Congenital ear lesion in a 10-year-old girl

A healthy 10-year-old girl presented to the clinic with an unusual, asymptomatic lesion on her right ear. Her parents had noticed the lesion since birth, but due to the absence of symptoms, they had not sought medical attention until now. The family is now seeking guidance on the nature and possible management of the lesion. Can you determine the diagnosis?

Figure. Congenital ear lesion in a 10-year-old girl (Image author provided)

Clinical presentation

History

The lesion was a small, pedunculated, flesh-colored papule located anterior to the left ear, present since birth. It was nontender, was unchanged in size over time, and had never discharged fluid. The child had no prior trauma, infection, or other symptoms associated with the lesion. Her medical history was otherwise unremarkable, with normal developmental milestones and no known congenital anomalies. There was no family history of congenital ear abnormalities or related syndromes.

Examination findings

Physical examination revealed a soft, mobile, nontender papule measuring approximately 5 mm in diameter located anterior to the left tragus. The rest of the auricular structures, including the contralateral ear, external auditory canals, and tympanic membranes, appeared normal. There were no other visible congenital anomalies and no signs of syndromic features, such as facial asymmetry, preauricular pits, or renal abnormalities.

Initial thoughts

The differential diagnosis for a preauricular nodule includes preauricular sinus, dermoid cyst, lipoma, epidermal inclusion cyst, and accessory tragus (Table).¹ Given the clinical history and examination, an accessory tragus was considered the most likely diagnosis.¹

Table. Differential Diagnosis¹

Investigations

Audiological assessment

Pure tone audiometry showed normal bilateral hearing. There was no evidence of conductive or sensorineural hearing loss, which excluded any auditory involvement.

Ultrasound examination

Ultrasound imaging revealed a well-defined subcutaneous papule with internal echogenicity consistent with cartilage.¹ No cystic or vascular features were noted. A neck ultrasound showed no abnormalities in cervical lymph nodes or salivary glands.

Diagnostic dilemma

The clinical and imaging findings pointed toward a diagnosis of accessory tragus.¹ However, it is essential to evaluate for associated congenital anomalies, especially of the renal and craniofacial systems.² What additional investigations would you recommend?

Further evaluation

Renal ultrasound

To assess for renal anomalies, a renal ultrasound was performed. The kidneys were normal in size and structure, with no evidence of cysts or other abnormalities.²

Genetic consultation

Due to potential syndromic associations, the family was referred for genetic evaluation. No chromosomal abnormalities or gene mutations were detected. The patient had no clinical indications of syndromic involvement.²

Diagnosis and management

Final diagnosis

Based on clinical evaluation, audiology, and imaging, the lesion was diagnosed as an isolated accessory tragus.¹

Management plan

Because the lesion was asymptomatic and benign, surgical excision was offered for cosmetic reasons. The lesion was excised under local anesthesia without complications.

Outcome

The patient recovered uneventfully, with no recurrence or complications noted at the 6-month follow-up.

Discussion

Accessory tragi are rare congenital anomalies resulting from aberrant development of the first and second branchial arches. Although often isolated, they may be associated with syndromes such as branchio-oto-renal syndrome and Goldenhar syndrome.^1,2

Clinical pearls

1. Differential diagnosis: The differential for preauricular lesions includes accessory tragus,¹ preauricular sinus, dermoid cyst, and epidermal inclusion cyst. A detailed clinical history and examination are essential.²
2. Syndromic associations: Accessory tragi can be indicators of syndromic conditions, including renal and craniofacial anomalies.² Careful systemic evaluation, including renal ultrasound and genetic consultation, is crucial.^3,4
3. Management: Isolated accessory tragi are benign and typically only require excision for cosmetic purposes. Surgical removal is simple and effective.

Conclusion

This case highlights the importance of considering a broad differential diagnosis when evaluating congenital ear lesions.¹ A structured clinical and diagnostic workup is critical to exclude associated syndromic anomalies. In isolated cases, accessory tragi are benign and effectively managed with cosmetic surgical excision.

References
1. Bahrani B, Khachemoune A. Review of accessory tragus with highlights of its associated syndromes. Int J Dermatol. 2014;53(12):1442-1446. doi:10.1111/ijd.12369
2. Chintalapati K, Gunasekaran S, Frewer J. Accessory tragus in the middle ear: a rare congenital anomaly. Int J Pediatr Otorhinolaryngol. 2010;74(11):1338-1339. doi:10.1016/j.ijporl.2010.08.008
3. Acosta-Rodríguez A, Reza-López SA, Aguilar-Torres CR, Hinojos-Gallardo LC, Chávez-Corral DV. A systematic review of congenital external ear anomalies and their associated factors. Front Pediatr. 2025;13:1520200. doi:10.3389/fped.2025.1520200
4. Jansen T, Romiti R, Altmeyer P. Accessory tragus: report of two cases and review of the literature. Pediatr Dermatol. 2000;17(5):391-394. doi:10.1046/j.1525-1470.2000.017005391.x