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.