High Le Fort I osteotomy for correction of mid-face deformity in Crouzon syndrome

Congenit Anom (Kyoto). 2016 Sep;56(5):240-2. doi: 10.1111/cga.12168.

Abstract

An 18-year-old woman with mild Crouzon syndrome was referred with malocclusion and mandibular protrusion. Examination revealed Class III canine and molar relationships, hypoplastic maxilla, 1-mm overbite, and -2-mm overjet. Analysis showed 69° sella-nasion-A, 73.6° sella-nasion-B, and -4.6° A point-nasion-B point angles. Polysomnography revealed respiratory disturbance and 6.3% oxygen desaturation indices of 5.4/h and 9.0/h. We performed double-jaw surgery using high Le Fort I osteotomy and bilateral sagittal split ramus osteotomy for midfacial deformity correction. Twelve months post-surgery, her measures were 70.8°, 72°, -1.2°, 3.0/h, and 6.1/h, respectively. Esthetics were satisfactory. High Le Fort I osteotomy is effective for midfacial deformity correction in patients with Crouzon syndrome.

Keywords: Crouzon syndrome; airway; high Le Fort I osteotomy; midfacial deformity; obstructive sleep apnea syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Cephalometry
  • Craniofacial Dysostosis / diagnosis*
  • Craniofacial Dysostosis / surgery*
  • Female
  • Humans
  • Maxilla / abnormalities*
  • Maxilla / surgery*
  • Osteotomy, Le Fort* / methods
  • Phenotype
  • Tomography, X-Ray Computed
  • Treatment Outcome