[Quadruple internal distraction with early frontal-facial avancement for faciocraniodysostosis]

Frontofacial monobloc advancement (FFMA), known as a high risk procedure, was evaluated in combination with distraction.

Twenty patients with faciocraniosynostosis, were treated with FFMA and quadruple internal distraction. Mean age at surgery was 3.2 Years and mean follow-up was 14 Months (3-32 Months). Four distractors were used per patient in combination with complete osteotomy FFMA. Rate of distraction was classical (0.5 mm to 1mm per day) starting on day 7. The rate of short-term complications was evaluated. Correction of exorbitism and the dental occlusion relationship were assessed to evaluate achievement of advancement. Rate of relapse was evaluated by measuring the orbital bony gap on horizontal CT slices, before and 6 Months after removal of the distractor. Respiratory impairment when present was also evaluated.

Distraction was completed in 18/20 patients. Fifteen patients completed their distraction uneventfully during the initial period. In three patients with CSF leaks, distraction was delayed at day 14 and 16, but was resumed and completed normally. Second operations were required for two patients during the early phase: the distractor failed in one requiring immediate reoperation to change the device; distraction was completed successfully. Device infection developed in two patients requiring removal of the device and abortion of distraction. One patient died the day after surgery from acute tonsillar herniation before distraction could be initiated. Exorbitism was clinically corrected in all cases in whom distraction was completed (n=18). Class I occlusion was obtained in 16/18 patients. When present, respiratory impairment was corrected in all cases. Septic frontal necrosis occurred in one patient two Months after distraction was completed and necessitated removal of the forehead flap. Distractors were easily removed through a coronal approach at 6 Months, but reossification was limited. Relapse was observed in three patients whose retaining phase was less than five Months. Rate of relapse was much lower when distraction was maintained more than six Months.

Internal distraction can provide early correction of respiratory impairment due to faciocraniosynostosis in infancy and can reduce the major risks of frontofacial monobloc advancement. Morbidity is higher in patients with prior surgery before FFMA. Further evaluation is necessary to determine whether the two stage treatment strategy for faciocraniosynostosis (fronto-orbital advancement before one Year of age, and later facial Lefort III type advancement) could be replaced by a routine FFMA procedure.