Treacher Collins Sydrome: Treatment

The Jaws
Children with TCS almost always require both braces and jaw surgery. Typically, children's upper jaw grows too far forward compared to the lower jaw. However, sometimes the reverse may occur. It is also fairly typical that the front teeth cannot close together. That is, the back teeth may hit, but up front there is a gap between the teeth. This condition is called an "open bite" and it can only be treated surgically, by operating both on the upper and lower jaws. It is a condition that is very difficult to treat, and may take more than one operation to get just right.

Some doctors recommend using a technique called "distraction" on the lower jaw in order to stretch it forward. This technique is not used at our center for a number of reasons (listed below). This technique involves making an osteotomy (cut in the bone) across the mandible (lower jaw) and attaching a metallic screw driven device to either side of this osteotomy. Pins are usually put through the skin into the bone, and the expansion device is attached to these pins. By turning a screw on the expansion device, a little each day, the bone slowly gets longer. There are also devices are completely buried under the skin, except for a small metal bar that pokes outside so that the screw can be turned. These distraction devices successfully lengthen bone, and the majority of doctors treating Treacher Collins syndrome recommend them. However, these devices are almost never used at the Craniofacial Center in Dallas. There is a long list of reasons why I chose not to use these devices for this condition:

  1. The overall complication rate for using these devices is about 1 out of three cases, as published in a recently survey of doctors. The complication rate was over 50% for doctors who had done fewer than 10 patients! Surveys typically underestimate complication rates, so that the actual complication rate may even be higher.
  2. These devices leave scars on the child's face that cannot be removed, and remain for a lifetime. Many doctors say that the scars will fade away and not be noticeable. I recommend seeing another child's scars first (in person, scars tend to not show up in photographs) before allowing this procedure to be done. There is an internal distraction device that does not leave facial scars, but this device has an even higher complication rate than the external device.
  3. In order to attach the distracter to the jaw, it is necessary to use metal pins or screws. It is very likely that the front pins or screws will go through the child's un-erupted permanent teeth. The result is that the permanent tooth will not come up and the child will need an artificial tooth.
  4. The upper jaw is not treated by distraction. This means that after the two operations are finished, one to put on the distracter and one to take it off, the upper jaw is still out of position, and will need surgery to correct it.

The alternative to using distraction devices is a single-staged orthognathic procedure. From inside the mouth (so there are no scars on the face) the upper and lower jaws may be cut and moved into a better position. It is best to delay this surgery until the child is older and wants to have the surgery done, unless the jaws are way out of alignment or the child has a tracheostomy. This operation is longer and more complicated than putting on a distracter, and not every doctor is able to do this procedure; but the final result is usually better. However, in very severe cases, in which the jaw is extremely small, there may be an indication for a new type of distraction.