Goldenhar syndrome (oculo-auriculo-vertebral spectrum – OAVS): findings on cone beam computed tomography – three case reports

Publication date: Available online 7 May 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Igor Maryanchik, Madhu K. Nair
BackgroundGoldenhar syndrome, also known as oculo-auriculo-vertebral spectrum (OAVS), is a variant of hemifacial microsomia, a congenital condition affecting primarily aural, oral, and mandibular development, with vertebral anomalies, ocular abnormalities, and epibulbar dermoids. As a complex and heterogeneous condition, this disorder varies from mild to severe, and involvement is unilateral in many cases, but bilateral involvement is also known to occur. It presents several complex problems in treatment, requiring an integrated, multidisciplinary approach.Clinical and Radiographic FindingsOAVS has a wide range of clinical manifestations. This report describes radiographic features of the syndrome in three patients, as visualized on maxillofacial cone beam computed tomography (CBCT). Pertinent radiographic findings include multiple substantial unilateral anomalies affecting the skull, cervical vertebrae, orbit, ear, maxilla, mandible, and teeth.Definitive InterpretationRadiographic features are consistent with those previously reported in the literature for OAVS.ConclusionThe relevance of imaging diagnosis and characterization of the syndrome and the significance of coordinated diagnosis and management approaches between oral and maxillofacial radiologists and medical/dental specialists must be emphasized in order to allow for planning and implementation of inter-disciplinary intervention at an early stage.



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