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Arthrogryposis Multiplex Congenita: Enhancing
Function through Physical Therapy
Maureen Donohoe PT, DPT, PCS
Arthrogryposis Multiplex Congenita
Arthrogryposis: a review and update. Bamshad M, Van Heest AE, Pleasure D.
Bone Joint Surg Am. 2009 July 1; 91(Suppl 4): 40–46.
Arthrogryposis: Infancy
 Body Types
 Predicting the future
 Early management of contractures
 Emphasis of therapy when the child is casted
Arthrogryposis: Infancy
Body Types
Arthrogryposis: Infancy
Predicting the future
Upper body strength
+/- upper body strength
+/- for upper body strength
+/- for upper body strength
Good upper body strength
Poor upper body strength
Poor upper body strength
trunk
Good alignment
Good trunk alignment
Scoliosis/ hyperlordosis
Good trunk alignment
Good trunk alignment
Scoliosis/ hyperlordosis
gluteals
Greater than 3/5
Greater than 3/5
Less than 3/5 strength
Less than 3/5 strength
Less than 3/5 strength
Less than 3/5 strength
quardiceps
Greater than 3/5
Less than 3/ 5 strength
Greater than 3/5
Less than 3/ 5 strength
Less than 3/ 5 strength
Less than 3/ 5 strength
Foot anomalies
+/-
+
+/-
+
Community
ambulatory with
or without
AFO‟s
Household to
limited
community
ambulation with
KAFO‟s.
Bracing above
the waist and
use of an
assistive device
for household
ambulation.
Initially bracing
above the waist
but work to
KAFO‟s
Transfers with
KAFO‟s
Limited
community
ambulation with
assistive device
Power mobility
for long
distances
Stand pivot
transfers with
assistance
+
Initially bracing
above the waist
but work to
KAFO‟s
Transfers with
KAFO‟s
Exercise
ambulator
+
Bracing to the
chest.
Transfer skills.
Power mobility
for function.
Exercise
ambulator
Arthrogryposis: Infancy
Early Management
3-4 month catch up window after fetal
crowding
Hall, 2009
Ponseti Method‟s Impact on
Arthrogrypotic clubfeet
Comparison of Management
Surgical Management
 Splinting for 8-20 months
 Posterior Medial Lateral
Release at 20-24 months of age.
 Second surgery if necessary
around 4-5 years of age, often
including osteotomies.
 Third surgery at end of growth
to fuse the foot in the best
position.
Minimal Surgical Management
 Casting for weeks
 Percutaneous tendoachilles
lengthening then casting for 4
weeks.
 Brace wear 23 hours a day for
weeks (abduction brace is
preferred)
 Relapse is expected through the
5th year and families should be
prepared for annual casting
episodes.
 Around age 4, anterior tibialis
transfer.
Comparison of Management
Surgical Management
Minimal Surgical Management
 Small feet
 Relatively normal sized feet
 Stiff feet
 Supple feet
 Painful feet
 Less report of painful feet
 Less time in casts
 Expected long periods of
casting
 Less time off of feet
 Fairly predictable
 Impacts on mobility
 Needs strong family
commitment
Arthrogryposis: Infancy
Emphasis of therapy when the baby is in casts
 Head control in a wide variety of positions
 Antigravity trunk control
 Positioning with hips in extension
 Sitting skills
 Upper body skills for play
Arthrogryposis: Young Children
 Standing balance: tool for a lifetime
 Bracing and balancing options
 Supports for ambulation
 Alternative mobility options
Arthrogryposis: Young Children
Standing balance: tool for a lifetime
Walking is icing on the cake but there
are plenty of cakes that are great
without the icing.
 The ability to stand without upper extremity support will
give a person much greater quality of life than the ability to
walk with support.
 Think long term function for transfer and clothing
management.
Arthrogryposis: Young Children
Bracing and balancing options
Biomechanical Approach
 AMC
– If the brace can not stand in the shoes, the child
can not stand in the brace and shoes.
– Equalize leg length
– Straighter is always better
Arthrogryposis: Adolescents and
Young Adults
 Activity expectations
 Changes in the body
 Use of assistive devices
 Skills needed for lifetime independence
Arthrogryposis: Adolescents and
Young Adults: Activity expectations
 Need to work on increasing independence for self care
 Increased social circle opportunities
 Longer distances need to be travelled
 Longer days
– Brace wear
– Sitting
Arthrogryposis: Adolescents and Young Adults:
Changes in the body
 Last growth spurt
– Changes with joint contractures
– Longer lever arms with the same amount of strength to
support those lever arms
 Increased body weight
 Increased self awareness
 Changes in mindset towards surgical intervention
Adolescents and Young Adults:
Use of assistive devices
 Many use a variety of options
 Power mobility is used for long distances.
 Those braced above the waist abandon walking.
 If possible work to get out of braces in time for a driver‟s
license.
 Some shun wheelchairs or motorized scooters, even if it is
easier and energy efficient.
Arthrogryposis: Adolescents and Young Adults
Skills needed for lifetime independence
.
Knee Flexion Contractures
 Yang , et al., 2010 . Ambulation gains after knee surgery in
children with arthrogryposis.
 Palocaren, et al, 2010. Anterior distal femoral stapling for
correcting knee flexion contracture in children with
arthrogryposis--preliminary results.
Important to consider quadriceps strength, amount of
contracture, and arc of motion before deciding most
appropriate surgical intervention.
Knee Extension Contractures
 Often have more mediolateral instability than flexion
extension
 Always have more quadriceps than hamstrings
 Often have bilateral hip dislocation too
 Excellent ambulation potential with minimal bracing
Borowski A, Grissom L, Littleton AG, Donohoe M, King M, Kumar SJ.
Diagnostic imaging of the knee in children with arthrogryposis and knee
extension or hyperextension contracture. J Pediatr Orthop. 2008, 28:466-70.
Arthrogryposis: Hip
 Flexion Contractures
 Rotational Deformities
 Dislocated Hips
 Extension Contractures
Arthrogryposis: Elbows
 Flexion
 Extension
Better alignment for facial
care and feeding
Better alignment for lower
body care and dressing
No good strategies for lower
body care
When elbow range is
available, external devices
are helpful
May need extension
osteotomy to improve
dressing opportunities.
Often coupled with a weak
hand.
Capsulotomies
Muscle transfers
What direction should
physical therapy support
for the person with AMC?
 Functional Mobility for Lifetime Independence
Orchestrate care/ Self advocacy
Transfer skills
 Skills for lifelong cardiac fitness
 Education
Therapy issues related to medically
based services based services
 Episodic care
 Equipment procurement
 Biomechanical approach
 Family education
Episodic Care
•Outpatient PT is the means to the next skill and the
extracurricular activity.
•It should not be the extracurricular activity.
•PT‟s give the child and the families the tools to
move to the next skill.
Equipment: Standing
Equipment: Walking
Parapoduim
Gait trainer
Shopping cart
Walkers
Crutches
Adapted canes
PVC pipe
Pillow case
Arthrogryposis: Young Children
Alternative Mobility Options
Equipment: Wheelchair
Equipment: ADL
Therapy issues related to educationally
based services
 Testing tools
 Functional skills for lifetime educational process with the
least amount of external support
 Opportunities for life long exercise and cardiovascular
fitness.
 Skills change with growth, changes in medical
management, and equipment changes.
Testing Tools
 Birth to 3
–
–
–
–
PDMS 2nd Ed
Bayley Scales
Carolina Curriculum
HELP 0-3
 Preschool
– PDMS 2nd Ed
– Carolina
Curriculum
– HELP 3-6
 School Age
– PDMS 2nd Ed
– School Function
Assessment (SFA)
– PEDI
•Hopping and jumping are not really options
•Basically the test that the agency requires to
qualify for service.
Testing for educationally based service
and planning for recommended placement
 Be prepared to have the
child not perform.
 Jumping hopping, and ball
skills are nice but not
necessary.
 Allow families to be active
participants.
 Establish realistic goals for
the team.
 Even if the school the child
is attending does not have
stairs, there needs to be a
plan for stairs.
 Every child should be able to
do 3 things independently on
the playground.
 Do not rely on the classroom
aide always being in place.
 Think safety first.
Functional skills for lifetime educational
process with least amount of external support
 These are bright kids who have excellent potential to go to college.
 Our job is to support the child in the gross motor skills necessary to
succeed in the educational process.
 If we really want to make sure “our kids” are successful in
education, they should have the gross motor skills necessary to go
to college.
 Just because you are smart enough to go to college, it does not
mean you have the financial opportunity to employ a personal care
assistant to allow that to happen.
Functional skills for lifetime educational process with
least amount of external support
 Book and assistive technology management.
 Classroom ergonomics and materials management.
 Independence in the cafeteria (including appropriate self advocacy).
 Independence in bus transportation including parking lot safety
skills.
 Transfer skills for personal care.
Functional skills for lifetime educational process
with least amount of external support
 Management of clothing appropriate for weather.
 Ability to access education even if the elevator is not
functioning.
 Knowledge of and opportunity to participate in a
variety of exercise opportunities both individual and
group activities.
 Ability to get off the floor if necessary.
 Basic wheelchair problem solving.
Opportunities for life long exercise and
cardiovascular fitness. Let‟s talk about sports
 Not just a section of the newspaper or the TV news.
 Physical activity improves circulation and cardiovascular fitness, and allows for
a sense of competition with others and oneself.
 Sports are a way to be involved in enjoyable activities without even thinking
about the secondary benefits gained through participation.
 Physical and occupational therapies are not the only safe contexts to work on
physical activity.
 Formal therapy can often be complemented or replaced by more pleasurable
avocational activities which provide similar physical benefits while allowing for
lifetime participation in an interesting sport.
A to Z of Adaptive Sports
 There are plenty of other options out there but this will give
you a good start
 Websites will help give you direction.
 Guidelines to participation
– Cost
– Accessibility
– Assistance
Adaptive Skiing





Growing area of winter sports
Paralympic sport
Can be performed from standing or sitting position
Can be done with and without support personnel.
For more information on adaptive ski equipment check
disabled sports USA adaptive equipment at
www.dsusafw.org
Aerobic Exercise Classes
 Low impact aerobics
 Aqua aerobics
 Pilates
 Yoga
 Kick boxing
 Video exercise programs
 Wii
 X-box 360
Archery
 Wide range of types
– Basic bow and arrow to high tech cross bow systems
– Paralympic sport
 The site set up by Buckmasters American Deer
Foundation at
www.badf.org/disabled_hunters/adaptiveequip.html has
an extensive array of possible adaptations available to
allow cross bow use to universally accessible
Basketball
 Can be as simple as shooting hoops in the
living room at a small hoop
 Wheelchair basketball
www.nwbba.org
Boating
 Wide range of activities
 Allows for a wide range of abilities
– Canoeing
– Kayaking
– Rowing
– Adaptive rowing
– World Rowing has more information available on adaptive
rowing and can be found at http://www.worldrowing.com, then
search the site with keyword: adaptive rowing.
Boating
– Motor boating
Types of motor operated boats
Considerations
– Sailing
Types of sail boats
Considerations
– For more information on adaptive sailing check into US
Sailing at http://www.ussailing.org
Bowling
 Traditional bowling
– No adaptations
– Bumper Bowling
 Ramp Bowling
 American Wheelchair Bowling Association
http://www.AWBA.org
Baseball
 Challenger Baseball or Miracle League Baseball
 "buddies" are assigned to help their challenged partners around the
base paths, if necessary.
 Often “buddies” are typically developing children who enjoy baseball.
 To find a Miracle League Baseball team, http://www.miracleleague.com
 To find a Challenger Baseball team, contact the local little league or
check out the Little League website at www.littleleague.org look under
“programs”
Cheerleading
 Keep and open mind when recommending
this activity.
 Works on extension
 Works on deep breathing and breath
control
 Does not necessarily need a high level of
gross motor skill if the team is open to
diversity
Cycling
 Wide variety of types of cycles
 Bicycle
 Tricycle
 Recumbent Bicycle/Tricycle
 Hand Tricycle
 Step N Go Cycle
 Stationary Cycles
Dancing
 Can be as formal and as informal as you want
 Ballet
 Ballroom dancing
 Adaptive dancing with wheelchairs
– Able bodied and disabled
– Totally disabled
 The adaptive dancing website at
http://www.adaptivedancing.com has more information on
wheelchair dancing with an able bodied partner.
Fishing
 Can be as simple as dropping a line in a pond or
as extravagant as a big ocean fishing trip with
high tech gear.
 Risk and amount of adaptation involved depends
on the type of fishing.
 More information on adaptations for fishing can
be found at Fishing has no Boundaries, inc. at
www.fhnbinc.org
Golf
 Types Available
–
–
–
–
Miniature golf
Driving range
Putting greens
Full 18 hole golf course
 Considerations to improve accessibility
– Consider the athlete’s strengths and weaknesses
– Golf cart
– Caddy
 US Adaptive Golf Association http://www.usagas.org/default.asp
Hockey
 Floor Hockey can be easily adapted for a gym class
 Power Hockey : Philadelphia Power Play
http://www.philadelphiapowerplay.com
 Sled hockey or Sledge Hockey is a fast growing paralympic sport.
 US Sled Hockey Association at www.sledhockey.org
 Atlantic District US Sled Hockey Association
http://www.sledhockey.net
Horseback Riding
 Hippotherapy
 Therapeutic Riding
 Recreational Riding
 Competitive Riding
Martial Arts
 Non contact martial arts work on balance, breathing, coordination,
flexibility, strength, and self control.
 Karate, Tae Kwon-do, Kung-fu, and Kempo are additional examples of
martial arts that use punching, blocking, and kicking, to defend and
attack .
 Tai Chi, a Chinese martial art that focuses on controlled movement.
 It is helpful to screen facilities when looking for a class, possibly
watching a class in action or participating, before signing up for an
inappropriate class. Avoid facilities that require patrons to buy into
lengthy contracts .
Quad Rugby
 Murder ball
 Must have 3 limb involvement
 Equipment
– Chairs
– Balls
– Gloves
 http://www.quadrugby.com
Racket Sports
 Involves many different types of sports
– Squash and Racket ball tend to have limited adapted options.
– Table tennis is a paraylmpic sport.
– Tennis and Badminton easily adapt to those with special needs.
 Wheelchair tennis is played virtually the same as able bodied
tennis. The exception is that the wheelchair player is allowed
two bounces instead of one bounce in which to hit the ball over
the net.
Soccer
 TOPS Soccer: The Outreach Program for Soccer
 Youth Soccer Program
http://www.usyouthsoccer.org/programs/topsoccer/index_E.html
Swimming
 Can be a life long avocational activity for those with or
without physical limitations.
 Paralympic sport
 Even scuba diving is able to be adapted to those with
alternative needs
Wheelchair Sports
 Wheelchair athletics emphasize strength, endurance, and
coordination. These activities are individual sports but
they can be dedicated to a team.
Wheelchair Races
Short distance
Long distance
Throwing Events
Shot put
Javelin
Discus
Resources for general information
 Disabled Sports USA http://www.dsusa.org offers nationwide
rehabilitation sports to those with a permanent disability.
 Sports „N Spokes http://www.sportsnspokes.com A magazine dedicated
to wheelchair sports.
 Paralyzed Veterans of America, Sports and Recreation Program
http://www.pva.org Resource designed specifically for veterans but has
links to adaptive sports opportunities that are open to all.
Resources for general information
 The American Alliance for Health Physical Education, Recreation, and Dance
(AAHPERD) http://www.aahperd.org Resource for parents who need help in
defining physical education and adaptive physical education when
advocating for their children.
 The National Center on Physical Activity and Disability http://www.ncpad.org
Website that is divided into disability, sport, and equipment. There are many
links to help the athlete learn more about specific sports of interest.
 The National Sports Center for the Disabled http://www.nscd.org has a site
that details winter and summer activities.
Physical Limitations do not mean Disability
 Adaptive Sports and recreation programs are in abundance
 Some times advocates are needed to help improve
accessibility
 Physical Education‟s responsibility is to educate children on
life time physical activity options, no matter what their
limitations are.
Life Long Activity Expectations
 Function
 Cardiovascular Fitness
 Weight Management
 Healthy Choices
–
–
–
–
Don’t smoke
Get vitamin D and calcium
Be sun smart
Limit alcohol
 Energy Conservation
– Prevent long term wear and tear on the joints
 Avocational Activity
Family Resources
 AMCSupport.org
 TAG : The Arthrogryposis Group
 Adaptive Clothing: http://www.disability-resource.com/clothing.html
 Office of Vocational Rehabilitation
 Yahoo Groups
 Facebook
Questions
Thank-You