Oral Rehabilitation of a Child with Goldenhar syndrome

Abstract

Goldenhar syndrome (GS) is a rare abnormality affecting the craniofacial region having extra cranial manifestations as well. The first observation of the anomaly was reported by Arlt F in 1881. Dr Maurice Goldenhar in 1952 gave the name Goldenhar syndrome. The etiology of this disease still remains unclear and occurs as sporadic. However, some researchers have suggested multifactorial inheritance caused by the interaction of many genes, possibly in combination with environmental factors. The incidence has been reported to range from 1 in 3,500 to 1 in 5,600 live births; however, in children with congenital deafness, it is much higher at 1 in 1000. Patients with this syndrome may exhibit a wide range of anomalies that includes eye anomalies (micropthalmos, coloboma, epibulbar dermoid and lipodermoid malformations); ear anomalies (microtia, atresia, ear appendices and fistulas); vertebral and congenital heart anomalies; cleft lip/cleft palate, facial asymmetry, developmental dental disturbances and mental retardation. VATER (Vertebral anomalies, Anal atresia, Tracheoesophageal fistula, Esophageal atresia, Renal anomalies and Radial Dysplasia) or CHARGE (Coloboma, Heart defects, Atresia chooanae. This case report presents the dental management of a 7 year old boy with characteristic combination of external ear anomalies and ipsilateral facial underdevelopment the hallmark of this syndrome. Key words: Corneal opacities, Goldenhar syndrome, Mandibular hypoplasia, Oral Rehabilitation, Periauricular tags.