Orthognathic treatment in Greig cephalopolysyndactyly syndrome: A case report

Tayfun Cıvak; Goksu Trakyali; Altan Varol

doi:10.1016/j.ajoms.2019.04.005

Orthognathic treatment in Greig cephalopolysyndactyly syndrome: A case report

Tayfun Cıvak, Goksu Trakyali, Altan Varol

Research output: Contribution to journal › Article › peer-review

Abstract

Typical Greig cephalopolysyndactyly syndrome (GCPS) is characterized by preaxial polydactyly or mixed pre- and postaxial polydactyly, true widely spaced eyes, macrocephaly. Individuals with mild GCPS may have subtle craniofacial findings. The diagnosis of GCPS is based on clinical findings and family history. GLI3 is the only gene known to be associated with GCPS. It has an autosomal dominant inheritance. The literature fails in documentations of any treatment protocol for defective jaw relationship in these patients. Therefore, we report on a patient with GCPS presenting polysyndactyly, frontal bossing, high forehead, skeletal Class III deformity due to maxillary retrognathism and mandibular prognathism, treated with orthognathic surgery by means of double jaw surgery and orthodontic treatment with fixed appliances.

Original language	English
Pages (from-to)	327-332
Number of pages	6
Journal	Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
Volume	31
Issue number	5
DOIs	https://doi.org/10.1016/j.ajoms.2019.04.005
Publication status	Published - Sept 2019

Keywords

Double jaw surgery
Greig syndrome
Orthognathic surgery

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10.1016/j.ajoms.2019.04.005

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title = "Orthognathic treatment in Greig cephalopolysyndactyly syndrome: A case report",

abstract = "Typical Greig cephalopolysyndactyly syndrome (GCPS) is characterized by preaxial polydactyly or mixed pre- and postaxial polydactyly, true widely spaced eyes, macrocephaly. Individuals with mild GCPS may have subtle craniofacial findings. The diagnosis of GCPS is based on clinical findings and family history. GLI3 is the only gene known to be associated with GCPS. It has an autosomal dominant inheritance. The literature fails in documentations of any treatment protocol for defective jaw relationship in these patients. Therefore, we report on a patient with GCPS presenting polysyndactyly, frontal bossing, high forehead, skeletal Class III deformity due to maxillary retrognathism and mandibular prognathism, treated with orthognathic surgery by means of double jaw surgery and orthodontic treatment with fixed appliances.",

keywords = "Double jaw surgery, Greig syndrome, Orthognathic surgery",

author = "Tayfun Cıvak and Goksu Trakyali and Altan Varol",

note = "Publisher Copyright: {\textcopyright} 2019 Asian AOMS(+) ASOMP(+) JSOP(+) JSOMS(+) JSOM(+) and JAMI",

year = "2019",

month = sep,

doi = "10.1016/j.ajoms.2019.04.005",

language = "English",

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T1 - Orthognathic treatment in Greig cephalopolysyndactyly syndrome

T2 - A case report

AU - Cıvak, Tayfun

AU - Trakyali, Goksu

AU - Varol, Altan

PY - 2019/9

Y1 - 2019/9

N2 - Typical Greig cephalopolysyndactyly syndrome (GCPS) is characterized by preaxial polydactyly or mixed pre- and postaxial polydactyly, true widely spaced eyes, macrocephaly. Individuals with mild GCPS may have subtle craniofacial findings. The diagnosis of GCPS is based on clinical findings and family history. GLI3 is the only gene known to be associated with GCPS. It has an autosomal dominant inheritance. The literature fails in documentations of any treatment protocol for defective jaw relationship in these patients. Therefore, we report on a patient with GCPS presenting polysyndactyly, frontal bossing, high forehead, skeletal Class III deformity due to maxillary retrognathism and mandibular prognathism, treated with orthognathic surgery by means of double jaw surgery and orthodontic treatment with fixed appliances.

AB - Typical Greig cephalopolysyndactyly syndrome (GCPS) is characterized by preaxial polydactyly or mixed pre- and postaxial polydactyly, true widely spaced eyes, macrocephaly. Individuals with mild GCPS may have subtle craniofacial findings. The diagnosis of GCPS is based on clinical findings and family history. GLI3 is the only gene known to be associated with GCPS. It has an autosomal dominant inheritance. The literature fails in documentations of any treatment protocol for defective jaw relationship in these patients. Therefore, we report on a patient with GCPS presenting polysyndactyly, frontal bossing, high forehead, skeletal Class III deformity due to maxillary retrognathism and mandibular prognathism, treated with orthognathic surgery by means of double jaw surgery and orthodontic treatment with fixed appliances.

KW - Double jaw surgery

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KW - Orthognathic surgery

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ER -

Orthognathic treatment in Greig cephalopolysyndactyly syndrome: A case report

Abstract

Keywords

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