Le Fort III Distraction Osteogenesis in Syndromic Craniosynostosis

Syndromic craniosynostosis as Crouzon or Apert syndrome is a rare autosomal dominant disorder with a very low prevalence and characterized by faciocraniostenosis. The reduced intracranial volume associated with intracranial pressure and midfacial hypoplasia is known as the typical pathognomonic symptoms requiring surgical treatment. Le Fort III distraction osteogenesis (DO) with an internal or external distraction device in patients with syndromic craniosynostosis is a powerful and valuable surgical procedure that reliably produces a more significant change of appearance than most other single procedures routinely performed by craniofacial surgeons. It is indicated for children who require advancements beyond those achievable with the standard operation. The Le Fort III osteotomy combined with distraction has become the preferred technique for midfacial advancement over traditional methods. Advantages of the distraction technique include elimination of the need for bone grafts and rigid fixation, the possibility of longer advancement distances, reduced need for blood transfusion, and shorter hospital stays. It effectively treated both, exorbitism and impaired airways, in faciocraniostenosis in the affected patients. Complication rates were acceptable and patients’ functional and aesthetic outcomes were high.