Objective Although most preauricular sinuses are located near the anterior limb of the ascending helix, some are located in unusual areas around the auricle, as shown previously in limited reports. This study analyzed the clinical manifestations of congenital periauricular fistulas with unusual fistula locations and the possible relationship with the classical preauricular sinus. Methods We reviewed the medical records of patients who underwent congenital periauricular sinus excision by three surgeons and followed them for more than 6 months. Clinical manifestations were compared between classical preauricular sinus (classical group) and congenital periauricular fistula (variant group) patients. Results The classical and variant groups included 192 and 20 ears each, respectively. In the variant group, the fistula locations were in the ascending helix crus (15 ears, most common), infra-auricular area (3 ears), supra-auricular area (1 ear), and anterior to tragus (1 ear). In ears with fistulas in the ascending helix crus, the fistulous tract most often showed a medial direction (9 ears). There were 4 ears in the variant group with fistulous tracts running toward the postauricular area. Conclusions Congenital periauricular fistulas can be located anywhere around the auricle, and also considered the variant of preauricular sinus because the whole tracts were limited to lateral side of temporalis muscle and parotid gland as well as not associated with external auditory canal and facial nerve. Among them, the most common variant was a fistula on the ascending helix crus with short fistulous tract directed medially.
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