Medicine:Accessory auricle

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Accessory auricle
SpecialtyDermatology

An accessory auricle is a congenital anomaly typically seen as a skin coloured nodule, most frequently just to the front of the ear.[1][2]

Signs and symptoms

Well-developed accessory auricle on the side of the neck

The general presentation is of a skin-covered nodule, papule, or nodule of the skin surface, usually immediately anterior to the auricle.[2] However, it may be anywhere within the periauricular tissues.[2] Bilateral presentation can be seen.[2]

Genetics

A study of a family with 11 affected showed the accessory auricle were inherited in an autosomal dominant manner.[3]

Diagnosis

File:Ear Accessory Auricle LDRT.tif The lesions presents as a nodule or papule, either sessile or pedunculated. They may be soft or have a cartilaginous structure. By histologic examination, it is a recapitulation of normal external auricle. There will be skin, cartilaginous structures, and cartilage (although the last is not seen in all variants of this disorder).[4][5] Some investigators believe that the tragus is the only hillock which is derived from the first branchial arch. This is clearly suggestive that true cases of accessory auricle represent a true duplication of the hillocks that were part of the second branchial arch.[6] The second ear appears as a mirror image folded forward and lying on the posterior cheek.

Differential diagnosis

Classification

The several components or degrees of development range from an ear tag, preauricular appendage, preauricular tag, or accessory tragus, to supernumerary ears or polyotia.[7] It is a relatively common congenital anomaly of the first branchial arch or second branchial arches. Other anomalies may be present concurrently, including cleft palate, cleft lip, or mandibular hypoplasia. There is a known association with Goldenhar syndrome (oculo-auriculo-vertebral syndrome)[8] and with Wildervanck syndrome.[9][10][11] There may also be an association with congenital cartilaginous rest of the neck.

Management

Simple surgical excision is curative.[12] The recommended treatment is that the skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed.[13] Normal appearance is achieved in majority of cases. The reconstruction successful in true cases of accessory auricle, as it also is in individuals with auricular appendages.[14][15]

Epidemiology

These lesions usually present in neonates, although they may not come to clinical attention until adulthood (for cosmetic reasons). There is no gender predilection. They are present in approximately 3–6 per 1000 live births.[16]

References

  1. ↑ Hwang, Jungil; Cho, Jaeyoung; Burm, Jin Sik (September 2018). "Accessory auricle: Classification according to location, protrusion pattern and body shape". Archives of Plastic Surgery 45 (5): 411–417. doi:10.5999/aps.2018.00430. PMID 30282411. 
  2. ↑ 2.0 2.1 2.2 2.3 Cardesa, Antonio (2016). "8. Ear and temporal bone". in Franchi, Alessandro; Slootweg, Pieter J.; Gale, Nina et al. (in en). Pathology of the Head and Neck. Springer. pp. 397. ISBN 978-3-662-49670-1. https://books.google.com/books?id=ag4bDgAAQBAJ&pg=PA397. 
  3. ↑ Yang, Y (2006). "A locus for autosomal dominant accessory auricular anomaly maps to 14q11.2–q12". Human Genetics 120 (1): 144–147. doi:10.1007/s00439-006-0206-1. PMID 16775710. 
  4. ↑ Jansen, T.; Romiti, R.; Altmeyer, P. (2000). "Accessory tragus: Report of two cases and review of the literature". Pediatric Dermatology 17 (5): 391–394. doi:10.1046/j.1525-1470.2000.017005391.x. PMID 11085670. 
  5. ↑ Brownstein, M. H.; Wanger, N.; Helwig, E. B. (1971). "Accessory tragi". Archives of Dermatology 104 (6): 625–631. doi:10.1001/archderm.1971.04000240049006. PMID 5131708. 
  6. ↑ Stevenson, Roger E.; Hall, Judith G. (2005). Human Malformations and Related Anomalies (2nd ed.). Oxford University Press. pp. 339–340. ISBN 978-0199748082. 
  7. ↑ Lam, J.; Dohil, M. (2007). "Multiple Accessory Tragi and Hemifacial Microsomia". Pediatric Dermatology 24 (6): 657–658. doi:10.1111/j.1525-1470.2007.00560.x. PMID 18035991. 
  8. ↑ Konaş, E.; Canter, H. I.; Mavili, M. E. (2006). "Goldenhar complex with atypical associated anomalies: Is the spectrum still widening?". Journal of Craniofacial Surgery 17 (4): 669–672. doi:10.1097/00001665-200607000-00011. PMID 16877912. 
  9. ↑ Tadini, G.; Cambiaghi, S.; Scarabelli, G.; Brusasco, A.; Vigo, P. (1993). "Familial occurrence of isolated accessory tragi". Pediatric Dermatology 10 (1): 26–28. doi:10.1111/j.1525-1470.1993.tb00006.x. PMID 8493161. 
  10. ↑ Gao, J. Z.; Chen, Y. M.; Gao, Y. P. (1990). "A survey of accessory auricle anomaly. Pedigree analysis of seven cases". Archives of Otolaryngology–Head & Neck Surgery 116 (10): 1194–1196. doi:10.1001/archotol.1990.01870100088019. PMID 2206506. 
  11. ↑ Resnick, K. I.; Soltani, K.; Bernstein, J. E.; Fathizadeh, A. (1981). "Accessory tragi and associated syndromes involving the first branchial arch". The Journal of Dermatologic Surgery and Oncology 7 (1): 39–41. doi:10.1111/j.1524-4725.1981.tb00591.x. PMID 7204730. 
  12. ↑ Pan, B.; Qie, S.; Zhao, Y.; Tang, X.; Lin, L.; Yang, Q.; Zhuang, H.; Jiang, H. (2010). "Surgical management of polyotia". Journal of Plastic, Reconstructive & Aesthetic Surgery 63 (8): 1283–1288. doi:10.1016/j.bjps.2009.06.037. PMID 19617017. 
  13. ↑ Scott-Brown's Otorhinolaryngology (7th ed.). Hodder Arnold. 2016-06-15. pp. 969. ISBN 978-0-340-808-931. 
  14. ↑ Ku, PK; Tong, MC; Yue, V (1998). "Polyotia- a rare external ear anomaly". International Journal of Pediatric Otorhinolaryngology 46 (1–2): 117–20. doi:10.1016/S0165-5876(98)00152-9. PMID 10190712. 
  15. ↑ Bendor-Samuel, RL; Tung, TC; Chen, YR (1995). "Polyotia". Annals of Plastic Surgery 34 (6): 650–2. doi:10.1097/00000637-199506000-00015. PMID 7661545. 
  16. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 894. ISBN 978-1-4160-2999-1. 

Further reading

Classification

Template:Congenital malformations and deformations of ears



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