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

BACKGROUND: Frontofacial monobloc advancement (FFMBA) is a powerful but high-risk procedure to correct both exorbitism and impaired airways of faciocraniosynostosis. PATIENTS AND METHODS: 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.
RESULTS: Seventy-six patients (72%) completed their distraction uneventfully in the initial period. COMPLICATIONS: - 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.
CONCLUSIONS: 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).