KidsMD Health Topics

Radial Club Hand

  • 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; Brian I. Labow, MD, principal investigator, Plastic Surgery, Boston Children's Hospital

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

    A diagnosis of radial dysplasia refers to a condition in which the radius bone of the forearm is underdeveloped or absent, resulting in a characteristic appearance of a hand and wrist that is angled toward the thumb-side of the forearm.

    • Radial dysplasia is a congenital (present at birth) condition in which the radius (the inner bone that connects the elbow to the forearm) did not form correctly in the womb. The radius may be malformed or missing.
    • In radial dysplasia:
      • The affected arm is shorter, with curving of the forearm and stiffness of the elbow and fingers.
      • thumb is either very small or missing.
    • The occurrence of radial dysplasia is usually sporadic (meaning that it occurs by chance, for no apparent reason), but it may be associated with any of several other defects or syndromes.
    • The condition occurs in between one in 55,000 and one in 100,000 babies.
    •  More than half of babies with radial dysplasia have the deformity in both limbs.
    • There are four types of radial dysplasia, corresponding to increasing degrees of severity and complexity. (See Tests for a classification of these types.)
    • Treatment usually begins in infancy with splinting, stretching and physical therapy.
    • Many affected children, except those with very mild cases may benefit from one or more surgeries, usually starting in the first year of life—but early splinting and stretching can help increase the effectiveness of later treatments.
    • Radial dysplasia can occur by itself (in isolation), or can be associated with genetic syndromes.
    • Radial dysplasia can recur following surgery in infancy, requiring additional surgery(ies).

    How Children’s Hospital Boston approaches radial dysplasia

    You can have peace of mind knowing that the skilled experts in our Orthopedic Center’s Hand and Orthopedic Upper Extremity Program and our Plastic Surgery Department’s Hand and Microsurgery Reconstructive 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.

    Our Department of Pediatric Plastic Surgery provides comprehensive care and treatment for a wide variety of congenital and acquired conditions, including hand deformities. As one of the largest pediatric plastic surgery centers in the United States, we perform more than 3,000 surgeries and care for more than 14,000 children every year.

    Radial dysplasia: Reviewed by Donald Bae, MD and Brian Labow, MD

    © Children’s Hospital Boston, 2011

    We offer orthopedic care in lots of places
    Boston Children’s physicians provide orthopedic care at Children’s satellite locations in Lexington, Peabody, Weymouth and Waltham, as well as at our main campus in Boston.
    Plastic surgery research and innovation

    Researchers in our Plastic Surgery lab are constantly developing improved treatments for children suffering from the conditions that require plastic surgery. Our aim is to translate the knowledge gained in the laboratory back to the clinic to improve care for your child.

    If you live far from the United States, we can help

    As an international pediatric orthopedics center, Children’s treats young patients from all over the world. Our International Center assists families residing outside the United States: We facilitate the medical review of patient records; coordinate appointment scheduling; and help families with customs and immigration, transportation, hotel and housing accommodations.

    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 
     fax: 617-739-1093

    Boston Children's Hospital at Waltham
    9 Hope Avenue
    Waltham MA 02453
     617-355-6021
     
    Boston Children's North
    10 Centennial Drive
    Peabody MA 01960
     617-355-6021
     

    The specialists in the Hand and Reconstructive Microsurgery Program at 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.
    Children's Hospital Boston
    300 Longwood Avenue
    Hunnewell 1
    Boston MA 02115
     617-355-7252

  • In-Depth

    At Children’s Hospital Boston, our orthopedic and plastic surgery teams know how concerned you are that your baby has a hand deformity. It may help you to know that we’ve developed innovative surgical and non-surgical treatments for children with all degrees of severity of radial dysplasia. 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 radial dysplasia?

    Radial dysplasia is a rare congenital (meaning your baby was born with it) difference in which the radius bone of the forearm did not form properly. This causes the affected hand to be bent inward toward the thumb-side of the forearm, often with limited movement.

    In addition to the curve of the wrist and forearm, your child may have a missing or small thumb.

    What does “radial” mean?

    When describing a specific side of the forearm, you may hear your child's doctor refer to the "radial side," which means the side of the arm on which the thumb lies, corresponding to the radius bone of the forearm. The doctor may also refer to the "ulnar side," which describes the side on which the little finger lies, corresponding to the ulna bone.

    How common is radial dysplasia?

    Radial dysplasia affects between one in 55,000 and 100,000 babies.

    How will having radial dysplasia affect my child?

    Functional consequences depend upon the severity of radial dysplasia. In the most severe cases, your child may have very limited range of motion at the wrist because her radius is completely absent. Other problems that cause limited function include:

    • an underdeveloped or missing thumb
    • abnormal muscles in the forearm, wrist, and hand
    • curvature and/or shortening of the ulna

    In mild cases, the radius is merely slightly smaller than the ulna and there is minimal wrist deviation. This generally does not cause many problems with your child’s development or hand function.

    Does radial dysplasiacause the baby pain?

    No, radial dysplasia isn’t usually painful.

    How is radial dysplasia diagnosed?

    Radial dysplasia develops early in pregnancy—sometime between the 28th and 56th day of gestation—when the bones of the hand and forearm are being formed. It can sometimes be picked up on a routine prenatal ultrasound. After the baby is born, the deformity is apparent, and the diagnosis is usually confirmed through a physical exam and x-rays.

    In diagnosing the condition, your child’s doctor will likely classify it as one of four types—with Type 1 the mildest and Type 4 the most severe.

    For details about these classifications, see Tests.

    Can radial dysplasiabe associated with other conditions?

    If your child is diagnosed with radial dysplasia, your doctor will check for other associated deformities or syndromes, including problems with the:

    • heart (such as Holt-Oram syndrome, also called hand-heart syndrome)
    • kidneys, spinal column and/or digestive system (such as VACTERL syndrome)
    • blood cells (such as Fanconi anemia, TAR)

    Additional anomalies can occur with radial dysplasia, including:

    • bone/joint anomalies
    • muscle/tendon anomalies
    • nerve/artery anomalies

    How is radial dysplasia treated, and at what age?

    Children’s orthopedic surgeons and plastic surgeons usually begin treating a child with radial dysplasia during infancy by some combination of splinting, casting, stretching and range of motion (ROM) exercises. If the condition persists, surgery is usually needed.

    For details, see Treatment & Care.

    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. Furthermore, radial dysplasia may recur after initial treatment, so she may need additional surgery(ies) as she grows.

    If she has a mild case, your child will need therapy as she grows in order to maintain alignment and strength—and she will likely have small limitations of her ability to move and use her arm. If she has a more severe case, her arm will have limited range of motion, strength and function. 

    Causes

    Radial dysplasia usually occurs sporadically (by chance). Doctors and scientists don’t know why radial dysplasia affects certain children.

    In some children, radial dysplasia is associated with other congenital anomalies or syndromes that are known to have a genetic component. Some associated deformities or syndromes include those of the:

    • heart (such as Holt-Oram syndrome, also called hand-heart syndrome)
    • kidneys, spinal column and/or digestive system (such as VACTERL syndrome)
    • blood cells (such as Fanconi anemia)
    • thrombocytopenia-absent radius syndrome (TAR)

    Symptoms

    Signs of radial dysplasia may include:

    • The affected arm is shorter, with curving of the forearm and stiffness of the wrist and fingers.
    • The thumb is either very small or missing.

    The arrangement of muscles and nerves may be unbalanced, and some muscles and nerves may even be missing.

    When to see a specialist

    Your child’s doctor will know by sight that your baby’s hand has a radial dysplasia. The doctor will refer you to a hand specialist, who will guide you to a more detailed diagnosis and treatment plan.

    Questions to ask your doctor

    If your child is diagnosed with radial dysplasia, you may feel overwhelmed with information. It can be 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 will x-rays reveal?
    • What actions might you take after you review my child’s x-rays? 
    • Is surgery necessary? Are there alternative therapies?
    • Will my child be OK after surgery?
    • Will my child recover full function of her hand? 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?

    Radial dysplasia glossary

    • absent radius: condition in which the inner forearm bone (radius) is completely missing; occurs in Type 4 radial dysplasia
    • The Center for Families at 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
    • camptodactylya deformity in the finger joints that causes a flexed finger or fingers; can be present in radial dysplasia
    • congenital: present at birth
    • embryonic development: development of the fetus in the womb
    • in utero: in the womb (uterus)
    • occupational and physical therapy: services offered by trained professionals to help restore function or (re)teach basic life skills, like dressing oneself or grasping objects
    • 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
    • passive motion: motion in a body part guided by a helper, rather than by the patient
    • pollicization: surgical procedure in which a thumb is constructed from an existing index finger
    • post-operative (post-op): occurring after surgery
    • prenatal (fetal) ultrasound:ultrasound performed at several stages of pregnancy; may detect radial dysplasia in the fetus
    • pre-operative (pre-op): occurring before surgery
    • radial dysplasia (radial longitudinal deficiency): a deformity in which the radius bone of the forearm doesn’t develop properly, causing the hand to angulate toward the thumb-side of the forearm with limited movement
    • radius: forearm bone on the thumb-side
    • range of motion (ROM) exercises: physical therapy exercises designed to improve or restore flexion and extension of joints 
    • 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
    • thumb aplasia: complete absence of the thumb
    • thumb hypoplasia: underdevelopment of the thumb; can occur with radial dysplasia
    • ulna: bone on the  little finger side of the forearm 
    • x-raysa diagnostic test that uses invisible ionizing radiation to produce images of internal tissues, bones, and organs onto film

     

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

    Radial dysplasia can sometimes be seen by ultrasound prenatally, and is apparent at birth. Your doctor will use a physical exam and x-rays to assess the underlying structure of your baby’s deformity and determine a course of treatment.

    If your child is diagnosed with a radial dysplasia, your doctor will check for other associated deformities or syndromes, including problems with her:

    • heart (such as Holt-Oram syndrome, also called hand-heart syndrome)
    • kidneys, spinal column and/or digestive system (such as VACTERL syndrome)
    • blood cells (such as Fanconi anemia)
    • thrombocytopenia absent radius (TAR)
    • other bones/joints
    • muscles/tendons
    • nerves/arteries

    Types of radial dysplasia
    In diagnosing the condition, your doctor will likely classify it as one of the following four types:

    • Type 1
      • mildest form of radial dysplasia
      • mild deviation of the wrist
      • underdevelopment of thumb may occur
      • problems that can result from more severe forms, such as loss of motion, usually don’t occur
      • typically, surgery required only to correct underdeveloped thumb (if present)
    • Type 2
      • limited growth of your child’s radius on both sides
      • wrist turned toward the radius, ulna bows out
      • underdevelopment of the thumb is usually more significant (if present)
    • Type 3
      • partial absence of the radius
      • wrist severely deviated, hand has limited support
      • ulna is thickened and bowed
      • associated problems with thumb and fingers, such as underdevelopment or camptodactyly, a deformity in the finger joints that causes a flexed finger or fingers (may be present)
    • Type 4
      • most common and most severe form
      • complete absence of the radius (absent radius)
      • complete or near-complete absence of the thumb (thumb hypoplasia/aplasia)
      • causes many limitations in the function of your child’s hand, wrist and forearm
      • ulna bowing is the most severe
      • index, long and ring fingers may be involved
      • elbow may have limited range of motion

    In most types of radial dysplasia, there can also be varying degrees of absent muscles, nerves and blood vessels—there’s a broad variability with each type and each case.

    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 Microsurgery Reconstructive Program at Boston Children's Hospital are experts in the management of congenital and acquired hand deformities.
  •  

    At Boston Children's Hospital, experts in our Orthopedic Center's Hand and Orthopedic Upper Extremity Program and our Plastic Surgery Department's Hand and Microsurgery Reconstructive Program provide comprehensive care—including evaluation, diagnosis, consultation, surgery, non-surgical therapies and follow-up care.

    The goals of treatment are to:

    • correct the radial deviation of the wrist
    • balance the wrist on the forearm
    • maintain wrist and finger mobility
    • maximize hand function
    • preserve forearm growth
    • improve appearance

    Exercises and splinting 

    During infancy, the goal of treatment is to allow your child's wrist to extend and her elbow to move into a normal position. Your child's doctor will guide you in performing stretching exercises for your child's wrist and elbow at home to help accomplish this goal. More severe cases may require casting or splinting to gradually stretch the contracted soft tissues.

    Once passive motion (motion guided by a helper) is achieved, your baby will likely need to wear a splint during the night throughout her infancy and during periods of rapid growth. Even if your child needs surgery, keep in mind that the range-of-motion exercises are extremely important.

    Any improvement in range of motion achieved through exercise will make subsequent surgery more effective and perhaps less complex.

    The decision of whether to operate

    Treatment for radial dysplasia can be complex. Surgical treatment is usually needed to any correct deviation of the wrist that persists after stretching and splinting. But doctors also must weigh the decision to perform surgery against any other problems that your child may be experiencing due to other associated deformities or syndromes, including those of the:

    • heart (such as Holt-Oram syndrome, also called hand-heart syndrome)
    • kidneys, spinal column and/or digestive system (such as VACTERL syndrome)
    • blood cells (such as Fanconi anemia)

    Also, if your child cannot flex her elbow, a bent wrist and short forearm can sometimes be an advantage, enabling your child to do things such as feed herself.

    Surgery

    Surgical treatment is often separated into several phases—with a specific surgical procedure to treat each area of the arm and hand that's affected by the radial dysplasia. Treatments may begin in the first year of life, and may take place over the course of months or years.

    Treatment options for the wrist

    Your child's doctor may recommend:

    • pre-surgical lengthening
      • used prior to surgery to gently stretch your child's hand into a straighter position
      • recommended in infancy in situations in which it's very difficult to stretch out the tight wrist
      • used in adolescence when there is recurrent deformity or marked shortening of the forearm
    • centralization surgery
      • indicated for the more severe forms of radial dysplasia (Types 2, 3 and 4—see Tests for classification of types)
      • more common than radialization procedure (see next bullet)
      • bones from your child's wrist are removed so that the hand sits straight on the end of the ulna, which is sometimes placed in a slot within the wrist
      • often the wrist position is maintained with the use of an internal pin
    • radialization surgery
      • less common than centralization procedure (see previous bullet)
      • hand is moved slightly closer to the ulnar border of the forearm and the tight muscles of the wrist are reorganized so that the hand is balanced on the end of the ulna
      • an “overcorrection” to compensate for the tendency of radial dysplasia to recur
    • splinting
      • usually necessary following centralization or radialization procedures
      • splint may be used for a year or even more after surgery, until the ulna gradually broadens and becomes a more stable platform on which the wrist can balance

    Treatment for the forearm

    • osteotomy
      • surgery to straighten your child's forearm bones
      • bones are cut and then rearranged into a straighter position
      • held in place by the same pin that holds the wrist

    Treatment for underdeveloped or absent thumb (thumb hypoplasia)

    • reconstructive surgery
      • maybe a procedure known as pollicization (constructing a thumb from an existing index finger) or by tendon transfers
      • usually performed after other procedures described above are completed

    Treatment for elbow

    • elbow surgery
      • rare in a child with radial dysplasia
      • to release the tight soft tissue structures at the back of the elbow that are causing the elbow to stiffen

     

    Caring for your child after surgery

    If the surgery to remove the extra digit is fairly complex, it can also involve your child's bone, ligament and tendon. If that's the case, your child may need to wear a cast for a few weeks. Your doctor may recommend occupational or physical therapy to help reduce scarring, stiffness and swelling and improve function.

    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. Her radial dysplasia may have a tendency to recur, so she may need additional surgery(ies) as she grows.

    If she has a mild case, your child will need therapy as she grows in order to maintain alignment and strength—and she will likely have small limitations of her ability to move and use her arm. If she has a more severe case, her arm will have limited range of motion, strength and function.

    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 radial dysplasia. 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 radial dysplasia? 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.

     

    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 change.

    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.
  • Research & Innovation

    Improving outcomes for upper extremity disorders

    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:

    • reconstruction of the congenitally deficient hand
    • the use of microsurgical techniques in reconstructive surgery for congenital upper extremity differences
    • 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 Children’s Clinical and Translational Study Unit, through the eyes of children who are “giving back” to science.
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