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The e-mémoires of the Académie Nationale de Chirurgie

Early frontofacial monobloc advancement with distraction for faciocraniosynostosis.

ARNAUD E | MARCHAC D | RENIER D

Seance of wednesday 21 march 2007 (CHIRURGIE PLASTIQUE PEDIATRIQUE NOUVELLES APPROCHES)

Abstract

Faciocraniosynostosis treatment usually involves a two stage strategy: afronto-orbital advancement before one year of age to treat the craniostenosisand a facial advancement to correct the facial retrusion, laterin life. Eventually several facial advancements may be necessary beforeadult age is reached. Frontofacial monobloc advancement (FFMA)corrects both the craniostenosis and the facial retrusion but it is knownas a high risk procedure in the classical approach. Osteodistraction isnow a well accepted technique which has gained popularity, and FFMAwas evaluated in combination with distraction.Fifty-five patients with faciocraniosynostosis, were treated with FFMAand 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 incombination with a FFMA complete osteotomy. In some patients atransfacial pin was used. Rate of distraction was classical (0.5mm to1mm per day) started at day 7. The rate of short term complications wasevaluated. The achievement of advancement was evaluated clinically onexorbitism correction and dental occlusion relationship. The relapserate was evaluated by measurements of orbital bony gap in horizontalCT cuts, before and 6 months after removal of distractor. Respiratoryimpairment when present was also evaluated. The distractors were leftup to six months.The exorbitism was clinically corrected in all cases in which distractionwas completed (94%). Class I occlusal relationship was obtained in75%, but often with an open bite. When respiratory impairment waspresent, hypoxemia was corrected in all cases, but a residual sleep apneasyndrom might remain untreated. The rate of infectious complicationswas around 4% in primary cases, and 8% in secondary cases. Easyremoval of distractors was possible after a 6 months delay through acoronal approach, but reossification was limited. A relapse has beenobserved in three patients in whom the retaining phase was under 5months, but was much smaller when it was greater than six months.Internal distraction could allow early correction of respiratory impairmentof faciocraniosynostosis in infancy and could limit the major risksof frontofacial monobloc advancement. Previous surgeries performedprior to the FFMA increased its morbidity. Further evaluation is necessaryto decide whether the two stage strategy of treatment of faciocraniosynostosis(frontoorbital advancement before one year of age, andlater facial Le Fort III type advancement) could be replaced by a routineFFMA procedure.