Treatment of the Syndromic Mid Face with Rigid External Distraction Le Fort III: a Long-term Assessment at Skeletal Maturity
Jeffrey A. Fearon, MD, Niyant Patel, MD.
The Craniofacial Center, Dallas, TX, USA.
Mid facial advancements improve obstructive sleep apnea (OSA) and help normalize appearance in children with syndromic craniosynostosis, yet they carry substantial surgical risks, and ideally are performed as infrequently as possible. We are unaware of any long-term outcome analyses following advancements utilizing rigid external distraction Le Fort III techniques (RED LFIII) at skeletal maturity. We sought to evaluate long-term functional efficacy and determine how many procedures are actually required over a child’s lifetime.
Following IRB approval, a retrospective review was performed of all children undergoing RED LFIII advancements who had achieved skeletal maturity. Physical examinations, photographic scoring, cephalometric, anthropometric and polysomnography data were collected, and appropriate statistical testing was performed.
Over a 15-year period (1998-2012) 104 patients underwent RED LFIII’s for syndromic craniosynostosis: 32 patients (20 Apert, 8 Crouzon, 3 Pfeiffer and 1 Saethre-Chotzen) met our criteria for skeletal maturity at a mean age of 18.6 years (range: 14.9-23.9 years). Follow up averaged 9.4 years. 11 patients (34%) had first been treated with a conventional Le Fort III at a mean age of 5.6 years, 10 by other surgeons. The surgical mean age of primary RED LFIII’s was 8.4 years (range: 4.7 to 13.1 years).
Ten children presented preoperatively with OSA, including 3 with tracheostomies, 8 (80%) normalized post distraction (including three decanulations), and 2 significantly improved but did not normalize. Over time, 2 (20%) developed recurrent OSA, and 1 other developed OSA de novo.
The mean estimated RED LFIII distraction was 25.6 mm (range, 15 to 37). Per anthropometry, the mean sagittal advancement was 18.3 mm, producing an average overcorrection of 4.3 mm (range, -10.9 to 17.3 mm) with respect to age/gender matched norms. The mean sagittal growth after RED LFIII was -0.5 mm and the mean vertical growth was 2.8 mm.
Almost half (15/32) of all patients underwent two mid facial advancements; however, following a primary RED LFIII advancement only 5 (16%) required a subsequent repeat advancements. Associated with the need for a repeat distraction was a more severe presenting mid facial hypoplasia score (-2.4 vs. -1.6, p=0.01) and performance of RED LFIII at a younger age (7.1 vs. 9.5 years, p=0.04). Also associated with the need for a repeat procedure was failure to overcorrect the mid facial position (p=0.03). Twenty (63%) patients underwent subsequent Le Fort I advancements to maximize occlusal relationships, and three received alloplastic malar augmentation.
Our analyses revealed that sagittal mid facial growth ceases post advancement in children with syndromic synostosis. Although some have suggested that OSA does not worsen with growth, we found that while RED LFIII advancements effectively eliminated OSA in most patients, ventilatory degradation leading to recurrent, or de novo, OSA was noted in some patients with subsequent growth. These findings suggest the need for continued monitoring of patients through skeletal maturity. We also found that performing this procedure at less than 8 years of age, and not distracting to an overcorrected position, were both associated with need for a subsequent repeat mid facial advancement.
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