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Syndactyly

Syndactyly, the most common congenital hand difference, is an abnormal connection of the fingers to one another. In babies born with syndactyly, the fingers are webbed or fused together because they did not separate normally during development.

In complex syndactyly, the bones in the affected fingers are fused together.

Courtesy of Shriners Hospital for Children—Northern California

The condition can take a number of forms, including:

• Simple syndactyly. In this form, the fingers are joined by either skin or other soft tissue, which may appear webbed. The webbing may be either:
  • Complete — extending from the base of the finger all the way to the tip
  • Incomplete — stopping at any point before the tip
• Complex syndactyly. In this form, the bones of the fingers are fused together. This form of syndactyly is more difficult to treat because, in addition to bone, the fingers may also share a nail, nerves, muscles, blood vessels, and tendons.

Syndactyly often runs in families and can occur alone or as part of a medical condition such as Apert syndrome or Poland syndrome.

Polydactyly

Polydactyly, with an extra little finger.

Courtesy of Shriners Hospital for Children—Northern California

Polydactyly is the presence of one or more extra fingers on the hand. An extra finger may be small and nonfunctional — made of only skin and soft tissue — or may be fully formed with bones of its own.

Most commonly, an extra finger is adjacent to the thumb or little finger. Rarely, an extra finger is between the other fingers.

Polydactyly often runs in families and is sometimes associated with other medical conditions or syndromes. In some cases, babies born with polydactyly will have syndactyly as well.

Limb Deficiencies

A baby with an upper limb deficiency may be born with hands or arms that are smaller than normal or are missing. Some of the more common limb deficiencies are described below.

Radial Deficiency

In a radial deficiency, the radius bone and soft tissues in the forearm fail to develop properly. This causes the affected hand to be bent inward toward the thumb side of the forearm, giving it the appearance of a club hand.

In a radial deficiency the hand is turned inward, giving it a club-like appearance.

Courtesy of Shriners Hospital for Children—Northern California

The forearm may also be shortened, and the thumb may be smaller and weaker than normal or missing — a condition known as thumb hypoplasia.

Thumb hypoplasia may cause a child's thumb to be absent or underdeveloped.

Courtesy of Shriners Hospital for Children—Northern California

Some types of radial deficiency run in families, while others are related to congenital differences in the heart, kidneys, and blood system. To determine whether a radial deficiency is caused by an underlying medical condition, the doctor may recommend that your child undergo thorough evaluation by a pediatrician and/or a geneticist.

Transverse Deficiency

In a transverse deficiency, all of the elements below a certain level in the patient's arm are missing. The condition has an appearance similar to that of an amputation and is sometimes known as congenital amputation.

Doctors believe that a transverse deficiency occurs when a vascular problem, such as a blood clot in the limb, causes the affected arm to stop developing. The most common type of transverse deficiency is one in which the elements below the elbow are missing.

A child with a below-the-elbow deficiency, the most common type of transverse deficiency.

Reproduced from Song K (ed): Orthopaedic Knowledge Update Pediatrics 4. Rosemont, IL. American Academy of Orthopaedic Surgeons, 2011, p. 149.

Physical Examination

Most congenital hand differences can be detected soon after birth.

A pediatrician will perform a careful examination of your child's hand and arm and, in most cases, will be able to diagnose the specific type of hand difference.

Some cases are more complex and have more than one feature, however, and the pediatrician may refer your child to a specialist — such as a pediatric orthopaedic surgeon or pediatric hand surgeon — to ensure the correct diagnosis.

Tests

Your child's doctor may order tests to confirm a diagnosis or to help plan your child's treatment.

X-rays. X-rays provide images of dense structures, such as bone. Your child's doctor may order X-rays to determine which bones in your child's hands and arm are affected.

Genetic testing. An evaluation by a genetic specialist may be recommended for children with certain hereditary hand differences or differences caused by medical conditions or syndromes.

Additional tests. If the hand difference is associated with a medical condition or syndrome, your child's doctor may recommend additional tests to determine if other parts of your child's body are affected, such as the heart or kidneys.

Nonsurgical Treatment

Nonsurgical treatment for hand differences often includes one or more of the following:

• Therapeutic recreation. Play, sports, and other activities can help build confidence and improve a child's emotional well-being.
• Physical therapy. Specific exercises can strengthen the arm and hand and improve motion in the wrist and fingers.
• Occupational therapy. Exercise and training can prevent stiffness in the hand and improve handwriting and other skills necessary for daily activities.
• Assistive or adaptive devices. Using specialized devices can make eating, dressing, and other daily activities easier.
• Prosthetics. Artificial devices can be used to replace or augment missing parts or limbs.

Surgical Treatment

There are several surgical procedures used to treat congenital hand differences. The procedure your child's doctor recommends — as well as the age at which the procedure is performed — will depend upon your child's specific condition and the type of surgery.

Surgical procedures for hand differences include:

• Separation of webbed or joined fingers
• Removal of extra fingers
• Reconstruction of fingers or other parts of the hand/upper extremity

Peer Support

Establishing peer contacts for both your child and your family is a very important part of treatment. Meeting other children with hand differences and their families will provide reassurance that you are not alone and can be an invaluable source of information and support.

You can find many support groups and discussion groups online. In addition, some children's hospitals and other organizations sponsor Hand Camps for children with differences, such as the nonprofit Hands to Love. These camps provide a safe and encouraging environment for children to socialize and participate in camp activities with other children who have congenital hand differences.