Accessory Tragi: An Association of Goldenhar Syndrome

Abstract

Accessory Tragus also known as preauricular ear tag, supernumerary pinna, and rudimentary ear, is a congenital developmental anomaly due to a minor aberration in the first branchial arch leading to external ear malformation.[1] It has been associated with syndromes such as Goldenhar syndrome, Treacher-Collins syndrome, VACTERL syndrome, Townes-Brocks syndrome, and Wolf-Hirschhorn syndrome.[2] An 18-month-old female child presented with three skin-colored asymptomatic, firm, pedunculated lesions over the left side of the face, two near the left ear, in the pre-auricular area and one, a few centimeters away from the angle of the mouth [Figure 1]. A pigmented growth was seen over the lateral aspect of the epibulbar region, suggestive of an epibulbar dermoid [Figure 2]. There was also mild left-sided malar hypoplasia confined to the midportion alone [Figure 3]. There were no vertebral [Figure 4] or facial nerve abnormalities. A probable diagnosis of Goldenhar syndrome was made based on the clinical features mentioned above.Figure 1: Clinical image of accessory tragi over the left side of the faceFigure 2: Epibulbar dermoidFigure 3: Left-sided malar hypoplasiaFigure 4: Clinical image of the back showing no abnormalitiesGoldenhar syndrome is an autosomal dominant disorder in which accessory tragus is a constant finding. It was initially described by Goldenhar as a triad of epibulbar dermoids, preauricular appendages, and pretragal fistulae and was later expanded to include vertebral anomalies, imparting the term, “Oculoauriculovertebral dysplasia.” This syndrome is also associated with microtia, microphthalmia, anophthalmia, facial asymmetry, mandibular hypoplasia along with renal, cardiac, gastrointestinal, hearing, and speech abnormalities.[3] There have been no fixed diagnostic criteria or age of presentation of the various clinical features of this syndrome thus calling for mandatory lifelong follow-up and a multidisciplinary approach. This case has been reported to highlight the importance of identifying the syndromic associations of a child with accessory tragi. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.