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.