Arthrogryposis Multiplex Congenita (AMC)-

Signs, Causes & Treatment

What is Arthrogryposis Multiplex Congenita?

It is a condition in which a newborn child is affected with multiple joint contractures, stiffness & muscular weakness in the body. It is a non-progressive condition. This problem occurs because of many reasons. This is a rare disorder, occurs in 1 out of 3000 live births. These children will have normal speech & normal intelligence.

How to Diagnosis the AMC? 

Diagnosis of Arthrogryposis Multiplex Congenita is made by clinical examination by a pediatric orthopedic surgeon or pediatrician after birth. Before delivery, ultrasound can identify the limb deformity, but it is complicated to confirm the diagnosis & prognosis before delivery. Genetic analysis can help only in identifying the cause of this problem.

What are the Causes of AMC? 

There is no single cause of this complex problem. One essential factor which causes this problem is fetal akinesia, in which fetal movement in the mother’s uterus decreases. If it persists for a long duration, then joint stiffness and muscle contracture develop. Most of the time, causes are not clear, but in 30% of cases, you can find sporadic genetic causes. But the familial transmission is rare.

Extrinsic causes: Insufficient space in the uterus due to low amniotic fluid, abnormal uterus structure or trauma, etc

Intrinsic: Neurological causes (malformed brain/ spinal cord), connective tissue disorder(tendon, ligament, bone, joint structure, etc.)

Arthrogryposis Multiplex Congenita Sign & Symptoms?

The newborn child will present with signs & symptoms of multiple joint & limb deformity, contracture. A child can have a combination of foot deformities like club foot/ vertical talus, knee deformity like congenital dislocation of knee/ flexion deformity, and hip dislocation lower limb. In the upper limb, the child can have elbow deformity, wrist, hand, and finger deformity, etc. in severe cases child can have spinal deformity and facial deformity.

How AMC is treated?

Most arthrogryposis congenital children will have normal speech and a good IQ. There is no absolute cure for this problem. The different methodologies of treatment can improve the quality of life. Prognosis depends upon the severity of the problem and area of involvement. Treatment modalities include-

Physical & occupational therapy:

With the help of physical and occupational therapy joint mobility, power and balance can be improved. But it may be required for a longer duration. The child may need some orthopedic intervention intermittently.

Orthopedic intervention:

Most of the deformities and contractures in AMC are very rigid and stiff, so they need repeated manipulation plaster or tapping. Several plasters are more compared to the ordinary course in a similar type of problem. Some joint deformity which is very rigid they will require surgery. A situations like congenital dislocation of the knee, the congenital hip dislocation will require surgery. Club foot require manipulation and plaster application. Careful planning and execution of proper surgery is a must. After surgery, a brace and therapy for a longer duration are required. All this planning should be done by a pediatric-orthopedic surgeon, who has sufficient experience in treating this type of problem.

 

Leg Deformities X-Ray - 1
Limb Deformities X-Ray - 2

Frequently Asked Questions

How does AMC developed?

AMC involves a variety of non-progressive conditions characterized by multiple joint contractures (stiffness) and involves muscle weakness found throughout the body at birth.

Are there different types of AMC?

Common types of  AMC are Amyoplasia, Distal Arthrogryposis & Syndromic Arthrogryposis.

Will my AMC child be able to live independently as an adult?

Many people with AMC despite their physical limitations, will have a positive life experience and learn to live independently. Some people may need to live with assistance even in their adult life.

MEDICALLY REVIEWED AND EDITED BY DR. JITENDRA KUMAR JAIN
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Dr. Jitendra Kumar Jain is a famous name in the field of childhood physical disability pediatric orthopedic problems in North India. He has been an MS Orthopedics, DNB Orthopedics, Pediatric orthopedic surgeon & also a cerebral palsy specialist for over 21 years.

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