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

How is ulnar longitudinal deficiency diagnosed?

Ulnar longitudinal deficiency is diagnosed after inspection of the forearm and an x-ray. Your child's doctor will want to check for other congenital deformities or syndromes that are associated with ulnar longitudinal deficiency.

Further tests may depend on whether the doctor suspects any associated problems, including problems with heart, kidneys, vertebral column, blood cells and digestive system.

In diagnosing the condition, the doctor will want to note the severity and will likely classify it as one of four types.

What are the treatment options for ulnar longitudinal deficiency

Exercises and splinting

During infancy, the first goal of treatment is to allow your child's wrist to extend and the elbow to move into a normal position. Your child's doctor will guide you in performing gentle but firm and frequently repeated passive stretching exercises for the wrist and elbow to help accomplish this goal.

More severe cases may require casting or splinting to gradually stretch the contracted soft tissues. Once passive motion is achieved, your baby will likely need to wear a splint during the night throughout 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.

Surgical treatment for ulnar longitudinal deficiency

Surgical treatment is usually required to correct deviation of the wrist that persists after stretching and splinting. However, the decision to perform surgery is weighed against any other problems that your child may be experiencing due to associated medical conditions.

Sometimes, if your child cannot flex his elbow, a bent wrist and short forearm can be an advantage, enabling your child to do things like feed himself.

Surgery is divided into phases. Each problem is treated in separate surgical procedures that may take place over the course of months or years, depending on the problem.

There are a number of procedures your child's doctor may recommend for the wrist:

  • Lengthening: This procedure is used prior to surgery to gently stretch your child's hand into a straighter position. Lengthening is recommended in infancy if it's difficult to stretch out the tight wrist. It is also used in adolescence when there is recurrent deformity or marked shortening of the forearm.
  • Centralization: This procedure involves removing bones from your child's wrist so that the hand sits straight on the end of the radius, which is sometimes placed in a slot within the wrist.he time of surgery, surgeons will likely create an internal splint — a pin that is placed through your child's wrist and radius. This splint may remain in place for a year or even more after surgery, until the ulna gradually broadens and becomes a more stable platform on which the wrist may balance.

For the forearm bones, your doctor may recommend an osteotomy. Your child's surgeon may want to straighten her forearm bones by cutting them into wedges, then rearranging them into a straighter position. These will be held in place by the same pin that holds the wrist.

For an underdeveloped or absent thumb, reconstructive surgery, either through a procedure known as pollicization or by tendon transfers, is usually performed after other procedures described above are completed.

Surgery on the elbow is uncommon in ulnar longitudinal deficiency. However, in some cases, your child's doctor may want to perform an operation to release the tight soft tissue structures at the back of the elbow that are causing the elbow to stiffen.

What's my child's long-term outlook?

It depends on the severity of the deformity. In mild cases, intermittent therapy throughout growth is required to maintain alignment and strength. These children will have minor limitations of motion, function and strength. 

In the more severe cases, there will be marked limitation of motion, strength and function. In these situations, growth will also be limited in that forearm.