Craniofacial dysostosis

Craniofacial dysostosis is the term used to describe a number of conditions which, like craniosynostosis, involve the overgrowth and undergrowth of the structures of the head and face.

Examples of craniofacial dysostosis include:  
•  Crouzon syndrome
•  Apert syndrome
•  Pfeiffer syndrome

What surgery is available, and what techniques are involved?

Children with these conditions will often exhibit abnormal facial growth, and will require surgery to move their facial bones forward.
The timing of this type of facial surgery is a matter of great debate, and will vary considerably from patient to patient and from unit to unit. Ideally, most of these operations are best left until late adolescence. At this stage, facial growth is more or less complete, and surgeons can be reasonably sure that bones they reposition will stay fixed in place.
However, it is often the case that surgery needs be carried out sooner. This may be to correct a functional problem, such as feeding or dislocation of the eyes, or it may be to improve the patient’s appearance as they grow up, particularly during adolescence. When surgery is carried out before growth is complete, further operations are often needed at the end of adolescence in order to position the teeth correctly.
Operations to move the facial bones forward fall under the broad heading of LeFort osteotomies. LeFort osteotomies are numbered according to the level in the face at which they are carried out, as follows:
•  LeFort I advancement moves the tooth-bearing part of the upper jaw only
•  LeFort II advancement moves the tooth-bearing part of the upper jaw and the nose
•  LeFort III advancement moves the whole of the upper jaw, nose, cheek bones and eye sockets forwards. 

Sometimes a LeFort III type osteotomy is extended into the skull to move the forehead region forward at the same time, and this is known as monobloc advancement. In more severe conditions, such as Apert syndrome and Pfeiffer syndrome, the face is deliberately split vertically along the line of the nasal bridge during monobloc advancement. This procedure, known as facial bi-partition, is done in order to bring the eyes closer together, expand the upper jaw and rotate the two halves of the face into a more normal position.

What should I expect as a patient/parent of a patient?

Before most LeFort osteotomies there is a period of orthodontic preparation, during which the teeth are moved into a position that will be most suitable for surgery. This process generally takes about a year to complete, and there is likely to be a period of orthodontic treatment following the operation. This is only appropriate in older children who have their permanent adult teeth.
When the facial bones have been moved they are usually fixed in position by a combination of bone grafts and titanium screws, possibly assisted with small titanium plates. The bone most commonly used for grafting is taken from the hip, but may sometimes be taken from the skull.
Often the teeth of the upper and lower jaw are fixed together at the time of surgery in order to locate the position of the facial bones accurately, and in most cases this fixation will be taken off at the end of the operation to enable the jaws to move. However, during the recovery period elastic bands are sometimes inserted between splints on the upper and lower jaw. Patients and parents should note that oral hygiene is extremely important, both during the period of preparation and post-operative recovery. A dental hygienist will assist with this, but patient cooperation is essential.
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