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Radioulnar synostosis

  • Overview

    Combining training in adult and pediatric orthopedics, hand surgery, plastic surgery and microsurgery allows our surgeons to provide a comprehensive level of care unmatched in most other hospital settings

    Donald S. Bae, MD, associate in Orthopedic Surgery, Boston Children's Hospital

    If your baby was born with radioulnar synostosis, we know that you and your family are concerned. So, please know that at Boston Children’s Hospital, we will approach your child’s treatment and care with sensitivity and support—for your child and your whole family.

    Radioulnar synostosis is usually congenital (something your child was born with); but it may also occur after a forearm fracture or trauma. Please note that these pages discuss the congenital form of the condition.

    • If your child has radioulnar synostosis, it means that she has an abnormal bony or soft tissue connection between the two bones of her forearm—the radius (forearm bone on the (thumb) side) and ulna (forearm bone on the (little finger) side).
       
    • “Synostosis” means a union or fusion of bones that are usually distinct from each other—in this case, the radius and ulna near the elbow.
       
    • Signs and symptoms of your child’s condition can range from a minor to a major limitation in his/her ability to rotate his/her arm from a pronated (palms down) position to a supinated (palms up) position. She may also carry her elbow at an abnormal angle or have a shortened forearm.
       
    • The end of the radius bone closest to the elbow (radial head) may be, or may become, dislocated.
       
    • Diagnosis is usually confirmed by a physical exam, x-rays and/or CT scans.
       
    • Children whose forearms lie in functional positions may never need surgery. Children who have the problem in both arms, and/or whose forearms are fixed in a position that limits their ability to use their arm, may benefit from surgery.
       
    • Children with mild to moderate cases often compensate for their limited arm-rotation by wrist and shoulder motions.
       
    • Radioulnar synostosis can occur:
      • by itself (in isolation)
      • in association with other skeletal abnormalities (about one-third of the time)
      • in association with problems of the heart, kidneys, nervous system or gastrointestinal system
      • in association with certain genetic syndromes, such as Holt-Oram syndrome (also called hand-heart syndrome) and fetal alcohol syndrome
    • The condition affects both of a child’s arms about 60 percent of the time.
    • The average age at diagnosis is about 6 years old.
    • Boys and girls are equally affected.
    • The condition isn’t usually painful for a baby. But if left untreated or undiagnosed, teens may begin experiencing pain if the end of the radius (radial head) becomes dislocated.


    How Boston Children's Hospital approaches radioulnar synostosis

    You can have peace of mind knowing that the skilled experts in our Orthopedic Center’s Hand and Orthopedic Upper Extremity Program have treated thousands of babies and children with hand problems, ranging from the routine to the highly complex. So we can provide your child with expert diagnosis, treatment and care—as well as the benefits of some of the most advanced clinical and scientific research in the world.

    Our Orthopedic Center is nationally known as the preeminent center for the care of children and young adults with a wide range of developmental, congenital, neuromuscular, sports related, traumatic and post-traumatic problems of the musculoskeletal system.

    Radioulnar synostosis: Reviewed by Donald Bae, MD
    © Boston Children's Hospital, 2011

    Hand and Orthopedic Upper Extremity Program

    The Hand and Orthopedic Upper Extremity Program provides comprehensive care involving occupational and physical therapy, splinting, casting and reconstructive surgeries for infants, children and adolescents with complex congenital, neuromuscular, sports-related oncologic and traumatic upper limb conditions. 

    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 2
    Boston MA 02115 

    617-355-6021

    Boston Children's Hospital at Waltham
    9 Hope Avenue
    Waltham MA 02453 

    Boston Children's North
    10 Centennial Drive
    Peabody MA 01960 

    617-355-6021 

    Hand and Reconstructive Microsurgery Program 

    The specialists in the Hand and Reconstructive Microsurgery Program at Boston Children's are experts in the management of congenital and acquired hand deformities. We recognize the social elements involved in pediatric hand surgery, so an essential part of these operations has been making the child's hand as symmetrical as possible with his unaffected hand. 

    Boston Children's Hospital
    300 Longwood Avenue
    Hunnewell 1
    Boston MA 02115

    617-355-7252 

  • In-Depth

    At Boston Children's Hospital, our orthopedic and plastic surgery teams know how concerned you are that your baby has a congenital forearm difference. It may put your mind at ease to know that we’ve developed innovative surgical and non-surgical treatments for children with all degrees of severity of radioulnar synostosis. Learning more about this condition will help you feel more confident and in control as we treat—and work toward healing—your child.
     

    What is radioulnar synostosis?

    Congenital radioulnar synostosis is a rare congenital difference in which there’s an abnormal bony or soft tissue connection between the two bones of the forearm—the radius and the ulna.
     

    What signs or symptoms might my child have?

    Radioulnar synostosis can be mild to severe. Signs and symptoms of your child’s condition can range from a minor to a major limitation in her ability to rotate her arm from a pronated (palms down) position to a supinated (palms up) position. She may also carry her elbow at an abnormal angle or have a shortened forearm.

    Can radioulnar synostosis be associated with other conditions?

    Yes, radioulnar synostosis can occur as part of an underlying syndrome or with other developmental abnormalities, such as:

    • other skeletal abnormalities (about one-third of the time)
    • problems of the heart, kidneys, nervous system or gastrointestinal system
    • certain genetic syndromes, such as Holt-Oram syndrome (also called hand-heart syndrome) and fetal alcohol syndrome

    How does congenital radioulnar synostosis happen?

    When your baby is still in the womb, her upper limb develops between the fifth and eighth week of gestation. Initially, the radius and ulna bones are connected. Then, separation occurs and the individual radius and ulna bones form. Congenital radioulnar synostosis results if these bones don’t separate during this time.


    Why does it happen?
    While most cases of congenital radioulnar synostosis occur by chance (sporadically), about one in five cases is associated with a family history of the same condition.

    Congenital radioulnar synostosis can also occur as part of an underlying syndrome or with other developmental abnormalities.

    How common is radioulnar synostosis?

    The exact incidence of this condition is unknown. Boys and girls are equally affected, and about 60 percent of patients have the condition in both arms.
     

    Does radioulnar synostosis cause my baby pain?

    No, radioulnar synostosis isn’t usually painful for a baby. But if left untreated or undiagnosed, teens may begin experiencing pain if the top of the radius (radial head) becomes dislocated.

    How is radioulnar synostosis diagnosed?

    Your child’s doctor will do a thorough medical history and careful physical examination. X-rays and/or CT scans may be used to confirm the diagnosis.

    In milder cases, doctors may diagnose radioulnar synostosis late in the patient’s childhood or adolescence, since the child rarely experiences pain, and some children don’t experience too much limitation of movement. In fact, your child may notice only subtle differences in her ability to rotate her forearm.

    In more serious cases, forearm rotation is much more limited and obvious, so the diagnosis can be made at an earlier age—typically around age 6.

    How is congenital radioulnar synostosis treated?

    In general, your child’s doctor will determine a treatment plan based on whether your child is having problems using her arm, rather on than the absolute position of the forearm.

    Some children’s forearms lie in functional positions, and never require surgery. But others who have the problem in both arms, and/or in whom the forearm is fixed in a position that limits their ability to use the arm, may benefit from surgery.

     

    Will my child be OK? What’s her long-term outlook?

    Your child’s long-term outlook depends on the severity of her deformity, as well as the prognosis for any associated conditions she may have.

    If she has a mild case, your child may experience only a slight limitation on her ability to rotate her arm, and can do quite well without surgery. Many kids compensate for their lack of rotational ability with hyper-mobility in their wrists and shoulders.

    If she has a more severe case and her forearm(s) is more or less fixed in a pronated (palms down) position, she will need surgery to remove the abnormal soft tissue or bony connection or to position the forearm in a more functionally acceptable position.


    FAQ

    Q: What is radioulnar synostosis?
    A:
    Radioulnar synostosis is a rare congenital difference in which there’s an abnormal bony or soft tissue connection between the two bones of the forearm, the radius and ulna.

    Q: What are the signs and symptoms of radioulnar synostosis?
    A:
    Signs and symptoms of your child’s condition can range from a minor to a major limitation in her ability to rotate her arm from a pronated (palms down) position to a supinated (palms up) position. She may also carry her elbow at an abnormal angle or have a shortened forearm.

    Q: How is radioulnar synostosisusually diagnosed?
    A:
    Your child’s doctor will do a thorough medical history and careful physical examination. X-rays and/or CT scans may be used to confirm the diagnosis of radioulnar synostosis.

    Q: How does Boston Children’s treat radioulnar synostosis?
    A:
    Treatment, if needed, is usually by surgery. Many cases are mild enough that no surgery is needed.

    Q: If my child has surgery for radioulnar synostosis, will she be OK?
    A:
    Your child’s long-term outlook depends on the severity of her deformity, as well as the prognosis for any associated conditions she may have.

    While it’s rare that surgery can reconstruct a “normal” joint between the radius and ulna, some children with serious cases of radioulnar synostosis see significant improvement in function from osteotomy (bone-cutting) procedures, in which the forearm bones are surgically repositioned.

    Q: What is Children’s experience treating radioulnar synostosis?
    A:
    The experts in our Orthopedic Center’s Hand and Orthopedic Upper Extremity Program have treated thousands of babies and children with hand problems ranging from the simple to the highly complex, including radioulnar synostosis. So we can provide your child with expert diagnosis, treatment and care—as well as the benefits of some of the most advanced clinical and scientific research in the world.


    Causes

    When your baby is still in the womb, her upper limbs develop between the fifth and eighth week of gestation. Initially, the radius and ulna are connected. Then separation occurs, and the individual radius and ulna bones form. Congenital radioulnar synostosis results if these bones don’t separate during this time.

    While most cases of congenital radioulnar synostosis occur by chance (sporadically), about one in five cases is associated with a family history of the same condition.

    Congenital radioulnar synostosis can also occur as part of an underlying genetic syndrome, such as Holt-Oram syndrome (also called hand-heart syndrome) and fetal alcohol syndrome, or with other developmental abnormalities.


    Signs and symptoms

    Radioulnar synostosis occurs on a spectrum of severity. Signs and symptoms of your child’s condition can range from a minor to a major limitation in her ability to rotate her arm from a pronated (palms down) position to a supinated (palms up) position. She may also carry her elbow at an abnormal angle or have a shortened forearm.


    When to see a specialist

    Your child’s doctor will know by physical exam and x-rays that your baby has radioulnar synostosis. The doctor will refer you to an orthopedic surgeon, who will guide you to a more detailed diagnosis and treatment plan.


    Questions to ask your doctor

    If your child is diagnosed with radioulnar synostosis, you may feel overwhelmed with information. It’s easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

    Some of the questions you may want to ask include:

    • What is happening to my child, and why?
    • What do the x-rays reveal? How serious is my child’s condition?
    • What actions might you take after you review my child’s condition?
    • Is surgery necessary? Are there alternative therapies?
    • Will my child be OK after surgery?
    • Will my child recover full function of her arm? Will it look OK?
    • Will there be restrictions on her activities or capabilities?
    • What will be the long-term effects?
    • What can we do at home?
       

    Long-term outlook

    Your child’s long-term outlook depends on the severity of her deformity, as well as the prognosis for any associated conditions she may have.

    While it’s rare that surgery can reconstruct a “normal” joint between the radius and ulna, some children with serious cases of radioulnar synostosis see significant improvement in function from osteotomy (bone-cutting) procedures, in which the forearm bones are surgically repositioned.


    For parents

    Many parents whose babies are born with radioulnar synostosis feel frustrated that the birth of their child didn’t turn out the way they’d dreamed it would. Your child’s treatment and recovery may be fairly easy. Or, the journey may be more complicated—requiring surgery and care for your child as she grows.

    Even though you understand the importance of therapy for your child, you still might experience her treatment and care as a stressful time. If you feel frustrated or depressed, speak to your doctor or counselor to get help. Professionals in Children’s Center for Families can provide you with important resources and referrals.
     

    Radioulnar synostosis glossary

    • The Center for Families at Boston Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
    • congenital: present at birth
    • CT scan (computed tomography): detailed three-dimensional images of your child's bone, tissue and blood vessels. CT exams in Children’s powerful multi-detector scanners are quick, painless and non-invasive.
       
    • embryonic development: development of the fetus in the womb
    • in utero: in the womb (uterus)
    • orthopedics: the medical specialty concerned with diagnosing, treating, rehabilitating and preventing disorders and injuries to the spine, skeletal system and associated muscles, joints and ligaments
    • orthopedic surgeon, orthopedist: a physician specializing in surgical and non-surgical treatment of the spine, skeletal system and associated muscles, joins and ligaments
    • osteotomy: surgery that involves cutting bone
    • post-operative (post-op): occurring after surgery
    • pre-operative (pre-op): occurring before surgery
    • pronated (forearm position): “palms down” position
    • proximal (opp. distal): next to or nearest the point of attachment or origin (i.e., closest to the trunk of the body); opp. distal: situated away from the point of attachment or origin (i.e., furthest away from the trunk of the body)
    • radial head: top (most proximal part) of the radius bone closest to the elbow joint; important for rotating the forearm
    • radioulnar synostosis: a deformity in which the forearm bones (radius and ulna) are connected on the proximal side by bony or soft tissue, instead of having completely separated into two distinct bones in utero
    • radius: forearm bone on the thumb side
    • reconstructive surgery: surgery performed to repair and/or restore a body part to normal or as near normal as possible
    • sporadic: occurring without apparent genetic cause
    • supinated (forearm position): “palms up” position
    • synostosis: a union or fusion of bones that are usually distinct from each other
    • ulna: forearm bone on the little finger side
    • x-rays: a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
       

    Our plastic surgery programs and services include …


    Our complete orthopedic team

    Children’s is the primary pediatric teaching hospital of Harvard Medical School, where our physicians hold faculty appointments. We’re the largest pediatric orthopedic center in the nation, with 13 specialty clinics; an onsite brace shop; a plaster room; and a clinical team of orthopedic surgeons, orthopedic residents and fellows, certified physician assistants, nurse practitioners, registered nurses, physical/occupational therapists, brace technicians and cast technicians.

  • Tests

    At Boston Children's Hospital, we understand that the first step to treating your child is obtaining an accurate, timely and thorough diagnosis.

    Your child’s doctor will do a thorough medical history and careful physical exam. X-rays and/or CT scans may be used to confirm the diagnosis, assess the underlying structure of the baby’s deformity, check for related conditions and determine a course of treatment.

    In milder cases, radioulnar synostosis may not be noticeable and doctors may diagnose it during a child’s later school age years or adolescence, since the child rarely experiences pain, and some children don’t experience too much functional limitation of the limited forearm movement. In fact, your child may notice only subtle differences in her ability to rotate her forearm.

    In more serious cases, a child’s forearm rotation is much more limited and obvious, so the diagnosis can be made at an earlier age—typically around age 6.

    congenital radioulnar synostosis x-ray Elbow x-rays of a child’s arm with congenital radioulnar synostosis


     

     

    Our Orthopedic Clinical Effectiveness Research Center (CERC)

    Children’s Orthopedic Clinical Effectiveness Research Center (CERC) was established by our
    Orthopedic Center to improve the quality of life for children with musculoskeletal disorders. This collaborative clinical research program is unique in the nation and is playing an instrumental role in establishing, for the first time, evidence-based standards of care for pediatric orthopedic patients throughout the world.


    Children’s Hand and Reconstructive Microsurgery Program

    The doctors and staff in the Hand and Reconstructive Microsurgery Program at Boston Children's Hospital are recognized experts in the management of congenital and acquired hand deformities.

  • At Boston Children's Hospital, our Orthopedic Center's Hand and Orthopedic Upper Extremity Program provides comprehensive care—including evaluation, diagnosis, consultation, surgery, non-surgical therapies and follow-up care.

    In general, your child's doctor will determine treatment based on whether your child is having problems using her arm, rather than the absolute position of the forearm.

    • Some children's forearms lie in functional positions, and never need surgery.
    • Children who have the problem in both arms, and/or in whom the forearm is fixed in a position that limits their ability to use their arm, may benefit from surgery, preferably before they reach school age.

    Surgery can be performed either to remove the abnormal soft tissue or bony connection or to position the forearm in a more functionally acceptable position.

    While it's rare that surgery can reconstruct a “normal” joint between the radius and ulna, some children see significant improvement in function from osteotomy (bone-cutting) procedures, in which the forearm bones are surgically repositioned.


    Coping and support

    At Boston Children's Hospital, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit The Center for Families for all you need to know about:

    • getting to Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family


    In particular, we understand that you may have a lot of questions when your child is diagnosed with radioulnar synostosis. Will this affect my child long term? Will she be able to enjoy regular activities? Children's can connect you with extensive resources to help you and your family through this stressful time, including:

    • patient education: From doctor's appointments to physical therapy and recovery, our nurses and physical therapists will be on hand to walk you through your child's treatment and help answer any questions you may have—Why will my child need surgery? Are there non-surgical options? How long will her recovery take? How should we manage home exercises and physical therapy? We'll help you coordinate and continue the care and support your child received while at Children's.
    • parent-to-parent: Want to talk with someone whose child has been treated for radioulnar synostosis? We can often put you in touch with other families who've been through the same process that you and your child are facing, and who will share their experiences.
    • faith-based support: If you're in need of spiritual support, we'll connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy— representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
    • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.
       

    The Experience Journal

    Designed by Children's psychiatrist-in-chief David DeMaso, MD, and members of his team, the Experience Journal is an online collection of thoughts, reflections and advice from kids, parents and other caregivers about a variety of medical experiences, including hip problems.
     

    Children's Teen Advisory Committee

    To help teenagers take a more proactive role in their treatment and to have their needs recognized, Children's developed the Teen Advisory Committee. The group—made up of current Children's patients, ages 14 to 21—serves as a team of peers who can listen to other patients' needs, ensure their voices are heard and advocate for any changes they think can improve their hospital stay.

  • Research & Innovation

    The Orthopedic Center's Hand and Orthopedic Upper Extremity Program is dedicated to the comprehensive care of all upper limb conditions in infants, children and adolescents. Under the direction of Peter M. Waters, MD and Donald S. Bae, MD, patients with congenital, neuromuscular, sports-related, oncologic and traumatic or post-traumatic conditions of the upper extremity receive multi-disciplinary care that includes occupational and physical therapy, splinting/casting and reconstructive surgery.

    In addition, our Hand and Orthopedic Upper Extremity Program is committed in its efforts to improve clinical care through continued clinical and basic science research. The program is studying the treatment of a wide variety of upper limb conditions, including:

    • the use of microsurgical techniques in reconstructive surgery for congenital upper extremity differences
    • reconstruction of the congenitally deficient hand
    • instability of the sternoclavicular and glenohumeral joints of the shoulder
    • post-traumatic stiffness and deformity of the elbow
    • surgical correction of forearm deformity following previous fracture
    • ligament and/or cartilage injuries of the wrist
    • the use of microsurgical techniques in reconstructive surgery following limb salvage for bone and soft-tissue tumors

    Many of these investigations have resulted in national and international presentations or peer-reviewed medical publications—and all have increased our ability to provide the highest standard of patient care.

    Children speak about what it’s like to be a medical research subject

    View a video of a day in the life of Boston Children’s Clinical and Translational Study Unit, through the eyes of children who are “giving back” to science.

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