Clinical-embryological and radiological correlations of oculo-auriculo-vertebral spectrum using 3D-CT

The purpose of this paper is to present a variety of imaging findings of oculo-auriculo-vertebral spectrum (Goldenhar syndrome) using three-dimensional reconstructed images from computed tomography (3D-CT), associating clinical and embryological patterns of the syndrome. The study population consisted of 10 patients with oculo-auriculo-vertebral spectrum with clinically identified hemifacial microsomia. The patients were examined using spiral CT, and abnormal imaging features were grouped under facial, ear and temporal bone, vertebral, and skull base anomalies. The original CT data were transferred to a networked computer workstation with a computer graphics system to generate 3D-CT volume rendered images of the skull and vertebra. Two observers analysed the bone and muscular setting protocols to assess the relationship between bone and muscular structures. Asymmetric underdevelopment was a characteristic pattern of this syndrome resulting from hypoplasia of the mandibular ramus and condyle, the zygomatic, sphenoid and auricular conduct bones, and the temporal and masseter muscles. The syndrome was associated with local atrophy seen on 3D-CT images using specific bone and muscles protocols in all cases. Understanding the aetiology, embryology and wide imaging spectrum of this syndrome is essential to make a correct diagnosis, for treatment planning, and for evaluation when associated with a 3D-CT computer graphics system.