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Craniofacial Stability in Patients With Crouzon or Apert Syndrome After Le Fort III Distraction Osteogenesis. 2013

Jacobus H Reitsma, and Edwin M Ongkosuwito, and Peter H Buschang, and Léon N A V Adrichem, and Birte Prahl-Andersen

Objective : Le Fort III osteotomy with distraction osteogenesis (DO) is used to improve the retruded midface in patients with Crouzon or Apert syndrome. This study aimed to evaluate sagittal and vertical preoperative and postoperative cephalometric changes of DO of the midface in patients with Crouzon or Apert syndrome. Design : Population-based case-control study. Patients and Methods : Records of patients with the syndrome of Crouzon (N = 6) or Apert (N = 7) were compared, before and after Le Fort III DO, with a nonsyndromic untreated control group (N = 486). Main Outcome Measures : Sagittal and vertical cephalometric maxillary landmarks and measurements were used to predict and measure midface advancement and rotation after Le Fort III DO. Cephalograms were taken before surgery (T0), 4 months after surgery at removal of the distraction device (T1), and 1 year after removal of the distraction device (T2). Analysis : Z scores were performed to compare cephalometric measures of syndromic patients with control subjects. Results : Cephalograms of 13 patients with Crouzon syndrome (N = 6) or Apert (N = 7) (age range 8.2 to 19.8 years) were evaluated. Treatment changes (T1-T2) showed statistically significant maxillary advancement, with no significant differences between the patients with the Crouzon or Apert syndrome. Conclusions : DO of the midface in patients with Crouzon or Apert syndrome seems to be stable in the sagittal direction after follow-up. Although Crouzon and Apert differ after DO, anteroposterior craniofacial dimensions were significantly improved and were closer to patterns of normal subjects.

UI MeSH Term Description Entries
D002508 Cephalometry The measurement of the dimensions of the HEAD. Craniometry
D003394 Craniofacial Dysostosis Autosomal dominant CRANIOSYNOSTOSIS with shallow ORBITS; EXOPHTHALMOS; and maxillary hypoplasia. Crouzon's Disease,Dysostosis, Craniofacial,Craniofacial Dysarthrosis,Craniofacial Dysostosis Syndrome,Craniofacial Dysostosis Type 1,Craniofacial Dysostosis, Type I,Crouzon Craniofacial Dysostosis,Crouzon Disease,Crouzon Syndrome,Craniofacial Dysarthroses,Craniofacial Dysostoses,Craniofacial Dysostosis Syndromes,Craniofacial Dysostosis, Crouzon,Crouzons Disease,Dysarthroses, Craniofacial,Dysarthrosis, Craniofacial,Dysostoses, Craniofacial
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000168 Acrocephalosyndactylia Congenital craniostenosis with syndactyly. Apert Syndrome,Pfeiffer Syndrome,Saethre-Chotzen Syndrome,Acrocephalosyndactyly (Apert),Acrocephalosyndactyly III,Acrocephalosyndactyly, Type 1,Acrocephalosyndactyly, Type 3,Acrocephalosyndactyly, Type I,Acrocephalosyndactyly, Type II,Acrocephalosyndactyly, Type III,Acrocephalosyndactyly, Type V,Acrocephaly, Skull Asymmetry, and Mild Syndactyly,Apert-Crouzon Disease,Chotzen Syndrome,Craniofacial-Skeletal-Dermatologic Dysplasia,Dysostosis Craniofacialis with Hypertelorism,Kurczynski Casperson Syndrome,Noack Syndrome,Syndactylic Oxycephaly,Acrocephalosyndactylias,Acrocephalosyndactylies, Type 1,Acrocephalosyndactylies, Type 3,Acrocephalosyndactylies, Type I,Acrocephalosyndactylies, Type II,Acrocephalosyndactylies, Type III,Acrocephalosyndactylies, Type V,Acrocephalosyndactyly IIIs,Apert Crouzon Disease,Disease, Apert-Crouzon,Noack Syndromes,Saethre Chotzen Syndrome,Syndactylic Oxycephalies,Syndrome, Apert,Syndrome, Chotzen,Syndrome, Kurczynski Casperson,Syndrome, Noack,Syndrome, Pfeiffer,Syndrome, Saethre-Chotzen,Syndromes, Noack,Type I Acrocephalosyndactylies,Type I Acrocephalosyndactyly,Type II Acrocephalosyndactylies,Type II Acrocephalosyndactyly,Type III Acrocephalosyndactyly,Type V Acrocephalosyndactylies,Type V Acrocephalosyndactyly
D016022 Case-Control Studies Comparisons that start with the identification of persons with the disease or outcome of interest and a control (comparison, referent) group without the disease or outcome of interest. The relationship of an attribute is examined by comparing both groups with regard to the frequency or levels of outcome over time. Case-Base Studies,Case-Comparison Studies,Case-Referent Studies,Matched Case-Control Studies,Nested Case-Control Studies,Case Control Studies,Case-Compeer Studies,Case-Referrent Studies,Case Base Studies,Case Comparison Studies,Case Control Study,Case Referent Studies,Case Referrent Studies,Case-Comparison Study,Case-Control Studies, Matched,Case-Control Studies, Nested,Case-Control Study,Case-Control Study, Matched,Case-Control Study, Nested,Case-Referent Study,Case-Referrent Study,Matched Case Control Studies,Matched Case-Control Study,Nested Case Control Studies,Nested Case-Control Study,Studies, Case Control,Studies, Case-Base,Studies, Case-Comparison,Studies, Case-Compeer,Studies, Case-Control,Studies, Case-Referent,Studies, Case-Referrent,Studies, Matched Case-Control,Studies, Nested Case-Control,Study, Case Control,Study, Case-Comparison,Study, Case-Control,Study, Case-Referent,Study, Case-Referrent,Study, Matched Case-Control,Study, Nested Case-Control
D019340 Osteotomy, Le Fort Transverse sectioning and repositioning of the maxilla. There are three types: Le Fort I osteotomy for maxillary advancement or the treatment of maxillary fractures; Le Fort II osteotomy for the treatment of maxillary fractures; Le Fort III osteotomy for the treatment of maxillary fractures with fracture of one or more facial bones. Le Fort III is often used also to correct craniofacial dysostosis and related facial abnormalities. (From Dorland, 28th ed, p1203 & p662) LeFort Osteotomy,Osteotomy, LeFort,Le Fort Osteotomy
D019857 Osteogenesis, Distraction Bone lengthening by gradual mechanical distraction. An external fixation device produces the distraction across the bone plate. The technique was originally applied to long bones but in recent years the method has been adapted for use with mandibular implants in maxillofacial surgery. Callotasis,Distraction Osteogenesis,Callotases,Distraction Osteogeneses,Osteogeneses, Distraction

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