ARTHROGRYPOSIS
Arthrogryposis refers to a term used to describe a variety of conditions involving multiple joint contractures (or stiffness). A contracture refers to a condition where the range of motion of a joint is limited — causing it to not fully extend or bend.
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Arthrogryposis
Multiple joint contractures that occur throughout the body during fetal development are the hallmark of the condition known as Arthrogryposis or Arthrogryposis Multiplex Congenital (AMC). When a joint stiffens up and stuck in a bent or straight posture, it develops a contracture, which restricts the joint’s range of motion. Although several causes of joint contracture are known, the reasons why AMC occurs are not fully understood. Others, including genetic effects, are still under investigation. Finally, this web page describes Arthrogryposis, including some of the disorder’s possible origins, symptoms, and treatment. Also, you will have a better understanding of how doctors identify and treat AMC and what to expect during pregnancy.
Is Arthrogryposis Rare?
Arthrogryposis has a birth defect rate that occurs in approximately 1 in 3,000 to 5,000 live births in the United States and affects both men and women of all ethnic backgrounds. Arthrogryposis multiplex congenital (AMC) is the name given to the condition when more than one body component is afflicted. For example, clubfoot is a congenital contracture that affects only the foot. Additionally, this is an isolated congenital contracture, not arthrogryposis. There are over 150 types of AMC, with muscles not developing correctly being the most common, accounting for over 40% of all cases of AMC.
Arthrogryposis Causes
The main cause of arthrogryposis is generally a reduction in fetal movements while in the womb. To develop muscles and joints, the fetus needs to move its limbs. If the joints are immobile, more connective tissue grows around the joint to stabilize it. Among the factors contributing to fewer fetal movements are:
- Central nervous system defects or malfunctions, such as spina bifida, brain anomalies, or spinal muscular atrophy.
- A hereditary neuromuscular condition like multiple sclerosis, myasthenia gravis, or myotonic dystrophy
- Infection in the mother during pregnancy, such as rubella or rubeola (German measles).
- Protracted maternal fever over 102.2 degrees Fahrenheit or an elevation in body temperature brought by prolonged hot tub use in mothers
- Maternal exposure to chemicals like alcohol, narcotics, or the anti-seizure medicine phenytoin can pass through the placenta and damage the developing baby (Dilantin).
- Lack of amniotic fluid or persistent amniotic fluid leakage may make it difficult for the fetus to move about.
QUESTIONS AND ANSWERS
What is Arthrogryposis?
Arthrogryposis, also known as arthrogryposis multiplex congenita (AMC), appears as a rare congenital condition characterized by joint contractures and limited movement in multiple joints. Doctors discover the condition at birth and it can affect different parts of the body, leading to varying degrees of joint stiffness and muscle weakness.
What causes Arthrogryposis?
Doctors do not know the exact cause of arthrogryposis but they know it can result from a combination of genetic and environmental factors. Some cases may link to underlying genetic mutations that affect muscle or nerve development. Other factors, such as reduced fetal movement in the womb, maternal illness, or exposure to certain medications, can also contribute to the condition.
How do doctors treat Arthrogryposis?
Treatment for arthrogryposis focuses on improving joint mobility, muscle strength, and overall function. The approach varies depending on the severity and specific joints affected. Early intervention remains crucial and may involve physical therapy, occupational therapy, and orthopedic interventions. For instance, splints, casts, or surgical procedures are used to release tight joint structures. Assistive devices, adaptive technologies, and mobility aids may help to enhance daily activities. The goal of treatment must maximize the individual’s independence and quality of life by addressing the unique challenges posed by arthrogryposis.
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How is the Body Affected by Arthrogryposis?
Several kinds of arthrogryposis have varying effects on the musculoskeletal system. Scoliosis or curvature of the spine is possible. Upper and/or lower extremities can affect the whole body, including shoulders, elbows, wrists, fingers, hips, knees, and ankles. Sometimes, only the hands and feet appear affected. A condition with several joint contractures may include arthrogryposis (e.g., cervical spine, wrist, hips, and knees).
A baby with arthrogryposis may have clubfeet, congenitally dislocated hips, or vertical tali (rocker-bottom feet). It is possible to hold the upper and lower extremities in particular positions that indicate common patterns found in patients with this issue. Children affected with Arthrogryposis face difficulty in straightening or bending their wrists, hips, or knees. Instead of the usual wrinkles around the joints, these joints may show smooth, tight skin. At the elbow and knee joints, a baby with Arthrogryposis may show skin webbing. Abnormal fibrous and fatty tissue may take the place of healthy skeletal muscle.
Nonsurgical Treatments for Arthrogryposis in Children
Nonsurgical treatment of joint contractures is minimally effective in restoring the range of motion and improving function in large joints such as hips, knees, shoulders, and elbows. Physical and occupational therapy can help improve joint range of motion, mobility, function, and activities of daily living. Also, hand braces and clubfoot casts often help straighten the wrists and feet into a better position. Long-term bracing may become necessary to maintain gains. Improved joint mobility and deformity correction might need some surgical intervention. Successful treatment corrects the deformity, improves movement, and improves normal mechanical alignment. This increases mobility and function.
Surgery on the lower extremities has been shown to enhance function and reduce discomfort. To restore better postures, this can necessitate lengthening of short or tight tissues (tendons and ligaments) or shortening osteotomy, which involves removing tiny wedges from close to the joints. Even after surgery, patients may require ongoing brace use, assistive device use, physical therapy, and occupational therapy to maintain motion acquired and to help with ambulation, mobility, and daily living tasks. Treatment for arthrogryposis in infancy or early childhood can increase muscular strength, flexibility, and range of motion.
Physical and Occupational Therapy
The most popular forms of treatment for infants and young children with arthrogryposis are physical and occupational therapy. Children who have difficulties crawling, walking, climbing stairs, or engaging in sports might benefit from physical therapy. Also, the goal of occupational therapy is to help children get better at doing daily tasks including eating, grooming, and using the restroom. Therefore, these treatments are often the most successful in resolving mobility and strength issues brought on by hyper-extended or flexed knees, hips, elbows, wrists, or hands when initiated early—even in newborns. Additionally, contracture-related abnormalities in the trunk, arms, and legs are treated and prevented through physical and occupational treatments, which also lowers the likelihood that an adult would have discomfort.
To increase range of motion, muscle strength, and flexibility, physical and occupational therapists employ a variety of exercises and specialized techniques. To make your infant more comfortable with rolling over, crawling, and walking, you could also do some other activities. Children who find it difficult to walk or move about might benefit from aids like wheelchairs or walkers. Children who have trouble holding a pen or pencil, feeding themselves, or taking care of themselves might benefit from other gadgets.
Positioning Devices
Splints and casts, which act as positioning aids, can keep joints elongated for a few weeks or more. In order to promote flexibility, increase range of motion, and avoid long-term injury, using a series of splints and casts can also assist your child’s bones to align correctly and relieve tight joints during growth. Orthotics, braces, standing frames, chair inserts, and other positioning aids support your child’s alignment while they are sitting or standing. Physical or occupational therapists or orthopedists typically fit serial casts. The most typical arthrogryposis treatments are as follows:
- Stiff joint-moving casts.
- Exercise that reduces muscle atrophy and enhances joint motion (when the muscles waste away).
- Joint manipulation refers to a sort of therapy that involves gentle movement of the joints.
- Stretching activities improve the range of motion and flexibility.
- Surgery on the wrists, elbows, hips, knees, or ankles can potentially widen the range of motion. By separating the bones from the tissues that prevent them from moving and forcing the muscles to flex and move, the aim is to improve your child’s function. Surgery is uncommon.
Experts advise using a multidisciplinary strategy and doctors use several treatment options for this condition.
The Outlook for Arthrogryposis (AMC)?
The long-term prognosis for those with AMC is influenced by a number of factors, such as:
- The severity of Arthrogryposis.
- The underlying reason for arthritis.
- How your child’s body responds to treatment.
Most people with arthrogryposis mature into self-sufficient adults. Then through treatment, individuals are able to live lifestyles that are comparable to those of those without arthrogryposis.
Does Arthrogryposis Get Worse?
Arthrogryposis is not progressive. Although arthrogryposis will not get worse, underlying conditions can get worse. As pediatric orthopedic doctors and surgeons at Medical City Children’s Orthopedics and Spine Specialists, we are considered experts with children including treating them with Arthrogryposis.
Medical City Children’s Orthopedics and Spine Specialists with offices in Arlington, Dallas, Flower Mound, Frisco, and McKinney, TX exclusively focus on infants, children, and adolescents, providing specialized care tailored to the unique needs of young patients. Their experience and training in pediatric orthopedics ensure that children receive the most appropriate and effective treatments for their specific medical conditions. By choosing Medical City Children’s Orthopedics and Spine Specialists for their child’s health, parents can rest assured that their little ones will receive expert care from a team dedicated to ensuring the best possible outcomes.
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