Surgical Treatment Options for Radial Dysplasia (radial club hand, radial longitudinal deficiency) in Children

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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)

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.

Surgical Options

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

Pre-surgical lengthening is used prior to surgery to gently stretch your child's hand into a straighter position. This option is recommended in infancy in situations in which it's very difficult to stretch out the tight wrist. Pre-surgical lengthening is also used in adolescence when there is recurrent deformity or marked shortening of the forearm.

Centralization surgery

Centralization surgery is indicated for the more severe forms of radial dysplasia (Types 2, 3 and 4 — see tests for more information). This surgery is 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

Radialization surgery is often less common than centralization procedure (see previous bullet). The 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" is to compensate for the tendency of radial dysplasia to recur.

Splinting

Splinting is usually necessary following centralization or radialization procedures. A 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

Osteotomy is a surgery to straighten your child's forearm bones.  The 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

Reconstruction surgery for an underdeveloped or absent thumb is also known as pollicization (constructing a thumb from an existing index finger) or by tendon transfers. This surgery is usually performed after other procedures described above are completed.

Treatment for elbow

Elbow surgery

Elbow surgery is rare in a child with radial dysplasia. If recommended, this procedure releases 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.

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- Sandra L. Fenwick, President and CEO

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