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Arthrogryposis Multiplex Congenita (Amc) : Tapan Kumar Paraseth

Arthrogryposis multiplex congenita (AMC) is a condition present at birth characterized by multiple contracted muscles and joints, limiting mobility and range of motion. It has various causes but typically results from lack of fetal movement in the womb. Occupational therapy aims to improve range of motion, motor skills, independence in daily activities, and quality of life through exercises, splinting, adapted equipment and positioning, and engagement in meaningful occupations.

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145 views24 pages

Arthrogryposis Multiplex Congenita (Amc) : Tapan Kumar Paraseth

Arthrogryposis multiplex congenita (AMC) is a condition present at birth characterized by multiple contracted muscles and joints, limiting mobility and range of motion. It has various causes but typically results from lack of fetal movement in the womb. Occupational therapy aims to improve range of motion, motor skills, independence in daily activities, and quality of life through exercises, splinting, adapted equipment and positioning, and engagement in meaningful occupations.

Uploaded by

Tapan Paraseth Studies
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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ARTHROGRYPOSIS MULTIPLEX

CONGENITA (AMC)

Tapan Kumar Paraseth


Introduction
 Arthrogryposis multiplex congenital is a non-
progressive syndrome characterized by poorly
developed and multiple contracted muscles
and deformed joints present at birth.
 It involves contractures of at least two joints in
two different body regions.
 Other terms are amyoplasia congenita and
congenital arthromyodysplasia.
 The muscles involved are partially or
completely replaced by fat and fibrous
tissue.
 The sensory system is not affected.
 Upper limbs usually present internal rotation
of the shoulder, elbow extension, wrist flexion
and ulnar deviation.
 Hyperadducted thumb, and contractures of
the distal interphalangeal joints.
Epidemiology

 1 in 3,000 live births.


 The condition is usually detected at birth or before
by ultrasound examination.
 It is often secondary to other conditions.
 2:1 male to female
 More common in some isolated communities in
Edinburgh, Finland and Israel.
Etiology
 It usually occurs due to absence of active
fetal movements (akinesia), normally
appearing in the 8th week of pregnancy.

 Fetal akinesia lasting over 3 weeks may


be sufficient to result in contracture of
muscles.

 It results in thickened joint and capsules.


The conditions may be classified as below and
broadly under the following headings:

 Disorders characterised mainly by limb


involvement.
 Disorders that involve the limbs and other body
parts.
 Disorders with limb involvement and central nervous
system dysfunction.
 Other associated syndromes and conditions.
• Meningomyelocele
• spinal muscular atrophies
• congenital myopathies
OCCUPATIONAL THERAPY
INTERVENTION
 Assessment
 Range of motion.
 Muscle strength.

 Developmental of gross and fine motor skills.

 Developmental of hand skills, grasp, and prehension

 Balance and postural control

 Self-perception

 Family and social support

 Daily living skills

 Play skills
Problems Identification
 Motor:
 Rigidity and/or contractures of the joints.
 Shoulders are adducted and internally rotated
 Elbows are extended but occasionally flexed
 Forearms are pronated
 Wrists are flexed and ulnary deviated
 Fingers are flexed, and the thumb is in the palm
 Hips may be dislocated and are usually flexed and
externally rotated
 Knees are extended but occasionally flexed
 Feet are in equinovarus.
 Severely limited range of motion in all major
joints.
 Muscle weakness usually occurs.

 The person may have absence or atrophy of


individual muscle or muscle groups.
 The person may have gross abnormalities of the
chest and spine.
 Other problems include webbing on the ventral aspects
of flexed joints, cleft palate, scoliosis, torticollis, facial
palsy, limb deformity, and congenital amputation.
 Achievement of gross and fine motor skills is usually
delayed
 Development of hand functions is usually delayed.

Sensory
 Sensory system is intact, but perceptual skills, such as
stereognosis, could be delayed due to lack of
opportunity.
 Cognitive

 Cognition is normal and intelligence is average to above


average, but learning could be delayed due to lack of
opportunity and many surgical procedures.
 Self-Care
 Development of self-care skills is usually delayed
because the patient's hand functions and fine
motor skills are now develop.
 Productivity
 Play skills are usually underdeveloped.
The occupational goals
 Increase and maintain joint range of motion
 Develop functional sitting and standing posture
 Foster achievement of gross motor competence
 Achieve maximum independence in the skills
required for activities of daily living

Children should be managed


with a multidisciplinary approach, consisting
physician, physical therapist, nurse, orthotist, social
worker, family, and community resources.
These goals to be accomplished by means of
a program which emphasized

 Range of motion exercises.


 Appropriate splinting.
 Adapted seating with trunk and lower-limb
orthoses as required.
 Special equipment for feeding, dressing,
toileting.
 Play and school activities, whenever
necessary.
Approaches

 Biomechanical
 Motor Learning
Treatment/Management
 Motor
 Increase and maintain range of motion through
activities, such as exercise and stretching.
 Promote achievement of gross and fine motor
skills.
 Improve posture and positioning through the use of
adapted equipment, such as corner seats, strollers,
swivel buckets, or parapodiums.
 Provide splints to maintain gains in range of
motion or serial casts to increase range of motion.
 COGNITIVE
 learning and encourage child to explore problem-solving
methods.
 Educate the parents about the disorder and encourage
participation in the therapy program.
 Know importance of having the child wear splints until bone
matures
 Continuing range of motion activities everyday so the child
maintains use his/her body
 Engage the child in daily activities , Play , Selfcare
 Intrapersonal
 Provide opportunities to develop self-perception
through the use of creative activities, such as art, crafts,
drama, dance, music, and games.
 Encourage parents to express feelings and concerns
about the child's condition and welfare.
 Provide instructions in stress-management techniques.
 Sensory
 Provide opportunities to experience sensory input
through playful activities – perceptual ,
propripceptive, kinesthetic etc
 Interpersonal
 Encourage parent-infant bonding.
 Encourage parents to participate in self-help
groups.
 Self-Care
 Provide self-help devices to assist in the performance of
daily living skills.
 Adapted equipment may be necessary for grooming,
such as extended handles on combs, brushes and
toothbrushes.
 Clothing adaptation may be needed to account for
various deformities, using Velcro and elastic. Providing
large rings on zippers or loops to grasp and large
button is also useful.
 Productivity
 Promote development of play skills, especially
exploratory and manipulative play.
 Assist teachers in determining what, if any,
adapted equipment or devices may be needed to
assist a child with academic activities. Computers
with adapted keyboards may be helpful. the child
should be able to attend regular classroom
instruction, except for physical education.
 Leisure
 Usually no special goals or objectives are
necessary. The child or an adult will seek those
activities that are within the child's functional
abilities.
 Precautions
 Bone structure is fragile. Range of motion and
stretching exercises should be carefully monitored.
 Prognosis/Outcome
 The person has maximum range of motion possible given
structural limitations.
 The person achieves gross and fine motor skills, although
the development of skills does not progress normally.
 The person has functional hand skills, although the use of
the hands may be unorthodox.
 The person has independently mobility with or without
powered mobility.
 The person achieves independence in self-care and daily
living skills.
 The person develops productive skills.

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