Goldenhar Syndrome

Resident: Swan
Syndrome: Goldenhar Syndrome, AKA Oculo-Auriculo-Vertebral (OAV) syndrome
Etiology: congenital defect characterized by incomplete development of the ear, nose, soft palate, lip, and mandible, usually unilateral. Often synonymous with hemifacial microsomia. It is associated with anomalous development of the first and second branchial arches
Diagnosis: No genetic test available. Diagnosis made by physician after evaluation of characteristic symptoms.
Systemic/Medical Conditions: Some patients will have growing problems with various organs, especially heart, kidney, lungs. Usually, the organ will either not be present on one side or will be underdeveloped. Also, possible scoliosis, hearing loss, limbal dermoids (benign congenital tumor of the eye, where cornea meets sclera), strabismus is common, abnormal ear formation, tissue tags near the ear.
Oral Manifestations: hypoplastic mandible/maxilla, possible cleft lip/palate, facial asymmetry, high vaulted palate, class III malocclusions, anterior open bite.
Dental needs/considerations: possible distraction osteogenesis with bone grafting necessary