Accessory Tragi and Associated Syndromes Involving the First Branchial Arch

Abstract

Although most deformities in front of the tragi are limited abnormalities, they may be signs of certain associated congenital syndromes that result from maldevelopment of the mandibular arch.

Familial Occurrence of Isolated Accessory Tragi

“…Accessory tragi are mostly an isolated developmental defect. They may be associated with other anomalies of the first branchial arch, such as cleft lip and palate, branchial fistulae, and hyperplasia of the mandible, or they can be a feature of more complex developmental abnormalities, such as oculoauriculovertebral syndrome (Goldenhar syndrome), mandibulofacial dysostosis (Treacher Collins syndrome), and otomandibular dysostosis (hemifacial microstomia) (1)(2)(3)5). Of these, accessory tragi are a constant component of Goldenhar syndrome only, in which they contribute to the pathognomonic triad together with epibulbar dermoids or lipodermoids and vertebral anomalies (6,8).…”

“…Accessory tragi are a developmental abnormality of the first branchial arch (1,2). They appear at birth as sessile or pedunculated, skin-colored papules or nodules, usually located in front of the auricle (3,4).…”

“…Most often the lesion is single, but it may be multiple, unilateral, or bilateral. It occurs as an isolated anomaly or may rarely be part of a complex developmental disorder (1)(2)(3).…”

Review of accessory tragus with highlights of its associated syndromes

“…30 Accessory tragus is also associated, although uncommon, with mandibulofacial dysostosis (Treacher-Collins syndrome), Townes-Brocks syndrome, VACTERL syndrome, and 4p syndrome (Wolf-Hirschhorn syndrome). 26,27,31 Treacher-Collins syndrome is a condition distinguished by disorders relating to the ear, eye, maxilla, and mandible, with several potential dental implications. 32 In Townes-Brocks and VACTERL syndromes, there is anal, ear, and renal congenital defects present.…”

The Accessory Tragus—No Ordinary Skin Tag

“…The incidence is higher when the accessory tragus is close to the mouth 1 . The only disorder with which accessory tragi are consistently found is the oculo‐auriculo‐vertebral syndrome (Goldenhar's syndrome), which is a triad of epibulbar dermoid cysts, vertebral defects, and accessory tragi 8, 9 . Since these defects are not occult, the presence of an accessory tragus does not necessitate an extensive search for rare defects.…”