and Prevention. Centers for Disease Control and Prevention, 14 Mar. 2011. Web. 21 Oct. 2012.<http://www.cdc.gov/ncbddd/spinabifida/index.html>.
15. "NINDS Tethered Spinal Cord Syndrome Information Page." Tethered Spinal Cord Syndrome. National Institute of Neurological Disorders and Stroke, n.d. Web. 22 Oct. 2012. <http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm>.
16. Narayanan. Management of children with ambulatory cerebral palsy: an evidence-based review. J Pediatr Orthop, 2012. 32; S172-181
17. WebMD. What causes Cerebral palsy? http://www.webmd.com/brain/understanding-cerebral-palsy-basic-information Web 23 Oct. 2012
18. Diagnosis of and Tests for Cerebral Palsy. http://cerebralpalsy.org/about-cerebral-palsy/diagnosis/. Web 23 Oct. 2012
19. The Luncina Foundation. http://www.lucinafoundation.org/birthdefects-spinabifida.html. Web 20 Oct 2012
20. MOMS. Management of Myelomeningocele Study. http://www.spinabifidamoms.com/english/about.html. Web 20 Oct 2012
21. Cuccurullo S, editor. Physical Medicine and Rehabilitation Board Review. New York: Demos Medical Publishing; 2004. Spina Bifida.Available from: http://www.ncbi.nlm.nih.gov/books/NBK27180/