Faciocraniosynostosis: monobloc frontofacial osteotomy replacing the two-stage strategy?

Frontofacial monobloc advancement (FFMBA) is a powerful but high-risk procedure to correct both exorbitism and impaired airways of faciocraniosynostosis.

One hundred and five children with faciocraniosynostosis (mean 4.9 years, 7 months-14 years) were evaluated prospectively after FFMBA and quadruple internal distraction. The advancement was started at day 5 (0.5 mm/day). Mean follow-up was 61 months (maximum 10.5 years). Relapse was evaluated by the comparison between the evaluation at the time of removal of distractors and 6 months later.

Seventy-six patients (72%) completed their distraction uneventfully in the initial period.

- One death at D1 from acute tonsillar herniation before beginning of distraction. - Cerebrospinal fluid leaks managed conservatively (11 patients) and with transient lumbar drainage (eight patients). - Revision surgery (dysfunction/infection) of distraction devices (nine patients, subsequently four completed the distraction). Ninety-nine out of 104 patients finally completed their distraction, resulting in exorbitism correction. Respiratory impairment, when present, was corrected and class I occlusal relationship was obtained in 77% of the cases. Reossification was limited at the orbital level but relapse could be prevented by a retention phase of 6 months. Pfeiffer syndrome, previous surgeries, and surgery before 18 months of age were risk factors.

Internal distraction allows early correction of respiratory impairment and exorbitism of faciocraniosynostosis. In order to limit the risks, we advise: - Preliminary craniovertebral junction decompression if needed - Four devices to customize the distraction - Double pericranial flap to seal the anterior cranial fossa - Systematical external transient drainage if CSF leak - Slow rate of distraction (0.5 mm/day) - Long consolidation phase (6 months).