title: Analysis of midface retrusion in Crouzon and Apert syndromes.
abstract: BACKGROUND
  Midface retrusion is the hallmark of the syndromic dysostoses (i.e., Crouzon and Apert). Lack of forward projection and/or structural deficiency could be responsible, but neither has been adequately assessed three-dimensionally. The authors examined both the cranial base/facial interface and the midface volume to provide an understanding of the etiopathogenesis of midface deficiency.
  
  
  METHODS
  Children with computed tomographic scans in the absence of any surgical intervention were included. Demographic information was recorded for three groups: Apert, Crouzon, and control. Scans were digitized and manipulated using Materialise software (Surgicase CMF). Craniometric data relating to the midface and sphenoid were collected. Volumetric assessment of the midface was tabulated. Statistical analysis was performed using the t test.
  
  
  RESULTS
  Thirty-six scans were included (control, n=17; Crouzon/Apert, n=19). All children were in the early mixed dentition stage. The anterior cranial fossa proved to be shorter and wider in Crouzon/Apert patients compared with controls. The cranial base angles (N-S-BA, N-S-SO, N-SO-BA, S-SO-BA, and N-S-AR) were not statistically different across the groups. The Crouzon/Apert group showed angles more obtuse between the greater wings of the sphenoid, and more obtuse (more splayed) between the pterygoid plates. Nasion-sella-pterygomaxillary fissure angle was more obtuse (flatter) in the Crouzon/Apert group. There was no volumetric difference in the maxilla, zygoma, and sphenoid comparing the Crouzon/Apert group to controls.
  
  
  CONCLUSIONS
  Midface retrusion in the Crouzon/Apert group is associated with altered sphenoid morphology (widened and retruded pterygoid plates), with a flatter and wider maxilla, suggesting diminished growth inferiorly and anteriorly. There is no volumetric deficiency in Crouzon/Apert patients compared with controls.
  
  
  METHODS
  Risk, II.
authors: New Haven, Conn.; and São Paulo, Brazil From the Section of Plastic and Reconstructive Surgery, Yale School of Medicine; and the Department of Plastic Surgery, University of São Paulo.
authors: Antonio Jorge
authors: AJ
authors: Forte
authors: Nivaldo
authors: N
authors: Alonso
authors: John A
authors: JA
authors: Persing
authors: Miles J
authors: MJ
authors: Pfaff
authors: Eric D
authors: ED
authors: Brooks
authors: Derek M
authors: DM
authors: Steinbacher
mesh_terms: D000168:Acrocephalosyndactylia / Q000000981:diagnostic imaging / Q000473:pathology*; D016022:Case-Control Studies; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D005145:Face / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009929:Organ Size; D012189:Retrospective Studies; D019291:Skull Base / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed