Abstract

AGNOL, Mauro Antonio Dall et al. Goldenhar Syndrome in a pediatric patient: a case report and review of literature. RGO, Rev. gaúch. odontol. (Online) [online]. 2016, vol.64, n.4, pp. 472-483. ISSN 1981-8637.

Goldenhar Syndrome is a rare, generally sporadic condition, whose physical manifestations include asymmetric incomplete facial development, epibulbar dermoids, auricular malformations e auricular appendices, vertebral anomalies, central nervous system disturbances, ocular irregularities and visceral abnormalities. Its etiology is not fully understood, with its incidence ranging from 1:3500 to 1:5600 live births, and a gender ratio of 3:2 (male: female), presenting more frequently in children with congenital deafness. The purpose of this case report was to describe clinical presentation of Goldenhar syndrome in a five-year old male with no significant family history, where almost all of the classic signs of the condition were present. Considering its involvement in craniofacial structures, its rarity, and its wide spectrum of symptoms and associated systemic abnormalities, knowledge of this condition is paramount for the dental surgeon.

Keywords : Craniofacial abnormalities.; Diagnosis.; Goldenhar syndrome.; Anormalidades craniofaciais.; Diagnóstico oral.; Síndrome de Goldenhar..