Abstract
Arthrogryposis multiplex congenita is a disease involving multiple joint contractures with deficient musculature. Arthrogryposis is a rare disorder that occurs in about 1 in 3000 to 1 in 10000 live births.
Congenital contractures are divided into three groups according to associated system anomalies. The most common type of arthrogryposis (group 1) is termed amyoplasia or classic arthrogryposis.
Distal arthrogryposis is characterized by restriction of motion of the distal joints of the hand, feet and sometimes the knees and has been classified into six types. Autosomal dominant transmission has been noted especially in type I distal arthrogryposis.
Amyoplasia differs from spinal degenerative diseases by 3 symptoms: 1. it does not deteriorate after the birth, 2 sensation is not affected, 3 intelligence is normal. The severity of the neurogenic or myogenic disability is determined by electromyography examination.
In most patients with arthrogryposis the two major goals of treatment are independent ambulation and independent function of the upper extremities for activities of daily living. The surgical treatment of contractures of the lower extremities is indicated in the first year of life - reduction of dislocated hips, flexion contractures of the knee, hyperextension and dislocation of the knee, rigid equinovarus deformity or talus verticalis.
The surgical treatment of contractures of the upper extremities is indicated around four years of life - the first goal is to achieve passive elbow flexion. Procedures to achieve active elbow flexion - muscles transfer may be indicated.
The result of the therapy depends on the severity congenital contractures, timeliness and comprehensiveness therapy, which involved an orthopaedic surgeon, a physical therapist. In the presence of other associated congenital defects also by other specialists.
Adequate