Abstract
ObjectivesGoldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. Material and methodsThe clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. ResultsAll patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. ConclusionOur orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities.
Highlights
Goldenhar syndrome (GS), known as oculoauriculovertebral dysplasia or hemifacial microsomia has a wide range of clinical manifestations, including craniofacial, vertebral, cardiac, renal, and central nervous system anomalies1
This paper describes the clinical features of GS, with emphasis on the oral manifestations, by presenting WKH FDVHV RI SDWLHQWV DQG EULHÀ\ UHYLHZLQJ WKH literature about this genetic disorder
Several terms are used to describe GS due to the wide spectrum of anomalies that may be associated with this condition
Summary
Goldenhar syndrome (GS), known as oculoauriculovertebral dysplasia or hemifacial microsomia has a wide range of clinical manifestations, including craniofacial, vertebral, cardiac, renal, and central nervous system anomalies. The classic facial aspect of GS patients, described as hemifacial microsomia, and the other anomalies of this syndrome are probably caused by development GHIHFWV RI WKH ¿UVW DQG VHFRQG EUDFKLDO DUFKV12. The causes for these developmental defects seem to be heterogeneous. This paper describes the clinical features of GS, with emphasis on the oral manifestations, by presenting WKH FDVHV RI SDWLHQWV DQG EULHÀ\ UHYLHZLQJ WKH literature about this genetic disorder
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
