Abstract

GOLDENHAR SYNDROME WITH UNILATERAL TESSIER’S TYPE 7 CLEFT: REPORT OF AN UNCOMMON CASE

*Dr. Saraswathi Gopal K. M. D. S. and Dr. Supriya Manoharan

ABSTRACT

Goldenhar syndrome, a variant of hemifacial microsomia, is a well- known developmental anomaly of maxillofacial skeleton that is apparent at birth. The first and second branchial arch involvement during early embryonic development results in a wide spectrum of anomalies that may include macrostomia and lateral facial clefts. Though clefts of the orofacial region are among the most common congenital facial defects, the occurrence of lateral facial clefts (Tessier’s type 7 cleft) in Goldenhar syndrome is reported only in (5%) of the cases. The lateral facial cleft which results because of improper development of the perioral muscles of the face, gives an appearance of macrostomia giving rise to potential psychological, aesthetic and feeding problems. This clinical report throws light on an one year old male infant with Goldenhar’s syndrome.

Keywords: Goldenhar syndrome, Occuloauriculovertebral Syndrome, Maxillofacial, Ocular dermoid, Preauricular ear tags and Tessier‘s type 7 cleft.