Arthrogryposis Multiplex Congenita
Multiple Congenital Contractures (MCC), Myodystrophia Foetalis,
Amyoplasia Congenita
AMC is a nonprogressive syndrome characterized by:
- Rigid and deformed joints.
. - Muscle absence or atrophy
. - Cylindrical or ellipsoid joints with skin crease loss and subcutaneous atrophy
. - Contractures of capsules and periarticular structures
. - Dislocation of joints like hip and knee
. - Normal mentality and intact sensation
Causes
1
Intrauterine immobilization of joints at various stages of
development is due to:
Myopathic
Autosomal recessive
Neurogenic causes
due to reduced number or improper organization of anterior horn cells, peripheral nerves & motor end plates, weakness of muscles, etc
Mechanical causes
like breech, twins, oligohydramnios amniotic
bands, etc. which reduce the intrauterine space
2
Classification
3
8 types
upper limb
2
lower limb
6
types
4
neurogenic
myogenic
Clinical Features:
Deformities
Foot
- planovalgus and
- equinovarus.
Knee
- Flexion contracture
- and fixed in extension.
Hip
- extension,
- abduction,
- external rotation
Shoulder
Medial rotation of shoulder
Elbow and wrist
flexed
Investigations
5
Muscle biopsy.
Electromyography
Nerve conduction studies.
Radiograph for scoliosis, dislocation, etc.
Chromosomal studies
6
Treatment
7
Principles of Orthopedic Treatment
- Muscle balance is to be restored if tendons are available for transfers.
- Recurrence is the rule due to tough inelastic capsule and soft tissues
- Tenotomies should be accompanied by capsulotomy and capsulectomy
- Osteotomies are to be carried out once skeletal growth is over, otherwise recurrence occurs.
- Maximum correction is to be obtained during the initial surgery. There is no role of wedging, etc.