Arthrogryposis

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 is typical. 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. 

Arthrogryposis does not get worse over time. For most children, treatment can lead to big improvements in how they can move and what they can do. 

Most children with arthrogryposis have typical thinking and language skills. Most have a normal life span. Most lead independent, fulfilling lives as adults. However, some need lifelong help with daily activities. Some walk, and others use a wheelchair.

The main cause of arthrogryposis is fetal akinesia. This means the baby does not move around inside the womb as much as is typical. Starting in early pregnancy, moving helps a baby’s joints, muscles and tendons develop. If a baby doesn’t move much, these parts may not develop well. Extra tissue may form in the joints, making movement harder. 

There are many reasons why fetal akinesia might happen, including: 

• Nerve signals don’t reach the baby’s muscles because of problems with the baby’s central nervous system (CNS). 
• There isn’t enough room inside the womb for the baby to move. This may happen if the womb is not the typical shape or if amniotic fluid leaks out of the womb (oligohydramnios). 
• The baby’s muscles don’t form in the typical way and are weak, or their tendons, bones or joints don’t form typically. 

Fetal akinesia usually has nothing to do with what the parents did or did not do before or during pregnancy. 

About 1 baby in 3,000 is born with arthrogryposis. 

Most families who have a child with arthrogryposis are not at greater risk for having another child with it. In about 1 in 3 children with this condition, doctors do find a genetic cause. The families of these children may be at greater risk. Your child’s doctor can explain what this means for your family. 

To get more information, your child may need 1 or more tests, such as these: 

• X-ray to check their bones and joints
• Ultrasound to check their central nervous system (CNS) and organs 
• CT (computed tomography) scan to check their bones
• MRI (magnetic resonance imaging) scan to check their CNS or muscles
• Muscle biopsy to see if their muscle fibers are typical
• Skin biopsy or blood tests to check their chromosomes for anything that is not typical 
• Electromyogram and nerve conduction velocity (EMG/NCV) studies to tell if a problem with their nervous system or muscles is the cause and to tell how quickly signals travel along their nerves  

Genetic tests can tell your doctor more about some forms of arthrogryposis, so your child’s team may suggest these tests, too.

Sometimes doctors can diagnose arthrogryposis based on what they see during an ultrasound before a baby is born (prenatally). If this happens, talk with your healthcare team about what they can tell from the ultrasound and what it may mean for you and your baby. Our team at Seattle Children’s is here to provide prenatal consultations to talk with you about your baby’s condition.

Physical therapists (PTs) use stretches and exercises to increase your child’s range of motion and muscle strength so they can move better. 

PTs also work with your child to improve their gross motor skills. This includes finding ways for your child to roll, sit, crawl, stand, walk and play. 

If a wheelchair, walker or other device might help your child be more mobile, PTs suggest these devices and teach your child how to use them. 

Occupational therapists (OTs) teach your child skills to take care of themselves (such as how to eat and how to get dressed) and ways to move around (such as how to get out of a chair and how to use crutches). 

They also work with your child to develop fine motor skills, such as writing. 

Many tools (adaptive devices) may help your child do things on their own. OTs suggest these tools and teach your child how to use them. OTs also make customized splints and adaptive devices. 

Splints and casts are rigid supports that go around a joint to hold it in place. PTs and OTs use splints and casts as part of therapy. These supports can help line up your child’s bones so your child can move better. Splints and casts also help keep joints stretched, and they can improve or prevent contractures. 

Your child may need different splints or casts at different times. Some splints are worn only at night. Often children go through a series of splints or casts that are changed as their range of motion changes. Your child’s team will teach you how to work with splints and casts during daily activities, such as doing your child’s therapy exercises at home or getting your child dressed. 

Our OTs make splints and casts for the arms and hands. Our orthotists make them for the legs and feet.

Arthrogryposis affects each child in different ways, so your child may need other types of care, too. For example, some children need speech therapy. This could help them learn to speak more clearly if their muscles used for speech are weak or if the feeling inside their mouth is not typical. Some children need a gastrostomy tube or a tracheotomy if jaw, mouth or throat problems make it hard for them to eat or breathe. Some families work with a dietitian to make sure their child gets good nutrition. Your child’s team will put together a complete plan based on your child’s needs.

• The arthrogryposis team includes doctors, surgeons, physician assistants, nurse practitioners, therapists, dietitians and other experts from Rehabilitation Medicine, Orthopedics, Genetics, , Physical Therapy, Nutrition, Social Work and .
• We can successfully treat arthrogryposis in many children with physical and occupational therapy, sometimes along with splints and casts. We have the largest team of physical therapists and occupational therapists in the Pacific Northwest who specialize in the care of babies, children, teens and young adults. We also have onsite orthotists to build and fit splints and casts for our young patients.
• If your child needs surgery, our pediatric orthopedic surgeons know how to plan the order of their surgeries to get the best results for your child. Many of the surgeons have expanded fellowship training in areas such as foot and ankle conditions, hip problems, upper extremity surgery, limb deformity, neuromuscular diseases and spine problems.
• The doctors at Seattle Children’s who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to give anesthesia to children safely. They know how to handle special issues, such as how to intubate a child who has problems with their jaw or airway.
• Arthrogryposis and the conditions it may be linked with can affect more than a child’s muscles and joints. That’s why we connect you and your family with any expert your child may need across Seattle Children’s. For example, specialists in metabolic bone diseases may be involved in your child’s care.

• For some families, our work begins even before their baby is born. If a prenatal (before birth) ultrasound suggests that your developing baby may have arthrogryposis, we work with Seattle Children’s Fetal Care and Treatment Center. Together, our experts provide diagnostic and counseling services to you and your family. 
• We specialize in caring for kids. This means our experts have the knowledge, training and skills to treat the youngest patients through young adults. We have treated many children of all ages with arthrogryposis. At Seattle Children’s, your child’s team has special training in the medical, surgical, emotional and social needs of young people. 
• Babies, children and teens are still developing, so they may need different care than adults do, like treatment that takes their growth plates into account. When we evaluate your child’s condition, design their treatment and provide their care, we carefully consider their growth. We think about how growth may affect your child’s joints and movement over time. We also consider how their arthrogryposis may affect the rest of their development and health. 
• As your child grows, we adapt their treatment to meet their changing needs. Along the way, we consider all aspects of what your child and family need to thrive — from learning practical skills for daily life to coping with feelings.  
• If your child needs imaging that uses radiation, we use the lowest amount possible (PDF) to make the best image. We have a low-dose radiation X-ray machine, called the EOS. It makes safer full-body images. We also have the largest group of  board-certified pediatric radiologists in the Northwest.

• The team at Seattle Children’s helps connect you with other families of children with arthrogryposis if you want. We also have a yearly arthrogryposis get-together for families.
• We know it can be stressful to have a child with arthrogryposis and to find the treatment they need. Everyone at Seattle Children’s works to make your experience here as easy as we can on your whole family.
• Your child’s team does more than plan and provide care for your child. We also make sure you and your child understand your child’s condition and treatment options.
• Seattle Children’s supports your family with a range of resources. Our Child Life specialists, Family Resource Center and Guest Services are here to help

• Seattle Children’s takes part in the CoULD Registry for congenital upper limb differences. A registry is a place where researchers keep information about people who have a certain condition so they can learn more about it. CoULD connects researchers from children’s hospitals around the country to study treatments and improve life for kids born with arms and hands that aren’t typical.  
• Learn more about current orthopedics research at Seattle Children’s.