[Surgical correction of a patient with Goldenhar syndrome].

Abstract

The syndrome has been described at first in 1952 by Goldenhar. Today, after the completion by Gorlin in 1963, the Goldenhar anomaly and the hemifacial microsomia are considered variants of a single dysmorphogenetic entity. The main characteristics of the syndrome are: 1. Epibulbar dermoids, 2. auricular appendages or fistulae, 3. vertebral anomalies. More recently, other malformations have been documented in association with the Goldenhar complex including CNS, cardiac, pulmonary and renal anomalies. We present the surgical correction of a patient with Goldenhar syndrome, with a combination of maxillofacial and plastic procedures. A review of the literature concerning the pathogenetic mechanisms and the surgical intervention is also presented.