Arthrogryposis Multiplex Congenita
Terms in this set (113)
What is AMC?
Nonprogressive neuromuscular syndrome that is present at birth
AMC is characterized by?
1. severe jt. contractures
2. muscle weakness
AMC occurs in 1 out of _________ live births
What is the Cause AMC?
AMC insult happens in the _______ of pregnancy.
What is the most common form of arthrogryposis?
Amyoplasia is related to _____ and is ________
What are the forms of Arthrogryposis?
Amyoplasia and Heterogenous group
What does distal arthrogryposis primarily affect?
hands and feet
What is highly responsive to treatment?
Disal arthrogryposis has a ________ basis?
Genetic (inherited as an autosomal dominant trait)
AMC c neurogenic and myopathic disorders typically exhibit?
Motor weakness that immobilized the fetal joints--> jt. contractures
The fetus with AMC with neurogenic and myopathic disorders may have an imbalance in _________.
Strength of oppositional muscle groups--> tendency to certain postures.
What are maternal factors that could lead to AMC?
Fever greater than 100, fever for 1-2 days in first trimester, prenatal viral infection, vascular compromise of the blood supply between mom and baby, uterine fibroid tumors, septum in the uterus
In AMC prenatal diagnosis is found by?
Ultrasound that shows prenatal AMC exhibits:
nuchal edema, thin undercalcified bone, small lungs, decreased mvmt time after 11 wks gestation limitations
AMC prevalence of severely affected body parts:
Most; hip, foot
Least: elbow, shoulder
Lack of muscle development
What are 2 common variations of AMC?
Child has abducted and ER hips, flexed knee, clubfeet, IR shoulders, extended elbows, flexed and ulnarly deviated wrists
Child has flexed and dislocated hips, extended knees, clubfeet (equinovarus), IR shoulders, flexed elbows,flexed and ulnarly deviated wrist
AMC associated characteristics
-Common to both type: clubfeet, flexed, and ulnarly deviated wrists, IR shoulders
Dimpling of skin over joints
Absent or decreased finger creases
Congenital heart disease
With AMC what is usually normal?
Intelligence and speech
Clubfoot management 1 month after child is born?
Child naturally stretched out and gets family in routine
After 1st month of clubfoot management what do you do?
Months of Serial casting and position devices ( allows plantigrade ft in time for appropriate upright activity)
What surgery can be used for clubfoot?
PMLR: posteriormediolateral release of clubfoot
With Clubfoot if there is not response to serial casting or reoccurence the 2nd PMLR ___________, or a ______ is performed.
PMLR shortens the cuboid, distal wedge osteotomy performed
In clubfoot at the end of growth what is performed?
What could you use to fix a club foot?
Clubfoot surgical correction result in?
Stiff painful feet in adulthood
With clubfoot what other surgery can be performed?
Hip dislocations and subuxation occur due to?
Poor acetabular development
Surgical repair of hips causes?
With AMC it is possibly better to have mobile ___________
With AMC what is most commonly associated with capsular changes within the joint?
With AMC patellar realignment is done before ______ to allow the femoral groove to develop with growth.
Later in life surgery for extension contractures if at least ______________
25 degrees of knee ROM present
What do you do to wrists if conservative splinting and stretching management is unsuccessful?
Fuse in functional positions
Fuse wrist in _____ positions
What do you do before wrist fusion?
Cast wrist for 1 week in position ( to assure ADLS are benefitted by position)
AMC may also be associate with what condition?
How can you manage scoliosis?
1 in 5 children/ adolescents with AMC develop?
Long C-shaped thoracolumbar scoliosis
What do you do for congenital scoliosis?
Vertical expandable prosthetic titanium rib implant
vertical expandable prosthetic titanium rib implant allows for?
In PT eval take photographs and videos to document changes from ___ &______
Growth and splinting
Repeat photographs and videos how often?
Every 4 mos for 2 years
Muscle grades for infants and very young children are ascertained during _________, observations of the ability of extremities to ________, and elevations of _______
Move against gravity
Gross motor function
Less than 3/5 fair muscle strength in hip extensors require?
Bracing above the hip
Less than 3/5 fair strength in knee extensors will require?
Bracing above knee
Corrected clubfeet require _________ during growth to minimize problems of recurrent clubfeet
Children with poor UE function and weak LE may not be functional _________
Children with poor UE function and weak LE may not be functional community ambulators as a results of ________________
Decreased motor control and protective responses
What is the developmental tests for children with AMC?
Children with AMC score lower than average on __________ due to ___________.
formal gross motor tests, due to decreased ROM and strength
AMC never achieve ______
Some milestones (creeping)
AMC children cognitively score
score average or above average
How do AMC ambulate
Assistive device (manual or powered WC)
Having limb segments aligned for mechanical advantage maximizes ________ &_______
Function and participation
What are STG and LTG for AMC Children?
Sx and bracing (ortho)
Incorporate family= maximize Ind. c ADL and mobility
Infanct PT examination includes:
PROM, strength, developmental assessment/ motor milestones
In infants c AMC what developmental assessments can you uses?
1. Bayley Scales of Infant Development
2. Alberta Infant Motor scale
In infants what do you do to measure activity and participated?
Could use pediatric evaluation of disability inventory and the WeeFim
What are infant goals?
Enhance general sensorimotor development
Education of family
Education of Family emphasizes?
Positioning, stretching techniques, avoidance of potentially harmful activities that feed into deformity
AMC interventions of infants
Foot abduction braces
Thermoplastic serial splinting
Address: developmental skills, compensatory strategies, maximize participation in age appropriate activities
What are first type interventions in infants?
Ambulate assisted, c device and LE orthotics by 18 mos.
AMB I by 2.5 yrs
Encourage stretching of hips and prone positions
Impossible to assume quadruped
scoots on bottom or roll
Ambulate assisted with device and LE orthotics by _____
AMB independently by _______
Encourage stretching of ______ and _____ positions
Infants are nearly impossible to assume______
Infants tend to .....
scoot on bottom or roll
What are infants second type interventions?
1. Amyoplasia: prone with wedge, towel roll due to elbow contractures
2. Velcro band in supine to bring hips in
3. Prone positioning with towel roll or wedge under chest
Amyoplasia prone with ____, towel roll due to _______
Velcro bands in supine to....
bring hips in
Infants with 2nd type interventions are in prone positioning with ______ or _______ under chest
towel roll or wedge
In caregiver stretching program do ______ sets and _______ reps in each set
3-5 sets per day, 3-5 reps in each set
How long should stretch be held?
2-30 secs per stretch
Do UE stretches when?
During feeding times
Stretching should be a ________ but most important in first _______
1st two years
Splints to maintain range should redo every
4-6 wks in infants
AFOs should be worn how many hours?
22 hours per day
In AFO calcaenous should be in what position?
Knee splints are ___________ for first ____ mos
thermoplastic for 3-4 wks.
Wear knee splints ______ hrs/day
Older infants with knee extension contractures should not?
Wear knee flexion splint greater than 50 degrees due to encouraging hip flexion contractures
At 6 mos you should remove knee splints to control ___________ for 6 hours/ day to allow emerging skills.
Describe a Day wrist splint
Dorsal cock-up splints with a palmar arch in the position or neutral deviation and a slight stretch into extension to allow toy manipulation
Describe Night wrist splint
Dorsal cock-up splint with a pan to allow finger stretching when child asleep
Splint elbow in _____ while sleeping
What splints are most function and worn during the day?
Elbow flexion splints and elbow flexion-assist splints
What do you do in Preschool Age PT exam?
Orthotics/ splints for stretch
Functional muscle strength, formal MMT if can understand verbal instruction
MMT in preschool are often strong in ________ but unable to move extremity to ___________********
Shortened end range
Where does the child need to work the muscles to maintain stretch of antagonist muscle?*********
Shortened end range
Preschool age goals emphasis is on assisting the child through ________ rather than through ________
Problem solving rather than through physcial assistance
What are ultimate goals of Preschool Age?
-Ind. Ambulations c min bracing and AD
Preschool stretching should be done?
twice daily during dressing and bathing
In preschool stretching teach child to __________
Count during reps
What type of orthotics do children with AMC use?
Lightweight polypropylene orthotics
What is the 1st of LE braces?
HKAFO with pelvic band
Pelvic band encourages?
Neutral rotation and abduction of LES
Pelvic band facilitates ________, can be locked in _____
Full available extension, can be locked in extension
Pelvic band allows a _______ for the trunk to move into _____ to help with _______
posterior weight shift on stance side during gait
In adolescent period what may be needed?
Power WC or scooter
An adolescent with AMC should be able to be efficient and independent in?
Dressing, feeding, and toileting abilities
In adolescence you should maintain _______ through growth spurts
Joint contracture management
In what period does school therapist act as a team member?
Adolescent use what splint?
What is significant in aging?
Adults with AMC may get what symptoms?
Arthritis and over use syndromes ,
Carpal tunnel, neuropathologies from prolonged def.
Adults with AMC will have painful stiff feet from?
What AD is most common in adults with AMC?
Manual/ power WC
In adults what skills do you work on?