Early frontofacial monobloc advancement with distraction for faciocraniosynostosis

Faciocraniosynostosis treatment usually involves a two stage strategy: a fronto-orbital advancement before one year of age to treat the craniostenosis and a facial advancement to correct the facial retrusion, later in life. Eventually several facial advancements may be necessary before adult age is reached. Frontofacial monobloc advancement (FFMA) corrects both the craniostenosis and the facial retrusion but it is known as a high risk procedure in the classical approach. Osteodistraction is now a well accepted technique which has gained popularity, and FFMA was evaluated in combination with distraction. Fifty-five patients with faciocraniosynostosis, were treated with FFMA and quadruple internal distraction. Mean age at surgery was 3.2 years (range 5 months to 14 years of age). Mean follow-up was 30 months (60 months to 3 months). Four distractors per patients were used in combination with a FFMA complete osteotomy. In some patients a transfacial pin was used. Rate of distraction was classical (0.5mm to 1mm per day) started at day 7. The rate of short term complications was evaluated. The achievement of advancement was evaluated clinically on exorbitism correction and dental occlusion relationship. The relapse rate was evaluated by measurements of orbital bony gap in horizontal CT cuts, before and 6 months after removal of distractor. Respiratory impairment when present was also evaluated. The distractors were left up to six months. The exorbitism was clinically corrected in all cases in which distraction was completed (94%). Class I occlusal relationship was obtained in 75%, but often with an open bite. When respiratory impairment was present, hypoxemia was corrected in all cases, but a residual sleep apnea syndrom might remain untreated. The rate of infectious complications was around 4% in primary cases, and 8% in secondary cases. Easy removal of distractors was possible after a 6 months delay through a coronal approach, but reossification was limited. A relapse has been observed in three patients in whom the retaining phase was under 5 months, but was much smaller when it was greater than six months. Internal distraction could allow early correction of respiratory impairment of faciocraniosynostosis in infancy and could limit the major risks of frontofacial monobloc advancement. Previous surgeries performed prior to the FFMA increased its morbidity. Further evaluation is necessary to decide whether the two stage strategy of treatment of faciocraniosynostosis (frontoorbital advancement before one year of age, and later facial Le Fort III type advancement) could be replaced by a routine FFMA procedure.