Treatment strategy in Goldenhar syndrome

J Craniofac Surg. 2014 Jan;25(1):177-83. doi: 10.1097/SCS.0000000000000387.


Goldenhar syndrome is a rare congenital defect characterized by ocular symptoms including (epibulbar dermoids, microphthalmia, anophthalmia, eyes asymmetry/dysmorphy, cleft eyelid, exophthalmia, strabismus), auricular symptoms (dacryocystitis), preauricular appendages, preauricular fistulas, ear asymmetry, microtia, atresia of the external auditory canal), craniofacial deformities (cleft face, cleft lip, cleft palate, macrostomia, bifid tongue, hypoplasia of the mandible, hypoplasia of the maxilla, asymmetry of the mandible and maxilla, malocclusion, tooth discrepancies, agenesis of third molars and second premolars, supernumerary teeth, enamel and dentin malformations, delay in tooth development), and skeletal abnormalities (cleft spine, microcephaly, dolichocephaly, plagiocephaly, vertebral defects) or abnormalities of internal organs. The degree of abnormalities vary from severe to mild. In this article, we present a long-term observation of a patient with Goldenhar syndrome. During the patient's life, the intensification of anomalies varied. We describe preoperative and postoperative orthodontic treatment and surgical correction procedures of maxillofacial deformation.

MeSH terms

  • Adult
  • Esthetics*
  • Goldenhar Syndrome / diagnosis*
  • Goldenhar Syndrome / surgery*
  • Humans
  • Imaging, Three-Dimensional
  • Male
  • Malocclusion / diagnosis*
  • Malocclusion / surgery*
  • Mastication / physiology*
  • Maxillofacial Abnormalities / diagnosis*
  • Maxillofacial Abnormalities / surgery*
  • Tomography, X-Ray Computed