Apert Sydrome: Treatment

Surgery of the Midface:

There are three operations to bring the mid face forward, the LeFort I, the LeFort III and the monobloc. The LeFort I brings the lower mid face forward, from the level of the upper teeth, to the nostrils. The LeFort III brings the entire mid face forward, from the upper teeth to just above the cheekbones. The monobloc brings both the entire mid face and the forehead forward together at the same operation. Your surgeon will discuss what is he or she thinks is best for your child. In general, the LeFort I is not performed until children are in their teenage years. This leaves the LeFort III and the monobloc. Some surgeons believe the monobloc is a very good operation for Apert syndrome. Among the advantages of this procedure is that the forehead and mid face are brought forward at the same time; saving a child one operation. However, this operation does have a high risk for very serious infections, which is the reason that many surgeons, including myself, choose to not perform this procedure (see publications [#8], [#24]).

The LeFort III is the most commonly performed operation for treating the mid face in Apert syndrome. Of all the operations done for Apert syndrome, this procedure has the greatest effect on improving a child's appearance. If this operation is not being done to treat sleep apnea, it is usually being performed to improve a child's appearance. Many surgeons will recommend this surgery when your child is between 6 and 8 years old. Children do not really begin teasing each other about appearance until age 6. Depending on how a child with Apert syndrome is developing and coping with his or her appearance will help determine the timing for this procedure. This operation is one of the bigger operations a child will undergo. The procedure is performed through the same incision, on the top of the child's head, used for the skull operation. The bones of the mid face are cut across the top of the nose, along the floor of the orbit (under the eye), and down the sides of the cheekbones. No scars are put on the child's face. After the bones are cut, the mid face is moved forward and held in position with bone grafts (taken from the skull) that are held in place with plates and screws. In young children, the teeth are frequently wired together for 4-6 weeks. In older children a LeFort I may be done at the same time as a LeFort III.


Since 1998, I have been using a halo-distraction technique for the LeFort III. This is a technique, which I developed, that utilizes a device called the RED system (see publications [#17, 2001], and [#24, 2005]). The device is not actually red, but is purple, and is gets its name for being a rigid external distraction device. This device is used primarily in children, because it does not offer much for teenagers unless the mid face is really far back. With the RED system, the bones of the mid face are basically cut as described above, with some small differences. Then, instead of pulling the mid face forward and filling in the gaps with skull bone, the skin is closed, and a halo is attached to the outside of the skull with 8-10 screws. A splint (U-shaped piece of plastic) is attached to the upper teeth and two wires extend forward from this splint to attach to the halo. The forward pull of the mid face comes from the dental splint; I do attach any wires to the bones of the face, which go through the skin (this would leave facial scars that are unnecessary). The parents, or the child, turn two screws daily, in order to slowly (and painlessly!) bring the mid face forward. The children are allowed to eat soft foods, and may go to school. Eight weeks later the device is removed with a 15 minute anesthetic. One advantage of using the RED device is that it is a slightly smaller operation, and is better tolerated by the children (compared to those who undergo the traditional technique and need to have their jaws wired shut). The greatest advantage of the RED device is that it enables surgeons to move the mid face more than three times further forward than is achieved on average with the traditional technique (based on a study done at our center). I am very excited about this new technique and have made many improvements in the way this operation is done, since I first began using it in early 1998. So far, I have treated over 60 children with the RED device, but have continued to perform the standard LeFort III in older children.