Staged Reconstruction for Adult Complete Treacher Collins Syndrome

Abstract

Treacher Collins syndrome is a congenital craniofacial malformation affecting the structures derived from the first and second brachial arches. For the complete form, the deformities can be severe; both the orbital-zygomatic region and mandible as well as the soft tissues should be reconstructed. To explore the surgical management of this kind of deformities, we proposed our protocol for the staged reconstruction: (1) upper-facial reconstruction with specially designed outer calvarial table, (2) mandibular lengthening by distraction osteogenesis technique and orthognathic surgery to correct the birdlike facial appearance and anterior open bite after distraction, and (3) lipofilling for the correction of residual depressive deformities. Two adult patients have undergone this protocol, and the results were satisfactory. Even though many authors advised using pedicled bone flap for the reconstruction of the orbit and zygomatic complex in young patient because free bone graft has the tendency of bone resorption and multiple onlay bone graft may need to be added in later stage, for adult patient, however, the reconstructed bone framework using outer cranial table can well maintain the bony facial contour of the orbital zygomatic region. The residual depressive deformities in the temporal and cheek regions can be treated with lipofilling. Compared with the technique of mandibular advancement osteotomy, bilateral mandibular lengthening by gradual distraction is relatively safe and can effectively elongate the mandible and the surrounding soft tissues. After the distraction, the open bite can be closed with subapical osteotomy, and the chin projection can be further added by advancement genioplasty.