Ectrodactyly: What is Cleft Hand and How is it Treated?
What is Ectrodactyly or Cleft Hand?
Ectrodactyly or Cleft Hand is the name given to a collection of congenital hand deformities in which some of the fingers or in some cases the entire finger in the middle of the hand are missing which leaves an indentation or a space in the middle of the hand in the shape of "V" sign which is referred to as a cleft.1 Ectrodactyly or Cleft Hand can be present in one or in both hands. In some cases, a child with a Cleft Hand has a family history of this disorder and may have clefts in both the hands as well as the foot.
What is the Cause of Ectrodactyly or Cleft Hand?
Ectrodactyly or Cleft Hand has been proved to be a genetic disorder which is autosomal dominant type. This means that if the parent has a Cleft Hand then the chances of the offspring having the same deformity is about 50%. This disease can also occur with other genetic conditions like a cleft lip or cleft palate. How severe is the condition depends on the functionality of the hand. The condition is said to be mild if the child is able to use the affected hand in a normal fashion and if the child is not able to perform normal function with the affected hand then the condition is said to be severe.
What are the Symptoms of Ectrodactyly or Cleft Hand?
Since Ectrodactyly or Cleft Hand is a visible deformity, it can be seen as soon as the child is born. A child born with Ectrodactyly or Cleft Hand will have the following:
- Missing fingers in the middle of the hand with the child having a V shaped gap in the middle of the hand.2
- The V shaped gap is usually in the middle of the hand, although in some cases a radial or an ulnar cleft is also seen.
How is Ectrodactyly or Cleft Hand Diagnosed?
As stated Ectrodactyly or Cleft Hand is a visible deformity and a diagnosis is almost always made at the time of birth. In some cases this deformity can be seen readily on ultrasound before birth. How severe the deformity is checked by the physician by conducting a close physical examination of the child. Radiological studies in the form of an x-ray or CT scan will be done to look at the state of the bones and tissues of the affected hand. Every anatomic structure of the hand will be inspected by the physician in order to identify which bones or tendons are missing in the affected hand in order to formulate a treatment plan for the child. If Ectrodactyly or Cleft Hand is identified the physician will also look for accompanying conditions like a cleft lip or a cleft palate, any abnormalities of the foot, any abnormalities in the function of the digestive tract, any cardiac abnormalities.
How is Ectrodactyly or Cleft Hand Treated?
The main treatment for Ectrodactyly or Cleft Hand is surgery but not all children need surgery for correction. If the child is able to use the hand in a normal fashion with no issues then surgery is not recommended. Surgery is recommended only for those cases in which the deformity is far too severe and the child is not able to use the hands for any activity. The surgery is performed very early in the life of the child if the deformity is severe and has accompanying deformities like syndactyly. Even such conditions are not present then surgery can be delayed until the child is about two years old. There are different approaches to surgery for Ectrodactyly or Cleft Hand. This condition may require multiple surgeries and the approach differs from case to case. The aim of the surgery is to close the gap or the cleft so that use of the hand is made easier, create a space between the thumb and the index finger for allowing decent motor function, reorganize the skin and soft tissues, realign the bones so that the hand functions normally, correct any other deformity that may be present.
When it comes to the overall prognosis and whether the child with Ectrodactyly or Cleft Hand will be able to use the hands normally depends on the how good the reconstruction procedure was done and how severe the deformity of the child was in the first case. In majority of the cases, post procedure the child with Ectrodactyly or Cleft Hand may have close to a normal function of the hand and will be able to grip, grasp and pinch objects. The child will also have better alignment of the fingers of the hand. With passage of time as the child grows some of the deformities may recur which may require additional surgery to for further correction of Cleft Hand.