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Case Reports
. 2016 May-Aug;20(2):328.
doi: 10.4103/0973-029X.185907.

Goldenhar Syndrome: A rare case report

Affiliations
Case Reports

Goldenhar Syndrome: A rare case report

Ruchi Bhuyan et al. J Oral Maxillofac Pathol. 2016 May-Aug.

Abstract

Goldenhar Syndrome or oculoauriculovertebral spectrum is a complex syndrome characterized by an association of maxillomandibular hypoplasia, deformity of the ear, ocular dermoid and vertebral anomalies and the most severe form of hemifacial microsomia. Here, we describe a 26-year-old male patient with unilateral hemifacial microsomia, preauricular ear tags, macrosomia on the right side of the face.

Keywords: Hemifacial microsomia; ocular dermoid; preauricular ear tags.

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Figures

Figure 1
Figure 1
Lateral profile view of the patient showing preauricular skin tags, nodes and unilateral macrosomia
Figure 2
Figure 2
Orthopantomogram showing underdeveloped mandible on the right side
Figure 3
Figure 3
Front view demonstrating unilateral macrosomia, hypoplasia of the zygomatic area
Figure 4
Figure 4
Medial aspect of eye demonstrating epibulbar dermoid
Figure 5
Figure 5
Intra-oral view showing high-arched palate
Figure 6
Figure 6
Patient started with orthodontic alignment

References

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    1. Gorlin RJ, Jue KL, Jacobsen U, Goldschmidt E. Oculoauriculovertebral dysplasia. J Pediatr. 1963;63:991–9. - PubMed
    1. Santos DT, Miyazaki O, Cavalcanti MG. Clinical-embryological and radiological correlations of oculo-auriculo-vertebral spectrum using 3D-CT. Dentomaxillofac Radiol. 2003;32:8–14. - PubMed
    1. Bielicka B, Necka A, Andrych M. Interdisciplinary treatment of patients with Goldenhar Syndrome – Clinical reports. Dent Med Probl. 2006;43:458–62.

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