title: Le Fort III distraction osteogenesis in syndromal craniosynostosis.
abstract: Midface distraction osteogenesis has become a valuable technique. Although outcome data are available from several craniofacial centers, information regarding perioperative and immediate postoperative course is scarce. This report describes seven children, aged 4 to 17 years, with syndromal craniosynostosis in need of midface advancements. Most were rather complex cases with several previous craniofacial surgeries (mean 4.4 times, range 1-8). The Modular Internal Distraction System (Howmedica Leibinger, Inc., Rutherford, NJ) was used for the gradual Le Fort III advancements. The average midface advancement obtained was 23 mm (range 15-30 mm), resulting in improved facial profile, normalized or improved dental occlusion, reduced exophthalmos, and cessation or a significant decrease in preoperative respiratory problems. The mean length of operation was 354 minutes (range 300-535 minutes), and the mean amount of perioperative blood transfusion needed was 1251 mL (range 450-1800 mL) or 46.0 mL/kg (range 8.2-121.4 mL/kg). Complications included subcutaneous infections inferolaterally to the eye (N = 3) and forehead (N = 1). One patient had worsening of her facial profile and underwent a subsequent fronto-orbital advancement. One patient developed a deviation of the nasal septum and needed a corrective rhinoplasty. One patient developed marked trismus and one needed reoperation and trimming of the anchoring titanium plate on the malar process. In one patient, the lacrimal sac was lacerated, leading to transient epiphora. In most patients, Le Fort III distraction led to a significant improvement in the facial profile. However, surgery is still a major undertaking, with several potential complications. In our hands, the rate of complications is not less than for monobloc advancement. Thus, the choice of operation method is not based on which method is the least risky but on which is best suited for the individual patient.
authors.affiliation: Department of Neurosurgery, the National Hospital, University of Oslo, Oslo, Norway. torsteinrmeling@mailcity.com
authors.forename: Torstein R
authors.initials: TR
authors.lastname: Meling
authors.forename: Hogevold
authors.initials: H
authors.lastname: Hans-Erik
authors.forename: Skjelbred
authors.initials: S
authors.lastname: Per
authors.forename: Bernt J
authors.initials: BJ
authors.lastname: Due-Tonnessen
mesh_terms: D000168:Acrocephalosyndactylia / Q000601:surgery; D000293:Adolescent; D016063:Blood Loss, Surgical; D001860:Bone Plates / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000601:surgery; D003398:Craniosynostoses / Q000601:surgery*; D005094:Exophthalmos / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005624:Frontal Bone / Q000601:surgery; D006801:Humans; D007765:Lacrimal Apparatus / Q000293:injuries; D007902:Length of Stay; D008297:Male; D009300:Nasal Septum / Q000601:surgery; D009915:Orbit / Q000601:surgery; D019857:Osteogenesis, Distraction / Q000379:methods*; D019340:Osteotomy, Le Fort / Q000379:methods*; D012120:Respiration Disorders / Q000601:surgery; D013530:Surgical Wound Infection / Q000209:etiology; D013997:Time Factors; D014313:Trismus / Q000209:etiology