Background: Surgical treatment of faciocraniosynostosis is a complex problem that includes both function and aesthetics. Treatment goals are to prevent further neurologic disorders and to correct the morphologic impairment.
Methods: Thirty-six patients with faciocraniosynostosis (mean age, 5.2 years) were evaluated prospectively after frontofacial monobloc advancement and quadruple internal distraction. Four distractors were used in combination with a frontofacial monobloc advancement osteotomy. Complications and advancement were evaluated clinically and radiographically. Respiratory status was evaluated by polysomnography. Relapse was evaluated by comparing results at the time of distractor removal to 6-month values. The mean follow-up was 30 months.
Results: Distraction was completed in 35 patients. Twenty-eight patients (80 percent) completed their distraction uneventfully in the initial period. In seven patients, a problem related to the distraction devices required revision surgery, and subsequently six of them completed the distraction. One patient died the day after surgery from acute brain tonsillar herniation before distraction was begun. The exorbitism was corrected clinically in all patients in whom distraction was completed (n = 34). A class I occlusal relationship was obtained in 28 of 35 patients (80 percent). When respiratory impairment was present, it was corrected in all but two cases (14 of 16). A septic frontal osteonecrosis occurred in one patient 2 months after distraction was completed (frontal bone loss in one of 35 patients). Reossification at time of distractor removal was limited. Relapse has been observed predominantly at the occlusal level (six of 35) and more frequently in Pfeiffer syndrome.
Conclusions: Internal distraction allows for early correction of respiratory impairment and exorbitism of faciocraniosynostosis, and limits the major risks of frontofacial monobloc advancement. Previous operations performed before the frontofacial monobloc advancement increased its morbidity.