Zygomatic repositioning and Le Fort II distraction with intraoral devices in Apert syndrome: A case report. 2022

Sven Erik Nørholt, and Josephine Sköldstam, and Johan Blomlöf, and Sujeeva Karunahara, and Thomas Klit Pedersen
Department of Oral and Maxillofacial Surgery, Aarhus University Hospital, Aarhus, Denmark; Section of Oral and Maxillofacial Surgery and Oral Pathology, Aarhus University, Aarhus, Denmark.

The aim of this case report is to describe the surgical technique and outcome using internal intraoral distraction devices in LeFort II distraction with zygomatic repositioning (LF2ZR). In Apert syndrome the midface is characterized by a complex hypoplasia, with the central part being more affected than the lateral orbito-zygomatic complex. In LF2ZR, the zygomas are repositioned and internally fixated, and the central midface is further advanced through a LeFort II distraction. In previous publications, the distraction has been performed using external halo-based devices. It seems that the LF2ZR procedure can be planned and performed with adequate accuracy using virtual surgical planning tools. Knowledge about the possibility of using internal intraoral distraction devices in LF2ZR is important, as the inconspicuous placement of intraoral devices can be advantageous for some patients.

UI MeSH Term Description Entries
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
D015050 Zygoma Either of a pair of bones that form the prominent part of the CHEEK and contribute to the ORBIT on each side of the SKULL. Cheek Bone,Jugal Bone,Malar Bone,Zygomatic Arch,Arches, Zygomatic,Zygomatic Arches,Arch, Zygomatic,Bone, Cheek,Bone, Jugal,Bone, Malar,Bones, Cheek,Bones, Jugal,Bones, Malar,Cheek Bones,Jugal Bones,Malar Bones,Zygomas
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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