Impairments

Question 1

MSK

Answer 1

Postural Issues: forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, ant pelvic tilt, rotational deformities of hip/tibia, flexed hips/knees, pronated feet
–Important to examine balance and WB in multiple positions, static/dynamic, sleeping posture and sitting positions, look for habitual positions that could cause postural or joint deformities

Postural alignment: (see note card for specific levels)

• Crouched standing common across all levels- characterized by persistent hip/knee flexion and increased lumbar lordosis
• Crouched posture occurs d/t mm weakness (insufficient soleus to maintain tibia vertical) and orthopedic deformities (calcaneal valgus- results in obligatory IR and knee flexion)–> hip/knee flexion contractures usually occur secondary to adaptive shortening
• Crouch posture negatively affects task requirements and torque generating capacity of MSK system —> results in increased demands on MSK system = negative impact on function and increased risk of additional impairments

Joint alignment: stress often seen during dynamic activities (walking or w/c propulsion), important to look at weight patterns on shoes or orthotics to determine abnormal stress or malalignment

• • supple deformities may positively respond to stretching
• • fixed deformities may respond to serial casting, but often require surgery

15d recline of seat in w/c = decrease stress on lumbar spine

Question 2

Osteoporosis

Answer 2

decreased bone mineral density occurs secondary to hypotonic or flaccid musculature with decreased loading of long bones from altered mobility– common in children with MM often results in osteoporotic fx

Standing programs are beneficial, 60 min 4-5x/week increased bone mineral density and improve acetabular development

Children who are ambulatory have higher bone mineral density

Fractures- occur subacutely d/t lack of sensation with swelling and warmth at fracture site, low grade fever are common s/s
- Want to keep immobilization to a minimum, want to resume WB as soon as possible to avoid risk of additional fractures

Question 3

Motor Paralysis

Answer 3

MM- paraplegia d/t spinal cord malformation
- Upper limb weakness can also occur in this pop regardless of lesion level, s/s of progressive neurologic dysfunction

Motor level = lowest intact, functional neuromuscular segment

• • Ex. L4 = fourth lumbar nerve and myotome are functioning, segments below L4 are not intact
• – look at table 22-1 for motor levels
• – MM spinal lesions can be asymmetric when motor or sensory functions of right and left of the body are compared

Neuromuscular involvement in MM can manifest in 3 ways:

1. lesions resembling complete SC transection
   - – normal function down to a particular level below which there is a there is flaccid paralysis, loss of sensation, or absent reflexes
2. incomplete lesions
   - – mixed manifestations of spasticity and volitional control
3. Skip lesions
   - – observed where more caudal segment are functional despite the presence of one or more nonfunctional segments interposed btwn the intact more cephalad spinal segments
   - – individual skip motor lesions manifest either with isolated fxn on mm noted below the last functional level of the lesion or with inadequate strength of mm groups that have innervation higher than the the lowest functioning group

Question 4

Sensory Deficits

Answer 4

Not clear cut in this population bc sensory levels do not correlate with motor levels and may be skip areas that lack sensation

Education regarding sensory deficits is important for safety, pressure relief, etc.

may also lack proprioception and kinesthetic awareness
- may want to use patellar tendon bearing orthosis may facilitate foot placement for individuals with innervation through L3 bc contacts skin area with intact sensation

Question 5

Hyrdocephalus

Answer 5

excessive accumulation of CSF in the ventricles of the brain, 25% of children with MM have hydrocephalus, 60% develop it following closure of spinal lesion

If left untreated can result in continued expansion of ventricles causing loss of cerebral cortex with additional cognitive and functional impairment

Cerebellar hypoplasia with caudal displacement though foramen magnum = Arnold Chiari Type II malformation—-> usually associated with hydrocephalus

80-90% of children will need to be shunted
- Shunt moves excess CSF from lateral ventricles to peritoneal space where it is reabsorbed

Must be aware of shunt malfunction: headache, lethargy, changes in speech, fever, decreased activities, irritability, decreased coordination, onset or increase in seizures

Question 6

Cognitive Dysfunction

Answer 6

Improved outcomes in cognition with early closure of spinal lesion and treatment with antibiotics to prevent meningitis and improved CSF shunt intervention

Children without hydrocephalus or those with uncomplicated hydrocephalus, lower lesions will have intellect WNL

Children with CNS infections = lower intelligence scores

Dyscoordination and memory deficits contribute to cognitive dysfunction

“cocktail party personality” = behavioral disorder associated with hydrocephalus regardless of age or intelligence level

• articulate, verbose, and superficially appear to have high verbal skills
• frequent and inappropriate use of cliches or jargon , misuse of individual words

Question 7

Language Dysfunction

Answer 7

MM and hydrocephalus

High frequency of irrelevant utterances and poorer performance of abstract rather than concrete language

Question 8

Latex Allergy

Answer 8

73% of children with MM have latex allergy

occurs d/t exposure to latex frequently and early on in development, exact cause is unknown

Question 9

Upper Limb Dyscoordination

Answer 9

Greater in children with hydrocephalus

3 possible causes

1. cerebellar ataxia likely related to chiari 2
2. motor cortex or pyramidal tract damage due to hydrocephalus
3. motor learning deficits resulting from use of upper limbs for balance and support rather than manipulation and exploration

Lack of smooth movement patterns (motor apraxia), heavy reliance of visual feedback and kinesthetic sense

Repetition of fine motor tasks is beneficial

delayed development of hand dominance in children with MM

Question 10

Visuoperceptual deficits

Answer 10

studies have not determined if these deficits are common in children with MM

Children with MM may have lower scores on tests looking at hand eye coordination—may be due to upper limb dyscoordination

Removal of ue coordination= children with mm performed at normal level when just looking at visual component

Question 11

Cranial Nerve Palsies

Answer 11

Chiari, hydrocephalus, and dysplasia of the brainstem can cause cranial nerve palsies

Abducens- ocular mm palsy, lateral rectus eye weakness, esotropia (eye turns inward) on the involved side

Glossopharyngeal and Vagus- pharyngeal and laryngeal (croupy, hoarse cry) dysfunction or swallowing difficulties
- Apneic episodes, and bradycardia– sever symptoms of chiari 2 malformation

Question 12

Spasticity

Answer 12

mm tone in infants and young children with MM can range from flaccid to spastic

Some kids with MM with demonstrate UMN lesion signs

Question 13

Progressive Neurologic Dysfunction

Answer 13

Minor improvements in strength or development are rare but can occur as late as the 4th decade of life

Deterioration from neuro changes that are due to treatable complications
- includes UE’s and LE’s- loss of sensation, loss of strength, pain at side of sac repair, pn radiating along dermatome, initial onset or worsening of spasticity, scoliosis, BB control issues

Deterioration can be due to shunt malfunction, hydromyelia, growth of dermoid or lipoma at site of repair, subarachnoid cyst, tethered cord

Tethered cord- occurs d/t scarring of neural placode or spinal cord to the overlying dura or skin with resultant traction on neural structures
- may result from other congenital anomalies

Acquired cause of spinal cord deterioration is herniated disc

May treat these dysfunctions surgically

Question 14

Seizures

Answer 14

10-20% of those with MM

Associated with brain malformation. shunt malfunction/infection, residual brain damage from shunt infection or malfunction

Meds used as prophylaxis against seizures
–con to meds is they can accentuate cognitive deficits or dyscoordination that is already present

Untreated seizures = permanent cognitive or neurological functional loss or death

Question 15

Neurogenic Bowel

Answer 15

<5% with MM develop voluntary control of urinary or anal sphincter

Abnormal or absent spinal segments S2-4 = incontinence

Anal sphincter can be flaccid, hypotonic, or spastic

Impaired anorectal sensation–unable to feel imminent bowel movement

Constipation/impaction can also occur

Need to develop a bowel program» minimizes incontinence and constipation

Presence of bulbocavernosus or anal cutaneous reflex predictive of success with bowel training program

Bowel program before age 7 = improved outcomes and compliance

Question 16

Neurogenic Bladder

Answer 16

Goal is infection free social continence with preservation of renal function

Vesicoureteral reflux- Retrograde flow of urine from bladder up the ureters to the kidneys, can occur without evident s/s until irreversible renal failure occurs

Inadequate emptying of the bladder with residual urine retention w/in bladder = increased risk of bacteria for recurrent UTI’s or sepsis

Bladder dysfunction can begin in utero typically d/t dyssenergy btwn detrusor mm and external urethral sphincter (bladder contracts but sphincter does relax allowing release of urine)

Bladder intervention = clean intermittent catheterization on a voiding schedule

• • mastery of self-cath achieved at 6-8 y/o
• • may also be put on oral meds for spastic detrusor mm

Recc follow up with urology every 6 mo until age 2 and yearly after that throughout life span

Question 17

Skin Breakdown

Answer 17

Decubitus ulcers and other skin break down occurs in up to 95% of all children with MM

greatest risk for skin breakdown seen in children who are not toilet trained

young children have increased risk for friction burns

education regarding insensate areas and later develop independence in self-care

pressure ulcers can lead to decreased ambulation and participation in activities

Question 18

Obesity

Answer 18

common in children with MM impacts fitting or orthotic and w/c, impacts independence in mobility and self-care

burn less calories bc not as ambulatory as typical children

as obesity develops = less participation

Thoracic and high lumbar lesions = increased incidence of obesity

encourage physical activity and nutritional education

Question 19

Postural Deviations based on Level

Answer 19

Thoracic to L2: hip flexion, and, and ER contractures, knee flexion contracture, and ankle PF contractures, lordotic lumbar spine

L3-L5: hip/knee flexion contractures, increased L lordosis, genu and calcaneal valgus malalignment, pronated foor in wb, walk with crouched gait and WB on calcaneus

Sacral: mild hip/knee contractures and increased lumbar lordosis, ankle/foot can be in varus or valgus combined with a pronated or supinated forefoot, may walk with a mild crouched gait and may WB on calcaneus unless PF mm are at least 3/5

Question 20

Spinal deformities

Answer 20

Scoliosis occurs with MM can be congenital or acquired

• congenital underlying vertebral anomaly, curve is not flexible
• Acquired- curve is flexible until skeletal maturity is reached, little progression of curve is noted after maturity is reached
• More frequently observed in higher level lesions

Kyphosis

• Congenital- 10-15% of infants with MM
• Paralytic- acquired in 1/3 by early adolescence
• can occur in lumbar spine or can be more diffusely dispersed throughout spine
• lordosis and kyphosis- often seen in higher level lesions
• severe scoliosis or kyphosis negatively impact chest wall expansion resulting in respiratory compromise–> restrictive lung condition that can limit exercise tolerance and can be life threatening

Lordosis
- Hyperlordosis common

Treatment of Spinal deformities

• orthotics- helpful but does not decrease progression of acquired deformities
• surgery- spinal fusion, must be done at a time when child is at appropriate age, surgery at younger age results in increased rate of instrumentation failure and trunk shortening—> ideal age 10-11 in F, 12-13 in M, SB reach puberty early compared to typical pop

Question 21

Hip Deformities

Answer 21

Children with MM prone to hip deformities

Fixed flexion deformities require surgical intervention d/t negative impact on gait and orthotic fit
High lumbar lesion have unopposed flexion and adduction forces that push femoral head superior and posterior = contractures and bony deformities = increased risk of dislocation and subluxation, 1/3 of children with MM

Subluxation/Dislocation- may not have to have reduced in order to allow for ambulation– level pelvis and good ROM and more important for function than hip reduction
– many facilities operate only on children at or below L3 with quad mm function present– b/c more likely to be community ambulators

Hip contractures- increase significantly by adolescence
- thoracic and high lumbar lesions = higher incidence and greater severity of contractures

Question 22

Knee deformities

Answer 22

Frequent contractures or deformities, both flexion and extension (flexion primarily occurs in w/c ambulators, extension due to immobilization)
– if significant may need surgical release

Varus/valgus occur as well, often seen in L3 and above

Contractures increase with increased age

Knee contractures are greatest in thoracic and high lumbar lesions, incidence decreases with lower levels

Question 23

Ankle/foot deformities

Answer 23

Occurs in both ambulatory and nonambulatory children, m/c in children with lesions of L5 and above

Deformity is determined by partial innervation and consequent mm imbalances

• even with surgical intervention these deformities with recur, unless deforming forces are removed
• progressive deformities are often observed in conjunction with development of spasticity and motor strength loss associated with tethered cord
• “Skip” Lesions (occurs with tethered cord)– prone to progressive foot deformities

Foot deformities- ankle equinovarus (clubfoot), forefoot varus or valgus, forefoot supination or pronation , calcaneal varus/valgus, pes cavus/planus, claw toe deformities
– club feet = thoracic and high lumbar, common congenital deformity, surgery is indicated when child is developmentally ready to stand
– claw toes = sacral level
– ankle valgus- seen when there is presence of fibular shortening (correlated with paralysis of soleus)- surgery is indicated when the deformity cannot be functionally alleviated with orthotics
M/c contracture = PF

Torsional deformities- excessive foot pronation angles, windswept position of lower limbs is often present as result of hip anteversion/retroversion or tibial torsion
- affects standing/sitting balance, weight distribution and walking