Radial Longitudinal Deficiency (Radial Club Hand) | Diagnosis & Treatment

How is radial longitudinal deficiency diagnosed?

Radial longitudinal deficiency 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 radial longitudinal deficiency, 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 longitudinal deficiency, including:

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

How is radial longitudinal deficiency treated?

The goal of treatment is 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 their 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 their 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.

Surgery for radial longitudinal deficiency

Treatment for radial longitudinal deficiency 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. Also, if your child cannot flex their elbow, a bent wrist and short forearm can sometimes be an advantage, enabling your child to do things such as feed themselves.

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 longitudinal deficiency. Treatments may begin in the first year of life, and may take place over the course of months or years.

The are a number of options that your child's doctor may recommend for the wrist.

  • 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 is indicated for the more severe forms of radial longitudinal deficiency (Types 2, 3 and 4 ). 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 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 longitudinal deficiency to recur.
  • 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.

To straighten your child's forearm bones, your doctor may recommend an osteotomy. During this surgery, the bones are cut and then rearranged into a straighter position, held in place by the same pin that holds the wrist.

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

Elbow surgery is rare in a child with radial longitudinal deficiency. If recommended, this procedure releases the tight soft tissue structures at the back of the elbow that are causing the elbow to stiffen.

What is the long-term outlook for radial longitudinal deficiency?

Your child's long-term outlook depends on the severity of their deformity, as well as the prognosis for any associated conditions they may have. Their radial longitudinal deficiency may have a tendency to recur, so your child may need additional surgery(ies) as they grows.

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