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Sunday, February 13, 2011

ARTHROGRYPOSIS MULTIPLEX CONGENITA

Arthrogryposis multiplex congenita is a non-progressive disorder of congenital origin; characterised by marked stiffness and contracture of joints affecting the limbs and trunk.

Classification
There are two distinct types:
a.The neurogenic type, which is more common
b. The myogenic type.
The neurogenic type is due to degeneration of anterior horn cell in certain segments of the spinal cord, and intrauterine paralysis of the corresponding muscles.
The myogenic type is due to myodystrophy in the intrauterine state with replacement of muscles by fibrofatty tissue.

Clinical manifestation
In the myogenic type the legs are in flexion, abduction at the hips and flexion at the knees. The feet may be in gross equinovarus deformity. The hips often show congenital dislocation. Intelligence is normal and there is no sensory deficit. Contractures are produced from non-progressive muscular dystrophy.

In the neurogenic type, joint contractures develop from muscle imbalance in early intrauterine life. The hips are in flexion and the knees in hyperextension (genu recurvatum) with the feet in calcaneovalgus. In upper limb the elbow may be in extension, or in flexion with skin webs, pronation of forearm and flexion at the wrist.

Treatment
Deformities in arthrogryposis are difficult to correct; surgical correction of the deformities must be followed by splinting and exercise therapy. For lower limb deformities, club foot is corrected by talectomy, genu recurvatum by quadriceps plasty and open reduction with Kirschner's wire fixation, hip treated with open reduction, and scoliosis with braces and plasters.

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