Treacher-Collins Syndrome

Treacher-Collins Syndrome or mandibulofacial dysostosis is a genetically inherited condition which may vary in its facial deformity from mild to severe. It manifests bilaterally (on both sides) to involve the eyes, cheek bones, external ears and lower jaw. Dr. Paul Tessier considered this condition a grouping of facial clefts or defects which resulted in a downward slant of the lateral (outer) eyes, deficiencies of the malar (cheek) bones, absent or malformed external ears, deficiencies of the mandible (lower jaw) and other facial anomalies. A cleft lip and/or palate may also be present.

The UCLA Craniofacial Clinic Protocol for correction of Treacher-Collins may vary depending on the individual patient and the severity of the deformity. However, guidelines for timing of the procedures are as follows:

1) 3 months: Repair of Cleft lip and/or 1 year: Repair of cleft palate; If airway obstruction is problematic as an infant, surgically procedures may be used for correction of this including: a tracheostomy, mandibular or hyoid advancement or another procedures.
2) 6-8 years: Reconstruction of malar region-Zygomatic arch, lateral orbital wall and orbital floor with cranial bone graft and eyelid correction;
3) 6-8 years: External ear reconstruction;
4) 5-8 years: Mandibular lengthening with distraction osteogenesis using intraoral devices;
5) 14-18 years (facial skeletal maturity) Orthognathic (Jaw) surgery
6) 14-18 years(after jaw surgery): Septorhinoplasty, Laser removal of sideburn hair or other ancillary ('finishing') procedures.