Arthrogryposis Multiplex Congenita: Diagnosis and treatment - Children Orthopaedic Care Institute Pravira Hospital - Orthopaedic Hospitals - Book Appointment Online - Orthopaedic Hospitals in Wardha Road, Nagpur

Arthrogryposis Multiplex Congenita

Arthrogryposis is a term used for a various of conditions that have common diminished fetal movement with congenital (by birth) joint stiffness and varying degrees of muscle weakness. The most common type of arthrogryposis is termed amyoplasia or classical arthrogryposis.Distal arthrogryposis is characterised by restriction of motion of the distal joints of the hands, feet & sometime the knees and legs. Classic arthrogryposis or amyoplasis,Present with Striking musculoskeletal abnormalities. The most frequent posture is that of elbow extension wrist flexion and ulnar deviation, knee extension or flexion and equinovarus foot deformities. The arms and legs are thin & atrophic and the joint lack flexion creases.

About 1 baby in 3,000 is born with arthrogryposis. Most contractures happen in the arms and the legs. They can also happen in the jaw and the spine.

Arthrogryposis in Children

Each child with arthrogryposis is different. In some children, the condition is mild. It affects only a few joints, and these joints have almost as much movement as normal. In other children, the condition is more serious. It affects more joints and restricts their movement more. In extreme cases, arthrogryposis affects nearly every joint.

Type Of Arthrogryposis Multiplex Congenita

The HIP

The dislocation of Hip & Hip abduction & adduction contractures are occasionally encountered and the resulted pelvic obliquity may contribute to development of scoliosis.

The Knees

There considerable variation in knee deformities at birth. Flexion contracture are most frequent but extension contracture, hyper extension and varus & valgus deformity are also encountered.

The Feet

The most frequent deformity is a rigid equinovarus deformity which is more severe and rigid than an idiopathic clubfoot. This needs an urgent treatment in form of Ponseti technique after birth. May need major surgeries once child s is 1-2 year old.Virtually any other deformity may present Vertical Talus,Equinovalgus and cavus being the most common.

The Shoulder

The Shoulder is often contracted into adduction & internal rotation by joint incongruity and soft tissues contractures typically there is little active flexion abduction of the shoulder.

The Elbow

The Elbow deformity is usually one of the full extension without active flexion but with same active triceps function retained. If elbow flexion is limited to 45 degree or less a triceps-plasty is indicated to gain functional range of motion.

The Wrist

The wrist in arthrogryposis is usually flexed and devited ulnarward and a small range of motion is available.Early stretching & splinting has been recommended but the efficacy is uncertain.Surgical treatment is indicated to reorient the wrist into a neutral position while maintaining any available motion.

The Hand

Flexion contractures of the fingers considerable limit of function of the hand.Passive stretching and splinting rarely add significant range of motion.With current methods of surgery many non ambulatory children can become ambulatory.

Treatment

Achievement

The pelvic harness is the preferred method of treating neonatal DDH. Use of the harness is most effective where there is strong support system to educate the parents and to closely monitor neonate’s progress.

If the hip remains dislocated after 3-4 weeks of harness wear, use of harness should be discontinued and the hip should be managed with either closed or open reduction.

The child who presents with dislocated hip between ages 6 months to 2 years & the child in whom initial splintage has failed are managed in same manner.The goals of the treatment are to obtain & maintain reduction of the hip without damaging the femoral head.

The two principles methods of treatment are closed reduction & open reduction.Treatment of children between 2 & 6 years of age with hip dislocation is more challenging.The femoral head is usually in more proximal location in the older child, and the muscles that cross the hip are more severely contracted.

Femoral shortening is an essential part of management of the older child, and with higher dislocation, greater shortening is necessary. The open reduction from anterior approach is suggested. The acetabular dysplastic part is corrected with acetabuloplasty.

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