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Arthrogryposis Multiplex Congenita - Coggle Diagram
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
1
Causes
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
Muscle biopsy.
Electromyography
Nerve conduction studies.
Radiograph for scoliosis, dislocation, etc.
Chromosomal studies
6
5
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.