Branchial Arch and Oral-Acral Disorders

Abstract

Abstract Oculo-auriculo-vertebral spectrum (hemifacial microsomia, Goldenhar syndrome) In the 1960s, hemifacial microsomia was defined as a condition affecting primarily aural, oral, and mandibular development. The disorder varies from mild to severe, and involvement is limited to one side in many cases, but bilateral involvement is also known to occur, with more severe expression on one side. Goldenhar syndrome is considered a variant of this complex, characterized additionally by vertebral anomalies and epibulbar dermoids (55). The condition is now known to be extremely complex and heterogeneous. Thus, in the present edition we have employed the term oculo-auriculo-vertebral (OAV) spectrum.