Radial Club Hand

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INTRODUCTION:-

In radial club hand, a baby’s radius was not formed properly in the womb, which in turn causes the wrist to be in a fixed bent position toward the thumb side of the hand. There may be a deformity or absence of the thumb as well. The condition is sometimes called “radial dysplasia” which simply means abnormal development of the radius. In most forms of radial club hand, not only is the bone abnormal but the soft tissues and flesh of the forearm are also abnormal. The arrangement of muscles and nerves may be unbalanced and some muscles and nerves may even be absent. The most severe cases lead to significant problems in the function of the hand, fingers and elbow. The entire arm will be shorter, with marked curving of the forearm, stiffness of the elbow and fingers. In these situations, the thumb will either be very small or absent.
Radial club hand is a birth defect.
Radial club hand develops early in pregnancy, sometime between the 28th and 56th day of gestation, when the bones of the hand and forearm are being formed.
Degrees of malformation
In the most severe cases, the radius is completely absent, leading to very limited range of motion at the wrist. The elbow joint may also be disturbed or even fused with no motion. There may also be underdevelopment or absence of the thumb, which interferes with hand function.
In the mildest cases, the radius is merely slightly smaller than the ulna and there is minimal deviation at the wrist.
The severity of radial club hand is also affected by the presence of an abnormal bar of fibrous tissue that may appear in any case where the distal part (hand end) of the radius is absent. This fibrous tissue which connects the remnants of the radius to the hand, is known by the German word anlage and has a very limited ability to grow. It is attached to the radial side of the hand and wrist. As the ulna grows in the mothers’ womb, the lack of growth in the radial anlage draws the hand into a deviated “club” position. This may also cause the ulna to bow.
The degree to which the hand and wrist deviate at birth, depends on two factors: the degree to which the support of the radius is lacking and the degree to which the anlage tethers the wrist, allowing the ulna to push the wrist further and further into deviation as it grows.
Causes
Radial club hand occurs sporadically with no known cause. It occurs between 1 in 30,000 and 1 in 100,000 live births. Several theories have been raised, such as maternal drug exposure, compression of the uterus, and vascular injury, but none of these have been proven. There is no known genetic link to radial club hand except when the problem is associated with other congenital anomalies or syndromes that do have a known genetic component. It has been associated with many congenital syndromes including those affecting cardiac, gastrointestinal and renal systems. It has also been linked to some chromosomal abnormalities including Trisomy 13, 18 and 21.

Diagnosis
Radial club hand 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 radial club hand.

Further tests may depend on whether the doctor suspects any of these associated problems, including concern regarding the 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 the following four types

  • Type 1: This is the mildest form of radial club hand, involving mild deviation of the wrist throughout life. Problems that can result from more severe forms, such as loss of motion, usually do not occur. Type 1 involves a defective growth plate (physis) on the distal end (hand side) of the radius. This defect leads to a minor foreshortening of the radius and a prominent distal ulna. Surgical intervention is usually not required. However, some surgery may be required to correct underdevelopment (hypoplasia) of the thumb, an associated problem that sometimes occurs in Type 1 cases.
  • Type 2: This involves limited growth of the radius on both its distal and proximal sides. The wrist is therefore more deviated toward the radius, and the ulna bows out. Underdevelopment of the thumb is usually more significant with more deficiency in the bones of the wrist.
  • Type 3: This involves the absence of two thirds of the radius on the hand side. The wrist is more severely deviated and the hand has limited mechanical support. The ulna is thickened and bowed. Associated problems with the thumb and fingers, such as underdevelopment or camptodactyly, a deformity in the finger joints that leads to a flexed finger or fingers.
  • Type 4: This type of radial club hand is most common and the most severe, causing profound limitation of hand, wrist and forearm function. It involves the complete absence of the radius along with complete or near complete absence of the thumb. The ulna bowing is also most severe. The index, long and ring fingers may be involved and the elbow may have limited range of motion.

Physioline’s Treatment
Treatment for radial club hand can be very complex. Specific treatment will vary from child to child and will be determined by your child’s physician based on:

  • your child’s age, overall health, and medical history
  • the severity of the condition
  • any other deformities or syndromes associated with the problem
  • your child’s tolerance for specific medications, procedures, or therapies
  • your opinion or preference
Physioline’s Exercises and splinting
During infancy, the first goal of treatment is to achieve passive extension of the wrist and elbow into a normal position.
More severe cases may require casting or splinting to gradually stretch the contracted soft tissues. Still, a rigorous stretching program is an important component of any treatment plan.
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.
More severe cases, in which there is no support for the wrist without the splint, usually require surgery. However, even if your child needs surgery, keep in mind that the range of motion exercises are extremely important. Even if wrist therapy alone doesn’t resolve the wrist contracture (shortening) and deviation, this should not discourage you from continuing the exercise regimen. Any improvement in range of motion achieved through exercise will make subsequent surgery more effective and perhaps less complex.
Long-term goals of physioline
The long-term outlook is dependent 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.
DETAILED TREATMENT:- Kindly contact Physioline for the detailed assessment and treatment program which is individualised according to every patient.