TY - JOUR
T1 - Orthognathic treatment in Greig cephalopolysyndactyly syndrome
T2 - A case report
AU - Cıvak, Tayfun
AU - Trakyali, Goksu
AU - Varol, Altan
N1 - Publisher Copyright:
© 2019 Asian AOMS(+) ASOMP(+) JSOP(+) JSOMS(+) JSOM(+) and JAMI
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
KW - Greig syndrome
KW - Orthognathic surgery
UR - http://www.scopus.com/inward/record.url?scp=85066049233&partnerID=8YFLogxK
U2 - 10.1016/j.ajoms.2019.04.005
DO - 10.1016/j.ajoms.2019.04.005
M3 - Article
AN - SCOPUS:85066049233
SN - 2212-5558
VL - 31
SP - 327
EP - 332
JO - Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
JF - Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
IS - 5
ER -